PEDIATRIC ASTHMA
OVERVIEW
In childhood asthma, the lungs and airways become easily inflamed when exposed to certain triggers. Such triggers include inhaling pollen or catching a cold or other respiratory infection. Childhood asthma can cause irritating daily symptoms that interfere with play, sports, school and sleep. In some children, unmanaged asthma can cause dangerous asthma attacks.
Childhood asthma isn't a different disease from asthma in adults, but children face unique challenges. The condition is a leading cause of emergency department visits, hospitalizations and missed school days.
Unfortunately, childhood asthma can't be cured, and symptoms can continue into adulthood. But with the right treatment, you and your child can keep symptoms under control and prevent damage to growing lungs.
Asthma is a lung disease that causes your airways to swell and narrow, making it very hard to breathe. If asthma isn’t well controlled, it can cause a variety of issues and complications. It can cause your child to miss school and even end up in the hospital. It’s important to have an asthma action plan to help manage your child’s condition.
What is childhood asthma?
Asthma is a long-term (chronic) lung disease that affects your airways. Your airways are the tubes that carry air in and out of your lungs. When you have asthma, you can’t get air into your lungs because your airways swell and get too narrow.
Like a pinched straw, this makes it hard for you to breathe, which can cause wheezing, coughing and chest tightness. Certain triggers can set off or worsen these symptoms, causing an asthma attack. Attacks can come on fast or develop slowly, and they may be life-threatening.
Asthma can begin at any age, but it most often starts during childhood when your child’s immune system is still developing. Most children who get asthma have their first symptom by age 5. Asthma can cause your child to miss school and even end up in the hospital. It’s important to have an asthma treatment plan to help manage your child’s condition.
How common is asthma in childhood?
Asthma is the leading cause of chronic illness in children. It affects about 7.5 million children in the United States. The rate of the condition in children is steadily increasing. It’s also one of the main causes of missed school for children and missed work for parents.
Why are more children getting asthma?
Researchers believe several factors may be leading to more and more children developing asthma. These factors include:
Exposure to more allergens such as dust, secondhand smoke and toxins in the air.
Not enough exposure to childhood illnesses that build up their immune systems.
Lower rates of breastfeeding, which prevent babies from receiving important immune system substances.
CAUSES
Childhood asthma causes aren't fully understood. Some factors thought to be involved include having:
A tendency to develop allergies that runs in the family.
Parents with asthma.
Some types of airway infections at a very young age.
Exposure to environmental factors, such as cigarette smoke or other air pollution.
Increased immune system sensitivity causes the lungs and airways to swell and produce mucus when exposed to certain triggers. Reaction to a trigger can be delayed, making it more difficult to identify the trigger. Triggers vary from child to child and can include:
Viral infections such as the common cold.
Exposure to air pollutants, such as tobacco smoke.
Allergies to dust mites, pet dander, pollen or mold.
Physical activity.
Weather changes or cold air.
Sometimes, asthma symptoms occur with no apparent triggers.
Researchers don’t know the exact cause of asthma, but it often develops during childhood when your child’s immune system is still developing. Many factors may affect how your child’s lungs develop or how their body fights germs. These include:
Genetics: Biological family history, such as a parent who has asthma.
Allergens: Things in the environment that affect your child, such as dust or tobacco smoke.
Viral infections at a young age: Respiratory infections that affect breathing, such as the common cold.
Is asthma contagious?
No, asthma isn’t contagious. Germs such as bacteria and viruses don’t cause the condition, so it can’t spread from person to person.
Common triggers include:
Airway infections: These include colds, COVID-19, flu, pneumonia, and sinus infections.
Allergens: Your child might be allergic to things such as cockroaches, dust mites, mold, pet dander, and pollen.
Irritants: Things such as air pollution, chemicals, cold air, odors, or smoke can bother their airways.
Exercise: It can lead to wheezing, coughing, and a tight chest.
Stress: It can make your child short of breath and worsen their symptoms
SYMPTOMS
Common childhood asthma symptoms include:
A whistling or wheezing sound when breathing out.
Shortness of breath.
Chest congestion or tightness.
Frequent coughing that worsens when your child:
Has a viral infection.
Is sleeping.
Is exercising.
Is in the cold air.
Childhood asthma also might cause:
Trouble sleeping due to shortness of breath, coughing or wheezing.
Bouts of coughing or wheezing that get worse with a cold or the flu.
Delayed recovery or bronchitis after a respiratory infection.
Trouble breathing that hampers play or exercise.
Fatigue, which can be due to poor sleep.
Asthma symptoms vary from child to child and might get worse or better over time. Your child might have only one symptom, such as a lingering cough or chest congestion.
It can be difficult to tell whether your child's symptoms are caused by asthma. Wheezing and other asthma-like symptoms can be caused by infectious bronchitis or another respiratory problem.
Not all children have the same asthma symptoms. And symptoms can vary from episode to episode in the same child. Childhood asthma symptoms may include:
Frequent coughing spells. Coughing fits may occur while your child is playing or laughing. They may also occur at night or right after your child wakes up. Coughing may be their only symptom.
Less energy during play.
Rapid breathing or shortness of breath (dyspnea).
Complaining of chest tightness or their chest “hurting.”
A whistling sound (wheezing) when your child breathes in or out.
Retractions. When the area between your child’s ribs and neck area sinks in when they try to exhale. Retractions are a sign your child is working hard to breathe.
Feelings of weakness or tiredness.
Irritability.
Trouble feeding (sucking or eating).
When your child has an asthma attack (asthma exacerbation), their symptoms may get much worse. The attacks may come on slowly or quickly. Sometimes, they can be life-threatening (status asthmaticus). If your child has any of the following warning signs of a severe attack, you should get medical help right away:
Severe coughing.
Rapid worsening of shortness of breath or wheezing.
Serious breathing problems.
Increase in respiratory rate at rest.
Turning pale or bluish in their face, lips and/or fingernails.
Trouble speaking, inability to speak in sentences or not being able to speak at all.
PEDIATRIC ASTHMA DIAGNOSIS
Your child’s asthma symptoms may be gone by the time you get to the doctor’s office. You have an important role in helping your doctor understand what’s going on. A diagnosis will include:
Questions about medical history and symptoms. 
Your doctor will ask about any breathing problems your child may have had, as well as any family history of asthma, allergies, eczema, or other lung disease. Describe your child's symptoms in detail, including when and how often they happen.
Physical exam. 
Your doctor will listen to your child's heart and lungs and look in their nose or eyes for signs of allergies.
Tests. 
Your child might get a chest X-ray. If they’re 6 or older, they may have a simple lung test called spirometry. It measures the amount of air in your child’s lungs and how fast they can blow it out. This helps the doctor find out how severe their asthma is. Other tests can help find asthma triggers. They may include allergy skin testing, blood tests such as immunoglobulin E (IgE) test or radioallergosorbent test (RAST), and X-rays. These tests can help your doctor check if sinus infections or gastroesophageal reflux disease (GERD) are making asthma worse. A test that measures the level of nitric oxide (eNO) in your child’s breath can also point to inflamed airways.
PEDIATRIC ASTHMA TREATMENT
Based on your child's history and how severe their asthma is, their doctor will develop a care plan, called an asthma action plan. This describes when and how your child should use asthma medications, what to do when asthma gets worse, and when to seek emergency care. Make sure you understand this plan and ask your child's doctor any questions you may have.
Your child's asthma action plan is important for controlling their asthma. Keep it handy to remind you of your child's daily management plan, as well as to guide you when your child has asthma symptoms. Give its copies to your child’s caregivers, teachers, and even the bus driver so they’ll know what to do if your child has an asthma attack away from home.
In addition to following your child's asthma action plan, you want to avoid or limit your child's exposure to their asthma triggers.
Most asthma medications that work for adults and older children can also be safely prescribed to toddlers and younger children. Drugs that are approved for younger children are given in doses adjusted for their age and weight. In the case of inhaled drugs, they may need a different delivery device based on their age and ability. For instance, many children can’t coordinate their breathing well enough to use a standard inhaler.
There are two main types of asthma medications:
Quick-relief medications help with sudden symptoms. Your child will take them for fast help during an asthma attack. They need to keep this medicine with them all the time and use it when they have symptoms. Common reliever medicines include:
Inhaled corticosteroids, which reduce swelling in their airways.
Inhaled short-acting beta2-agonists (SABAs), which open their airways so air can flow through more easily. These can have side effects, such as tremors and rapid heart rate.
Short-acting anticholinergics, which open their airways quickly. These may not work as well as SABAs, but they are used in people who have trouble with side effects.
Long-acting medications prevent airway inflammation and keep asthma under control. Your child will probably take them every day. Common controller medicines include:
Corticosteroids by mouth (oral steroids) to reduce inflammation throughout their body.
Inhaled long-acting bronchodilators, such as long-acting beta2-agonists (LABAs) or long-acting muscarinic antagonists (LAMAs), which help prevent their airways from narrowing.
Leukotriene modifiers, which reduce swelling and help keep their airways open. This is usually an oral medicine rather than an inhaler.
Inhaled mast cell stabilizers, which help prevent swelling in their airways when they need to be around allergens or other things that trigger their asthma.
Biologic medicines, which help control asthma that's hard to control. These are generally injections.
Allergy shots (also called subcutaneous immunotherapy), which help turn down their response to allergens so they don't have as many asthma attacks. 
If an infant or older child has symptoms of asthma that require treatment with a bronchodilator medication more than twice a week during the day or more than twice a month at night, most doctors recommend daily anti-inflammatory drugs.
Many asthma medications contain steroids, which could have side effects. They can irritate your child’s mouth and throat. Some research shows that over time, they might result in slow growth, bone problems, and cataracts. After your child takes them, their body might not be able to make as many natural steroids. But without treatment, asthma can lead to health problems and hospital visits. You and your doctor should talk about the pros and cons of medication when you make an asthma action plan.
Kids with asthma need to get a flu shot every fall because flu can make their asthma symptoms worse.
How do I give my child asthma medication?
Your child's doctor will tell you how often to give your child breathing treatments, based on how severe their asthma is.
You may give your child (usually for children under 4) asthma medications using a home nebulizer, also known as a breathing machine. A nebulizer delivers asthma drugs, usually bronchodilators, by changing them from a liquid to a mist. Your child gets the drug by breathing it in through a face mask. These breathing treatments usually take about 10-15 minutes and are given several times a day.
To use the nebulizer:
Wash your hands.
Put the medicine in the nebulizer.
Connect the tubes from the compressor to the base.
Attach the mouthpiece or mask.
Turn the compressor on and watch for a light mist to come from the nebulizer.
Put the mask on your child’s face, or put the mouthpiece in their mouth and have them close their lips around it.
Have them breathe in and out until their treatment time is up.
Turn the nebulizer off when the medicine is gone.
Tell your child to cough to clear out any mucus.
There are guidelines for managing asthma in children up to age 4. This includes the use of quick-relief medications (such as albuterol) for off-and-on symptoms. A low dose of an inhaled steroid or montelukast (Singulair) is the next step. After age 4, the focus shifts from symptom control to disease management. If your child’s asthma is under control for at least 3 months, the doctor may lower their treatment.
PREVENTION
Careful planning and avoiding asthma triggers are the best ways to prevent asthma attacks.
Limit exposure to asthma triggers. Help your child avoid the allergens and irritants that trigger asthma symptoms.
Don't allow smoking around your child. Exposure to tobacco smoke during infancy is a strong risk factor for childhood asthma, as well as a common trigger of asthma attacks.
Encourage your child to be active. As long as your child's asthma is well controlled, regular physical activity can help the lungs work more efficiently.
See your child's health care provider when necessary. Check in regularly. Don't ignore signs that your child's asthma might not be under control, such as needing to use a quick-relief inhaler too often.
Asthma changes over time. Consulting your child's provider can help you make needed treatment adjustments to control symptoms.
Help your child maintain a healthy weight. Being overweight can worsen asthma symptoms, and it puts your child at risk of other health problems.
Keep heartburn under control. Acid reflux or severe heartburn might worsen your child's asthma symptoms. To control acid reflux, your child may need prescription medicines or medicines you can buy off the shelf.
Can childhood asthma be prevented?
You can’t prevent childhood asthma because the exact cause of the condition is unknown. In addition, your child may develop asthma if their immune system is still developing.
How can I lower my child’s risk?
Although childhood asthma isn’t preventable, there are some steps you can take to lower your child’s risk of developing it. These steps include:
Keeping your home free of mold and dampness.
Avoiding smog as much as possible.
Helping your child maintain a healthy weight.
PROGNOSIS
Childhood asthma patterns are strong predictors of long-term outcomes. Episodic asthma tends to result in better adult outcomes, whereas persistent childhood asthma often leads to ongoing symptoms and modest lung function impairment in adulthood. Research suggests that 30% to 70% of children with asthma experience significant improvement or become symptom-free by early adulthood. However, nearly 75% of those with asthma and wheezing during adolescence continue to experience symptoms into adulthood. Persistent asthma is associated with factors such as atopy, low lung function, and increased airway hyperresponsiveness, with sensitization and exposure to indoor allergens posing a 3-fold higher risk.
Effective asthma management is crucial for long-term prognosis. The goals of asthma management include reducing the risk of future exacerbations, preventing hindered lung development in children, preserving lung function, and minimizing adverse medication effects. Factors such as a history of exacerbations within the past year, poor adherence to asthma medication, improper inhaler technique, reduced lung function, smoking or vaping, elevated FENO levels, and blood eosinophilia all contribute to an elevated risk of exacerbations and poorer prognosis.
What can I expect if my child has asthma?
There’s no cure for asthma. But most children can manage their asthma with appropriate treatment and prevention strategies. Untreated asthma can lead to long-term complications such as permanent lung damage.
How will you know when your child's asthma is well-managed?
You’ll know that your child’s asthma is well-managed if, while on medication, your child:
Lives an active, normal life.
Has few troublesome symptoms.
Doesn’t miss school because of symptoms.
Performs daily activities without difficulty.
Has had no urgent visits to their pediatrician, emergency department or hospital.
Has few or no side effects from their medications.
Does childhood asthma go away?
Once a person’s airways become sensitive, they remain that way for life. About half of children who have asthma have a noticeable decrease in symptoms by the time they become adolescents. Therefore, they appear to outgrow childhood asthma.
However, childhood asthma can come back. About half of the children who seem to outgrow their asthma will develop asthma symptoms again in their 30s or 40s. Unfortunately, there’s no way to predict whose symptoms will decrease during adolescence and whose will return later in life.
POSSIBLE COMPLICATIONS
Asthma can cause a number of complications, including:
Severe asthma attacks that require emergency treatment or hospital care.
Permanent decline in lung function.
Missed school days or falling behind in schoolwork.
Poor sleep and fatigue.
Symptoms that interfere with play, sports or other activities.
Complications associated with asthma can stem from the condition itself or from medications and therapeutic interventions. The following lists outline potential complications of asthma:
Complications of Asthma
Pneumonia
Interference with school and sports
Lung remodeling
Poor sleep and fatigue
Death
Complications due to Endotracheal Intubation
Hypotension
Pneumothorax (also a complication of asthma)
Myopathy
Pneumomediastinum (also a complication of asthma)
Pneumoperitoneum
Subcutaneous emphysema
Aspiration
Subglottic stenosis
Infection
Gastrointestinal bleeding due to stress ulcers
Complications due to Medications
Potential neuropsychiatric symptoms such as agitation, depression, insomnia, and suicidal thoughts or actions associated with montelukast, which is a LTRA
Dysphonia and oral candidiasis resulting from ICS
Rare occurrences of adrenal insufficiency attributed to ICS 
Slight reduction in linear growth velocity due to ICS use 
Glaucoma, cataracts, adrenal insufficiency, and hyperglycemia due to OCS
Decreased serum potassium, phosphate, and magnesium, and increase in serum glucose associated with albuterol
Stress-induced or takotsubo cardiomyopathy associated with the treatment of status asthmaticus 
WHEN TO SEE A DOCTOR
Take your child to see a health care provider if you suspect that your child has asthma. Early treatment will help control symptoms and possibly prevent asthma attacks.
Make an appointment with your child's provider if you notice:
Coughing that is constant, is intermittent or seems linked to physical activity.
Wheezing or whistling sounds when your child breathes out.
Shortness of breath or rapid breathing.
Complaints of chest tightness.
Repeated episodes of suspected bronchitis or pneumonia.
Children who have asthma may say things such as, "My chest feels funny" or "I'm always coughing." Listen for coughing in children, which might not wake them, when they are asleep. Crying, laughing, yelling, or strong emotional reactions and stress also might trigger coughing or wheezing.
If your child is diagnosed with asthma, creating an asthma plan can help you and other caregivers monitor symptoms and know what to do if an asthma attack occurs.
When to seek emergency treatment
In severe cases, you might see your child's chest and sides pulling inward when breathing is difficult. Your child might have an increased heartbeat, sweating and chest pain. Seek emergency care if your child:
Has to stop in midsentence to take a breath.
Is using abdominal muscles to breathe.
Has widened nostrils when breathing in.
Is trying so hard to breathe that the abdomen is sucked under the ribs during a breath.
Even if your child hasn't been diagnosed with asthma, seek medical attention immediately if you notice troubled breathing. Although episodes of asthma vary in severity, asthma attacks can start with coughing, which progresses to wheezing and labored breathing.
Red Flag Warnings (Signs of Severe or Life-Threatening Asthma)
Severe difficulty breathing or gasping for air
Inability to speak full sentences or complete words
Use of accessory muscles (neck, chest) to breathe
Cyanosis (blue lips or face)
Rapid worsening of symptoms despite using a rescue inhaler
Silent chest on auscultation (severe obstruction)
Decreased level of consciousness or confusion
Persistent coughing or wheezing that does not improve
If any red flags are present, immediate emergency medical attention is required.
General Advice for Managing Pediatric Asthma
Identify and avoid triggers: common triggers include allergens (dust mites, pollen, pet dander), respiratory infections, cold air, exercise, smoke, and strong odors.
Medication adherence: ensure regular use of controller medications (like inhaled corticosteroids) and proper use of rescue inhalers (like albuterol) when needed.
Asthma action plan: work with healthcare providers to develop a written plan outlining daily treatment, how to recognize worsening symptoms, and steps to take during an attack.
Regular follow-up: routine check-ups with a pediatrician or asthma specialist to monitor control and adjust treatment.
Education: teach the child and family about asthma, inhaler techniques, and when to seek help.
Environmental control: keep living areas clean, use air filters if needed, and avoid smoke exposure.
DIFFERENTIAL DIAGNOSIS
The following list includes the differential diagnoses for asthma in children aged 12 or younger:
U pper Airway Diseases
Allergic rhinitis and sinusitis 
Large Airway Obstruction
Foreign body aspiration
Vascular ring or laryngeal webs
Laryngomalacia
Tracheomalacia
Lymphadenopathy
Mass
Epiglottitis
Vocal cord dysfunction 
Small Airway Obstruction
Bronchiolitis or wheezing associated with respiratory infections
Cystic fibrosis
Primary ciliary dyskinesia
Bronchopulmonary dysplasia 
Other Causes
Congestive heart failure
Gastroesophageal reflux disease
Anaphylaxis
Angioedema
Chronic obstructive pulmonary disease (more likely in adults)
Pulmonary embolism
Recurrent aspiration
Immunodeficiency
Pulmonary edema
Cardiomegaly
Atypical infection with Mycoplasma pneumonia
PEDIATRIC BRONCHIOLITIS
OVERVIEW
Bronchiolitis is a common lung infection in young children and infants. It causes swelling and irritation and a buildup of mucus in the small airways of the lung. These small airways are called bronchioles. Bronchiolitis is almost always caused by a virus.
Bronchiolitis starts out with symptoms much like a common cold. But then it gets worse, causing coughing and a high-pitched whistling sound when breathing out called wheezing. Sometimes children have trouble breathing. Symptoms of bronchiolitis can last for 1 to 2 weeks but occasionally can last longer.
Most children get better with care at home. A small number of children need a stay in the hospital.
Bronchiolitis is a viral infection that targets the small airways of a child’s lungs. It causes wheezing, coughing and a slight fever. It’s most common during colder months. Contact your child’s healthcare provider or emergency services if your child has trouble breathing.
CAUSES AND RISK FACTORS
Bronchiolitis happens when a virus infects the bronchioles, which are the smallest airways in the lungs. The infection makes the bronchioles swollen and irritated. Mucus collects in these airways, which makes it difficult for air to flow freely in and out of the lungs.
Bronchiolitis is usually caused by the respiratory syncytial virus (RSV). RSV is a common virus that infects just about every child by 2 years of age. Outbreaks of RSV infection often happen during the colder months of the year in some locations or the rainy season in others. A person can get it more than once. Bronchiolitis also can be caused by other viruses, including those that cause the flu or the common cold.
The viruses that cause bronchiolitis are easily spread. You can get them through droplets in the air when someone who is sick coughs, sneezes or talks. You also can get them by touching shared items such as dishes, doorknobs, towels or toys and then touching your eyes, nose or mouth.
A virus causes a bronchiolitis infection. Viruses that can lead to bronchiolitis include:
Respiratory syncytial virus (RSV).
Influenza (flu) virus.
Adenovirus.
Parainfluenza.
Metapneumovirus.
SARS-Cov-2 (the virus that causes COVID).
RISK FACTORS
Bronchiolitis usually affects children under the age of 2 years. Infants younger than 3 months have the highest risk of getting bronchiolitis because their lungs and their ability to fight infections aren't yet fully developed. Rarely, adults can get bronchiolitis.
Other factors that increase the risk of bronchiolitis in infants and young children include:
Being born too early.
Having a heart or lung condition.
Having a weakened immune system. This makes it hard to fight infections.
Being around tobacco smoke.
Contact with lots of other children, such as in a child care setting.
Spending time in crowded places.
Having siblings who go to school or get child care services and bring home the infection.
Bronchiolitis most often affects children younger than 2 years old. Adults can get bronchiolitis, but it’s rare. Your child may be more at risk of developing severe bronchiolitis if they:
Were born before 37 weeks of pregnancy.
Have a congenital (present at birth) lung or heart condition.
Have a compromised immune system.
Live or play in group settings like daycare.
SYMPTOMS
For the first few days, the symptoms of bronchiolitis are much like a cold:
Runny nose.
Stuffy nose.
Cough.
Sometimes a slight fever.
Later, your child may have a week or more of working harder than usual to breathe, which may include wheezing.
Many infants with bronchiolitis also have an ear infection called otitis media.
Some children have few or mild symptoms.
Bronchiolitis begins as a mild upper respiratory infection. Within 2 to 3 days, the child develops more breathing problems, including wheezing and a cough.
Symptoms include:
Bluish skin due to lack of oxygen (cyanosis) - emergency treatment is needed
Breathing difficulty including wheezing and shortness of breath
Cough
Fatigue
Fever
Muscles around the ribs sink in as the child tries to breathe in (called intercostal retractions)
Infant's nostrils get wide when breathing
Rapid breathing (tachypnea)
Early signs and symptoms of bronchiolitis resemble those of the common cold, including:
A runny nose.
A slight fever (under 101 degrees Fahrenheit or 38 degrees Celsius).
A cough.
Fatigue.
Fussiness or irritability (infants).
A bronchiolitis infection targets your child’s airways and can cause the following symptoms that affect their breathing:
Rapid or shallow breathing.
Wheezing.
Grunting noises when they breathe.
Flaring of the nostrils.
If your child has trouble breathing or you notice the following symptoms of severe bronchiolitis, call their healthcare provider or visit the emergency room immediately: 
Difficulty sucking and/or swallowing (unable to feed).
Flaring (widening) nostrils when breathing.
Chest retracts during breathing (their skin pulls down tightly against their rib cage and makes their chest look like it’s pulling inward).
Blue, gray or pale skin tone on their lips, fingers or toes (cyanosis).
Dry mouth, not urinating (peeing) or crying without producing tears (dehydration).
DIAGNOSIS METHODS
How is bronchiolitis diagnosed?
Your child’s healthcare provider will diagnose bronchiolitis after a physical exam and testing. During the exam, your child’s provider will check their vital signs and temperature and listen to their breathing through a stethoscope (auscultation).
You can expect your child’s provider to ask you questions about their symptoms, like:
How long has your child had symptoms?
Has your child had a fever?
Has your child been around others who were sick?
Your child’s provider may place a pulse oximeter (pulse ox) on their fingertip or toe to determine how much oxygen is in their blood (blood oxygen level).
Testing might include a nasal fluid culture, which is a swab that collects fluid from the inside of your child’s nose to detect viruses. If your child has severe symptoms, a chest X-ray might be necessary.
Do symptoms of bronchiolitis look similar to other conditions?
Bronchiolitis has symptoms that are similar to other respiratory infections like bronchitis and pneumonia. It may seem like asthma, which can cause wheezing and trouble breathing. You might wonder if your child breathed in (aspirated) something other than air.
Any time that you notice that your child has problems breathing, you should call their healthcare provider. They’ll be able to diagnose one breathing issue from another.
Your child's health care provider can usually diagnose bronchiolitis by the symptoms and listening to your child's lungs with a stethoscope.
Tests and X-rays are not usually needed to diagnose bronchiolitis. But your child's provider may recommend tests if your child is at risk of severe bronchiolitis, if symptoms are getting worse or if the provider thinks there may be another problem.
Tests may include:
Chest X-ray. A chest X-ray can show if there are signs of pneumonia.
Viral testing. A sample of mucus from your child's nose can be used to test for the virus causing bronchiolitis. This is done using a swab that's gently inserted into the nose.
Blood tests. Occasionally, blood tests might be used to check your child's white blood cell count. An increase in white blood cells is usually a sign that the body is fighting an infection. A blood test also can show if the level of oxygen in your child's bloodstream is low.
Your child's provider may look for symptoms of dehydration, especially if your child has been refusing to drink or eat or has been vomiting. Signs of dehydration include dry mouth and skin, extreme tiredness, and making little or no urine.
TREATMENT OPTIONS
At home, fluids by mouth
In the hospital, oxygen therapy and fluids by vein
Home treatment
Most children can be treated at home with fluids and comfort measures.
During the illness, frequent small feedings of clear fluids may be given. Increasing difficulty in breathing, bluish or grayish skin discoloration, fatigue, and dehydration indicate that the child should be hospitalized. Children who have congenital heart disease or lung disease or an impaired immune system may be hospitalized sooner and are far more likely to become quite ill from bronchiolitis.
Hospital treatment
In the hospital, oxygen levels are monitored with a sensor attached to a finger or toe, and oxygen is given by an oxygen tent, nasal tube (cannula), or face mask (see Oxygen administration). Rarely, a ventilator (a breathing machine that helps air get in and out of the lungs) may be needed to assist breathing.
Fluids are given by vein if the child cannot drink adequately.
Inhaled medications that open the airways (bronchodilators) may be tried. Although these medications relieve wheezing and airway narrowing caused by asthma, their effectiveness in treating bronchiolitis is questionable. Corticosteroids (to suppress inflammation) may be beneficial for some children.
Doctors no longer use the antiviral medication ribavirin (given by nebulizer) except for children whose immune system is extremely weak and whose infection is severe. Antibiotics are not helpful unless the child also has a bacterial infection.
Bronchiolitis usually lasts for 1 to 2 weeks but symptoms occasionally last longer. Most children with bronchiolitis can be cared for at home with comfort measures. It's important to be alert for problems with breathing that are getting worse. For example, struggling for each breath, not being able to speak or cry because of struggling to breathe, or making grunting noises with each breath.
Because viruses cause bronchiolitis, antibiotics — which are used to treat infections caused by bacteria — don't work against viruses. Bacterial infections such as pneumonia or an ear infection can happen along with bronchiolitis. In this case, your child's health care provider may give an antibiotic for the bacterial infection.
Medicines called bronchodilators that open the airways don't seem to help bronchiolitis, so they usually aren't given. In severe cases, your child's health care provider may try a nebulized albuterol treatment to see if it helps. During this treatment, a machine creates a fine mist of medicine that your child breathes into the lungs.
Oral corticosteroid medicines and pounding on the chest to loosen mucus, a treatment called chest physiotherapy, have not been shown to be effective for bronchiolitis and are not recommended.
Hospital care
A small number of children may need a stay in the hospital. Your child may receive oxygen through a face mask to get enough oxygen into the blood. Your child also may get fluids through a vein to prevent dehydration. In severe cases, a tube may be guided into the windpipe to help breathing.
The main focus of treatment is to relieve symptoms, such as difficulty breathing and wheezing. Some children may need to stay in the hospital if their breathing problems do not improve after being observed in the clinic or emergency room.
Antibiotics do not work against viral infections. Medicines that treat viruses may be used to treat very ill children.
At home, measures to relieve symptoms can be used. For example:
Have your child drink plenty of fluids. Breast milk or formula is fine for children younger than 12 months. Electrolyte drinks, such as Pedialyte, are also OK for infants.
Have your child breathe moist (wet) air to help loosen sticky mucus. Use a humidifier to moisten the air.
Give your child saline nose drops. Then use a nasal suction bulb to help relieve a stuffy nose.
Be sure your child gets plenty of rest.
Do not allow anyone to smoke in the house, car, or anywhere near your child. Children who are having trouble breathing may need to stay in the hospital. There, treatment may include oxygen therapy and fluids given through a vein (IV).
PREVENTION
Because the viruses that cause bronchiolitis spread from person to person, one of the best ways to prevent infection is to wash your hands often. This is especially important before touching your baby when you have a cold, flu or other illness that can be spread. If you have any of these illnesses, wear a face mask.
If your child has bronchiolitis, keep your child at home until the illness is past to avoid spreading it to others.
To help prevent infection:
Limit contact with people who have a fever or cold. If your child is a newborn, especially a premature newborn, avoid being around people with colds. This is especially important in the first two months of life.
Clean and disinfect surfaces. Clean and disinfect surfaces and items that people often touch, such as toys and doorknobs. This is especially important if a family member is sick.
Wash hands often. Frequently wash your own hands and those of your child. Wash with soap and water for at least 20 seconds. Keep an alcohol-based hand sanitizer handy to use when you're away from home. Make sure it contains at least 60% alcohol.
Cover coughs and sneezes. Cover your mouth and nose with a tissue. Throw away the tissue. Then wash your hands. If soap and water aren't available, use a hand sanitizer. If you don't have a tissue, cough or sneeze into your elbow, not your hands.
Use your own drinking glass. Don't share glasses with others, especially if someone in your family is ill.
Breastfeed, when possible. Respiratory infections are less common in breastfed babies.
Can bronchiolitis be prevented?
It’s difficult to prevent bronchiolitis since the viruses that cause it are common. You can take steps to lower your child’s risk of developing bronchiolitis by:
Avoiding others who are sick.
Practicing good handwashing.
Washing and sanitizing frequently touched surfaces or objects like toys.
Not sharing cups, forks or spoons.
Until your child is better, keep them home from daycare or other environments where they’re in close contact with others. The virus that caused their illness is contagious.
Most cases of bronchiolitis cannot be prevented because the viruses that cause the infection are common in the environment. Careful hand washing, especially around infants, can help prevent the spread of viruses.
A medicine called palivizumab (Synagis) that boosts the immune system may be recommended for certain children. Your child's provider will let you know if this medicine is right for your child.
PROGNOSIS
The prognosis of bronchiolitis is generally favorable, with most infants recovering within 5 to 7 days. Although some studies suggest an increased risk of asthma following bronchiolitis, only a small percentage of affected children develop asthma. A history of recurrent wheezing and a positive family history of asthma, allergies, or atopic dermatitis may increase the likelihood of asthma development in these patients in the future.
What can I expect if my child has bronchiolitis?
Your child may have symptoms for up to a week if they have bronchiolitis. During their illness, they may have trouble eating full meals or lose their appetite. To help your child eat when they don’t want to, try feeding them multiple small meals throughout the day instead of larger meals less often. It’s important to keep your child hydrated since they’re at a high risk of dehydration during their illness.
To alleviate your child’s symptoms, talk to their healthcare provider to see what’s safe for your child to take, like over-the-counter (OTC) medications to reduce a fever. Don’t give your child aspirin, as it can lead to Reye’s syndrome.
If your child has symptoms that don’t improve after one week or get worse, contact their healthcare provider. If your child has trouble breathing, contact emergency services or visit the emergency room immediately.
Some children develop asthma as they grow if they had bronchiolitis when they were infants. While less common, some children may develop pneumonia after bronchiolitis.
Can you have bronchiolitis more than once?
Yes. Since there are many viruses that can cause bronchiolitis, your child can develop the infection more than once.
Most children recover at home in 3 to 5 days. However, wheezing and coughing may continue for 2 to 4 weeks. With proper care, the chance of developing serious consequences due to bronchiolitis is low, even for children who need to be hospitalized.
Some children have repeated episodes of wheezing after having had bronchiolitis in early childhood.
POSSIBLE COMPLICATION
Complications from bronchiolitis are uncommon but can be severe, particularly in infants who are premature or have underlying pulmonary, cardiac, or immunocompromised conditions. Even healthy infants may experience acute or chronic complications, although most recover without incident.
 Acute complications of bronchiolitis include:
Aspiration
Respiratory failure
Apnea
Secondary bacterial infections
Death
Chronic complications of bronchiolitis include:
Recurrent episodes of wheezing
Bronchiolitis obliterans 
Complications of bronchiolitis include:
Pneumonia.
Asthma.
Respiratory failure.
Bronchiolitis can be life-threatening if your child has severe symptoms that affect their ability to breathe. If you notice your child has difficulty breathing, contact 911 or local emergency services or visit the emergency room immediately.
WHEN TO SEE A DOCTOR
If symptoms become serious, call your child's health care provider. This is especially important if your child is younger than 12 weeks old or has other risk factors for bronchiolitis for example, being born too early, also called premature, or having a heart condition.
Get medical attention right away if your child has any of these symptoms:
Has blue or gray skin, lips and fingernails due to low oxygen levels.
Struggles to breathe and can't speak or cry.
Refuses to drink enough, or breathes too fast to eat or drink.
Breathes very fast in infants this can be more than 60 breaths a minute with short, shallow breaths.
Can't breathe easily and the ribs seem to suck inward when breathing in.
Makes wheezing sounds when breathing.
Makes grunting noises with each breath.
Appears slow moving, weak or very tired.
Red Flag Warnings (Signs of Severe Bronchiolitis)
Severe difficulty breathing or very rapid breathing (usually >70 breaths/min in infants)
Persistent grunting or nasal flaring
Cyanosis (blue lips, face, or nails)
Decreased level of consciousness or extreme lethargy
Inability to feed or drink due to breathlessness or fatigue
Apnea (pauses in breathing)
Poor oxygen saturation (usually below 90-92% on room air)
Severe chest retractions or use of accessory muscles
If any red flags are present, immediate medical attention is critical.
General Advice for Pediatric Bronchiolitis
Supportive care: Most cases are viral and self-limiting. Focus on hydration, nasal suctioning, and maintaining comfort.
Monitor breathing: Watch for increased work of breathing or worsening symptoms.
Hydration: Encourage frequent small feeds or fluids; consider IV fluids if oral intake is poor.
Avoid irritants: Keep away from smoke and other respiratory irritants.
Fever management: Use age-appropriate doses of fever reducers like acetaminophen or ibuprofen if needed.
Avoid unnecessary medications: Antibiotics are generally not recommended unless there’s a bacterial co-infection.
Hospitalization: Consider if the child shows severe respiratory distress, dehydration, or oxygen desaturation.
Prevention: Practice good hand hygiene, avoid contact with sick individuals, and consider prophylaxis (like palivizumab) for high-risk infants.
DIFFERENTIAL DIAGNOSIS
The differential diagnoses of bronchiolitis include:
Gastroesophageal reflux
Aspiration pneumonia
Foreign body aspiration into the lower airway
Congenital malformations, such as vascular rings or slings
Acute exacerbation of asthma
Acute anaphylaxis
PEDIATRIC PNEUMONIA
OVERVIEW
Pneumonia is an inflammation in one or both of the lungs that is almost always caused by a viral or bacterial infection.  The inflammation interferes with the body’s ability to deliver oxygen and remove carbon dioxide from the blood.  A person is more likely to get pneumonia as a child, known as pediatric pneumonia, than they are as an adult.
Symptoms of pediatric pneumonia depend on the cause of the infection and several other factors, including the age and general health of the child. Rapid breathing, a high temperature and coughing are three of the most common signs of the condition.  
Pneumonia in newborns and very young children is more likely to be caused by a viral, rather than a bacterial infection. Potential viral causes for pneumonia include respiratory syncytial virus or influenza infection. Bacterial infections become more common in school-aged children and young adolescents. The most common bacterial cause for pneumonia is a type of bacterium known as streptococcus pneumoniae, but there are several other bacterial infections that can also cause pneumonia. 
Diagnosis is generally based on a physical exam and several other tests, which may include blood tests and an X-ray. 
The prognosis for pediatric pneumonia is generally good. A bacterial infection can often be treated with antibiotics, such as amoxicillin. Viral pneumonia usually resolves on its own without the need for medication.   However, parents and guardians should be vigilant, as the condition is often hard to spot in children. Most deaths from pediatric pneumonia occur due to underlying health conditions, such as heart disease. ref1
Vaccination against bacterial infection is the best way of preventing the spread of pediatric pneumonia.  Children aged over six months old may also benefit from the influenza vaccine.
Pneumonia is a form of acute respiratory infection that affects the lungs. The lungs are made up of small sacs called alveoli, which fill with air when a healthy person breathes. When an individual has pneumonia, the alveoli are filled with pus and fluid, which makes breathing painful and limits oxygen intake.
Pneumonia is the single largest infectious cause of death in children worldwide. Pneumonia killed 740 180 children under the age of 5 in 2019, accounting for 14% of all deaths of children under 5 years old but 22% of all deaths in children aged 1 to 5 years. Pneumonia affects children and families everywhere, but deaths are highest in southern Asia and sub-Saharan Africa. Children can be protected from pneumonia, it can be prevented with simple interventions, and it can be treated with low-cost, low-tech medication and care.
Pediatric pneumonia is an infection of the lungs in children, commonly presenting with symptoms like coughing, fever, and difficulty breathing, and is a leading cause of death in children globally, especially in developing regions. Diagnosis often involves physical exams, checking for rapid breathing or chest retractions, and may include chest X-rays or blood tests. Treatment varies depending on the cause, with antibiotics for bacterial pneumonia and supportive care for viral pneumonia
CAUSES AND RISK FACTORS
Pneumonia is an inflammation of the air sacs, also known as the alveoli, in the lungs, usually caused by infection that causes them to fill with fluid or pus. 
This inflammation interferes with the lungs’ ability to breathe and properly supply oxygen to the body, causing many of the symptoms described above.
Pneumonia is almost always caused by bacteria or a virus. In children below school age, viral infection is the most common cause. School-aged children and young adolescents are more likely to develop a bacterial infection. 
Potential viral infections
Viral infections that can cause pediatric pneumonia include: 
Respiratory syncytial virus (RSV), which is the most common viral cause
Influenza
Parainfluenza virus, which also causes croup
And less commonly:
Rhinovirus
Adenovirus
Human Metapneumovirus
Enterovirus
Coronavirus
Herpes simplex virus
Potential bacterial infections
The most common bacterial cause of pediatric pneumonia is Streptococcus pneumoniae bacteria. This bacterium is often carried underneath the nose of healthy people without causing any harm, but can develop into pneumonia if the bacterium spreads to those with vulnerable immune systems, such as children and the elderly. 
Another possible bacterial cause is Mycoplasma pneumoniae, which usually causes a milder form of the condition known as walking pneumonia or atypical pneumonia. Walking pneumonia is sometimes confused with the common cold and does not usually require bed rest. However, in some cases, symptoms following infection with Mycoplasma pneumoniae will be as severe as those caused by other types of bacteria.  
A few other types of bacteria can cause pneumonia in children, including group B streptococcus and staphylococcus aureus.
Other potential causes
In rare cases, there can be another cause for a child developing pneumonia. These include other potential infectious causes, such as:
Aspiration pneumonia, which is when food, saliva or stomach acid enters the lungs
Inhaling fungi, such as from soil
There are also non-infectious causes, such as inhaling harmful substances and chemicals.
Pneumonia is an acute respiratory infection of the lungs. It doesn’t have one single cause – it can develop from either bacteria, viruses or fungi in the air. When a child is infected, their lungs get inflamed, may fill with fluid or pus and it becomes difficult to breathe. Children whose immune systems are immature (i.e. newborns or premature babies) along with those with poor immunities (i.e. due to undernourishment or diseases like HIV) are more vulnerable to pneumonia.
Viruses: Most common cause in older infants and toddlers, and children aged 2-5 years. Respiratory syncytial virus (RSV) is a common culprit.
Bacteria: Streptococcus pneumoniae is a common bacterial pathogen, especially in infants. Other bacteria like group B streptococci and E. coli can affect newborns.
Infectious agents: Pneumonia is primarily caused by infections, with common culprits including bacteria (like Streptococcus pneumoniae), viruses (such as adenoviruses and influenza), and less commonly, fungi.
Aspiration: In some cases, pneumonia can result from inhaling foreign material or fluids into the lungs. 
Risk Factors for Pediatric Pneumonia:
Immune system vulnerability: Young infants, premature babies, and children with weakened immune systems due to malnutrition or pre-existing illnesses (like HIV or measles) are at a higher risk.
Environmental factors:
Indoor air pollution: Exposure to cooking fuels (like wood or dung) and cigarette smoke significantly increases the risk of pneumonia.
Crowding: Living in crowded conditions can facilitate the spread of infections.
Nutritional status: Malnutrition and sub-optimal breastfeeding practices are major risk factors, especially in low- and middle-income countries.
Other factors:
Inadequate immunization: Lack of essential vaccinations, such as the pneumococcal conjugate vaccine, increases susceptibility to specific types of pneumonia.
Low birth weight and prematurity: Babies born with low birth weight or prematurely are more vulnerable.
Presence of comorbidities: Existing chronic conditions can make children more prone to developing pneumonia.
Daycare attendance: Children attending daycare are at a higher risk due to increased exposure to infections.
SYMPTOMS OF PEDIATRIC PNEUMONIA
Symptoms of pediatric pneumonia depend on a number of factors, particularly the age of the affected child, and whether the cause of the infection is bacterial or viral. 
Symptoms in newborns
Newborns and babies under a month old are the only age group that rarely cough as a direct consequence of pneumonia. The most common symptoms are irritability and not feeding properly. A child of this age may also display: 
Abnormally fast breathing
Shortness of breath
Grunting sounds
Symptoms in babies over a month old
Once a baby is over a month old, then the most noticeable symptom of pneumonia is likely to be coughing. All of the symptoms that affect newborns will possibly be present too, although grunting becomes less common as the baby grows older. Other pneumonia symptoms observed in babies of this age include: 
Congestion, the feeling that the chest is full or clogged
Wheezing or heavy breathing
Fever, particularly during pneumonia caused by bacterial infection
Toddlers and preschoolers
Fever and cough are the most common symptoms for children over a year old. Other typical symptoms include: 
Abnormally rapid breathing
Congestion
Vomiting, particularly following coughing
Older children
Fever and coughing remain the most common signs of pneumonia in children of school age. They might also complain of the following symptoms: 
Chest pain
Tiredness
Vague stomach pain
Other possible symptoms of pneumonia at this age include:
Vomiting
Diarrhea
Sore throat
Ear ache
Other symptoms;
Fever (often high)
Cough (may be dry or productive)
Rapid breathing (tachypnea)
Difficulty breathing or shortness of breath
Chest pain, especially when coughing or breathing deeply (older children)
Wheezing or crackles heard on lung auscultation
Nasal flaring, grunting, or chest retractions (in infants and younger children)
Poor feeding or vomiting (especially in infants)
Lethargy or irritability
DIAGNOSIS 
The first step in a diagnosis of pediatric pneumonia is usually a physical exam, where a doctor assesses the child’s symptoms. Their temperature will typically be taken and the doctor will listen to their chest with a stethoscope. 
A particular challenge is finding out whether the pneumonia is bacterial or viral in origin. A doctor may ask questions about recent travel history and check their vaccination history.
A chest X-ray is sometimes used to confirm the diagnosis or to look for any complications that pneumonia may have caused around the lungs. In recent years, there has been promising progress in the use of ultrasound, a scan that uses sound waves to produce an image of the inside of the body, to diagnose pneumonia. Ultrasound may replace X-ray testing at some point in the future. 
Other potential tests include:   
A blood test can help determine whether an infection is present, the extent of its spread and possibly the cause
A sputum test, which is when a sample of spit or phlegm is laboratory-tested. This might confirm if a certain type of bacterium is causing the pneumonia
A pulse oximetry, which is a test to measure oxygen levels in the blood
A bronchoscopy, which is when a tube with a camera and light attached to the end is guided into the lungs so a doctor can look inside. This is rare and generally only used in complicated pneumonia cases
Laboratory evaluation in children suspected of having pneumonia should ideally start with non-invasive, rapid bedside testing, including nasopharyngeal swab assays for influenza, respiratory syncytial virus, and human metapneumovirus when available and appropriate. This can help minimize unnecessary imaging and antibiotic treatment in children with influenza or bronchiolitis. Children who present with severe disease and appear toxic should have complete blood count (CBC), electrolytes, renal/hepatic function testing, and blood cultures performed. These tests are generally not required in children who present with mild disease. Inflammatory markers do not help distinguish between viral and bacterial pneumonia in the pediatric population. However, these tests may be obtained to trend disease progression and serve as prognostic indicators. Children who have been in areas endemic to tubercluosis, or have an exposure history, and present with signs and symptoms suspicious of pneumonia should have sputum samples or gastric aspirates collected for culture.
Sputum Gram stain and culture are not productive as the samples are often contaminated by oral flora. Blood cultures can be done but are often negative. Today, serology is being used to determine the presence of mycoplasma, legionella, and chlamydia species. Polymerase chain reaction is becoming available in most hospitals, but still, the results take 24 to 48 hours. There are no clear guidelines for the routine use of chest x-ray in the pediatric population. Although the chest x-ray can be helpful in the diagnosis and confirmation of pneumonia, it carries with it risks, including radiation exposure, healthcare-associated costs, and false-negative results, increasing the use of unwarranted antibiotics. Imaging should be restricted to children who appear toxic, those with a recurrent or prolonged course of illness despite treatment, and infants ages 0 to 3 months with a fever, suspected foreign body aspiration, or congenital lung malformation. Imaging can also be considered in children younger than 5 years old who present with fever, leukocytosis, and no identifiable source of infection. Imaging may also be useful in those with acute worsening of upper respiratory infections or to rule out underlying mass in children who have "round pneumonia." 
TREATMENT
Treatment of pediatric pneumonia depends on the child’s age and health, as well as the cause of the infection. In most cases, particularly with school-age children, pneumonia can be treated or managed at home. Children with bacterial infections will generally be given antibiotics, whereas viral infections usually resolve themselves without the need for additional medication.
Sometimes a child may need to be hospitalized for treatment. The decision whether to hospitalize is typically based on factors such as:   
The child’s breathing ability
The age of the child
The risk of complications due to the type of pneumonia or any underlying health conditions the child may have
The level of oxygen in the blood
The presence of any unusual symptoms, such as altered mental states
Hospitalization will usually involve giving the affected child supplemental oxygen, monitoring their condition and treatment with antibiotics. Any complications may also need to be addressed.
Pneumonia medication
Children being treated at home for bacterial pneumonia infections will generally be prescribed an antibiotic drug called amoxicillin, which can come in tablet or liquid form. If this is unsuccessful, then a doctor may recommend alternative antibiotics.  
Macrolide antibiotics, such as azithromycin, may be recommended if it is thought the child is affected by atypical pneumonia. 
The antibiotics used in hospital will generally vary from those recommended for use at home. A doctor will base the antibiotic treatment on several factors, such as whether a child has been immunized. In hospital, the antibiotics may be delivered directly into the bloodstream using an intravenous drip.  
Antibiotics are not a useful treatment for children with viral infections. However, if the pneumonia has been caused by an influenza infection, antiviral therapy may be used to fight the spread of the disease.
A child’s fever symptoms might be helped with acetaminophen whether they are in the hospital or being treated at home.
Care tips at home
If a doctor decides a child should be treated at home, their caregiver should take the following steps:
Keep them hydrated by offering plenty of fluids.
If a child’s cough is producing mucus, encourage spitting the phlegm out to clear the airwaves. If the child is too young to understand, then an adult should lay the infant across their lap and pat their back when coughing fits occur
Relieve chest pain with a heated pad or warm compress on the chest area
If a child’s lips or nails are bluish or grey then this may indicate that they are not getting enough oxygen. This is a sign that medical professionals should be contacted.
Treatment should be targeted to a specific pathogen that is suspected based on information obtained from history and physical exam. Supportive and symptomatic management is key and includes supplemental oxygen for hypoxia, antipyretics for fever, and fluids for dehydration. This is especially important for non-infectious pneumonitis and viral pneumonia for which antibiotics are not indicated. Cough suppressants are not recommended.
If bacterial pneumonia is suspected, treat empirically with antibiotics, keeping in mind significant history and bacterial pathogens that are common to specific age groups. Neonates should receive ampicillin plus an aminoglycoside or third-generation cephalosporin; however, not ceftriaxone, as it can displace bound bilirubin and lead to kernicterus. Atypical pneumonia is common in infants 1 to 3 months old, and this group should have additional antibiotic coverage with erythromycin or clarithromycin. For infants and children over 3 months old, S pneumoniae is the most common, for which the drug of choice is high-dose oral amoxicillin or another beta-lactam antibiotic. In children older than 5, atypical agents have a more important role, and macrolide antibiotics are usually first-line therapy. 
Special attention should be given to children with chronic illnesses, as these might alter choices for antibiotics. Children with sickle cell anemia will need cefotaxime, macrolide, and vancomycin if severely ill. Children with cystic fibrosis will require piperacillin or ceftazidime plus tobramycin. Treat fulminant viral pneumonia as indicated, depending on the virus identified. For Varicella, use acyclovir, and for the respiratory syncytial virus (RSV), use ribavirin for high-risk patients. Patients with human immunodeficiency virus should be treated with sulfamethoxazole/trimethoprim and prednisone, and for cytomegalovirus, ganciclovir, and gamma globulin are the preferred agents. If methicillin-resistant S aureus (MRSA) is suspected, clindamycin or vancomycin may be given.
It is important to have a high index of suspicion for complications, especially in patients returning for repeat evaluation. For patients sent home with symptomatic or supportive management for suspected viral pneumonia, consider a secondary bacterial infection or other diagnoses upon re-evaluation. Children with uncomplicated bacterial infections who fail to respond to treatment within 72 hours should be assessed for complications, including pneumothorax, empyema, or pleural effusion. Other systemic complications of pneumonia include sepsis, dehydration, arthritis, meningitis, and hemolytic uremic syndrome.
Neonates and infants younger than 90 days old should be hospitalized for treatment, in addition to children who are immunocompromised or have other underlying chronic diseases like sickle cell anemia or cystic fibrosis. Children with social factors that preclude access to care have failed outpatient therapy, or present with presumed tuberculosis should also be hospitalized. Admission is often required for patients with respiratory distress and low oxygenation. In most cases, the presence of a parapneumonic effusion requires admission. Children with severe respiratory distress may require chest therapy or even mechanical ventilation. A large pleural effusion requires drainage for diagnostic and therapeutic purposes. In patients with empyema, early video-assisted thoracic surgery (VATS) correlates with decreased mortality, hospital stay, and ionizing radiation from CT scans. It is essential to ensure that clear discharge instructions and return precautions are given to parents or caregivers of children being discharged home in addition to close pediatrician follow-up.
PREVENTION
There are two vaccines that the Centers for Disease Control and Prevention (CDC) recommends in the U.S. to protect against Streptococcus pneumoniae bacteria, the most common cause of bacterial pneumonia.  It can also cause a number of other conditions, including bronchitis and meningitis. Conditions caused by infection with Streptococcus pneumoniae bacteria are known as pneumococcal disease.
The PCV13 vaccine is recommended for all children under the age of two. It protects against 13 of the most common strains of pneumococcal bacteria. The first shot should be given at two months old, the next two at four months and six months old. A final booster shot should be administered when the child is between 12 and 15 months old. For children older than this age, a single shot is usually all that is needed.
The PPSV23 vaccine is recommended for children older than two, who have certain medical conditions, such as leukemia or chronic cardiovascular disease. PPSV23 protects against 23 types of pneumococcal bacteria. Children with these medical conditions will generally need two doses of PPSV23.
Side effects are rare. Allergic reactions to the vaccine are thought to occur only in one in a million cases. Nevertheless, a doctor should be told if a person has had an allergic reaction to any other vaccine. Taking a vaccine should usually be delayed if the child feels ill, but a doctor will be able to advise on this.
The introduction of a vaccine against Streptococcus pneumoniae in the U.S. has coincided with a significant decline in pneumococcal disease in children under the age of five.
Influenza vaccination
One of the potential viral causes of pneumonia is influenza infection, also known as the flu. The influenza vaccination cannot stop all children from getting the flu, but it is the best means of defending against infection.
The CDC recommends that all children aged over six months should get a flu vaccine shot once a year before the end of October. A new vaccination is needed every year, because the specific influenza viruses that are spreading through the population change as time passes.
Babies younger than six months old are too young to be vaccinated. The best way to ensure that they are protected against the flu is to make sure everyone around them is vaccinated.
Other names for pediatric pneumonia
Childhood lung infection
Pneumonia, children
Pneumonia, infants
PROGNOSIS
For most children, the prognosis is good. Viral pneumonia tends to resolve without treatment. Long-term sequelae are rare. However, both staphylococcal and varicella pneumonia have guarded outcomes in children. Children with tuberculosis are at high risk for disease progression if the condition is not treated. Immunocompromized children have the worst prognosis. Each year, roughly 3 million children die from pneumonia, and the majority of these children also have other comorbidities like congenital heart disease, immunosuppression, or chronic lung disease of prematurity.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis for pediatric pneumonia includes:
Alveolar proteinosis
Aortic stenosis
Aseptic meningitis 
Asphyxiating thoracic dystrophy 
Aspiration syndromes 
Asthma 
Atelectasis
AV septal defect, complete 
AV septal defect, unbalanced 
Bacteremia
Birth trauma
POSSIBLE COMPLICATIONS
Complications of pediatric pneumonia include the following:
Empyema
Pleural effusion
Lung abscess
Necrotizing pneumonia
Sepsis
WHEN TO SEE A DOCTOR
You should seek medical attention for a child with suspected pneumonia if they exhibit symptoms like a persistent cough, fever, difficulty breathing (wheezing, grunting, rapid breathing), chest pain, vomiting, or loss of appetite. Seek immediate care if you notice signs of severe pneumonia such as extreme sleepiness, difficulty waking, dehydration, or a bluish discoloration of the lips or fingertips. 
When to Contact a Medical Professional:
Persistent or Worsening Cough: A cough that is severe, lasts longer than a few days, or brings up yellow or green mucus, especially with a fever.
Breathing Difficulties: Rapid breathing, grunting or wheezing sounds, labored breathing, nasal flaring, or the muscles under the ribcage drawing in with each breath.
High Fever: A fever that doesn't respond to medication or is accompanied by other concerning symptoms.
Vomiting and Dehydration: If your child is vomiting and unable to keep down liquids, which can lead to dehydration.
Chest Pain: Especially if it worsens with coughing or breathing.
Loss of Appetite or Poor Feeding: In infants, poor feeding can lead to dehydration.
Unusual Fatigue or Lethargy: If your child is excessively tired or unresponsive.
Signs of Severe Pneumonia: Seizures, a bluish color in lips or fingertips, or significant difficulty breathing requiring oxygen or mechanical ventilation.
Underlying Health Conditions: Children with weakened immune systems or chronic health conditions are at higher risk for severe pneumonia and should be seen by a doctor sooner. 
Important Considerations:
Early Intervention: Do not wait for symptoms to worsen before seeking medical advice.
Hospitalization: Severe pneumonia, respiratory distress, or inability to take oral medications may require hospitalization.
Viral vs. Bacterial: Viral pneumonia symptoms may last longer and cannot be treated with antibiotics, while bacterial pneumonia typically requires antibiotics and usually improves with appropriate treatment.
Avoid Cough Syrups: Cough medicines are generally not helpful for children with pneumonia.
Hydration: Encourage your child to drink small amounts of fluids frequently to prevent dehydration.
Red Flag Warnings (Signs of Severe or Complicated Pneumonia)
Very rapid breathing (age-specific cutoffs:
60 breaths/min if <2 months
50 breaths/min if 2–12 months
40 breaths/min if 1–5 years)
Severe chest indrawing or retractions
Cyanosis (blue discoloration of lips, face, or extremities)
Grunting or nasal flaring
Inability to feed or drink
Persistent vomiting
Decreased level of consciousness or severe lethargy
Signs of dehydration
Oxygen saturation below 90–92% on room air
Seizures or convulsions
If any red flags are present, urgent medical evaluation and treatment are needed.
General Advice for Pediatric Pneumonia
Medical evaluation: Always seek healthcare assessment for suspected pneumonia, especially if symptoms worsen or red flags are present.
Antibiotic treatment: Often required for bacterial pneumonia—prescribed by a healthcare provider.
Supportive care: Ensure adequate hydration and fever control (acetaminophen or ibuprofen).
Monitor breathing: Watch for worsening respiratory distress or decreased oxygen levels.
Rest and nutrition: Encourage rest and appropriate feeding.
Hospitalization: May be necessary for severe cases or infants, especially if breathing is compromised or oxygen is low.
Prevention: Ensure up-to-date vaccinations (e.g., pneumococcal, Hib, influenza), good hand hygiene, and avoid exposure to smoke.
PEDIATRIC CROUP
OVERVIEW
Croup refers to an infection of the upper airway, which becomes narrow, making it harder to breathe. Croup also causes a cough that sounds like barking.
The cough and other signs and symptoms of croup are the result of swelling and irritation around the voice box (larynx), windpipe (trachea) and bronchial tubes (bronchi). When a cough forces air through this narrowed passageway, the swollen vocal cords produce a noise like a seal barking. Taking a breath often produces a high-pitched whistling sound called stridor.
Croup most often occurs in younger children. It usually isn't serious. Most children can be treated for croup at home.
Croup (laryngotracheobronchitis) is a respiratory infection that affects young children. Viral infections are the most common cause of the condition. Croup causes swelling of your child’s voice box (larynx) and windpipe (trachea). This swelling causes the airway below their vocal cords to narrow, which makes their breathing noisy and difficult.
Croup in babies is most common, along with children younger than 3 years old. As children get older, croup isn’t seen as often. This is because their windpipes get larger and swelling is less likely to get in the way of their breathing.
Croup causes a distinctive cough that may sound similar to the call of a seal. The condition is usually mild but symptoms can become severe and life-threatening.
RSV vs. croup — what’s the difference?
RSV (respiratory syncytial virus) and croup are both respiratory illnesses that can affect babies and young children. RSV is a viral infection that can affect both children and adults. It causes coughing, sneezing and other cold-like symptoms.
While RSV is its own illness, the respiratory syncytial virus is also one of the viruses that can lead to croup.
Whooping cough vs. croup — what’s the difference?
Whooping cough (pertussis) and croup are both respiratory infections that can affect babies and children. Both conditions cause a distinctive cough, although the sound of whooping cough is a more high-pitched gasping or “whooping” noise.
Whooping cough is a bacterial infection whereas a viral infection usually causes croup. Therefore, no vaccines can prevent croup and antibiotics can’t treat it. (Antibiotics can’t kill viruses.) There’s a vaccine to prevent whooping cough, but it doesn’t go away quickly on its own as croup usually does.
How common is croup?
Croup affects about 3% of U.S. children every year. The condition accounts for 7% of all hospitalizations in children younger than 5 years old. It’s more common in males. Healthcare providers define about 85% of croup cases as mild. They consider less than 1% of cases severe.
CAUSES AND RISK FACTORS
Croup is usually caused by a viral infection, most often a parainfluenza virus.
Your child may get a virus by breathing infected respiratory droplets coughed or sneezed into the air. Virus particles in these droplets may also survive on toys and other surfaces. If your child touches a surface with a virus on it, and then touches the eyes, nose or mouth, an infection may follow.
The most common cause of croup is a viral infection. Croup viruses include parainfluenza, influenza, respiratory syncytial virus (RSV), measles and adenovirus. Viral croup causes your child’s upper airways to swell, making it difficult for them to breathe. However, these viruses are common and most children with viral infections don’t develop croup. Rarely, bacteria can complicate the viral infection and make it more difficult to breathe.
Is croup contagious?
Yes, croup is highly contagious because the viruses that lead to the condition are easily spreadable.
How do you get croup?
The viruses that cause croup spread easily through the air. When someone with a viral or bacterial infection that can cause croup sneezes or coughs, they send respiratory droplets containing croup-causing germs into the air. When your child breathes in these droplets, they can catch an illness that’ll cause croup. Your child can also get croup by touching objects contaminated by germs that can cause croup.
How long is croup contagious?
Your child is contagious for three days after their symptoms first appeared or until their fever is gone. You should keep your child home from school until 24 hours have passed without a fever and without using fever-reducing medication.
RISK FACTORS
Children between 6 months and 3 years of age have the highest risk of getting croup. Because children have small airways, they're likely to have more symptoms with croup. Croup rarely occurs in children older than 6 years of age.
Infants and children may be at increased risk of croup if they:
Are under 5 years of age
Are male
Have had croup before
Croup is more prevalent in the fall and winter than in the spring and summer, like many other viruses. Therefore, infants and children may be more likely to get croup during colder months of the year.
SYMPTOMS OF PEDIATRIC CROUP
What does croup sound like?
The croup cough sounds like a harsh “barking” sound. This is the most common symptom of croup. Your child may also have stridor, which is a raspy, vibrating sound that occurs when your child is breathing in.
What are the other symptoms of croup?
Croup is typically mild and lasts less than one week, but symptoms can get more severe. Symptoms normally start slowly and may begin with a runny or stuffy nose. Over the next 12 to 48 hours, symptoms can worsen and the barking cough may start. Symptoms are usually worse at night.
Other mild croup symptoms include:
Hoarseness.
Fever.
Rash.
Eye redness (conjunctivitis).
Swollen lymph nodes.
Symptoms of moderate to severe croup may include:
Difficulty breathing.
Restlessness or nervousness.
Retractions (sucking in the skin around your child’s ribs and the top of their breastbone).
Cyanosis (blue-tinged skin).
Croup often begins as an ordinary cold. If there's enough swelling, irritation and coughing, a child can develop:
Loud barking cough that's made worse by crying and coughing, as well as anxiety and distress, setting up a cycle of worsening symptoms.
Fever.
Hoarse voice.
Noisy or labored breathing.
Symptoms of croup are often worse at night and usually last for 3 to 5 days.
Infants and children who have croup often experience:
Coughing that sounds like a barking seal
Sneezing
Runny nose
Congestion
Low-grade fever
Hoarseness
A high-pitched whistling noise when the child inhales (stridor)
Difficulty breathing
Retraction (sinking in) of the chest when inhaling
Bluish lips
It’s common for croup symptoms to become worse in the evening and overnight. Sometimes, difficulty breathing or other symptoms can awaken a child from sleep. Symptoms typically improve in the morning and worsen again in the evening.
Other symptoms;
Barking cough (often described as "seal-like")
Hoarseness
Stridor (a high-pitched, wheezing sound heard when inhaling, especially during crying or agitation)
Fever (usually low-grade)
Runny nose and congestion (early signs)
Respiratory distress in more severe cases (nasal flaring, chest retractions)
Symptoms typically begin suddenly, often worsening at night, and peak over 2–3 days before gradually improving.
DIAGNOSIS 
Croup is usually diagnosed by a health care provider. The provider:
Observes your child's breathing.
Listens to your child's chest with a stethoscope.
Examines your child's throat.
Sometimes X-rays or other tests are used to rule out other possible illnesses.
How can I tell if my child has croup?
You can usually tell if your child has croup based on their signs and symptoms. The most common symptoms are a barking cough and stridor. This condition is especially widespread in the fall and winter months. If your child’s condition is severe, a healthcare provider may order X-rays and laboratory tests, but this is rare.
Because there are telltale sounds associated with croup, your child may be diagnosed after a doctor listens to the cough and learns about their medical history. Your child should also receive a physical exam, and may require diagnostic tests to confirm the diagnosis or rule out other conditions.
When you’re asked to share details about your child’s medical history, tell your pediatrician about your child’s symptoms, including details about a barking cough or breathing difficulties. You may be asked if your child has had croup before or if the condition runs in the family.
During a physical exam, if your child has a cough that sounds like a seal’s bark and/or if they make high-pitched whistling sounds when they inhale, they may be diagnosed with croup. Other noteworthy signs include lips that look blue, as well as, on inhalation, nostrils that flare or a chest that that retracts (sinks in).
Sometimes, croup is diagnosed after a physical exam and medical history. Other times, diagnostic tests are recommended, such as a chest and neck X-ray. Doctors may be able to see a swollen or narrowed trachea on an X-ray, but this step isn’t necessary much of the time. An X-ray can also help doctors rule out other causes, such as a swallowed object that is blocking airflow in the trachea.
TREATMENT
Most children with croup can be treated at home. Still, croup can be scary, especially if your child needs a visit to the health care provider's office, emergency room or hospital. Treatment is usually based on how severe the symptoms are.
Comfort measures
It's important to comfort and calm your child because crying and distress can worsen airway swelling, making it harder to breathe. Hold your child, sing lullabies or read quiet stories. Offer a favorite blanket or toy. Speak in a soothing voice.
Also, make sure that your child drinks plenty of fluids to stay hydrated.
Medicines
Your child's health care provider may prescribe these medicines:
Corticosteroid. A corticosteroid such as dexamethasone may be given to reduce swelling in the airway. Symptoms will usually start to improve within a few hours. Your child may take pills over several days. Or your child may get a single dose of dexamethasone as a shot because of its long-lasting effects.
Epinephrine. Epinephrine is effective in reducing airway swelling with more-severe symptoms. The medicine may be given in an inhaled form using a nebulizer. It's fast acting, but its effects wear off quickly. Your child likely will need to be observed in the emergency room for several hours to see if a second dose is needed before going home.
A stay in the hospital
For severe croup, your child may need to spend time in a hospital to be monitored and receive more treatments.
How is croup treated?
Croup treatment depends on the severity of your child’s condition and the risk of it rapidly worsening. If your child has a history of respiratory problems or was born prematurely, that may also affect the treatment approach.
Mild croup
You can usually treat mild croup at home. Home treatment includes using a cool mist humidifier to help soothe dry and irritated airways. You can also sit with your child in a bathroom filled with steam generated from hot water running in the shower. (Don’t sit in the shower or let your child near the hot water.) If your child’s condition doesn’t improve with mist treatment, you should contact their healthcare provider.
Other croup home remedies include:
Letting your child breathe cool air at night by opening a door or window.
Treating your child’s fever with an over-the-counter (OTC) medication such as acetaminophen (Tylenol®) or ibuprofen (Advil®).
Treating your child’s cough with warm, clear fluids to help loosen the mucus on their vocal cords.
Avoiding smoking in your home, as smoke can worsen your child’s cough.
Keeping your child’s head elevated with an extra pillow. (Don’t use pillows with infants younger than 12 months old.)
You may wish to sleep in the same room as your child so you’re there if they start to have trouble breathing.
Moderate to severe croup
For moderate to severe croup, you should take your child to the nearest urgent care center or emergency room (ER). Severe croup can be life-threatening, and you shouldn’t delay taking your child in. Treatment for moderate to severe croup will vary based on your child’s symptoms. Croup treatments may include:
Humidified air or oxygen.
IV fluids for dehydration.
Specific croup medication
If you take your child to their provider’s office or the emergency room, their provider will give them a glucocorticoid and a nebulized breathing treatment (epinephrine).
Glucocorticoids Monitoring of vital signs, including oxygen levels, breathing and heart rate.
Croup medication, including steroids (glucocorticoids) and nebulized breathing treatments (epinephrine).
Placement of a breathing tube (mechanical ventilation). This is rare.
Glucocorticoids are a type of steroid that decreases the swelling of your child’s voice box (larynx), typically within six hours of the first dose. For a child with mild croup, glucocorticoids may reduce the need for a repeat visit to their provider’s office or the emergency room.
The glucocorticoids healthcare providers use most often are dexamethasone and prednisolone. Your child will usually only need one dose taken by mouth (orally). If your child is vomiting or can’t keep the medicine down, their provider can also give dexamethasone intravenously (IV) or through an intramuscular (IM) injection.
Nebulized breathing treatment (epinephrine)
Your child will receive epinephrine as an inhaled mist (nebulizer). This also reduces the swelling in your child’s airways and usually starts working within 10 minutes. Epinephrine works for two hours or less, and your child may receive this treatment every 15 to 20 minutes for severe symptoms.
How is croup treated?
Treatments for croup vary, depending on whether it’s mild, moderate, or severe.
For mild croup, you may care for a child at home. Make sure your child drinks plenty of fluids to stay hydrated. Keeping your child calm is also important because crying can make it more difficult for them to breathe easily.
When a child has mild croup, some parents put a humidifier in their bedroom or run a hot shower so the child can sit in the bathroom to breathe in the steamy mist. Other parents take their child outside at night to breathe in cool air. While these treatments shouldn’t be harmful, they also aren’t proven by research to be helpful.
It’s important to note that over-the-counter cough and cold medications should not be given to a child with croup.
Moderate and severe croup requires medical attention. Doctors may prescribe:
Dexamethasone, a corticosteroid. This treatment can resolve a child’s symptoms more quickly and shorten the duration of illness. In certain cases, other corticosteroids may be used.
Epinephrine, a medication that is often given for life-threatening allergic reactions. When a child has severe croup and breathing is extremely difficult, epinephrine helps to open the airways. It’s typically nebulized (turned into a mist), so that a child may inhale the medication.
Dexamethasone plus epinephrine. For severe cases, doctors may prescribe both drugs.
Oxygen, to help patients breathe more easily.
Neuraminidase inhibitors, such as Tamiflu, but only when croup is caused by flu.
Antibiotics, but only in rare cases when croup is caused by bacteria, not a virus. Antibiotics may also be used if a child has a bacterial infection in addition to having viral croup.
Intubation, in very rare cases, when a child is unable to breathe unassisted.
PREVENTION
How can the spread of croup be prevented?
Croup can spread by physical contact or through the air. To help prevent its spread:
Wash and dry your hands thoroughly after caring for your child.
Wash toys between each use.
Encourage your child to cover their mouth and nose when coughing and sneezing.
Keep your child home from school or daycare when they’re ill or if outbreaks occur.
Throw used tissues away.
To prevent croup, take the same steps you use to prevent colds and flu.
Frequent hand-washing with soap and water for at least 20 seconds is the most important step.
Keep your child away from anyone who's sick.
Encourage your child to cough or sneeze into the elbow.
Clean frequently touched surfaces.
To prevent more-serious infections that may cause croup, keep your child's vaccinations up to date. The diphtheria and Haemophilus influenzae type b (Hib) vaccines offer protection from some of the rarest but most dangerous upper airway infections. There isn't a vaccine yet that protects against parainfluenza viruses.
PROGNOSIS
Patients with mild croup have an excellent prognosis. Hospital admission and mortality rates are low and vary significantly between communities. Less than 0.5% of intubated children died in a 10-year study of hospitalized children with croup.
When should I worry about croup?
Croup can be mild, moderate or severe, depending on how difficult it is for your child to pull air into their lungs. The size (diameter) of their windpipe and the amount of narrowing due to the swelling determine the severity of your child’s condition. In addition, your child’s condition may become more severe if they become upset.
Mild croup
A child with mild croup may have a barking cough and stridor. Symptoms can worsen throughout your child’s illness, especially during the evening hours. So it’s important to keep an eye on their breathing, but you can usually treat their condition at home.
Moderate croup
A child with moderate croup may have stridor along with retractions (sucking in the skin around their ribs and the top of their breastbone). They may also be slightly agitated or disoriented and may have moderate trouble breathing. You should take your child to see a healthcare provider for treatment.
Severe croup
A child with severe croup has stridor and retractions. They may also be agitated, anxious or fatigued. Cyanosis (blue-tinged skin) is common. Severe croup is a life-threatening condition. Take your child to the emergency room immediately.
How long does croup last?
Symptoms of croup usually clear up in most children within two days. However, symptoms can persist for up to one week.
When can my child go back to school?
Croup is very contagious. Your child should stay home from school until after their fever is gone.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis of croup includes other causes of stridor and respiratory distress, such as bacterial tracheitis, epiglottitis, foreign body aspiration, hemangioma, peritonsillar abscess, neoplasm, retropharyngeal abscess, and smoke inhalation. Rarely, neurological causes can cause stridor that mimics croup. In a report, 3 children presented with stridor and were treated for croup but were eventually found to have a neurological cause of stridor. 
Acute Epiglottitis
Acute epiglottitis is rare due to vaccination against Haemophilus influenzae type B. The patient does not have a barking cough but has anxiety out of proportion to the degree of respiratory distress. Symptom onset is generally rapid; the child appears febrile and ill-appearing. Affected children prefer to sit upright in a tripod position, and cough is rare. The thumb sign due to a swollen epiglottis may be noted on a radiograph. A cough is highly sensitive and specific for croup, whereas drooling may indicate bacterial epiglottitis. Other symptoms include acute onset of dysphagia, odynophagia, high fever, and a muffled voice. 
Distinguishing croup from epiglottitis is essential because patients with the latter may deteriorate rapidly. If acute epiglottitis is suspected, it is imperative to keep the child calm and avoid asking them to open their mouth wide, as this could precipitate fatal airway obstruction. An emergent ear, nose, and throat (ENT) consultation for airway evaluation in the operating room is essential for suspected epiglottitis. The Haemophilus influenzae vaccine, licensed in 1985, decreased cases of epiglottitis, but with the current rates of vaccine refusals, acute epiglottitis cases are likely to occur. All patients with epiglottitis should be admitted to the intensive care unit for close monitoring. 
Bacterial Tracheitis
Bacterial tracheitis is also called bacterial croup. An exudative bacterial infection invades tracheal soft tissue and occurs as a primary infection or secondary to viral croup. In secondary bacterial infection, mild viral croup symptoms worsen, and patients exhibit high fevers, a toxic appearance, and severe respiratory distress. Radiographs may show nonspecific edema and irregularities of the tracheal wall. 
Deep Neck Space Abscesses
Patients with peritonsillar, parapharyngeal, or retropharyngeal abscesses typically lack symptoms of croup, such as barking cough and stridor. Children may present with drooling, fever, difficulty swallowing, neck stiffness, enlargement of cervical lymph nodes, and a toxic appearance. With deep neck space abscesses, cellulitis of the cervical prevertebral tissues may occur. Children with a retropharyngeal abscess typically have fever, drooling, dysphagia, and odynophagia but also complain of neck pain with a bulging posterior pharyngeal wall on neck radiography. Children with a peritonsillar abscess often complain of a sore throat, fever, and a classic hot potato voice. 
Foreign Body
A previously healthy child presents with a sudden onset of choking and upper airway obstruction symptoms. A foreign body lodged in the larynx causes hoarseness and stridor. A large foreign body in the upper esophagus can pressurize the extra-thoracic trachea, resulting in a barking cough and inspiratory stridor. Late onset of stridor can occur due to the ingestion of a nonobstructive erosive foreign body, such as a button battery. 
Allergic Reaction or Acute Angioneurotic Edema
Patients present with a sudden onset of symptoms such as swollen lips and tongue without preceding respiratory symptoms or fever. The associated symptoms include urticarial rash, difficulty swallowing, and inspiratory stridor. Patients may have a history of allergies and a history of similar episodes in the past. 
Congenital and Acquired Anomalies
Congenital and acquired anomalies and upper airway injury can cause stridor. Anatomic abnormalities include laryngeal webs, papillomas, laryngomalacia, subglottic stenosis, hemangiomas, bronchogenic cysts, and vocal cord paralysis. Most patients present with stridor as a chronic symptom without other upper respiratory symptoms or fever.
POSSIBLE COMPLICATIONS
Most cases of croup are mild. In a small number of children, the airway swells enough to cause problems with breathing. Rarely, a bacterial infection of the windpipe can occur in addition to the viral infection. This can result in trouble breathing and requires emergency medical care.
Only a small number of children seen in the emergency room for croup require a stay in the hospital.
Symptoms of mild croup subside in the majority of children within 2 days. However, a few patients have symptoms that persist for up to 1 week. Moderate-to-severe croup symptoms need treatment with epinephrine in addition to glucocorticoids. Complications such as pneumonia, pulmonary edema, and secondary bacterial infection may occur, leading to tracheitis, requiring antibiotics.
WHEN TO SEE A DOCTOR
Contact your child's health care provider if symptoms are severe, worsen, last longer than 3 to 5 days or aren't responding to home treatment.
Seek immediate medical attention if your child:
Makes noisy, high-pitched breathing sounds when breathing both in and out.
Makes high-pitched breathing sounds when not crying or upset.
Begins drooling or has problems swallowing.
Seems anxious, upset and restless or extra tired and has no energy.
Breathes at a faster rate than usual.
Struggles to breathe.
Develops a blue or gray tint around the nose, in or around the mouth, or on the fingernails.
When should I take my child to see their healthcare provider?
You should call your child’s healthcare provider if:
Your child has a fever that lasts for more than three days.
Your child has symptoms of mild croup that last for more than one week.
You have questions or are concerned about your child’s condition.
When should I go to the ER?
If your child develops symptoms of severe or worsening croup, seek immediate medical attention. These symptoms include:
Difficulty breathing.
Blue-tinged skin (cyanosis).
Severe coughing spells.
Drooling or difficulty swallowing.
Inability to cry or speak due to trouble taking a breath.
A noisy, high-pitched whistling sound while breathing.
Sucking in the skin around your child’s ribs and the top of their breastbone (retractions).
Red-Flag Warnings (When to Seek Immediate Medical Attention)
Stridor at rest (not just with crying or activity)
Rapid or labored breathing
Bluish or grayish discoloration around the lips or fingernails (cyanosis)
Inability to speak or make sounds
Drooling or difficulty swallowing (could suggest epiglottitis or another emergency)
Severe agitation or fatigue (may indicate low oxygen levels or impending respiratory failure)
Persistent high fever or signs of worsening infection
These signs suggest moderate to severe croup, and the child may need urgent medical evaluation or hospitalization.
General Advice for Parents and Caregivers
Stay calm and keep the child calm: Crying worsens airway narrowing.
Cool or moist air can help reduce airway inflammation:
Take the child outside in cool night air for a few minutes.
Use a humidifier in the child's room.
Hydration is important—encourage fluids.
Antipyretics like acetaminophen or ibuprofen can help with fever and comfort.
Do not use cough medicines, which are not recommended for young children.
Monitor breathing closely, especially at night.
Medical Treatment (as advised by healthcare providers)
Mild cases: Often managed at home with supportive care.
Moderate to severe cases: May require:
Dexamethasone (a steroid given orally or by injection to reduce airway inflammation)
Nebulized epinephrine in emergency settings for quick relief of airway swelling
Oxygen therapy or hospitalization in severe cases
PEDIATRIC CYSTIC FIBROSIS
OVERVIEW
Cystic fibrosis (CF) is a genetic disease that causes sticky, thick mucus to build up in your organs, blocking and damaging them. Many people think of CF as a lung disease because it affects your lungs and airways, which can make it hard to breathe and cause frequent infections. But it’s called cystic fibrosis because it also causes cysts and scarring (fibrosis) in your pancreas. This damage, plus the thick mucus, can block ducts that release digestive enzymes, making it hard to get nutrients from your digestive tract. CF can also affect your liver, sinuses, intestines and sex organs.
The mucus that lines your organs and body cavities, such as your lungs and nose, is thin and watery. In people with CF, a change in a gene (genetic mutation) leads to low levels of certain proteins, or proteins that don’t work properly. Because of these faulty proteins, minerals that move water into your mucus (which thins it out) get trapped inside cells, leaving the mucus thick and sticky.
People with cystic fibrosis are born with it. It’s a lifelong illness that gets more severe over time. Most people with CF don’t live as long as people without it.
Types of cystic fibrosis
There are two types of cystic fibrosis:
Classic cystic fibrosis often affects multiple organs. It’s usually diagnosed in the first few years of your life. 
Atypical cystic fibrosis is a milder form of the disease. It may only affect one organ or symptoms may come and go. It’s usually diagnosed in older children or adults. 
CAUSES
In cystic fibrosis, a change in a gene causes problems with the protein that controls the movement of salt and water in and out of cells. This gene is the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It affects the cells that make mucus, sweat and digestive juices. When the CFTR protein doesn't work as it should, the result is thick, sticky mucus in the respiratory, digestive and reproductive systems, as well as extra salt in sweat.
Changes in the CFTR gene that cause CF are divided into several different groups based on the problems they cause. Different groups of gene changes affect how much CFTR protein is made and how well it works.
To have cystic fibrosis, children must get one copy of the changed CFTR gene from each parent. If children get only one copy, they won't develop CF. But they will be carriers and could pass the changed gene to their own children. People who are carriers may have no symptoms of CF or a few mild symptoms.
What causes cystic fibrosis?
Changes to the CFTR gene — called variants or mutations — cause cystic fibrosis. CFTR makes a protein that works as an ion channel on the surface of a cell. Ion channels are like gates in a cell’s membrane that allow certain molecules to pass through. 
CFTR usually makes a gate for chloride ions, a type of mineral with a negative electrical charge. Chloride moves out of the cell, taking water with it, which thins out mucus and makes it more slippery. In people with CF, gene mutations in CFTR prevent this from happening, so the mucus stays sticky and thick. 
There are different categories (classes I to VI) of gene mutation in CFTR that depend on the effect they have. Some produce no proteins at all, some produce only small amounts of proteins, and some produce proteins that don’t work properly.
Are you born with cystic fibrosis?
Yes, cystic fibrosis is a genetic condition that you’re born with. People who have CF inherit two mutated CFTR genes, one from each biological parent (it’s inherited in an autosomal recessive manner). 
Your parents don’t have to have cystic fibrosis for you to have CF. In fact, many families don’t have a family history of CF. Someone with just one copy of the gene variant is called a carrier. About 1 in 31 people in the U.S. are carriers who have no CF symptoms.
Can adults get cystic fibrosis?
You’re born with the mutation in the gene that causes cystic fibrosis. But with mild symptoms, or symptoms that come and go, some people may go undiagnosed until later in life, even as adults.
SYMPTOMS OF PEDIATRIC CYSTIC FIBROSIS
In the U.S., because of newborn screening, cystic fibrosis can be diagnosed within the first month of life, before symptoms develop. But people born before newborn screening became available may not be diagnosed until the symptoms of CF show up.
CF symptoms vary, depending on which organs are affected and how severe the condition is. Even in the same person, symptoms may worsen or get better at different times. Some people may not have symptoms until their teenage years or adulthood.
People who are not diagnosed until adulthood usually have milder symptoms and are more likely to have symptoms that aren't typical. These may include repeated bouts of an inflamed pancreas called pancreatitis, infertility and repeated bouts of pneumonia.
People with CF have a higher than usual level of salt in their sweat. Parents often can taste the salt when they kiss their children. Most of the other symptoms of CF affect the respiratory system and digestive system.
Respiratory symptoms
In cystic fibrosis, the lungs are most commonly affected. The thick and sticky mucus that happens with CF clogs the tubes that carry air in and out of the lungs. This can cause symptoms such as:
A cough that won't go away and brings up thick mucus.
A squeaking sound when breathing called wheezing.
Limited ability to do physical activity before tiring.
Repeated lung infections.
Irritated and swollen nasal passages or a stuffy nose.
Repeated sinus infections.
Digestive symptoms
The thick mucus caused by cystic fibrosis can block tubes that carry digestive enzymes from the pancreas to the small intestine. Without these digestive enzymes, the intestines can't completely take in and use the nutrients in food. The result is often:
Foul-smelling, greasy stools.
Poor weight gain and growth.
Blocked intestines, which is more likely to happen in newborns.
Ongoing or severe constipation. Straining often while trying to pass stool can cause part of the rectum to stick out of the anus. This is called a rectal prolapse.
Cystic fibrosis symptoms include:
Frequent lung infections (recurrent pneumonia or bronchitis).
Loose or oily poop (stool).
Trouble breathing.
Frequent wheezing.
Frequent sinus infections.
A nagging cough.
Slow growth.
Failure to thrive (inability to gain weight despite having a good appetite and taking in enough calories).
Atypical cystic fibrosis symptoms
People with atypical cystic fibrosis may have some of the same symptoms as those with classic CF. Over time, you also might experience:
Chronic sinusitis.
Nasal polyps.
Dehydration or heatstroke from abnormal electrolyte levels.
Diarrhea.
Pancreatitis.
Unintended weight loss.
Symptoms can vary based on age and severity but commonly include:
Respiratory Symptoms:
Chronic wet cough
Recurrent lung infections (e.g., pneumonia, bronchitis)
Wheezing or shortness of breath
Nasal polyps or chronic sinusitis
Clubbing of fingers and toes (in long-standing cases)
Gastrointestinal Symptoms:
Poor weight gain/growth despite normal appetite
Frequent greasy, bulky, foul-smelling stools (steatorrhea)
Abdominal pain or bloating
Constipation or intestinal blockage (e.g., meconium ileus in newborns)
Other Signs:
Salty-tasting skin
Frequent dehydration
Delayed puberty or growth delay
Male infertility later in life (due to congenital absence of the vas deferens)
DIAGNOSIS METHODS
To diagnose cystic fibrosis, healthcare professionals typically do a physical exam, review your symptoms and do tests.
Newborn screening and diagnosis
Every state in the U.S. now routinely screens newborns for cystic fibrosis. Early diagnosis means that treatment can begin right away. Testing can include:
Newborn screening. In this screening test, a healthcare professional takes a few drops of blood from the baby's heel. A lab checks the blood sample for higher levels than expected of a chemical called immunoreactive trypsinogen (IRT). IRT is released by the pancreas and may suggest CF. A newborn's IRT levels also may be high because of premature birth or a stressful delivery. For that reason, other tests may be needed to confirm a diagnosis of cystic fibrosis.
Sweat test. To check if a baby has CF, a sweat test is done once the baby is at least 2 weeks old. A chemical that causes the skin to sweat is put on a small area of skin. Then the sweat is collected to test it and see if it's saltier than typical. Testing done at a care center accredited by the Cystic Fibrosis Foundation helps ensure results that can be trusted.
Genetic testing. Healthcare professionals also may recommend genetic testing to look for specific changes on the gene responsible for CF. Genetic testing may be used along with IRT levels to confirm the diagnosis.
Testing of older children and adults
Cystic fibrosis tests may be recommended for older children and adults who weren't screened at birth. Your healthcare professional may suggest genetic and sweat tests for CF if you have repeated bouts of an inflamed pancreas, nasal polyps, chronic sinus infections, lung infections, bronchiectasis or male infertility.
How is cystic fibrosis diagnosed?
Healthcare providers often test for cystic fibrosis during a newborn screening. Providers perform this test with a few drops of blood from your baby’s heel. A lab looks in the blood sample for immunoreactive trypsinogen (IRT), a chemical made in your pancreas. People with CF have higher levels of IRT in their blood. Babies are often tested for IRT shortly after birth and a few weeks later.
Some conditions — like preterm delivery — can raise IRT levels. So, a positive IRT test alone doesn’t mean your baby has CF. If your baby has higher levels of IRT than expected, your healthcare provider will order additional tests to make a final diagnosis.
In about 5% of cases, the newborn screen doesn’t detect elevated IRT levels in someone with CF. Or you may have been born before routine CF screening was available. If you or your child has symptoms of CF, a provider will perform a sweat test and follow up with additional tests as needed.
Tests for cystic fibrosis
Sweat test. The sweat test measures the amount of chloride in your body’s sweat. Chloride levels in sweat are higher in people who have CF. This is the most conclusive test for CF, but it may be normal in people with atypical CF.
Genetic tests. A provider tests blood samples for changes in the genes that cause CF.
Imaging. Providers use imaging, like sinus and chest X-rays, to support or confirm a CF diagnosis. Imaging alone can’t diagnose CF.
Pulmonary function tests. These tests measure how well your lungs are working.
Sputum culture. Your healthcare provider takes a sample of your sputum (mucus coughed up from your lungs) and tests it for bacteria. Certain bacteria, such as Pseudomonas, are most commonly found in people who have CF.
Pancreatic biopsy. This can tell your provider if you have cysts or damage to your pancreas.
Nasal potential difference (NPD). This test measures the small amount of electrical charge that’s usually present in the lining of your nose. The movement of ions creates this charge. People with CF don’t have as much ion movement because of the way CF affects their ion channels. 
Intestinal current measurement (ICM). A provider takes a sample of rectal tissue to perform this test. A lab uses the sample to measure how much chloride it secretes.
More diagnosis methods
Newborn screening
May also be suggested by a positive prenatal screening test result, family history, or symptomatic presentation
Confirmed by a sweat test showing elevated sweat chloride on 2 occasions
Identifying 2 CF-causing variants (1 on each chromosome) is consistent with the diagnosis
May rarely be confirmed, in atypical cases, by demonstrating abnormal ion transport across the nasal epithelium or abnormal intestinal current measurements
Most cases of CF are first identified by newborn screening, but up to 10% are not diagnosed until adolescence or early adulthood. Despite advances in genetic testing, the sweat chloride test remains the standard for confirming a CF diagnosis in most cases because of its sensitivity and specificity, simplicity, and availability.
Newborn screening
Universal newborn screening for CF is now standard in the United States. Screening is based on detecting an elevated concentration of immunoreactive trypsinogen (IRT) in the blood.
There are 2 methods of following up on an elevated IRT level. In one method, a second IRT is done, which, if also elevated, is followed by a sweat test. In the other, more commonly used method, an elevated IRT level is followed by CFTR mutation testing, and, if 1 or 2 variants are identified, then a sweat test is done. For diagnosis, both methods have 90 to 95% sensitivity.
Sweat testing
In this test, localized sweating is stimulated with pilocarpine, the amount of sweat is measured, and the chloride concentration is determined. Although the sweat chloride concentration increases slightly with age, the sweat test is valid at all ages:
Normal: ≤ 30 mEq/L (≤ 30 mmol/L) (CF is unlikely.)
Intermediate: 30 to 59 mEq/L (30 to 59 mmol/L) (CF is possible.)
Abnormal: ≥ 60 mEq/L (≥ 60 mmol/L) (This result is consistent with CF.)
The results are valid after 48 hours of life, but an adequate sweat sample (> 75 mg on filter paper or > 15 mcL in microbore tubing) may be difficult to obtain before 2 weeks of age. False-negative results are rare but may occur in the presence of edema and hypoproteinemia or an inadequate quantity of sweat. False-positive results are usually due to technical error. Transient elevation of sweat chloride concentration can result from psychosocial deprivation (eg, child abuse, neglect) and can occur in patients with anorexia nervosa. A positive sweat test result should be confirmed by a second sweat test or by identification of 2 CF-causing variants.
Intermediate sweat test results
A small subset of patients have a mild or partial CF phenotype and sweat chloride values that are persistently in the intermediate or even normal range. In addition, there are patients who have single-organ manifestations such as chronic or recurrent pancreatitis, isolated bronchiectasis, or congenital bilateral absence of the vas deferens along with findings suggestive of abnormal CFTR function. They do not meet criteria for a CF diagnosis and are classified as having a CFTR-related disorder. In some of these patients, the diagnosis of CF can be confirmed by the identification of 2 CF-causing variants, 1 on each chromosome. If 2 CF-causing variants are not identified, ancillary evaluations such as pancreatic function testing and pancreatic imaging, high-resolution chest CT, sinus CT, pulmonary function testing, urogenital evaluation in males, and bronchoalveolar lavage including assessment of microbial flora may be useful.
Additional potentially helpful diagnostic tests include expanded CFTR genetic analysis and measurement of nasal transepithelial potential difference (based on the observation of increased sodium reabsorption across epithelium that is relatively impermeable to chloride in patients with CF) and measurement of intestinal currents.
CFTR-related metabolic syndrome and CF screen positive, inconclusive diagnosis
Infants who have a positive newborn screening result and evidence of possible CFTR dysfunction but do not meet the diagnostic criteria for CF are classified as having CFTR-related metabolic syndrome (CRMS), also called CF screen positive, inconclusive diagnosis (CFSPID). CRMS/CFSPID is diagnosed in infants who have a positive newborn screen, are asymptomatic, and have either of the following:
Sweat chloride concentrations in the intermediate range and 0 or 1 CF-causing variant
Sweat chloride concentrations in the normal range and 2 CFTR variants, at least 1 of which has unclear phenotypic consequences
Most children with CRMS/CFSPID remain healthy, but over time around 10% will develop symptoms and meet criteria for a diagnosis of CF or a CF-related disorder. Patients with CRMS/CFSPID should be evaluated and monitored regularly in a CF care center.
Pancreatic tests
Pancreatic function should be assessed at the time of diagnosis, usually by measuring the concentration of human pancreatic elastase in stool. Human pancreatic elastase measurement is valid even in the presence of exogenous pancreatic enzymes. Infants who are initially pancreatic sufficient and who carry 2 "severe" variants should have serial measurements to detect progression to pancreatic insufficiency.
Respiratory assessment
Chest imaging is done at times of pulmonary deterioration or exacerbations and routinely every 1 to 2 years. High-resolution chest CT may be helpful to more precisely define the extent of lung damage and to detect subtle airway abnormalities. Chest x-rays and CT may show hyperinflation, mucoid impaction, and bronchial wall thickening as the earliest findings. Subsequent changes include areas of infiltrate, atelectasis, and hilar adenopathy. With advanced disease, segmental or lobar atelectasis, cyst formation, bronchiectasis, and pulmonary artery and right ventricular hypertrophy occur. Branching, fingerlike opacifications that represent mucoid impaction of dilated bronchi are characteristic.
Pulmonary function tests are the best indicators of clinical status and response to therapy. In patients over 5 years of age, spirometry should be done routinely and at times of clinical decline. In infants, respiratory status can be monitored by using a raised-volume rapid thoracoabdominal compression technique, which generates a partial flow-volume curve. In children 3 to 6 years of age, the multiple breath washout procedure can be used to generate a lung clearance index as a measure of ventilation inhomogeneity.
Pulmonary function tests done by spirometry indicate
A reduction in forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), forced expiratory flow between 25% and 75% expired volume (FEF25-75), and FEV1/FVC ratio
An increase in residual volume and the ratio of residual volume to total lung capacity
Fifty percent of patients have evidence of reversible airway obstruction as shown by improvement in pulmonary function after administration of an inhaled bronchodilator.
Screening oropharyngeal or sputum cultures should be done at least 4 times/year, especially in patients not yet colonized with P. aeruginosa. Bronchoscopy/bronchoalveolar lavage is indicated when it is important to precisely define the patient’s lower airway microbial flora (eg, to direct antibiotic selection) or to remove inspissated mucus plugs.
Carrier screening
CF carrier screening is available in the United States and is recommended for couples who are planning a pregnancy or seeking prenatal care. If both potential parents carry a CFTR variant, prenatal screening of the fetus can be done by chorionic villus sampling or amniocentesis. Prenatal counseling in such cases is complicated by the wide phenotypic variability of CF and incomplete information on the clinical consequences of many of the CFTR variants that are identified through screening
TREATMENT
There is no cure for cystic fibrosis, but treatment can ease symptoms, lessen complications and improve quality of life. Close monitoring and early, aggressive intervention is recommended to slow the worsening of CF over time. This can lead to a longer life.
Managing CF is complicated, so it's best to get treatment at a center with a multispecialty team of doctors and other healthcare professionals trained in CF. They can evaluate and treat your condition.
The goals of treatment include:
Preventing and controlling infections that occur in the lungs.
Removing and loosening mucus from the lungs.
Treating and preventing intestinal blockage.
Getting enough nutrition.
Medicines
Options include:
Medicines that target gene changes and improve how the CFTR protein works. These are called cystic fibrosis transmembrane conductance regulator (CTFR) modulators.
Antibiotics to treat and prevent lung infections.
Anti-inflammatory medicines to lessen swelling in the airways in the lungs.
Mucus-thinning medicines, such as hypertonic saline, to help cough up mucus. This can improve lung function.
Medicines breathed into the lungs called bronchodilators. These can help keep airways open by relaxing the muscles around the bronchial tubes.
Pancreatic enzyme capsules taken by mouth to help the digestive tract take in and use nutrients.
Stool softeners to prevent constipation or bowel obstruction.
Acid-reducing medicines to help pancreatic enzymes work better.
Specific medicines for diabetes or liver disease, when needed.
Medicines that target genes
For those with cystic fibrosis who have certain gene changes, cystic fibrosis transmembrane conductance regulator (CFTR) modulators may help. About 90% of people with CF may be helped by using these medicines. Gene testing is needed to find out which specific gene change you have and if a CFTR modulator may work for you.
CFTR modulators are newer medicines that many experts think are a breakthrough in the treatment of CF. The medicines help the CFTR protein work better. This can make lung function better, help digestion and weight, and lessen the amount of salt in sweat.
The U.S. Food and Drug Administration (FDA) has approved these CFTR modulators for treating CF in people with specific changes in the CFTR gene:
The newest combination medicine with elexacaftor, ivacaftor and tezacaftor (Trikafta) is approved for people age 2 years and older. Trikafta has been shown to be the most effective CFTR modulator.
The combination medicine with ivacaftor and tezacaftor (Symdeko) is approved for people age 6 years and older.
The combination medicine with ivacaftor and lumacaftor (Orkambi) is approved for people who are age 1 year and older.
Ivacaftor (Kalydeco) is approved for people who are 1 month and older.
Your healthcare professional may do liver function tests and eye exams before prescribing these medicines. While taking these medicines, you'll likely need testing on a regular basis to check for side effects such as liver function changes and clouding of the eye lenses called cataracts. Ask your healthcare professional and pharmacist for information on possible side effects and what to watch for.
Keep regular follow-up appointments so your healthcare professional can monitor you while taking these medicines. Tell your healthcare professional about any side effects that you have.
Airway clearance techniques
Airway clearance techniques, also called chest physical therapy, can help get rid of mucus blocking the airways. It also can help to lessen infection and inflammation in the airways. Airway clearance techniques loosen the thick mucus in the lungs, making it easier to cough up.
Airway clearing techniques are usually done several times a day. Different techniques, and often more than one method, can be used to loosen and remove mucus.
Clapping with cupped hands on the front and back of the chest. This is a common technique.
Special breathing and coughing activities.
Mechanical devices, such as a tube that you blow into, and a machine that pulses air into the lungs called a vibrating vest.
Vigorous exercise.
Your healthcare professional can give you instructions on the airway clearance techniques that are best for you and how often you should do them.
Pulmonary rehabilitation
Your healthcare professional may recommend a long-term program called pulmonary rehabilitation. The program may improve your lung function and your overall well-being. Pulmonary rehabilitation is usually done on an outpatient basis and may include:
Physical exercise that may improve your condition.
Breathing techniques that may help loosen mucus and make breathing easier.
Dietary counseling.
Mental health counseling and support.
Education about your condition.
Surgery and other treatments
Options for certain conditions caused by cystic fibrosis include:
Nasal and sinus surgery. Surgery can remove nasal polyps that get in the way of breathing. Sinus surgery may be done to treat repeated or long-term sinusitis.
Oxygen therapy. If there isn't enough oxygen in your blood, you may need supplemental oxygen. You can get this extra oxygen to your lungs through a mask or through plastic tubing with tips that fit into your nose. These attach to an oxygen tank. Lightweight, portable units that you take with you can help you be more mobile. Oxygen therapy may prevent high blood pressure in the lungs, a condition called pulmonary hypertension.
Noninvasive ventilation. Typically used while sleeping, noninvasive ventilation uses a nose or mouth mask to give positive pressure in the airway and lungs when breathing in. It's often used along with oxygen therapy. Noninvasive ventilation can increase air exchange in the lungs and lessen the work of breathing. The treatment also may help with airway clearance.
Feeding tube. CF interferes with digestion, so you can't take in and use nutrients from food very well. A feeding tube delivers extra nutrition. This may be a short-term tube placed through your nose and guided to your stomach. Or the tube may be surgically placed in the stomach through a small cut in the skin on your belly. A feeding tube gives extra calories during the day or night and does not keep you from eating by mouth.
Bowel surgery. If a blockage happens in the intestines, you may need surgery to remove it. If part of an intestine folds inside a nearby section of intestine, you may need surgery.
Lung transplant. If you have severe breathing problems or life-threatening lung complications, or if antibiotics no longer work to treat lung infections, a lung transplant may be an option. Because bacteria line the airways in diseases such as CF that cause permanent widening of the large airways, both lungs need to be replaced.
Cystic fibrosis does not recur in transplanted lungs. But other complications linked with CF, such as sinus infections, diabetes, pancreas conditions and osteoporosis, can still happen after a lung transplant.
Liver transplant. For severe CF-related liver disease, such as cirrhosis, liver transplant may be an option. In some people, a liver transplant may be done together with lung or pancreas transplants.
Other treatment options
Treatment of Cystic Fibrosis
Comprehensive, multidisciplinary support
Antibiotics, inhaled medications to thin airway secretions, and physical maneuvers to clear airway secretions
Inhaled bronchodilators and sometimes corticosteroids for responders
Usually pancreatic enzyme and vitamin supplementation
High-calorie diet (sometimes requiring supplemental enteral tube feedings)
In patients with specific variants, CFTR modulators consisting of a CFTR potentiator or combination of CFTR correctors and a CFTR potentiator
Comprehensive and intensive therapy should be directed by an experienced physician working with a multidisciplinary team that includes other physicians, nurses, dietitians, physical and respiratory therapists, mental health professionals, pharmacists, and social workers. The goals of therapy are maintenance of normal nutritional status, prevention or aggressive treatment of pulmonary and other complications, encouragement of physical activity, and provision of psychosocial support. The treatment regimen is complex and may take up to 2 hours each day. With appropriate support, most patients can make an age-appropriate adjustment at home and school.
Treatment of respiratory manifestations
Treatment of pulmonary manifestations centers on prevention of airway obstruction and prophylaxis against and control of pulmonary infections. Prophylaxis against pulmonary infections includes maintenance of pertussis, Haemophilus influenzae, varicella, Streptococcus pneumoniae, and measles immunity; annual influenza vaccination; and COVID-19 vaccination in accordance with current recommendations from the Advisory Committee on Immunization Practices (ACIP). In patients exposed to influenza, a neuraminidase inhibitor can be used prophylactically or at the first signs of infection. Giving nirsevimab, or when not available, palivizumab, to infants with CF for prevention of respiratory syncytial virus infection has been shown to be safe, but efficacy has not been documented.
Long-term daily inhalation therapy with dornase alfa (recombinant human deoxyribonuclease) or with 7% hypertonic saline is recommended (1, 2) and has been shown to slow the rate of decline in pulmonary function and to decrease the frequency of respiratory tract exacerbations (3).
Airway clearance measures consisting of postural drainage, percussion, vibration, and assisted coughing (chest physiotherapy) are recommended at the time of diagnosis and should be done on a regular basis. In older patients, alternative airway clearance measures, such as active cycle of breathing, autogenic drainage, positive expiratory pressure devices, and vest therapy (high-frequency chest wall oscillation), may be effective. Regular aerobic exercise is recommended; it may also help airway clearance. For patients with obstructive sleep apnea, continuous positive airway pressure may be beneficial.
For patients with reversible airway obstruction, bronchodilators may be given by inhalation. Corticosteroids by inhalation usually are not effective. Oxygen therapy is indicated for patients with severe pulmonary insufficiency and hypoxemia.
Mechanical ventilation or extracorporeal membrane oxygenation (ECMO) is typically not indicated for chronic respiratory failure. Their use is typically restricted to patients with good baseline status in whom acute reversible respiratory complications develop, in association with pulmonary surgery, or to patients in whom lung transplantation is imminent. Noninvasive positive pressure ventilation nasally or by face mask also can be beneficial.
Oral expectorants are sometimes used, but few data support their efficacy. Cough suppressants should be discouraged.
Pneumothorax can be treated with closed chest tube thoracostomy drainage. Open thoracotomy or thoracoscopy with resection of pleural blebs and mechanical abrasion of the pleural surfaces is effective in treating recurrent pneumothoraces.
Mild to moderate hemoptysis is treated with antibiotics (oral/aerosol or IV depending on severity of hemoptysis and severity of infection) and airway clearance. Massive or recurrent hemoptysis is treated by bronchial artery embolization or rarely by focal lung resection.
Oral corticosteroids are indicated in infants with prolonged bronchiolitis and in patients with refractory bronchospasm, allergic bronchopulmonary aspergillosis (ABPA), and inflammatory complications (eg, arthritis, vasculitis). Long-term use of alternate-day corticosteroid therapy can slow the decline in pulmonary function, but because of corticosteroid-related complications, it is not recommended for routine use. Patients receiving corticosteroids must be closely monitored for evidence of diabetes and linear growth retardation.
Allergic bronchopulmonary aspergillosis is also treated with systemic corticosteroids and an oral antifungal medication.
Ibuprofen, when given over several years at a dose sufficient to achieve a peak plasma concentration between 50 and 100 mcg/mL (242.4 and 484.8 micromol/L), has been shown to slow the rate of decline in pulmonary function, especially in children 5 to 13 years of age. The appropriate dose must be individualized based on pharmacokinetic studies.
Chronic rhinosinusitis is very common. Treatment options include nasal saline irrigation, low-pressure isotonic nasal irrigation, intranasal dornase alfa nebulization, and sinonasal topical antibiotics. Sinus surgery may be helpful in cases refractory to medical management. An intranasal corticosteroid spray is recommended to treat allergic rhinitis.
CFTR modulators
CFTR corrector and potentiator medications are indicated for about 90% of the variants carried by patients with CF. CFTR modulators are not available for patients with class I frameshift and nonsense mutations.
Ivacaftor is a small-molecule oral medication given chronically that potentiates the CFTR ion channel in patients with specific CFTR variants. It may be used in patients 1 month of age and older who carry at least 1 copy of a specific variant potentiated by ivacaftor.
Lumacaftor, tezacaftor, and elexacaftor are small-molecule oral medications that partially correct the defective CFTR protein by altering protein misfolding in patients who carry the F508del variant or other specified variants.
The combination of lumacaftor and ivacaftor can be given to patients 1 year of age and older who carry 2 copies of the F508del variant.
The combination of tezacaftor and ivacaftor can be given to patients 6 years of age and older who carry 2 copies of the F508del variant or other specified variants.
The triple combination of elexacaftor, tezacaftor, and ivacaftor can be given to patients 2 years of age and older who carry at least 1 copy of the F508del variant or 1 copy of certain rare variants (4, 5).
These medications can improve pulmonary function, increase weight, improve exocrine pancreatic function, decrease the frequency of pulmonary exacerbations and hospitalizations, improve quality of life, and reduce and sometimes normalize sweat chloride concentrations (6). The indications for ivacaftor, lumacaftor/ivacaftor, tezacaftor/ivacaftor, and elexacaftor/tezacaftor/ivacaftor are based on the patient's CFTR variants and age and are changing rapidly. Although all of these medications can be helpful, only ivacaftor and the combination of elexacaftor, tezacaftor, and ivacaftor are considered to be highly effective modulator therapy.
Treatment and prevention of infections
For mild pulmonary exacerbations, a short course of oral antibiotics should be given based on culture and sensitivity testing. The medications of choice for methicillin-sensitive staphylococcus are a penicillinase-resistant penicillin (eg, dicloxacillin), a cephalosporin (eg, cephalexin), or trimethoprim/sulfamethoxazole. Erythromycin, amoxicillin/clavulanate, a tetracycline, or linezolid may be used. For patients colonized with methicillin-resistant S. aureus (MRSA), a course of oral trimethoprim/sulfamethoxazole, clindamycin, linezolid, or a tetracycline may be effective. For patients colonized with P. aeruginosa, a short course of inhaled tobramycin or aztreonam (eg, 4 weeks) and/or an oral fluoroquinolone (eg, 2 to 3 weeks) may be effective. Fluoroquinolones have been used safely in young children.
For moderate-to-severe pulmonary exacerbations, especially in patients colonized with P. aeruginosa, IV antibiotic therapy is advised. Patients often require hospital admission, but carefully selected patients can safely receive some of the therapy at home. Combinations of the aminoglycoside tobramycin (or sometimes amikacin) plus a cephalosporin, extended-spectrum penicillin, fluoroquinolone, or monobactam with antipseudomonal activity are given IV, usually for 2 weeks. Higher doses may be required to achieve acceptable serum concentrations. Because of enhanced renal clearance in patients with CF, large doses of some penicillins may be required to achieve adequate serum levels. For patients colonized with MRSA, vancomycin or linezolid can be added to the IV regimen.
Eradication of chronic P. aeruginosa colonization is difficult. It has been shown, however, that early antibiotic treatment around the time the airways are initially infected with P. aeruginosa may be effective in eradicating the organism for some period of time. In patients who are chronically colonized with P. aeruginosa, antibiotics delivered by inhalation improve clinical parameters and possibly reduce the bacterial burden in the airways (7). The long-term use of alternate-month inhaled tobramycin or aztreonam therapy along with continuous (every month) oral azithromycin given 3 times a week may be effective in improving or stabilizing pulmonary function and decreasing the frequency of pulmonary exacerbations.
Patients who have a clinically significant nontuberculous mycobacterium infection may require long-term therapy with a combination of oral, inhaled, and IV antibiotics.
Patients with allergic bronchopulmonary aspergillosis (ABPA) or lower airways aspergillus infection may require prolonged oral or IV therapy with an antifungal azole and/or systemic corticosteroids.
Treatment of gastrointestinal manifestations
Neonatal intestinal obstruction can sometimes be relieved by enemas containing a hyperosmolar or iso-osmolar radiopaque contrast material; otherwise, surgical enterostomy to flush out the viscid meconium in the intestinal lumen may be necessary. After the neonatal period, episodes of partial intestinal obstruction (distal intestinal obstruction syndrome) can be treated with enemas containing a hyperosmolar or iso-osmolar radiopaque contrast material or acetylcysteine, or by oral administration of a balanced intestinal lavage solution. A stool softener such as dioctyl sodium sulfosuccinate (docusate) or lactulose may help prevent such episodes.
Ursodeoxycholic acid, a hydrophilic bile acid, is often used in patients with liver disease caused by CF, but there is little evidence to support its efficacy in preventing progression from bile stasis to cirrhosis.
Pancreatic enzyme replacement should be given with all meals and snacks to patients with pancreatic insufficiency. The most effective enzyme preparations contain pancrelipase in pH-sensitive, enteric-coated microspheres or microtablets. For infants, the capsules are opened and the contents are mixed with acidic food. After infancy, weight-based dosing is used. Doses > 2,500 IU lipase/kg/meal or > 10,000 IU lipase/kg/day should be avoided because high enzyme dosages have been associated with fibrosing colonopathy. In patients with high enzyme requirements, acid suppression with an H2 blocker or proton pump inhibitor may improve enzyme effectiveness.
Diet therapy includes sufficient calories and protein to promote normal growth—30 to 50% more than the usual recommended dietary allowances may be required (see table Recommended Dietary Reference Intakes for Some Macronutrients). Diet therapy also includes a normal-to-high total fat intake to increase the caloric density of the diet, a water-miscible multivitamin supplement in double the recommended daily allowance, supplementation with vitamin D3 (cholecalciferol) in patients with vitamin D deficiency or insufficiency, and salt supplementation during infancy and periods of thermal stress and increased sweating. Infants receiving broad-spectrum antibiotics and patients with liver disease and hemoptysis should be given additional supplemental vitamin K. Formulas containing protein hydrolysates and medium-chain triglycerides may be used instead of modified whole-milk formulas for infants with severe malabsorption. Glucose polymers and medium-chain triglyceride supplements can be used to increase caloric intake.
In patients who fail to maintain adequate nutritional status, enteral supplementation via gastrostomy or jejunostomy may improve growth and stabilize pulmonary function (see Overview of Nutritional Support). The use of appetite stimulants to enhance growth may be helpful in some patients.
Treatment of other manifestations
Cystic fibrosis–related diabetes (CFRD) is caused by insulin insufficiency and shares features of both type 1 and type 2 diabetes. Insulin is the only recommended treatment. Management includes an insulin regimen, nutrition counseling, a diabetes self-management education program, and monitoring for microvascular complications. The plan should be carried out in conjunction with an endocrinologist and a dietitian with experience in treating both CF and diabetes.
Patients with symptomatic right heart failure should be treated with diuretics, salt restriction, and oxygen.
Recombinant human growth hormone (rhGH) may improve pulmonary function, increase height and weight and bone mineral content, and reduce the rate of hospitalization. However, because of the added cost and inconvenience, rhGH is not commonly used.
Surgery may be indicated for localized bronchiectasis or atelectasis that cannot be treated effectively with medications, nasal polyps, chronic rhinosinusitis, bleeding from esophageal varices secondary to portal hypertension, gallbladder disease, and intestinal obstruction due to a volvulus or an intussusception that cannot be medically reduced.
Liver transplantation has been done successfully in patients with end-stage liver disease.
Often, discussion of lung transplantation is needed. In considering transplantation, patients need to weigh the merits of longer survival with a transplant against the uncertainty of getting a transplant and the ongoing (but different) burden of living with an organ transplant. Bilateral cadaveric lung and live donor lobar transplantation has been done successfully in patients with advanced pulmonary disease. Combined liver-lung transplantation has been done for patients with end-stage liver and lung disease.
Bilateral lung transplantation for severe lung disease is becoming more routine and more successful with experience and improved techniques. Among adults with CF, median survival posttransplant is about 9 years.
PREVENTION
If you or your partner have close relatives with cystic fibrosis, you both may choose to have genetic testing before having children. Testing done in a lab on a sample of blood can help find out your risk of having a child with CF.
If you're already pregnant and the genetic test shows that your baby may be at risk of CF, your healthcare professional can do other tests on your unborn child.
Genetic testing isn't for everyone. Before you decide to be tested, talk with a genetic counselor about the mental health impact the test results might have.
Can you prevent CF?
Since you’re born with CF, there’s no way to prevent it. If you’re a carrier of a CFTR gene variant, you can ask your provider about prenatal genetic testing and the chances that your biological children would have CF.
Living With
How do I take care of myself?
Taking care of yourself with CF includes developing a treatment plan with your healthcare team. You must follow this plan very closely to stay well, including:
Strictly following your airway clearance regimen.
Taking medications as prescribed.
Attending regular office visits with your team of CF providers.
Get recommendations from your providers about a healthy eating plan and physical activities that are safe for you. Ask your provider if pulmonary rehabilitation is a good idea for you.
You can reduce your risk of infections by avoiding people who are sick, practicing good handwashing techniques, and getting any recommended vaccinations.
You can also take part in clinical trials, which test new treatments for CF. Ask your provider if any would be a good fit for you. Make sure you get all the information about the benefits and risks of clinical trials.
PROGNOSIS
Patients with cystic fibrosis are estimated to live until about the fourth decade of life before requiring lung transplantation. Lung transplantation confers a median survival of 8.5 years.
Is cystic fibrosis a life-threatening condition?
Yes, cystic fibrosis can be life-threatening. Lung damage — from thick mucus and frequent lung infections — is the most common cause of death.
What is the life expectancy of cystic fibrosis?
Experts predict the life expectancy of someone born with cystic fibrosis in the past few years is around 50 years old. Improvements in treatment in recent years have increased this from a few years ago, when life expectancy was between 30 and 40 years old.
People with atypical cystic fibrosis tend to have longer life expectancies than those with classic CF.
What can I expect if I have cystic fibrosis?
There’s no cure for CF. You or your child will need lifelong treatments to manage it. This includes treating infections, maintaining nutrition and seeing a CF specialist frequently. But new treatment methods help children who have CF live well into adulthood and have a better quality of life.
Treatments work best when CF is diagnosed early, which is why newborn screening is so important. The addition of CFTR modulators at a young age may improve long-term health and increase life expectancy even more in the future.
DIFFERENTIAL DIAGNOSIS
Differential diagnoses of cystic fibrosis include the following:
Asthma
Bronchiolitis
Bronchiectasis
Celiac disease
Nutritional considerations in failure to thrive
Pediatric aspergillosis
Primary ciliary dyskinesia
Sinusitis
POSSIBLE COMPLICATIONS
Complications of cystic fibrosis can affect the respiratory, digestive and reproductive systems, as well as other organs.
Respiratory system complications
Damaged airways. Cystic fibrosis is one of the leading causes of damaged airways, a long-term lung condition called bronchiectasis. Bronchiectasis results in widening and scarring of the airways. This makes it harder to move air in and out of the lungs and clear mucus from the airways.
Ongoing infections. Thick mucus in the lungs and sinuses makes a place for bacteria and fungi to live and grow. Sinus infections, bronchitis or pneumonia are common and may happen repeatedly. Infections with bacteria that don't respond to antibiotics and are difficult to treat is common too.
Growths in the nose. Because the lining inside the nose is irritated and swollen, it can develop soft, fleshy growths called nasal polyps.
Coughing up blood. Bronchiectasis can occur next to blood vessels in the lungs. The combination of airway damage and infection can result in coughing up blood. Often this is only a small amount of blood, but rarely it can be life-threatening.
Collapsed lung. Also called pneumothorax, this condition happens when air leaks into the space that separates the lungs from the chest wall. This causes part or all of a lung to collapse. Collapsed lung is more common in adults with CF. Collapsed lung can cause sudden chest pain and trouble breathing. People often have a bubbling feeling in the chest.
Respiratory failure. Over time, CF can damage lung tissue so badly that it no longer works. Lung function usually worsens slowly over time and can become life-threatening. Respiratory failure is the most common cause of death with CF.
Bouts of worsening symptoms. People with CF may experience times when respiratory symptoms are worse than usual. These are called exacerbations (eg-zas-er-bay-shuns). Symptoms may include coughing with more mucus than usual and trouble breathing. Low energy and weight loss also are common during exacerbations. Exacerbations are treated with antibiotics. Sometimes treatment can be given at home, but a stay in the hospital may be needed.
Digestive system complications
Poor nutrition. Thick mucus can block the tubes that carry digestive enzymes from the pancreas to the intestines. Without these enzymes, the body can't take in and use protein, fats or fat-soluble vitamins and can't get enough nutrients. This can result in delayed growth and weight loss. An inflamed pancreas, a condition called pancreatitis, is common.
Diabetes. The pancreas makes insulin, which the body needs to use sugar. Cystic fibrosis raises the risk of diabetes. About 20% of teenagers and up to 50% of adults with CF develop diabetes.
Liver disease. The tube that carries bile from the liver and gallbladder to the small intestine may become blocked and inflamed. This can lead to liver problems, such as jaundice, fatty liver disease and cirrhosis, and sometimes gallstones.
Intestinal obstruction. Intestinal blockage can happen to people with CF at all ages. Sometimes, a condition in which a section of the intestine slides inside another nearby section of the intestine, like a collapsible telescope, also can happen.
Distal intestinal obstruction syndrome (DIOS). DIOS is partial or complete blockage where the small intestine meets the large intestine. DIOS requires treatment right away.
Reproductive system complications
Infertility in men. Almost all men with cystic fibrosis are not fertile. The tube that connects the testicles and prostate gland, called the vas deferens, is either blocked with mucus or missing entirely. Sperm is still made in the testicles even though it can't pass into the semen made by the prostate gland. Certain fertility treatments and surgical procedures sometimes make it possible for men with CF to become biological parents.
Lower fertility in women. Although women with CF may be less fertile than other women, it's possible for them to conceive and to have successful pregnancies. Still, pregnancy can worsen the symptoms of CF. Talk with your healthcare professional about the risks.
Other complications
Thinning of the bones. Cystic fibrosis raises the risk of developing a dangerous thinning of bones called osteoporosis. Joint pain, arthritis and muscle pain also may occur.
Out of balance electrolytes and dehydration. CF causes saltier sweat, so the balance of minerals in the blood may be upset. This raises the risk for dehydration, especially with exercise or in hot weather. Symptoms of dehydration include a fast heartbeat, extreme tiredness, weakness and low blood pressure.
Gastroesophageal reflux disease (GERD). Stomach acid repeatedly flows back up into the tube connecting the mouth and stomach, called the esophagus. This backwash is known as acid reflux, and it can irritate the lining of the esophagus.
Mental health conditions. Having an ongoing medical condition that has no cure may cause fear, depression and anxiety.
Higher risk of digestive tract cancer. The risk of cancer of the esophagus, stomach, small and large bowel, liver, and pancreas is higher in people with cystic fibrosis. Regular colorectal cancer screening should begin at age 40.
Complications of CF include:
Infections. Thick mucus can trap bacteria in your lungs and airways that you can’t clear out. This can lead to frequent infections.
Congenital bilateral absence of the vas deferens (CBAVD). In this condition, males don’t have the vas deferens (sperm ducts). They often need the help of fertility procedures if they want to have biological children.
Diabetes. Damage to your pancreas can cause cystic fibrosis-related diabetes.
Malnutrition. Thick mucus in your digestive tract and the lack of pancreatic enzymes to help you digest can put you at risk for malnutrition.
Osteopenia and osteoporosis. The inability to absorb nutrients in your digestive tract can lead to conditions that make your bones too thin.
Pregnancy complications. CF can affect your digestive tract and cause poor nutrition. This can increase your risk of pregnancy complications. Preterm (early) birth is the most common complication.
WHEN TO SEE A DOCTOR
If you or your child has symptoms of cystic fibrosis — or if someone in your family has CF — talk with your healthcare professional about testing for the condition. Make an appointment with a doctor who has skills and experience in treating CF.
CF requires regular follow-up with your healthcare professional, at least every three months. Call your healthcare professional if you have new or worsening symptoms, such as more mucus than usual or a change in the mucus color, lack of energy, weight loss, or severe constipation.
Get medical care right away if you're coughing up blood, have chest pain or trouble breathing, or have severe stomach pain and bloating.
Call 911 or your local emergency number or go to the emergency department at a hospital if:
You're having a hard time catching your breath or talking.
Your lips or fingernails turn blue or gray.
Others notice that you're not mentally alert.
When should I see my healthcare provider?
Keep all of your scheduled appointments with members of your healthcare team. Talk to your provider if you have any concerns about your treatment plan or symptoms you’re having. Ask them what to do if you have symptoms of an infection. You can also reach out to them if you need help with social or emotional issues.
When should I go to the ER?
Go to the emergency room if you have symptoms of severe illness, including:
High fever (over 103 degrees Fahrenheit/40 degrees Celsius).
Difficulty breathing.
Not peeing or peeing very little.
Pain in your chest or stomach (abdomen) that doesn’t go away.
Dizziness.
Confusion.
Severe muscle pain or weakness.
Seizures.
Bluish skin, lips or nails (cyanosis, which can be a sign of low oxygen levels in your blood or tissues).
Fever or cough that gets better or goes away but then get worse.
What questions should I ask my doctor?
You might want to ask your healthcare provider:
What are my treatment options?
What’s a healthy eating plan I can follow?
What can I do to manage my symptoms?
What signs of infection should I look out for?
When should I follow up with you?
What symptoms should I go to the ER for?
Should other family members get tested?
Red-Flag Warnings (When to Seek Immediate Medical Attention)
Difficulty breathing, fast breathing, or persistent wheezing
Severe cough with thick mucus, or coughing up blood
Signs of respiratory distress (nasal flaring, chest retractions)
High fever or signs of systemic infection
Sudden drop in appetite or energy
Severe abdominal pain or vomiting
Failure to gain weight despite adequate food intake
Signs of dehydration (dry mouth, no tears when crying, decreased urination)
Rectal prolapse (can occur in infants due to malabsorption and straining)
 General Advice for Parents and Caregivers
Ongoing Management:
Daily airway clearance therapy (e.g., chest physiotherapy, percussion vests)
Inhaled medications (bronchodilators, mucolytics like dornase alfa)
Pancreatic enzyme supplements with meals and snacks
High-calorie, high-protein diet with adequate fat and salt intake
Regular physical activity to help loosen mucus
Stay up to date on vaccines, especially influenza and pneumococcal
Infection Control:
Avoid contact with other CF patients (due to risk of cross-infection)
Practice good hand hygiene
Minimize exposure to respiratory viruses and contaminated water sources
Regular Monitoring:
Frequent clinic visits with a CF specialist (multidisciplinary team)
Lung function tests (e.g., spirometry)
Nutritional assessments and growth tracking
PEDIATRIC APNEA OF PREMATURITY
OVERVIEW
Apnea of prematurity is a breathing condition that affects babies born before 37 weeks gestation (preterm birth). Your baby’s body is still growing, and the parts that support breathing are still developing. So, your baby isn’t quite ready to breathe in a normal rhythm yet. Instead, they have periods where they don’t breathe (apnea). Providers call these periods apneic spells.
It can feel very scary to think your baby isn’t breathing right. But this condition is common among preterm babies. And providers know how to treat it. Your baby needs to spend some time in the neonatal intensive care unit (NICU). Providers monitor your baby’s vital signs and give them treatments to support breathing. Your baby can likely go home once they can breathe normally without treatments.
During the first few days of life, premature infants encounter problems with temperature regulation, acquisition of oral feeding skills, and the normal control of respiration. Resolution of apnea and establishment of a normal respiratory pattern is a major developmental milestone for many premature infants. The most widely used definition of apnea of prematurity (AOP) specifies a pause of breathing for more than 15–20 s, or accompanied by oxygen desaturation (SpO2 ≤ 80% for ≥4 s) and bradycardia (heart rate < 2/3 of baseline for ≥4 s), in infants born less than 37 weeks of gestation.
While AOP is a developmental disorder, the reasons behind the propensity for apnea in immature infants are not entirely clear. Although the pathogenesis of AOP is poorly understood, the immature pulmonary reflexes and breathing responses to hypoxia and hypercapnia likely contribute to the occurrence or severity of AOP. It may also be exacerbated by a number of coexisting factors or disease states.
Severe apnea that lasts longer than 20 s is usually associated with bradycardia or desaturation, which may lead to disturbances of cerebral hemodynamics and possibly affect neurodevelopmental outcome. However, it is difficult to prove a link between apnea and poor neurodevelopmental outcomes due to a number of comorbidities and confounding factors affecting neurological development in premature infants. Therefore, evaluating the consequences of AOP on long-term neurodevelopment remains a challenge.
Apnea means "without breath" and refers to breathing that slows down or stops from any cause. Apnea of prematurity refers to breathing pauses in babies who were born before 37 weeks of pregnancy (premature birth).
Most premature babies have some degree of apnea because the area of the brain that controls breathing is still developing.
CAUSES AND RISK FACTORS
Apnea of prematurity occurs because your baby’s airways and center for breathing in the brain aren’t fully developed yet. These parts of your baby’s body work together to allow them to breathe. Providers divide apnea in newborns into three main types according to what’s causing the breathing pauses:
Obstructive
Central
Mixed
Obstructive apnea
This is when there’s a blockage in your baby’s airways. These passages carry oxygen-rich air into your baby’s lungs and remove carbon dioxide from their body. Your baby’s airways need to stay open wide enough to let air pass through.
But your baby’s airways (typically, the part that passes through their neck) may not be developed enough to stay open all the time. This can cause breathing pauses.
Central apnea
This is when your baby’s brainstem (the center in the brain responsible for breathing) doesn’t send out certain signals when expected. Normally, your baby’s brainstem sends signals to your baby’s respiratory muscles (like their diaphragm and intercostal muscles). These muscles help your baby’s lungs pull in and push out air so they can breathe.
But if your baby’s brainstem isn’t fully developed yet, it’s not ready to send out signals in a predictable or reliable way. This can lead to pauses in breathing.
Mixed apnea
This is when your baby’s airways and brainstem aren’t working as expected. It’s the most common type of apnea among preterm infants.
There are several reasons why newborns, in particular those who were born early (prematurely), may have apnea, including:
The brain areas and nerve pathways that control breathing are still developing.
The muscles that keep the airway open are smaller and not as strong as they will be later in life.
Other stresses in a sick or premature baby may worsen apnea, including:
Anemia
Feeding problems
Heart or lung problems
Infection
Low oxygen levels
Temperature problems
RISK FACTORS 
While immature respiratory control is the primary cause of apnea in the premature infant, many coexisting factors can potentiate or worsen apnea. Apnea is a common presenting sign of both local and systemic infection. Apnea can be triggered by a number of central nervous system diseases, including intracranial hemorrhage, hypoxic-ischemic encephalopathy, and seizures. Thermoregulation may also play a role in apnea. Exposure to cooler temperatures decreased the duration and frequency of AOP, while elevated body temperature increased the incidence of AOP, suggesting that apnea is related to metabolic state and environmental temperature.
Other factors that have been associated with apnea in premature infants include glucose or electrolyte imbalance, as well as the presence of a patent ductus arteriosus with a large shunt. A number of medications, including narcotic analgesics and magnesium sulfate, can lead to apnea in infants. Anemia is also associated with apnea because of lowered oxygen-carrying capacity of red blood cells that leads to hypoxia, resulting in respiratory depression.
Gastroesophageal reflux and AOP are both occurring commonly in premature infants. However, the relationship between them remains controversial. Slocum et al.set up a reflux model with rapid infusions of graded volumes of air into the esophagus of newborns to study this association. They did not find an association with apnea. Esophageal pH monitoring is the conventional method to test for the presence of acid reflux from the stomach. Since infants receive frequent milk-based feedings, which continually buffer stomach acid, measured pH may be neutral or even alkaline in some cases. Therefore, esophageal pH may significantly underestimate the frequency of reflux episodes in premature infants and may not identify AOP events resulting from nonacid reflux. Multichannel intraluminal impedance technology has been used recently to monitor electrical impedance in the esophagus, which may provide a more comprehensive measure of reflux. In conjunction with pH measurement, multichannel intraluminal impedance can increase the sensitivity of reflux detection and the identification of both acid and nonacid reflux. Even with these improved detection methods, researchers have not found an association between gastroesophageal reflux and apnea. Therefore, the majority of apneic episodes do not appear to be related to gastroesophageal reflux, though in a specific subset of events, a causal relationship may exist. As a result, there is no evidence to support the use of anti-reflux medications for the treatment of AOP. On the other hand, apnea may periodically lead to increased reflux. Recently, researchers found that the lower esophageal sphincter pressure was decreased during apneic episodes.
Other factors—including neck flexion, nasal obstruction, and delayed gastric emptying—have also been linked to apnea. Neck flexion interferes with neuromuscular regulation of pharyngeal patency and can produce intermittent airway obstruction. Nasal edema or the presence of a nasogastric feeding tube also increases nasal airway resistance. Delayed gastric emptying can also increase apneic events since abdominal distension reduces lung volume and increase vagal afferent feedback.
SIGNS AND SYMPTOMS 
Apnea of prematurity signs and symptoms include:
Pauses in breathing that last 15 to 20 seconds or longer
Slow heart rate (bradycardia)
Low blood-oxygen level (hypoxemia), which can cause your baby’s skin to look blue, white, yellow-gray or gray — especially the lips and/or tongue (cyanosis)
Your baby may have breathing pauses that are shorter than 15 seconds. Providers consider these symptoms of apnea if they occur along with bradycardia and/or hypoxemia.
It’s important to distinguish these pauses from periodic breathing. Periodic breathing involves short pauses (less than 10 seconds) with no bradycardia or low oxygen level, followed by rapid breathing, and is considered normal.
The breathing pattern of newborns is not always regular and may be called "periodic breathing." This pattern is even more likely in newborns born early. It consists of episodes of either shallow breathing or short pauses in breathing lasting just a few seconds. These episodes are then followed by periods of regular breathing. This is generally considered a normal pattern and can be expected in less mature and even some full-term babies. However, the pattern of breathing, length of breathing pauses, and the age of the baby are both important when deciding if it needs to be further evaluated.
Apnea episodes or "events" that last longer than 20 seconds are considered serious. The baby may also have a:
Drop in heart rate. This heart rate drop is called bradycardia (also called a "brady").
Drop in oxygen level (oxygen saturation). This is called desaturation (also called a "desat").
DIAGNOSIS METHODS 
Healthcare providers diagnose apnea of prematurity in the neonatal intensive care unit (NICU). This is an area of the hospital for babies who need extra care and medical attention. If your baby is born preterm, they may need to spend some time in the NICU before they can go home.
While your baby is in the NICU, providers monitor their:
Heart rate
Breathing
Blood-oxygen level
Monitors go off if your baby’s heart rate drops or their breathing pauses for a certain amount of time. Data from these monitors along with nurses’ observations  help providers diagnose apnea.
Apnea in newborns is sometimes a sign of other medical conditions. These include metabolic disorders, brain bleeds and infections. Providers check your baby for signs and symptoms of other medical conditions. They rule out all other causes before diagnosing your baby with apnea of prematurity.
An apnea of prematurity diagnosis means your baby’s breathing pauses are due to early birth. Their nervous system and respiratory system aren’t fully developed yet. So, your baby needs a little extra help with breathing until their body is ready to handle things on its own.
All premature babies under 35 weeks gestation are admitted to newborn intensive care units, or special care nurseries, with special monitors because they are at higher risk for apnea. Older babies who are found to have apnea episodes will also be placed on monitors in the hospital. More tests will be done if the baby is not preterm and appears unwell.
Monitors keep track of breathing rate, heart rate, and oxygen levels.
Drops in breathing rate, heart rate, or oxygen level can set off the alarms on these monitors.
Baby monitors marketed for home use are not the same as those used in the hospital.
Alarms may occur for other reasons (such as passing stool or moving around), so the monitor tracings are reviewed regularly by the health care team.
TREATMENT OPTIONS
Apnea of prematurity treatment typically includes one or both of the following:
Medications (usually caffeine citrate)
Breathing support
Caffeine for apnea of prematurity
Caffeine citrate is a medication providers use to treat apnea of prematurity. Just like your morning coffee gives you a jolt, this medicine stimulates your baby’s nervous system. This “wake-up call” helps your baby’s brainstem and nerve cells send signals that regulate breathing.
Caffeine helps your baby have fewer apneic spells. It also helps shorten these pauses in breathing.
Breathing support
Your baby’s upper airway (pharynx and larynx) might need a little help staying open. If this is the case, providers often use continuous positive airway pressure (CPAP). With CPAP, your baby is still breathing on their own. But tiny prongs in their nose send air through their airway. This lowers the risk of breathing pauses from airway blockages.
If your baby continues to have severe apneic spells, they may need mechanical ventilation. This means a machine (a ventilator) does the work of breathing for your baby until their body can take over
How apnea is treated depends on:
The cause
How often it occurs
Severity of episodes
Babies who are otherwise healthy and have occasional minor episodes are simply watched. In these cases, the episodes go away when the babies are gently touched or "stimulated" during periods when breathing stops.
Babies who are well, but who are very premature and/or have many apnea episodes may be given caffeine. This will help make their breathing pattern more regular. Sometimes, the nurse will change a baby's position, use suction to remove fluid or mucus from the mouth or nose, or use a bag and mask to help with breathing.
Breathing can be assisted by:
Proper positioning
Slower feeding time
Oxygen
Continuous positive airway pressure (CPAP)
Breathing machine (ventilator) in extreme cases
Some infants who continue to have apnea but are otherwise mature and healthy may be discharged from the hospital on a home apnea monitor, with or without caffeine, until they have outgrown their immature breathing pattern.
PREVENTION TIPS
Prevention of pediatric apnea of prematurity (AOP)—a condition characterized by pauses in breathing lasting over 20 seconds primarily in preterm infants—relies on a combination of pharmacological and non-pharmacological interventions aimed at reducing the frequency and severity of apneic events. These methods address the underlying mechanisms such as decreased respiratory drive, immature diaphragmatic function, and increased work of breathing.
1. Pharmacological Prevention: Caffeine Therapy
Prophylactic caffeine administration is the most widely used and evidence-supported preventive treatment for AOP. Caffeine, a methylxanthine respiratory stimulant, enhances the respiratory drive by stimulating the central nervous system and diaphragm function. It has a large therapeutic window and fewer side effects compared to other methylxanthines like theophylline, making it the preferred agent.
Caffeine is typically started early in very preterm infants, especially those weighing less than 1250 grams, and continued until about 34 to 35 weeks' corrected gestation or until the infant remains apnea free without intervention for several days.
While some studies on prophylactic caffeine have mixed results on apnea frequency reduction, overall, caffeine use improves clinical outcomes, including reduced need for ventilatory support and better neurodevelopmental prognosis at 18–21 months.
2. Respiratory Support and Positioning
Non-invasive respiratory support like continuous positive airway pressure (CPAP) and flow-synchronized nasal ventilation can reduce apnea by maintaining airway patency and decreasing work of breathing.
Prone positioning in the hospital setting has been shown to reduce apnea frequency by maintaining airway openness and preventing airway obstruction. However, due to the increased risk of sudden infant death syndrome (SIDS), this position is used only under strict monitoring in the neonatal intensive care unit (NICU) and avoided after discharge, when supine sleeping is advised instead.
Maintaining stable and appropriate environmental temperature is important, as overheating and elevated ambient temperature can suppress ventilatory responses and increase apnea risk.
3. Sensory and Tactile Stimulation
Gentle tactile stimulation during apnea episodes is a longstanding method to terminate apneic events by promoting respiratory drive. Prophylactically, periodic tactile stimulation has shown some benefit in reducing apnea episodes, though it increases nursing workload and evidence is mixed for mechanical stimulation methods like oscillating mattresses.
Sensory stimuli (auditory, olfactory) such as pleasant scents (e.g., vanilla) have shown short-term reduction in AOP without adverse effects, but longer-term safety and efficacy data are limited.
4. Monitoring and Supportive Care
Infants are usually monitored in hospital until apnea resolves adequately. In rare cases, an apnea monitor may be used at home with caregivers trained in CPR and monitoring equipment use, although there is no evidence that home monitors reduce the risk of sudden infant death.
Treatment of underlying causes such as infections or anemia is critical to preventing secondary apnea episodes.
PROGNOSIS
Apnea of prematurity is a temporary condition. Symptoms improve as your baby grows. Your baby may need to spend several weeks or even a couple of months in the NICU. They’ll receive care not just for their breathing but also for any other issues related to preterm birth.
Providers will tell you how long your baby needs to stay in the NICU. They’ll also explain what needs to happen for your baby to safely head home.
In general, providers identify when it’s safe to stop giving your baby treatments. Then, after stopping treatment, they monitor your baby to see how their body responds. Your baby needs to go a certain number of days without apnea symptoms in order for providers to send them home. The length of this “observation period” depends on how early your baby was born and their overall health.
Most babies don’t need continued monitoring with devices at home. But if your baby does need monitoring, providers will explain exactly what’s involved. Ask if anything is unclear or you’re concerned about caring for your baby at home. Providers will make sure you get the information you need to feel comfortable with this transition.
Apnea is common in premature babies. Mild apnea does not appear to have long-term effects. However, preventing multiple or severe episodes is better for the baby over the long-term.
Apnea of prematurity most often goes away as the baby approaches their "due date." In some cases, such as in infants who were born very prematurely or have severe lung disease, apnea may persist a few weeks longer.
POSSIBLE COMPLICATIONS 
Possible complications of pediatric apnea of prematurity (AOP) include both short-term and long-term morbidities that can significantly affect the health and development of premature infants.
Short-term complications:
Bradycardia and oxygen desaturation: During apnea episodes, infants often experience a slow heart rate (bradycardia) and lowered blood oxygen levels (hypoxemia), which can lead to cardiovascular instability.
Decreased cerebral blood flow: Severe bradycardia can reduce cerebral blood flow velocity, potentially causing hypoperfusion of the brain, which increases the risk of hypoxic-ischemic brain injury.
Intraventricular hemorrhage and hydrocephalus: Prolonged or severe apnea episodes have been linked to increased incidence of brain hemorrhages and hydrocephalus, which are serious neurological conditions.
Prolonged mechanical ventilation: Babies with significant apnea may require longer ventilatory support, which itself carries risks of lung injury and infection.
Long-term complications:
Neurodevelopmental impairment: There is evidence showing that infants with frequent or prolonged apnea episodes have a higher risk of delayed mental and motor development, cerebral palsy, visual and hearing impairments, and abnormal neurological outcomes by 1 to 3 years of age.
Retinopathy of prematurity (ROP): Chronic intermittent hypoxia from apnea may contribute to the development of ROP, a potentially blinding eye disorder in premature infants.
Increased risk of infant mortality: Persistent apnea and associated hypoxemia have been linked to higher mortality rates in premature infants.
Respiratory failure: Apnea can predispose to respiratory failure requiring intensive care and interventions.
Other relevant risks:
Prolonged apnea beyond expected age: While apnea tends to resolve by 36 to 40 weeks’ postconceptional age, extremely premature infants (24–28 weeks gestation) may experience persistent apnea even beyond 44 weeks, increasing complications risk.
Sudden infant death syndrome (SIDS): Although premature infants have a higher baseline risk for SIDS, apnea of prematurity itself is not conclusively linked as a direct cause
WHEN TO SEE A DOCTOR
You should see a doctor for pediatric apnea of prematurity if your premature baby exhibits any of the following concerning signs, as these may indicate clinically significant apnea episodes requiring medical evaluation and treatment:
Pauses in breathing lasting more than 15 to 20 seconds, or shorter pauses accompanied by a slow heart rate (bradycardia) or low oxygen levels (hypoxemia). These episodes are abnormal and distinguish apnea of prematurity (AOP) from normal periodic breathing pauses, which usually last less than 10 seconds and do not cause heart rate or color changes.
A drop in heart rate below 80 beats per minute during breathing pauses, often seen as pale or bluish (cyanotic) skin tone or limpness in the baby, which indicates the baby is not getting enough oxygen.
Episodes of apnea that begin after the first week of life or increase in frequency or severity, particularly if accompanied by low oxygen saturation or significant bradycardia, signaling worsening respiratory function or potential underlying complications such as infection, hypoglycemia, or neurological issues that must be ruled out.
Visible changes in the baby’s color such as bluish discoloration around the mouth or limbs, or in babies of color, yellow-gray or pale mucous membranes inside the mouth or eyelids, indicating oxygen deprivation during apnea episodes.
If apnea episodes are associated with other signs of illness such as feeding difficulties, temperature instability, lethargy, or poor muscle tone, which may suggest additional health concerns exacerbating apnea.
Babies born very prematurely (less than 35 weeks gestation), especially those with very low birth weights (<1000 g), have a higher risk for apnea and should be monitored closely by healthcare providers, often in a hospital setting with apnea monitors that alarm when significant episodes occur
Red Flag
Be alert for these symptoms in premature infants, as they may indicate apnea of prematurity (AOP) or underlying complications:
Pauses in breathing lasting 20 seconds or longer.
Breathing pauses less than 20 seconds but associated with:
Slow heart rate (bradycardia) heart rate <100/min.
Low oxygen saturation (below 85%).
Bluish discoloration of skin or lips (cyanosis).
Poor muscle tone or appearing limp.
Episodes not resolving quickly with gentle stimulation.
Onset of apnea within the first day of life (potentially serious, may suggest a cause other than AOP, such as infection or brain injury).
Apnea developing after the first two weeks of life in an otherwise stable infant (could indicate infection, metabolic imbalance, or other disease).
Clusters of frequent and/or severe episodes especially if accompanied by:
Feeding difficulties.
Lethargy or poor responsiveness.
Fever, vomiting, or seizures (may signal drug toxicity or other illnesses
General Advice for Caregivers: Managing and Monitoring Apnea of Prematurity
1. Understand Apnea of Prematurity
Apnea of prematurity involves pauses in a baby's breathing (typically over 20 seconds), often accompanied by a slow heart rate or low oxygen levels.
It is common in infants born before 37 weeks and most often resolves as the nervous system matures.
2. Hospital-Based Monitoring
While in the hospital, premature babies are continuously monitored for breathing and heart rate abnormalities.
Alarms are set to notify healthcare staff of concerning changes, allowing for immediate intervention.
3. At-Home Care: General Recommendations
Safe Sleep: Always place the baby on their back to sleep, never on the stomach or side, to reduce the risk of sudden infant death. Avoid soft bedding and toys in the crib.
Feeding: Ensure regular and slow feedings; watch for signs of distress or pauses in breathing during or after feeding. Feed in an upright position if possible.
Temperature Management: Dress the baby comfortably; avoid overheating or chilling, as temperature extremes can trigger episodes.
Gentle Handling: Minimize overstimulation and handle the baby gently, as excessive stimulation can worsen apnea.
Monitor for Symptoms: Be observant for warning signs such as blue lips or skin, limpness, failure to resume breathing quickly after stopping, or overall poor color.
Follow Discharge Instructions: Adhere closely to the caregivers’ discharge plan, including medication schedules such as caffeine therapy (if prescribed), scheduled check-ups, and guidelines for home monitoring if needed.
4. Use of Apnea Monitors
Most infants do not require home monitoring unless specifically advised by a healthcare provider.
If a monitor is used:
Learn proper operation: Get training at the hospital for setup, alarm response, and troubleshooting.
Respond to alarms: Immediate gentle stimulation (touch or mild shake) may restore breathing. If the baby does not respond, seek emergency help right away.
5. When to Seek Immediate Medical Help
Pauses in breathing longer than 20 seconds.
Blue or pale skin color, especially around the lips or face.
Limpness or very low tone.
Difficulty waking or unusual sleepiness.
Any concerns about the baby's breathing or general appearance.
6. Communication and Support
Maintain regular communication with your healthcare provider for guidance, especially if episodes change in frequency or severity.
Keep a diary of apnea episodes, feeding habits, and any concerning symptoms to share during follow-up visits.
Seek emotional support or counseling if caring for a preterm infant feels stressful or overwhelming.
Careful adherence to these practices provides the best chance for your infant’s safe growth and development until apnea resolves naturally with maturity.
 DIFFERENTIAL DIAGNOSIS 
Infections: Sepsis (blood infection), meningitis (infection of the brain and spinal cord), and other infections can cause apnea. 
Hypoglycemia: Low blood sugar levels can lead to apnea. 
Central Nervous System (CNS) Pathology: Brain malformations, injuries, or seizures can manifest as apnea. 
Metabolic Disorders: Inborn errors of metabolism or other metabolic disturbances can be a cause. 
Respiratory Distress Syndrome (RDS): A common respiratory problem in premature infants. 
Other: Gastroesophageal reflux (GER), obstructive sleep apnea, and certain cardiac conditions may also be associated with apnea. 
PEDIATRIC CONGENITAL LUNG MALFORMATIONS
OVERVIEW 
Pediatric congenital lung malformations (CLMs) are rare developmental anomalies present at birth, arising from abnormal lung formation during fetal development. These malformations include cysts, solid masses, or mixed lesions affecting lung parenchyma, airways, and vasculature.
The main types of congenital lung malformations are:
Congenital Pulmonary Airway Malformation (CPAM), formerly known as Congenital Cystic Adenomatoid Malformation (CCAM): a benign mass characterized by abnormal overgrowth of lung tissue that forms cysts of various sizes. CPAM lesions usually occur in a single lung lobe and may impair normal alveolar development. They often stop growing around 26 weeks gestation and can shrink before birth. Large CPAMs may cause fetal hydrops due to increased blood flow, risking heart failure.
Pulmonary Sequestration (PS): solid masses of lung tissue that do not communicate with normal lung airways and have an aberrant blood supply usually from the aorta. PS can be intralobar (within the lung) or extralobar (outside the lung, in the chest or abdomen).
Congenital Lobar Emphysema: a condition where a blocked airway causes overinflation of one lung lobe, leading to compression of healthy lung tissue and respiratory difficulty.
Bronchogenic cysts: cystic masses often near the esophagus or trachea that may compress airways if large or infected.
Other variants, including hybrid lesions combining features of CPAM and PS, and bronchial atresia (partial airway blockage).
Diagnosis usually occurs prenatally by ultrasound or postnatally by imaging modalities such as chest radiography, CT, MRI, and ultrasound. Imaging plays a central role in diagnosis, management, and follow-up, though specific guidelines are still evolving.
Treatment depends on symptoms; symptomatic lesions typically require surgical resection, most commonly lobectomy. The decision to operate on asymptomatic cases remains debated. Post-surgery, some children experience acute or long-term complications, including decreased lung function, underscoring the need for long-term follow-up.
Incidence of CPAM ranges from approximately 1 in 8,300 to 1 in 35,000 births, reflecting the rarity of these conditions.
In summary, pediatric congenital lung malformations represent a spectrum of rare, diverse developmental anomalies that can affect respiratory function and often require specialized imaging assessment and surgical management with long-term clinical monitoring.
Congenital lung malformations (CLMs) are rare developmental anomalies of the lung, including congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestration, congenital lobar overinflation, bronchogenic cyst and isolated congenital bronchial atresia. CLMs occur in 4 out of 10,000 live births. Postnatal presentation ranges from an asymptomatic infant to respiratory failure. CLMs are typically diagnosed with antenatal ultrasonography and confirmed by chest CT angiography in the first few months of life. Although surgical treatment is the gold standard for symptomatic CLMs, a consensus on asymptomatic cases has not been reached. Resection, either thoracoscopically or through thoracotomy, minimizes the risk of local morbidity, including recurrent infections and pneumothorax, and avoids the risk of malignancies that have been associated with CPAM, bronchopulmonary sequestration and bronchogenic cyst. However, some surgeons suggest expectant management as the incidence of adverse outcomes, including malignancy, remains unknown. In either case, a planned follow-up and a proper transition to adult care are needed. The biological mechanisms through which some CLMs may trigger malignant transformation are under investigation. KRAS has already been confirmed to be somatically mutated in CPAM and other genetic susceptibilities linked to tumour development have been explored. By summarizing current progress in CLM diagnosis, management and molecular understanding we hope to highlight open questions that require urgent attention.
CAUSES AND RISK FACTORS 
1. Aberrant Embryological Development
The primary cause of CLMs is abnormal embryological lung development at different stages of gestation. Disruption in the normal process leads to malformations of airways, alveoli, or blood vessels.
The human lung develops through complex branching morphogenesis regulated by precise gene signaling. Any disturbance, especially during critical phases, can result in a spectrum of lesions such as:
Congenital Pulmonary Airway Malformation (CPAM)
Bronchopulmonary Sequestration (BPS)
Congenital Lobar Emphysema
Bronchogenic Cyst
2. Genetic and Molecular Factors
Gene signaling dysregulation: Research suggests that abnormal signaling in pathways, such as the Fibroblast Growth Factor 10 (FGF10) and Sonic Hedgehog (SHH), leads to cystic lesions by disrupting lung branching and epithelial differentiation. Overactive or disrupted FGF10/SHH signaling has been implicated, particularly in CPAMs.
DICER1 gene mutation: Preclinical studies show that mutations in the DICER1 gene may result in improper airway development, cyst formation, and features similar to pleuropulmonary blastoma, linking congenital lung malformations to genetic predispositions.
Other transcriptional/differentiation errors: The Ras pathway and other kinases have also been implicated in abnormal development due to gene expression irregularities.
3. Physical and Environmental Influences (Secondary Causes)
Oligohydramnios (low amniotic fluid): Low levels of amniotic fluid reduce lung distending forces, critically impeding normal lung growth and causing hypoplasia.
Thoracic space limitations: Conditions such as congenital diaphragmatic hernia, large pleural effusions, or thoracic masses can compress the developing lungs, leading secondarily to hypoplasia.
4. Timing of Insult
The type and severity of malformation depend significantly on the timing of the embryological insult:
Early insults (pseudoglandular stage) often produce more severe or extensive lesions.
Later insults may affect only certain lung regions or yield milder malformations
RISK FACTORS 
1. Genetic Predisposition
Although most cases are sporadic, a family history of congenital malformations or recognized syndromes (e.g., DICER1 mutations) increases risk. Other underlying gene mutations affecting lung growth factors, receptors (such as FGFR2), or transcription factors might contribute.
2. Maternal and Obstetric Factors
Oligohydramnios: Significantly raises the risk of pulmonary hypoplasia (underdeveloped lungs).
Maternal infections or exposures: There is no consistent evidence that infections or medications directly cause CLMs, but severe disturbances in intrauterine environment can theoretically impact lung development.
3. Male Predominance
Several population studies report a higher prevalence of certain malformations, like CPAM, in male infants; reasons remain unclear and may relate to molecular or hormonal differences during development.
4. Associated Anomalies
Thoracic deformities, congenital heart disease, and hernias: These conditions can restrict lung expansion and growth, indirectly contributing to malformations.
SIGNS AND SYMPTOMS 
Pediatric congenital lung malformations (CLMs) refer to a group of rare developmental anomalies of the lungs present at birth. The signs and symptoms vary widely depending on the specific type, size, location, and severity of the malformation, but primarily involve respiratory difficulties and recurrent respiratory infections.
Common Signs and Symptoms of Pediatric Congenital Lung Malformations
Respiratory Distress and Breathing Difficulties
Rapid breathing (tachypnea), grunting, and use of accessory muscles are common in newborns with significant lung malformations.
Shortness of breath or difficulty breathing can be present from birth or develop later as the lesion affects lung function.
Wheezing may occur especially when airways are obstructed or compressed by cysts or abnormal lung tissue.
Recurrent Pulmonary Infections
Children often experience repeated chest infections or pneumonia due to mucus trapping and impaired clearance in the malformed lung regions.
Chest pain and coughing, sometimes with hemoptysis (coughing up blood), may be symptoms of ongoing infection or irritation from the abnormal tissue.
Signs of Compromised Oxygenation
Cyanosis, a bluish tint of the skin and nail beds due to low oxygen levels, can occur in severe cases.
Chronic respiratory distress can also lead to failure to thrive and poor growth in infants.
Physical Findings Related to Lung and Heart Compression
An enlarged or asymmetrically expanded chest may be observed if a lung lobe is overinflated or cystically enlarged (e.g., congenital lobar emphysema).
Mediastinal shift (shifting of heart and other structures) can occur, compromising cardiac and lung function, sometimes causing severe respiratory failure and the need for ventilatory support.
Collapse of Lung Tissue
Pneumothorax (collapsed lung) may occur spontaneously due to rupture of cystic lesions leading to sudden worsening of breathing.
Specific Symptoms by Type of Malformation
Congenital Pulmonary Airway Malformation (CPAM): Often shows respiratory distress, recurrent infections, chest pain, and in severe cases respiratory failure at birth or early infancy.
Congenital Lobar Emphysema: Can present with wheezing, respiratory distress, cyanosis, and overexpansion of a lung lobe compressing normal tissue.
Pulmonary Cysts and Bronchogenic Cysts: Symptoms depend on size and location but generally include breathing difficulties and recurrent infections.
Pulmonary Sequestration: Presents typically later with repeated infections due to nonfunctional lung tissue not connected to normal airway or blood supply.
Summary Table of Key Symptoms
TREATMENT OPTIONS
Treatment options for pediatric congenital lung malformations (CLMs) depend on the type and severity of the lesion, as well as the presence of symptoms or complications such as hydrops or infection. Key treatment approaches include both prenatal interventions and postnatal management, with surgery being the cornerstone in most cases.
Prenatal Treatment Options
When CLMs are detected during pregnancy, the care team closely monitors the lesion’s size and the fetus’s condition via ultrasound:
Steroid Therapy: Administration of steroids like betamethasone to the mother can reduce the size of microcystic lesions and prevent or reverse fetal hydrops, a serious complication where fluid accumulates causing fetal distress.
Thoracoamniotic Shunting: For large macrocystic lesions causing fluid buildup around the fetal lung, a shunt tube may be inserted to drain fluid into the amniotic sac, relieving pressure on the fetal lungs, heart, and blood vessels.
Fetal Surgery: Rarely performed, this may involve lobectomy, the surgical removal of the lung mass in utero, or an EXIT procedure (ex utero intrapartum treatment) where surgery is done while the baby remains attached to the placenta for continued oxygenation. These are typically reserved for severe cases with hydrops or heart failure.
Postnatal Treatment Options
Most babies with CLMs will require surgery after birth to prevent infection, respiratory distress, or potential malignant transformation of the lesion:
Surgical Resection: The primary treatment is removal of the malformation, typically via lobectomy (removal of an affected lung lobe) or cystectomy, performed through a thoracotomy (incision in the chest). Segmentectomy is another surgical option depending on lesion size and location.
Postoperative Care: Following surgery, patients often require intensive care support, including mechanical ventilation for 48 to 72 hours, especially in neonates with congenital lobar emphysema, to help the remaining lung expand.
Conservative Management: In selected cases, such as small asymptomatic cysts or milder congenital lobar emphysema, observation without immediate surgery may be appropriate with close monitoring. However, surgery is usually recommended to prevent future complications like infections or malignancy.
Supportive care for pulmonary hypoplasia: Babies with underdeveloped lungs require respiratory support immediately post-birth, along with long-term management such as physiotherapy and nutritional support to aid respiratory function and growth.
Additional Options for Complex Cases
In cases refractory to conventional treatments or in older patients with complicated vascular malformations:
Endovascular Treatments: Surgical clipping, ligation, or endovascular occlusion devices are sometimes used, especially for vascular malformations.
Outcomes and Safety
Surgical resection of congenital lung malformations is generally safe and effective, with low morbidity and very low mortality rates reported in specialized centers. Early surgery helps avoid repeated infections, respiratory complications, and the small risk of malignancy associated with untreated lesions.
PREVENTION TIPS
Prevention Tips for Pediatric Congenital Lung Malformations
Understanding Congenital Lung Malformations (CLMs)
Congenital lung malformations are rare developmental abnormalities of the lungs that occur while a baby is still in the womb. Types include:
Congenital pulmonary airway malformation (CPAM)
Bronchopulmonary sequestration
Bronchogenic cysts
Congenital lobar emphysema
Pulmonary hypoplasia
These malformations result from abnormal embryologic development disruptions or errors in the formation of the airway and lung tissue during gestation.
Can CLMs Be Prevented?
Currently, there are no established or guaranteed methods to prevent congenital lung malformations. The causes are not fully understood, but most cases arise spontaneously, often without any clear familial or environmental risk factor.
Why Prevention Is Challenging
CLMs typically result from random errors in airway or lung development during embryogenesis.
While rare, some genetic factors or familial patterns may be involved, but these are not well-defined.
No consistent maternal behaviors, exposures, or infections have been directly linked as prevention targets in most cases.
Strategies and Recommendations
While specific prevention is not possible, certain general approaches may help reduce overall risk for congenital malformations (not just CLMs):
1. Preconception and Prenatal Health
Encourage optimal maternal health before and during pregnancy, including control of chronic illnesses, maintaining a healthy weight, and nutritional optimization.
Ensure adequate prenatal care for early detection and management of risk factors.
2. Avoidance of Teratogenic Substances
Avoid smoking, alcohol, illicit drugs, and unnecessary medications during pregnancy, as these can increase the risk of various birth defects.
3. Genetic Counseling
For families with a history of congenital malformations or genetic syndromes, genetic counseling may help evaluate risks in future pregnancies.
4. Environmental Safety
Minimize exposure to known environmental toxins (e.g., pesticides, radiation, some chemicals).
5. Prenatal Screening
First and second trimester ultrasounds can aid in early detection.
When a CLM is found, maternal-fetal specialists can manage the pregnancy to reduce complications, sometimes using steroids (betamethasone) to reduce fetal complications in select cases (e.g., large lesions with hydrops).
6. Research and Awareness
Encourage ongoing research to clarify causes and prevention for future generations.
Educate parents about the signs and symptoms of respiratory distress in newborns for prompt evaluation.
Key Points for Parents and Caregivers
Most CLMs are not preventable due to their origin early in fetal development.
Early, regular prenatal care and avoiding harmful exposures offer general protection against congenital anomalies.
When detected prenatally, collaboration with specialized care teams can optimize outcomes for both mother and child.
Congenital lung malformations remain largely unpreventable; however, healthy pregnancy practices and informed prenatal care play vital roles in reducing overall birth defect risk and ensuring the best outcomes where malformations do occur
PROGNOSIS
The prognosis of pediatric congenital lung malformations (CLMs) is generally favorable, but it depends on multiple factors including the type of malformation, the presence of symptoms, timing and type of treatment, and long-term follow-up.
Key Points on Prognosis:
Overall outlook: In the vast majority of children with congenital lung malformations, the prognosis is excellent. After appropriate treatment, most children grow to lead normal, healthy lives. Survival rates reported are high, around 93–95%, with low mortality mainly associated with specific severe cases or neonatal respiratory failure.
Types and presentation: The most common congenital malformations include congenital pulmonary airway malformation (CPAM), congenital lobar emphysema, bronchogenic cyst, bronchopulmonary sequestration, and bronchial atresia. Many children present with recurrent respiratory infections or respiratory distress, though some remain asymptomatic through childhood.
Treatment and outcomes: Surgical resection is the mainstay of treatment, especially for symptomatic cases. Lobectomy is the most common procedure and is generally curative, producing good long-term outcomes. Surgery in asymptomatic patients is more controversial but often preferred to avoid complications such as infection or malignancy. Early treatment is important to optimize lung and vascular growth.
Complications and long-term prognosis: Postoperative complications can occur in approximately 30% of patients in the short term, and long-term complications occur in a notable proportion (up to 70%) though these do not always correlate with specific patient or surgical factors. Pulmonary function may be affected, especially after extensive resection such as pneumonectomy, which can lead to measurable deficits in lung function.
Long-term monitoring: Given that some children experience clinical and functional respiratory complications following surgery, long-term follow-up with pulmonary function testing and clinical review is essential to ensure optimal management and timely intervention if problems arise.
Antenatal and neonatal factors: Antenatal detection and prognosis are influenced by lesion size; larger lesions carry higher risks including fetal demise in rare cases (3–4%). After birth, mortality is low (<1%) but may occur with severe respiratory failure in the neonatal period. Many malformations can spontaneously resolve prenatally or remain asymptomatic postnatally.
Pediatric Congenital Lung Malformations (CLMs) are a group of rare developmental anomalies of the lung that may cause various complications both before and after birth. These complications vary by malformation type, size, location, and treatment status.
Prenatal (Antenatal) Complications
Fetal hydrops: This is a severe and potentially fatal condition where excessive fluid accumulates in the fetus' tissues and body cavities. It occurs in about 5–30% of CLM cases and is associated with high mortality. Hydrops arises due to high blood flow demands on the fetal heart caused by large lung lesions like Congenital Pulmonary Airway Malformation (CPAM), leading to heart failure.
Pleural effusion: Collection of fluid in the fetal pleural space due to lesion-induced esophageal compression can impair normal fetal swallowing.
Polyhydramnios: Excess amniotic fluid accumulation occurs secondary to fetal swallowing difficulties caused by mass effect of lung malformations pressing on the esophagus.
Mediastinal shift: Progressive enlargement of lung lesions may displace the mediastinum, compressing adjacent lungs and vital structures, potentially leading to pulmonary hypoplasia and impaired cardiorespiratory function.
Neonatal and Postnatal Complications
Respiratory distress: Some neonates present with breathing difficulties due to mass effect or compromised lung tissue, though more than 75% of neonates with CLMs are asymptomatic at birth.
Recurrent infections: Undetected or untreated CLMs predispose to bacterial, fungal, and mycobacterial infections due to abnormal lung architecture and trapped secretions, often manifesting as recurrent pneumonia or chronic cough.
Bleeding and hemothorax: Lesions with abnormal vasculature (e.g., pulmonary sequestration) can bleed, causing hemothorax, a potentially life-threatening accumulation of blood in the pleural space.
Air embolism: Abnormal systemic arterial supply in malformations like bronchopulmonary sequestration may lead to air emboli entering circulation, risking cardiovascular complications.
High-output cardiac failure: Systemic arterial shunting through the malformation can overload the heart, causing failure, especially in large lesions.
Pneumothorax: Rupture of cystic components in malformations may cause air to escape into the pleural space, leading to lung collapse.
Growth and feeding difficulties: Some infants may experience poor feeding and failure to thrive, secondary to respiratory compromise.
Long-term and Rare Complications
Malignancy risk: There is ongoing debate and evidence that certain CLMs, particularly CPAM type 4, are associated with the development of malignancies such as pleuropulmonary blastoma (PPB), bronchioloalveolar carcinoma, and lung adenocarcinoma. PPB occurs at a higher rate in children with CLMs (up to 4%), with a 20% mortality rate. Malignancy can arise within cystic lesions or remaining lung tissue post-surgery.
Long-term respiratory impairment following surgery: Surgical resection (lobectomy, segmentectomy, or pneumonectomy) of symptomatic CLMs can lead to complications in about 31% of children soon after surgery and up to 74% may have long-term complications including reduced lung function. Pneumonectomy is significantly correlated with lung function deficits.
Chronic symptoms: Chronic cough, wheezing, and exercise intolerance may develop as sequelae, partly due to reduced lung capacity or persistent abnormalities.
Summary Table of Key Complications
Conclusion
Pediatric congenital lung malformations carry risks of serious prenatal complications like hydrops and mediastinal shift that can be life-threatening. Postnatal complications include respiratory distress, infections, bleeding, cardiac issues, and rare malignant transformation. Surgical resection can prevent many complications but also entails acute and chronic risks, necessitating long-term follow-up and functional assessment in affected children.
WHEN TO SEE A DOCTOR
You should see a doctor for pediatric congenital lung malformations (CLMs) if your child exhibits signs or symptoms commonly associated with these conditions, or if the malformation has been detected prenatally and requires ongoing monitoring. Here is an elaborate explanation of when to seek medical evaluation and care:
Prenatal Detection and Monitoring
Many congenital lung malformations (such as congenital pulmonary airway malformation (CPAM), congenital lobar emphysema, and pulmonary sequestration) are diagnosed during prenatal ultrasound examinations.
If a lung malformation is detected before birth, your obstetrician or fetal care team will monitor the fetus closely with serial ultrasounds, fetal MRI, or fetal echocardiograms to evaluate the lesion’s size and possible effects on the baby's heart and lungs.
Medical attention is warranted if the lesion grows significantly or causes complications like hydrops or heart failure risk (often measured by the CCAM volume-to-head circumference ratio, CVR, with 1.6 or greater considered higher risk).
Newborns or Infants with Symptoms
Seek medical care immediately if your newborn or infant shows signs of respiratory distress, including:
Difficulty breathing
Rapid breathing or grunting
Bluish tint to the skin or nail beds (cyanosis)
Enlarged chest or signs of heart and lung compression
These symptoms indicate that the lung malformation might be impairing lung function or causing airway compression and require urgent evaluation.
Symptoms That May Appear Later in Infancy or Childhood
Some children are asymptomatic at birth and are diagnosed later, either incidentally on imaging or when symptoms develop. You should see a doctor if your child develops:
Frequent or prolonged chest infections or pneumonia
Persistent or recurrent coughing, including coughing up blood
Wheezing or shortness of breath
Fever associated with respiratory illness
Chest pain
These symptoms suggest that the malformation may be causing recurrent lung infections or airway problems.
Routine Follow-Up for Known Lesions
If your child has a known congenital lung malformation, even without symptoms, regular follow-up with a pediatric pulmonologist or surgeon is important to monitor the lesion and lung function.
Imaging studies such as chest X-rays and CT scans will help assess the lesion’s size and whether any changes or complications are occurring.
Red Flag Warnings
Key Red Flag Warnings in Pediatric CLMs:
Respiratory distress: Rapid breathing (tachypnea), grunting, wheezing, shortness of breath, and difficulty breathing especially soon after birth are critical warning signs. Large lesions can compress normal lung tissue and displace mediastinal structures (heart, airways), impairing respiratory function.
Hydrops fetalis: In fetal or neonatal CPAM, a large malformation may cause excessive blood flow leading to heart failure and fluid accumulation in fetal compartments (hydrops), which can be life-threatening if untreated.
Pulmonary hypoplasia: Large malformations can prevent normal lung development on the affected side (hypoplasia), further impairing oxygenation and increasing risk of respiratory failure.
Recurrent or severe lung infections: Frequent or prolonged pneumonia, coughing with blood, and chest pain after birth may indicate infected or symptomatic lung malformations requiring early intervention.
Failure to thrive: Trouble feeding, poor weight gain, and systemic symptoms such as fever and irritability in infants with CLMs also point to potential complications from lung dysfunction or infection.
Mediastinal shift or compression: Significant shift of mediastinum due to large lesions can impair cardiovascular and respiratory function, necessitating urgent evaluation.
Air trapping and overinflation: Conditions like congenital lobar emphysema can cause progressive lung overinflation and respiratory distress, especially in the neonatal period.
Clinical relevance of red flags:
Red flags warrant close monitoring, diagnostic imaging (ultrasound, MRI), and possible prenatal or postnatal intervention, including surgery.
Timely recognition can prevent progression to respiratory failure or fatal outcomes such as hydrops.
Management is often multidisciplinary involving neonatologists, pediatric surgeons, pulmonologists, and radiologists.
In summary, pediatric CLM red flags are primarily related to respiratory distress, cardiac compromise (hydrops), repeated infections, failure to thrive, and mediastinal compression, signaling severe or complicated disease that requires prompt diagnosis and management to improve outcomes.
General Advice
Prenatal and Postnatal Monitoring
Most CLMs are diagnosed during routine prenatal ultrasounds, sometimes followed by fetal MRI or echocardiograms to assess lesion size and function.  
The cystic adenomatoid malformation volume-to-head circumference ratio (CVR) is used antenatally to assess risk of complications such as hydrops fetalis.
 Postnatal Diagnosis and Follow-up
After birth, infants suspected of having CLMs require chest X-rays and CT scans for detailed anatomical assessment.  
Some small malformations may remain asymptomatic but should be regularly monitored due to the risk of infection or malignancy in rare cases.
Management and general advise 
Prenatal monitoring: Serial ultrasounds assess lesion size and potential complications such as hydrops. In some severe cases, fetal interventions or early delivery may be considered.
Postnatal evaluation: Imaging (chest X-ray, CT scan) and pulmonary function testing are essential.
Surgical resection: Often recommended for symptomatic lesions or to prevent complications such as infection, respiratory distress, or potential malignancy. Timing of surgery in asymptomatic patients remains debated, requiring individualized multidisciplinary decision-making involving pediatric surgeons, pulmonologists, and neonatologists.
Long-term follow-up: Required to monitor lung function and detect late complications. Early intervention improves outcomes.
Parental Guidance
Educate families about signs of respiratory distress and infection.  
Emphasize importance of follow-up imaging and specialist evaluations.  
Support with nutritional and developmental monitoring if respiratory symptoms impact growth.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis of pediatric congenital lung malformations (CLMs) involves distinguishing among various lung anomalies that present with overlapping clinical and imaging features. CLMs encompass a spectrum of developmental lung abnormalities including congenital pulmonary airway malformation (CPAM, formerly congenital cystic adenomatoid malformation), pulmonary sequestration, bronchogenic cysts, congenital lobar emphysema, and others.
Key Congenital Lung Malformations to Differentiate
Congenital Pulmonary Airway Malformation (CPAM):
Characterized by multicystic abnormal lung tissue.
Classified into types 0 to 4 based on cyst characteristics and location.
Frequently detected antenatally on ultrasound.
May present as large multicystic masses or solid-appearing lesions.
Differential considerations within CPAM include macrocystic (large cysts, air-filled on imaging) versus microcystic lesions (appear as persistent opacities).
Pulmonary Sequestration:
Comprises nonfunctioning lung tissue that is not connected to normal bronchial tree.
Supplied by aberrant systemic arteries (often from the aorta).
Subtypes: intralobar (within lung lobe) and extralobar (separate, often pleural-covered mass).
Imaging shows mass with systemic arterial supply, often without communication with airways.
Bronchogenic Cyst:
Usually unilocular cystic lesions without communication with airways.
Typically appear as a solitary cystic lesion near the central airways.
Congenital Lobar Emphysema:
Characterized by overinflation of one lung lobe due to bronchial airway obstruction.
Appears as hyperlucent and hyperinflated lobe on imaging.
Unlike CPAM, it lacks cystic structures.
Important Differential Diagnoses Beyond CLMs
Congenital Diaphragmatic Hernia (CDH):
Can mimic cystic or solid lung lesions by herniation of abdominal contents into thorax.
Distinguished by presence of bowel gas patterns and displaced nasogastric tube position on imaging.
Essential to rule out due to significant clinical implications and different management.
Pleuropulmonary Blastoma:
A rare malignant tumor that may mimic cystic lung malformations.
Requires pathological confirmation.
Localized Congenital Cystic Bronchiectasis and Scarring:
Seen in older children with airway obstruction or infection; may appear cystic but differ in clinical context.
Diagnostic Approach
Prenatal ultrasound and fetal MRI:
Primary tools for antenatal detection.
Measurement such as CCAM volume-to-head circumference ratio (CVR) helps assess risk (CVR >1.6 predicts complications).
Ultrasound screening can detect cystic or solid lesions but may confuse CLMs with CDH.
Postnatal imaging:
Chest X-ray to identify cystic or solid lesions.
CT scan is gold standard for detailed evaluation of lesion type, size, communication to airways, and vascular supply.
Doppler imaging to identify aberrant systemic arterial supply indicating sequestration.
Other studies:
Bronchoscopy for airway assessment.
Echocardiogram to rule out associated cardiac anomalies or evaluate heart function.
OBSTRUCTIVE SLEEP APNEA
OVERVIEW
Pediatric obstructive sleep apnea is a condition in which a child's breathing is partly or completely blocked during sleep. Breathing can briefly stop and start again many times a night. The condition happens when the upper airway narrows or is blocked during sleep.
Obstructive sleep apnea can look different in children than it does in adults. Adults usually have daytime sleepiness. Children are more likely to have behavior issues, such as acting hyper or not paying attention. Risk factors also differ. In adults, the key risk factors are obesity and age. Although obesity can play a role in children, the main risk factor in children is having tonsils and adenoids that are larger than usual. The adenoids are two small pads of tissue in the back of the nose. The tonsils are two oval-shaped pads in the back of the mouth.
It's important for healthcare professionals to find and treat pediatric obstructive sleep apnea as soon as possible. Early treatment helps prevent other health conditions called complications. These can affect children's growth, learning, behavior and heart health. The first treatment may be surgery to remove enlarged tonsils and adenoids. But some children may get better using medical devices or taking medicines.
Pediatric obstructive sleep apnea (OSA) is a common, treatable condition. Obstructive sleep apnea causes breathing difficulties while sleeping. Some children do snore, only a small percentage (two percent) has obstructive sleep apnea.
Obstructive sleep apnea affects people of all ages. Children who have this condition can be harder to diagnose than older people. Children may snort or gasp while snoring and may “suck in” their chests. Their breathing starts and stops during sleep. This is caused by the throat narrowing or closing while they sleep. When breathing stops for short periods of time, it’s called apnea.
Children with pediatric obstructive sleep apnea have trouble sleeping at night. Because they’re tired, they often show behavioral problems during the day. If left undiagnosed, this condition can lead to problems at school and delayed growth. In extreme cases, it can cause heart failure. Heart failure happens when blood oxygen levels drop.
Obstructive sleep apnea syndrome (OSAS) is defined as a disorder of breathing during sleep characterized by prolonged partial airway obstruction and/or intermittent complete obstruction (obstructive apnea) that interrupts normal ventilation during sleep and normal sleep patterns. OSAS is usually considered as an extreme of "sleep-disordered breathing" spectrum encompassing primary snoring, upper airway resistance syndrome, obstructive hypoventilation, and OSAS. Sleep-disordered breathing occurs during sleep and is exacerbated by sleep. Primary snoring occurs when snoring is not associated with ventilatory abnormalities such as apnea, hypopnea, hypoxia, or hypercapnia. Upper airway resistance syndrome is characterized by increasing negative intrathoracic pressure during inspiration without apparent apneic or hypopneic events, resulting in increased respiratory arousals leading to increased sleep fragmentation and daytime sleepiness. Obstructive hypoventilation is common in obese children, and diagnosed by snoring, reduced ventilatory drive with hypercapnea without apparent sleep apneas, or respiratory arousals. Untreated sleep-disordered breathing in children has been reported to be related with various problems such as attention deficit/hyperactivity disorder, poor academic achievement, and behavioral problems. It may even cause more serious morbidities, such as growth failure, cor pulmonale, and systemic hypertension
CAUSES AND RISK FACTORS
Pediatric obstructive sleep apnea is caused by muscles in the back of the throat relaxing and blocking the upper airway. In children, this leads to pauses in breathing that last about twice as long as the typical breath.
When breathing stops, this triggers the brain to wake up so that the airway can open again. This makes it hard to get enough rest.
Various conditions can raise the risk of the upper airway becoming blocked during sleep. Commonly, enlarged tonsils in the back of the mouth and enlarged adenoids in the back of the nose can cause a blockage. Other possible causes include being born with a birth defect related to the shape of the face or head and certain health conditions.
The key causes and risk factors can be categorized as follows:
Enlarged tonsils and adenoids: These lymphatic tissues, located in the back of the throat and behind the nose, can grow large due to infections, allergies, or inflammation. When enlarged, they are the most common cause of airway obstruction in children, especially in younger kids.
Obesity: Excess body fat, particularly in obese children, contributes to fatty infiltration in the soft tissues around the throat. This narrows the airway and predisposes children to obstruction. Obesity is a significant risk factor, especially in adolescents and accounts for many cases.
Craniofacial abnormalities: Structural issues such as micrognathia (small jaw), retrognathia (receding jaw), midfacial hypoplasia, and other congenital or acquired abnormalities can reduce airway size or increase collapsibility.
Neuromuscular disorders and poor muscle tone: Conditions like Down syndrome, cerebral palsy, muscular dystrophies, and other neuromuscular diseases result in reduced muscle tone in the throat, which may lead to airway collapse during sleep.
Medications: Sedatives and opioids can relax throat muscles excessively, increasing the risk of airway obstruction.
Other factors: Allergic rhinitis causing nasal congestion, genetic syndromes such as Prader-Willi syndrome, sickle cell disease, and some rare congenital syndromes also contribute to airway obstruction
RISK FACTORS
The main risk factor for pediatric obstructive sleep apnea is enlarged tonsils and adenoids, especially in younger children. Obesity also is an important a risk factor, mainly among teenagers.
Other risk factors for pediatric sleep apnea include having:
A genetic condition such as Down syndrome or Prader-Willi syndrome.
Birth defects in the skull or face.
A group of conditions called cerebral palsy that affect movement and posture.
A group of inherited blood disorders known as sickle cell disease.
Conditions called neuromuscular disorders that affect the function of muscles due to problems with the nerves and muscles in the body.
A history of low birth weight.
A family history of obstructive sleep apnea.
Enlarged tonsils and adenoids
An abnormality in the face or jaw
Down syndrome or other congenital abnormalities
African American race
Obesity
Premature birth
Family history of OSA
Sedative medicines, which can promote snoring or slow breathing
 SIGNS AND SYMPTOMS
During sleep, symptoms of pediatric obstructive sleep apnea can include:
Snoring.
Pauses in breathing.
Restless sleep.
Snorting, gasping, coughing or choking.
Mouth breathing.
Nighttime sweating.
Bed-wetting that starts after a long period of dry overnights.
Infants and young children with obstructive sleep apnea don't always snore. They might just have disturbed sleep.
During the day, children with sleep apnea might:
Get headaches in the morning.
Breathe through the mouth or have trouble breathing through the nose.
Have trouble learning and paying attention.
Do poorly in school.
Have behavior issues such as acting hyper, impulsive or aggressive.
Have poor weight gain.
Talk about feeling sleepy, or fall asleep during school or during short car or bus rides.
The clinical presentation of a child with obstructive sleep apnea (OSA) is nonspecific and requires increased awareness by the primary care physician. OSA symptoms in children can include the following:
Abnormal breathing during sleep
Frequent awakenings or restlessness
Frequent nightmares
Enuresis
Difficulty awakening
Excessive daytime sleepiness
Hyperactivity/behavior problems
Daytime mouth breathing
Poor or irregular sleep patterns
Symptoms of pediatric obstructive sleep apnea can be hard to notice. They may include:
Sleeping in odd positions, often with the head propped up or off the bed
Snoring loudly and continuously
Losing weight or not gaining weight
Stopping breathing at night for a short period, followed by snorting and gasping or waking up
Sweating heavily during sleep
Acting out at home or school
Sleeping problems (restlessness)
Having trouble waking up even though the child should have had enough sleep
Having headaches during the day, particularly in the morning
Being irritable, aggressive and cranky
Falling asleep or daydreaming in school or at home
Wetting the bed at an unusual age
Inability to concentrate at school
Being hyperactive during the day
Pediatric obstructive sleep apnea can share symptoms with attention deficit hyperactivity disorder (ADHD). Because of this, some children with pediatric OSA are misdiagnosed as having ADHD. Some children may have both conditions. Pediatric obstructive sleep apnea can also sometimes worsen ADHD symptoms.
A wide range of symptoms and signs are associated with OSAS in children depending on their developmental stages. Snoring is the most common presenting complaint of children and adolescents with OSAS. Parents may describe chest wall retractions, paradoxical breathing, and sometimes pauses in breathing. Restless sleep with frequent changes of body position is also well described among children with OSAS, although many parents consider it a normal behavior during sleep in childhood. Excessive sweating during sleep may be related with labored breathing. Abnormal sleep positions such as hyperextension of the neck in infants and abnormal prone position may indicate the possibility of having sleep-disordered breathing. Night terror and sleepwalking are frequently accompanied by sleep-disordered breathing during slow-wave sleep in children and adolescents with a positive family history of parasomnias. Children with OSAS are high risk for enuresis, which may resolve when the OSAS is adequately treated. Excessive daytime sleepiness is a typical symptom in adolescents with OSAS, whereas hyperactivity or inattention is predominant in preadolescent children with sleep-disordered breathing. Morning headaches and poor appetite may present in OSAS, which may be due to carbon dioxide retention, sleep fragmentation, or gastroesophageal reflux.
Common Symptoms and Signs of Pediatric OSAS by Age
DIAGNOSIS METHODS
Diagnosis involves the steps that a healthcare professional takes to find out if your child has pediatric obstructive sleep apnea. A healthcare professional reviews your child's symptoms and health history and does a physical exam. Your child's healthcare professional likely will look at your child's head, neck, nose, mouth and tongue.
Other tests might be needed as well. The main test to check for sleep apnea in children is called a polysomnogram. This involves an overnight sleep test. Sensors are placed on your child's body. The sensors record brain waves, breathing patterns, snoring, oxygen levels, heart rate and muscle activity while your child sleeps. This test may take place at a sleep center.
Children and teens who snore should see a doctor to figure out if they have pediatric obstructive sleep apnea. In some cases, they may need to see a pediatric sleep specialist.
To diagnose this condition, a pediatric sleep specialist uses a test called polysomnography (PSG). This test records a child’s sleep activity for at least one night in a sleep lab. The study can determine the severity of the sleep apnea. It can also help doctors come up with the best treatment plan.
During the test, the specialist places sensors on the head and body to watch sleep patterns. The sensors record brain waves, leg and arm movements, muscle activity, heartbeat and breathing patterns. The sensors are not dangerous and are painless.
We encourage parents to stay with their children overnight in the sleep lab. This allows the parents to tell the staff if the study captured the child’s typical sleep and breathing patterns.
Diagnosis of OSAS in children is made on the basis of sleep history, physical examination, and polysomnographic findings. OSAS should be suspected on parental complaints about their children's sleep. Frequent napping or excessive sleepiness in the classroom is a major clue to sleep problems in older children. Clinicians can use a sleep log, sleep diary, or sleep questionnaire to efficiently identify the traits of a patient's sleep. Although a thorough history of sleep is very important in the diagnosis of sleep disorders, studies have shown that OSAS cannot be differentiated from primary snoring by history alone. A comprehensive physical examination of the upper airway from the nose to the oropharynx can help find any anatomical narrowing; that is, a deviated nasal septum, enlarged inferior turbinate, overcrowding of teeth, enlargement of adenoid with/without tonsillar hypertrophy, or the presence of a high and narrow hard palate. Mallampati score is helpful in evaluating the patency of airway related to tonsil size, particularly for older and obese children. An adenoid face with mouth breathing at the waking state is an important clue in detecting sleep-disordered breathing. Inspection of the lateral facial profile is helpful to evaluate for retrognathia, micrognathia, or midfacial hypoplasia.
TREATMENTS OPTIONS 
Your child's healthcare professional works with you to find the right treatment for your child's pediatric obstructive sleep apnea. Most often, the first treatment for the condition is surgery to remove enlarged tonsils and adenoids. This is called adenotonsillectomy (ad-uh-no-ton-sil-EK-tuh-me). But some children get better with medicines or medical devices.
The right treatment plan for your child depends on your child's sleep apnea symptoms and risk factors. For most children, treatment includes adenotonsillectomy, but your child's healthcare professional may recommend other treatments if this surgery isn't right for your child. Other treatments also may be needed if the surgery doesn't fully treat your child's obstructive sleep apnea.
Some kids get better without sleep apnea treatments. It's possible for some children with mild to moderate obstructive sleep apnea to outgrow the condition. A healthcare professional may recommend closely watching a child for up to six months to see if the symptoms get better. This is called watchful waiting. If the child also has allergies or other conditions that irritate the airway, watchful waiting can include treatment for those.
Medications
Topical nasal steroids might ease sleep apnea symptoms for some children with mild obstructive sleep apnea. These medicines include fluticasone (Flovent HFA, Xhance, others) and budesonide (Rhinocort, Pulmicort Flexhaler, others). For kids with allergies, montelukast (Singulair) might help relieve symptoms when used alone or with nasal steroids.
Therapies
Your child's healthcare professional may recommend use of devices such as:
Positive airway pressure therapy. Small machines gently blow air through a tube with treatments called continuous positive airway pressure (CPAP) and bilevel positive airway pressure (BPAP). The tube is attached to a mask that goes around your child's nose or nose and mouth during sleep. The machine sends air pressure into the back of your child's throat to keep your child's airway open. Often, positive airway pressure therapy is a treatment option if medicines or removal of adenoids and tonsils doesn't work.
Proper fitting of the mask and refitting as your child grows can make the mask more comfortable to wear.
Oral appliances. These devices go in the mouth. They include 
dental devices and mouthpieces. Oral appliances help to expand the roof of the mouth and nasal passages. They also might move your child's bottom jaw and tongue forward to keep the upper airway open. Only some children benefit from these devices.
Surgery or other procedures
Adenotonsillectomy to remove the tonsils and adenoids might improve obstructive sleep apnea by opening the airway. It's often a treatment option for children with moderate to severe obstructive sleep apnea. Your child's primary healthcare professional might refer you to a pediatric ear, nose and throat specialist to talk about surgery. Other forms of upper airway surgery might be recommended based on your child's condition.
Lifestyle and home remedies
You can take the following steps at home to help your child with pediatric obstructive sleep apnea:
Stay away from airway irritants and allergens. Allergens are things that cause allergies. Keep your child away from tobacco smoke and other indoor allergens or pollutants. This step is important for all children, but especially those with obstructive sleep apnea. Irritants and allergens can irritate the airway and cause congestion.
Weight loss. If your child is obese, ask your child's healthcare professional about a weight-loss plan. The healthcare professional can give you and your child information on healthy changes in diet and physical activity. You also may be referred to specialists in managing obesity because it is a complex disease. Weight-loss surgery is a treatment option for some teenagers who have obstructive sleep apnea and severe obesity.
Watchful waiting. Some children may outgrow their obstructive sleep apnea while their healthcare professionals track their health. This is especially true for kids with mild disease and no other risk factors
PREVENTION TIPS: 
Prevention tips for pediatric obstructive sleep apnea (OSA) focus largely on addressing risk factors, maintaining a healthy lifestyle, and minimizing airway irritants to reduce the occurrence or severity of the condition. The main approach is comprehensive and involves both environmental modifications and managing underlying health issues:
Avoid airway irritants and allergens: Exposure to tobacco smoke, indoor pollutants, and allergens should be minimized or eliminated as these can cause airway inflammation, leading to congestion and worsening of obstructive sleep apnea symptoms.
Weight management: Obesity is a significant risk factor for pediatric OSA, especially in teenagers. Promoting healthy eating habits and regular physical activity to maintain or achieve a healthy weight can substantially reduce the risk of OSA or alleviate mild symptoms. Structured weight-loss programs have been shown to improve sleep apnea outcomes in overweight or obese children.
Regular exercise: Encouraging consistent physical activity supports healthy weight and overall respiratory health, which helps minimize OSA risk.
Good sleep hygiene: Establishing consistent sleep schedules, creating a quiet and dark sleep environment, and avoiding stimulating activities before bedtime can improve sleep quality and help reduce OSA symptoms. A good sleep environment also involves controlling allergens and irritants near the child's sleeping area. Studies reported significant symptom improvement when sleep hygiene practices were followed.
Monitoring and early intervention: Since enlarged tonsils and adenoids are common causes in younger children, early medical evaluation and potential treatment (such as adenotonsillectomy) are important preventive and therapeutic steps. Regular check-ups are crucial to identify early signs of OSA and address other anatomical or genetic risk factors.
Avoid exposure to secondhand smoke: This is especially important for children who have nasal congestion or other respiratory conditions, as smoke aggravates airway inflammation and obstruction.
Special considerations: Children with genetic conditions (like Down syndrome or Prader-Willi syndrome), neuromuscular disorders, or craniofacial anomalies should be closely monitored and managed by specialists to prevent or address OSA due to their higher risk
PROGNOSIS:
The prognosis of pediatric obstructive sleep apnea (OSA) varies widely depending on factors such as severity, underlying causes, timely diagnosis, and treatment. OSA in children is characterized by repeated episodes of partial or complete obstruction of the upper airway during sleep, leading to disrupted sleep and intermittent hypoxia.
Key Prognostic Features and Outcomes:
Reversibility with early treatment: Many of the serious consequences of pediatric OSA, including neurobehavioral, cardiovascular, and growth impairments, are largely reversible when detected and treated early. Treatment often leads to significant clinical improvement.
Potential complications if untreated: Untreated pediatric OSA can result in:
Neurocognitive deficits such as attention deficit/hyperactivity disorder, poor academic achievement, emotional instability, and executive function impairments (e.g., behavioral inhibition, memory, problem solving).
Cardiovascular issues including mild pulmonary hypertension, systemic hypertension, and in severe cases cor pulmonale and heart failure.
Failure to thrive and delayed growth and development in younger children.
Behavioral problems and daytime sleepiness or hyperactivity.
Long-term risks: A 20-year follow-up study found that adults who had severe OSA in childhood exhibited higher body mass index, more snoring, and lower academic achievement than controls, suggesting that childhood OSA may have lasting consequences into adulthood.
Post-treatment prognosis:
Surgical removal of enlarged tonsils and adenoids (adenotonsillectomy) is the most common treatment and often leads to significant improvement or cure. However, only about 25% of children achieve complete normalization of symptoms post-surgery, highlighting that residual OSA or recurrence can occur.
Noninvasive ventilation (e.g., CPAP) is an alternative for children in whom surgery is not indicated or insufficient.
Following treatment, pulmonary hypertension and failure to thrive generally resolve, and neurobehavioral outcomes improve, although some cognitive deficits may persist.
Factors influencing prognosis:
Severity of obstruction, measured by the apnea-hypopnea index (AHI), and obesity at diagnosis are major determinants.
Younger children, those with comorbidities such as cardiopulmonary disease or craniofacial anomalies, and those with severe OSA are monitored more closely due to higher postoperative risks.
Mild cases: Some children with mild to moderate OSA may outgrow the condition as airway structures mature, possibly avoiding invasive treatments through observation and managing contributing conditions such as allergies
POSSIBLE COMPLICATIONS
Pediatric obstructive sleep apnea (OSA) can lead to a range of serious complications affecting multiple organ systems and overall development if left untreated.
Major Complications:
1. Growth and Developmental Issues
Failure to thrive and growth retardation are known consequences, likely due to disrupted sleep and increased work of breathing, particularly in infants and young children.
Developmental delays and cognitive difficulties arise due to compromised attention and executive function, influenced by sleep fragmentation, intermittent hypoxia, and hypercapnia impacting prefrontal brain areas. This can manifest as learning problems and behavioral issues such as hyperactivity and poor school performance.
2. Cardiovascular Morbidities
Pediatric OSA is associated with elevated blood pressure and systemic hypertension, influenced by increased sympathetic nervous system activity.
Pulmonary hypertension and right heart strain (cor pulmonale) may develop, especially if OSA is severe and untreated, but these conditions are usually reversible with treatment.
Endothelial dysfunction and chronic inflammation, indicated by raised C-reactive protein and adhesion molecule levels, also contribute to cardiovascular risks.
3. Metabolic and Endocrine Dysregulation
Obesity-related hypoventilation syndrome can co-occur, increasing risks of daytime hypoventilation and hypercapnia.
Children with OSA may exhibit metabolic syndrome features such as insulin resistance, dyslipidemia, and a predisposition to diabetes.
4. Neurocognitive and Behavioral Impact
Beyond executive dysfunction, OSA in children is linked to behavioral problems like inattention, irritability, and hyperactivity, which can worsen over time and impair quality of life.
Emotional regulation and memory can also be affected, interfering with academic and social performance.
5. Respiratory and Surgical Risks
Severe OSA may result in post-obstructive pulmonary edema following airway surgery and increase the risk of postoperative respiratory compromise requiring interventions such as intubation or noninvasive ventilation.
Chronic mouth breathing secondary to OSA can cause orofacial deformities, malocclusion, and poor oral health due to altered oral mechanics and increased salivary flow.
6. Other Complications
Enuresis (loss of bladder control) has been observed in some children with OSA.
Rarely, children with certain genetic syndromes suffering from severe OSA may face life-threatening complications.
WHEN TO SEE A DOCTOR
You should see a doctor for pediatric obstructive sleep apnea (OSA) if your child exhibits any signs or symptoms suggestive of this condition, especially if these symptoms are persistent or worsening. Key indications to seek medical evaluation include:
Frequent snoring, often loud, with pauses in breathing, snorts, gasps, or choking sounds during sleep.
Restless or disturbed sleep, including bedwetting that develops after a period of dryness.
Daytime symptoms such as morning headaches, mouth breathing, difficulty paying attention, learning problems, hyperactivity or behavioral issues (impulsivity, aggression), poor weight gain, excessive sleepiness, or falling asleep during school or car rides.
Because pediatric OSA can cause significant sleep disruption and lead to problems with learning, behavior, and growth, early evaluation by a healthcare professional is important if these symptoms are present.
A healthcare provider will conduct a detailed history and physical exam, considering symptoms and risk factors like enlarged tonsils or adenoids, obesity, craniofacial abnormalities, allergies, or certain medical conditions (e.g., Down syndrome).
To confirm diagnosis, children with suspected OSA usually undergo an overnight sleep study called polysomnography, which monitors breathing patterns, oxygen levels, and sleep stages. This helps determine the severity of airway obstruction and guides treatment.
Red Flag Warnings:
Cardiorespiratory failure or severe oxygen desaturation during sleep.
Significant daytime symptoms such as frequent choking or gasping episodes, persistent mouth breathing, and poor growth.
Behavioral problems, excessive daytime sleepiness, or developmental delays.
Children with complex or high-risk conditions require specialist referral without delay.
General Advice:
Adenotonsillectomy remains first-line treatment for most children with OSA caused by tonsillar hypertrophy.
Continuous positive airway pressure (CPAP) therapy is an alternative for children who are not surgical candidates or have residual OSA after surgery.
Non-invasive treatments such as night guards, palatal expanders, and anti-inflammatory medications may be useful adjuncts in selected cases.
A multidisciplinary approach involving pediatricians, pulmonologists, otolaryngologists, sleep specialists, and sometimes orthodontists improves outcomes.
Ongoing postoperative monitoring and reevaluation are important to assess treatment effectiveness and adjust management.
In mild cases, watchful waiting may be considered, while more severe or complex cases require active intervention.
In summary, early recognition, appropriate diagnosis with polysomnography, timely treatment primarily via adenotonsillectomy or CPAP, and addressing contributing factors like obesity are critical for managing pediatric obstructive sleep apnea and preventing long-term complications.
 Differential Diagnosis:
Distinguishing OSA from primary (benign) snoring is important; polysomnography (sleep study) is the gold standard diagnostic tool.
Other conditions to consider include central sleep apnea, neurological or neuromuscular disorders, and structural abnormalities of the airway such as craniofacial anomalies.
Genetic syndromes like Down syndrome or Prader-Willi syndrome often have associated airway obstruction contributing to OSA symptoms.
Comorbid conditions such as asthma or sickle cell disease may complicate diagnosis
PEDIATRIC PRIMARY CILIARY DYSKINESIA
OVERVIEW
Primary ciliary dyskinesia (PCD) affects your respiratory system and is a rare disorder. It’s due to issues with cilia, microscopic hair-like organs. Healthy cilia use wave-like motions to move cells and other substances within your body.
With primary ciliary dyskinesia, cilia may:
Be the wrong size.
Have an abnormal shape.
Be missing.
Move in an uncoordinated manner.
Not move.
What do cilia do?
Cilia line tissue in the respiratory system and other areas. Here, they help eliminate germs, waste and substances, like dust. They also play a role in the placement of developing organs in a fetis.
How can nonfunctioning cilia make me sick?
They can cause health issues beginning at birth or later in life, such as:
Abnormal organ placement: The heart, lungs or spleen may be facing the wrong direction. They may also be on the wrong side of your body.
Chronic, severe respiratory disease: Nonfunctioning cilia make it challenging to clear mucus. Instead, it builds up in your lungs and other passageways, causing infections.
Fertility issues: Men with PCD are infertile. Women may experience serious pregnancy complications, such as an ectopic pregnancy
CAUSES AND RISK FACTORS
Primary Ciliary Dyskinesia (PCD) in children is a rare genetic disorder caused by inherited mutations in genes responsible for the structure and function of cilia—microscopic, hair-like organelles that line the respiratory tract and other tissues.
Cause and Pathophysiology:
PCD is primarily caused by autosomal recessive mutations in genes encoding components of the motile cilia. The two most commonly implicated genes are DNAI1 and DNAH5, which code for proteins in the outer dynein arms crucial for ciliary beating.
Over 50 genes have been identified to date that, when mutated, disrupt ciliary structure or function, though not all genetic causes are known yet.
Cilia normally perform a coordinated, wave-like motion to clear mucus, bacteria, and debris from the respiratory tract, ears, and sinuses. In PCD, the cilia are either immotile or dyskinetic (move abnormally), leading to impaired mucociliary clearance.
This impaired clearance results in mucus accumulation, causing recurrent infections (sinusitis, otitis media, pneumonia) and chronic inflammation, which can lead to irreversible lung damage over time.
During embryonic development, specialized cilia called nodal cilia are responsible for establishing left-right body asymmetry. Dysfunction here causes randomized organ placement, leading to situs inversus totalis in about 50% of cases.
Ciliary dysfunction also affects sperm flagella, often causing male infertility due to immotile sperm.
Genetic Inheritance:
The disease usually follows an autosomal recessive inheritance pattern, meaning a child inherits two mutated gene copies (one from each carrier parent).
Rarely, X-linked or autosomal dominant inheritance may occur, but these are less common.
Being a carrier (one mutated copy) usually does not cause symptoms
RISK FACTORS
The risk factors of pediatric Primary Ciliary Dyskinesia (PCD) primarily center around its genetic etiology, with several contributing elements influencing the likelihood and severity of the disease:
Genetic Risk Factors
Inheritance pattern: PCD is mainly caused by genetic mutations inherited in an autosomal recessive manner, meaning a child must inherit faulty genes from both parents to develop the condition. Exceptions exist such as X-linked inheritance (e.g., mutations in the PIH1D3 gene) and rare cases of autosomal dominant mutations (e.g., FOXJ1 gene).
Genetic heterogeneity: More than 50 genes have been implicated in PCD, many encoding proteins necessary for ciliary structure and function, such as DNAH5 and DNAI1. Mutations disrupt ciliary motility leading to impaired mucociliary clearance.
Family history: Having a family history of PCD or related ciliopathies markedly increases risk.
Genes like CCDC40 are essential for proper ciliary motility and left-right organ patterning; mutations can cause specific PCD variants with organ laterality defects (e.g., situs inversus).
Biological and Demographic Factors
Age: PCD is typically diagnosed in childhood, often presenting in the neonatal period with respiratory distress, chronic cough, and ear infections. Diagnosis may be delayed due to symptom overlap with common illnesses.
Organ laterality defects: About 50% of affected children display situs inversus totalis (complete mirror organ arrangement), which is a hallmark of Kartagener syndrome (a PCD subset).
Gender: PCD affects males and females equally without gender predilection.
Ethnic/geographic factors: Some populations, such as those in Northern Europe, appear to have higher PCD prevalence due to genetic variations or founder mutations.
Environmental and Lifestyle Factors (Modifiers, Not Causes)
Although PCD is genetic, environmental factors such as exposure to pollutants, allergens, tobacco smoke, and recurrent respiratory infections can exacerbate symptoms or worsen respiratory outcomes.
A poor environment may increase the risk of complications like bronchiectasis but does not cause PCD itself.
Maintaining a healthy lifestyle with a balanced diet supports immune function, potentially mitigating disease severity but not the genetic risk
 SIGNS AND SYMPTOMS 
Symptoms are often present at birth and worsen over time.
PCD symptoms at birth
Congenital heart disease.
Cyst-like growths on organs, including the kidneys and pancreas.
Difficulty breathing (respiratory distress).
Heterotaxia, organs that are out of place, missing or not fully developed.
Humoral (antibody) deficiency, which can contribute to lung and sinus infections.
Lungs that cannot inflate properly (atelectasis).
Nasal congestion.
Situs inversus, organs that are a mirror image of where they should be.
Wet cough that does not go away.
Ongoing primary ciliary dyskinesia symptoms
Chronic cough.
Chronic sinusitis.
Ear infections.
Excess mucus and phlegm.
Infertility.
Fluid buildup in the brain (hydrocephalus).
Nasal polyps.
Severe pneumonia.
Upper respiratory infections.
DIAGNOSIS METHODS 
There is no single test that can confirm a PCD diagnosis. Evaluations include:
Physical exam to determine whether medical history and symptoms are consistent with primary ciliary dyskinesia.
Biopsy of tissue that contains cilia. Healthcare providers take a tissue sample from the nose or lungs and examine it under a microscope.
Genetic testing to check for mutations associated with primary ciliary dyskinesia. The majority but not all people with PCD have one of these mutations.
Other tests that may indicate a primary ciliary dyskinesia diagnosis include:
Exhaled nasal nitric oxide: A special device measures nitric oxide levels, a gas that’s present when you exhale. People with PCD have abnormally low levels.
Pulmonary function tests: These tests evaluate how well your lungs work.
Video microscopy: A healthcare provider views a sample of cilia through a microscope equipped with a high-powered video camera. Viewing video output in slow-motion enables healthcare providers to test cilia movement.
TREATMENT OPTIONS
There is no cure for PCD. But treatment can slow disease progression.
Therapies that help you eliminate mucus and fluids include:
Airway clearance: A special machine loosens mucus. There are also special coughing techniques.
Chest physical therapy: Some people wear a vest-like device that taps on their chest.
Ear tubes: Surgeons implant small tubes in the eardrums. They make it easier to clear fluid from the middle ear, lowering the likelihood of buildups.
Care may also include medications for infections and inflammation:
Antibiotics: These medications help your body fight infections. In severe cases, you may receive antibiotics through a vein in your arm (intravenously).
Azithromycin: This medication helps treat the inflammation in your lung. You take it on a daily basis.
Bronchodilators: Medications like albuterol make it easier to breathe.
Corticosteroids: These medications lessen inflammation by quieting chemical reactions in your body.
Mucus thinners: Medications you inhale to thin mucus in the airways
PREVENTION TIPS 
There are no preventative measures for primary ciliary dyskinesia (PCD) because it is an inherited disorder. However, early diagnosis and treatment can help delay its progression.
Management strategies focus on reducing symptoms, preventing complications, and improving quality of life. These strategies include:
Airway Clearance Techniques and medications help to clear mucus and lower the risk of infection. Staying hydrated and using a cool mist humidifier can also help thin mucus.
Infection Prevention Avoiding sick individuals, practicing frequent hand washing, and ensuring children receive routine immunizations, including annual flu shots and pneumococcal vaccines, are important steps. Early detection and treatment of respiratory infections can prevent severe complications.
Environmental Protection It is crucial to avoid exposure to tobacco smoke (both active and passive) and other environmental pollutants, as these can worsen lung damage.
Pulmonary Rehabilitation This program helps children learn how to prevent breathing problems and includes an aerobic exercise plan to strengthen the heart and lungs.
Regular Medical Care Consistent outpatient visits with specialists, typically two to four times annually, are recommended for monitoring and managing the condition.
PROGNOSIS 
The prognosis of pediatric Primary Ciliary Dyskinesia (PCD) is variable and depends on factors such as the severity of respiratory tract involvement, timing of diagnosis, and effectiveness of ongoing management. PCD is a rare, genetic disorder characterized by dysfunctional motile cilia, leading to impaired mucociliary clearance in the respiratory tract and other areas.
Key aspects of the prognosis:
Chronic Respiratory Morbidity: Children with PCD typically suffer from chronic upper and lower respiratory infections beginning in infancy or early childhood, which can lead to progressive lung damage such as bronchiectasis and reduced pulmonary function over time. Persistent respiratory symptoms like cough, pneumonia, and chronic otitis media are common from the neonatal period.
Lung Function Decline: Studies indicate that pulmonary function, measured by spirometry (e.g., forced expiratory volume in 1 second, FEV1), tends to decline with age in PCD patients. Longitudinal data show a gradual worsening, and cross-sectional analysis suggests this decline becomes more pronounced as patients grow older. Early diagnosis appears critical; children diagnosed before age 8 generally maintain better lung function throughout childhood compared to those diagnosed later, who have higher rates of pulmonary exacerbations and lower lung function.
Impact of Early Diagnosis and Management: While PCD symptoms often appear early in life, diagnosis is frequently delayed, sometimes until early childhood or beyond. Early and accurate diagnosis facilitates the initiation of comprehensive respiratory and otolaryngologic care, which can minimize long-term organ damage and improve outcomes. Poorly managed or undiagnosed cases tend to have worse pulmonary outcomes due to repeated infections and inflammation.
Long-term Outcomes & Quality of Life: Despite chronic symptoms, many children with PCD lead near-normal lives and some experience less problematic symptoms after the second decade of life. However, the disease can cause significant impairment in lung function and quality of life, and some individuals develop end-stage lung disease in early to mid-adulthood.
Mortality and Severe Cases: In severe cases, especially those with advanced lung disease, prognosis can be poor and may become fatal without interventions such as bilateral lung transplantation. Male infertility due to immotile sperm is a common associated problem but is not life-threatening.
No Cure, But Treatment Slows Progression: There is no cure for PCD. Treatment focuses on airway clearance, managing infections and inflammation, and preventing complications to slow disease progression. Many patients benefit from ongoing therapies and multidisciplinary monitoring. Genetic counseling may be advised for families since PCD is inherited.
In summary, the prognosis for children with PCD ranges from relatively mild disease with near-normal adult life to progressive respiratory failure in severe cases. Early diagnosis and proactive management are crucial to improving long-term pulmonary outcomes and quality of life in pediatric patients with PCD.
POSSIBLE COMPLICATIONS 
Pediatric Primary Ciliary Dyskinesia (PCD) is a genetic disorder characterized by defective ciliary function, leading to impaired mucociliary clearance and a range of respiratory and systemic complications. The possible complications in children with PCD are extensive and affect multiple organ systems, primarily the respiratory tract and ears, but also including other areas such as fertility and organ laterality.
Respiratory Complications
Chronic Respiratory Infections: Due to defective mucociliary clearance, children with PCD suffer recurrent or persistent infections involving the upper and lower airways. This includes chronic bronchitis, recurrent pneumonia, and sinusitis. Such infections can lead to ongoing inflammation and lung tissue damage.
Chronic Sinusitis: Sinonasal disease is common, with chronic sinus infections causing sinus pressure headaches, mucosal thickening, and frequent recurrences. Nasal polyps may develop in approximately 30% of cases.
Bronchiectasis: Over time, chronic infections and inflammation contribute to bronchiectasis, which is pathological dilation of the airways, predominantly in the middle and lower lung lobes. This is visualized on radiographs by peribronchial thickening, atelectasis, and air trapping.
Neonatal Respiratory Distress: Many children present early in life with respiratory distress (congestion, cough, tachypnea, hypoxia) often linked to impaired clearance of fetal lung fluid. Some may require hospitalization and oxygen support soon after birth.
Chronic Cough and Mucus Production: A daily, wet cough with increased mucus production is nearly universal, contributing to difficulty in clearing secretions and susceptibility to infections.
Ear-Related Complications (Otologic)
Recurrent Otitis Media: Frequent middle ear infections with effusion cause ear pain, hearing loss, and conductive hearing loss from thickened middle-ear fluid. This condition often requires repeated insertion of tympanostomy tubes, which may sometimes lead to chronic suppurative otitis media, otorrhea, and further complications like tympanosclerosis and cholesteatoma.
Hearing Loss: Associated conductive hearing loss is common in children due to chronic middle ear infections and effusions. Although hearing loss prevalence tends to decrease after adolescence, some patients experience persistent auditory impairment.
Other Systemic Complications
Laterality Defects and Heterotaxy: About 50% of patients with PCD have situs inversus or other organ laterality defects. PCD is also associated with an increased prevalence of heterotaxy syndromes, occasionally involving congenital heart disease.
Infertility: In older children and adults with PCD, infertility can occur due to impaired motility of spermatozoa or fallopian tube cilia, though infertility is not typically a direct pediatric complication.
Nasal Polyps and Chronic Rhinosinusitis: Beyond infections, mucosal changes such as swelling and polyposis can impair nasal function and sense of smell.
Hydrocephalus (Fluid Buildup in Brain): Though less common, some reports note hydrocephalus associated with PCD due to impaired ependymal cilia function affecting cerebrospinal fluid flow
WHEN TO SEE A DOCTOR 
When to see a doctor for pediatric Primary Ciliary Dyskinesia (PCD) is critical because early diagnosis and intervention can prevent irreversible lung damage, hearing loss, and other serious complications. PCD is a rare, inherited disorder where defective cilia impair mucociliary clearance, leading to recurrent respiratory infections and other systemic problems.
Parents and caregivers should seek medical evaluation promptly if a child exhibits two or more of the following signs and symptoms:
Newborn respiratory distress occurring within 12 to 24 hours of birth without a clear cause, sometimes requiring oxygen support.
Persistent daily wet cough or chronic cough that starts in early infancy or within the first year of life and does not improve, suggesting chronic airway problems.
Chronic nasal congestion or daily runny nose since infancy, often confused with allergies but persistent and severe.
Frequent and severe ear infections or chronic ear congestion, potentially leading to conductive hearing loss.
Recurrent sinus infections (sinusitis), bronchitis, pneumonia, or other chest infections, often recurring despite treatment.
Chronic wheezing or difficulty breathing not clearly explained by asthma or allergy, especially if unresponsive to conventional asthma treatment.
Abnormal organ placement (laterality defects) such as situs inversus totalis (mirror-image organ placement) or heterotaxy identified by imaging or clinical exam.
Neonatal respiratory problems with unclear cause; unexplained respiratory symptoms very early in life.
Other signs that should raise suspicion include digital clubbing (enlarged fingertips), abnormal fertility patterns later in life, or family history suggesting inherited ciliary dysfunction.
Because symptoms of PCD mimic many common childhood illnesses (asthma, bronchitis, recurrent ear infections), diagnosis is often delayed. If these signs and symptoms persist, worsen, or recur repeatedly despite standard treatments, it warrants evaluation by a pediatric pulmonologist or specialist experienced with PCD.
Diagnostic considerations include:
Nasal nitric oxide measurement (usually very low in PCD)
Ciliary function studies using biopsy samples under electron microscopy
Genetic testing for known PCD mutations
Chest X-rays or CT scans to evaluate for bronchiectasis or organ placement abnormalities.
In short, parents should seek medical attention when their child has persistent respiratory symptoms from birth or early infancy, especially if combined with multiple recurrent infections, abnormal organ arrangement, or poor response to usual treatments. Early diagnosis allows initiation of therapies to improve mucus clearance, treat infections aggressively, and prevent long-term complications.
Red Flag Warning
Red Flag Clinical Features in Children Suggestive of PCD
Neonatal Respiratory Distress
Respiratory distress shortly after birth in a full-term infant without an alternative explanation (e.g., transient tachypnea of the newborn) is an important early indicator, observed in over 80% of PCD cases.
This distress is often unexplained and persistent, signifying early ciliary dysfunction in clearing the airways.
Chronic, Year-Round Wet Cough and Nasal Congestion
Daily nasal congestion and chronic wet cough beginning soon after birth or in early infancy are hallmark signs reflecting ineffective mucociliary clearance.
The cough is typically productive and persistent, not resolving like common viral illnesses.
Recurrent and Chronic Respiratory Infections
Frequent episodes of pneumonia, bronchitis, and persistent rhinosinusitis are common, and bronchiectasis can develop in childhood.
Recurrent otitis media (middle ear infections) and related hearing problems are common, especially in young children, often before age 13.
Laterality Defects (Situs Inversus or Situs Ambiguus)
Approximately 50% of children with PCD have organ laterality defects, such as situs inversus totalis (mirror-image reversal of abdominal and thoracic organs) or heterotaxy.
This congenital abnormality in organ positioning alongside respiratory symptoms is a significant red flag.
Other Signs
Infertility or subfertility may be noted later in life, but this is less relevant in pediatric warning signs.
Congenital heart disease may sometimes accompany laterality defects.
Why Are These Signs of Red Flags?
These features distinguish PCD from more common childhood respiratory conditions because they reflect primary structural and functional abnormalities of cilia, rather than secondary infections or allergies.
Early recognition is crucial to avoid diagnostic delays that often occur due to symptom overlap with other conditions like asthma or cystic fibrosis.
Timely diagnosis allows for prompt management to reduce long-term lung damage and improve quality of life.
General Advice
General advice for managing pediatric Primary Ciliary Dyskinesia (PCD) focuses on multidisciplinary care aimed at preserving lung function, minimizing respiratory infections, and maintaining quality of life, as there is currently no definitive cure or gold-standard treatment. The main management principles include:
Regular multidisciplinary follow-up: Children with PCD should ideally be reviewed every 3 to 4 months by a specialist team including pulmonologists, otolaryngologists, physiotherapists, and other relevant experts to monitor lung function, detect complications early, and tailor care.
Airway clearance physiotherapy: Twice-daily chest physiotherapy to enhance mucociliary clearance is fundamental. Techniques may be intensified during respiratory exacerbations to prevent mucus stasis and bacterial colonization.
Use of mucolytic agents: Nebulized hypertonic saline (3% and possibly up to 7% depending on tolerance and age) may be used to thin airway secretions and assist clearance. Dornase alfa has also been considered in some cases, though evidence is limited.
Prompt and appropriate antibiotics: Respiratory exacerbations warrant early microbiological sampling (preferably sputum). A 2- to 3-week course of antibiotics targeting common respiratory pathogens such as Haemophilus influenzae, Streptococcus pneumoniae, and Staphylococcus aureus is recommended to reduce infection severity and frequency. Preventive antibiotic regimens may be considered in children with recurrent infections.
Avoidance of cough suppressants: Since effective cough is vital for mucus clearance, cough suppressants are generally discouraged.
Management of ear and sinus disease: Regular assessment and treatment for chronic otitis media and sinusitis are important to prevent hearing loss and further respiratory complications.
Lifestyle and psychosocial support: Encouraging healthy behaviors such as avoiding tobacco smoke and providing family counseling promote well-being and adherence to therapy. Positive outlook and family support play a key role in management.
Consideration of surgical options in select cases: Lobectomy may be an option for localized severe lung disease unresponsive to medical therapy, although this is reserved for carefully selected patients.
Patient and family education: Teaching families about the nature of PCD, its chronic course, and the importance of adherence to treatments and follow-up is essential
DIFFERENTIAL DIAGNONSIS
The differential diagnosis of pediatric Primary Ciliary Dyskinesia (PCD) involves distinguishing PCD from other respiratory and systemic conditions with overlapping clinical features, since PCD symptoms often mimic common illnesses and there is no single definitive diagnostic test. A comprehensive diagnostic approach combining clinical presentation, specialized investigations, and exclusion of other diseases is essential.
Key Conditions to Consider in the Differential Diagnosis of Pediatric PCD
Diagnostic Nuances
Situs inversus totalis or heterotaxy in about 50% of PCD patients is a key clinical clue distinguishing PCD from many other conditions with chronic respiratory symptoms.
Nasal nitric oxide (nNO) testing is markedly low in PCD and serves as a useful screening tool to exclude PCD in older children and adults, unlike most other respiratory conditions.
Electron microscopy (TEM) of respiratory cilia reveals hallmark ultrastructural defects in many—but not all—cases, distinguishing PCD from secondary ciliary dysfunction caused by infections or environmental factors.
Genetic testing confirming biallelic pathogenic variants in PCD-associated genes provides diagnostic certainty, differentiating PCD from phenocopies and related ciliopathies.
Clinical history focused on neonatal respiratory distress, chronic wet cough from infancy, recurrent otitis media, and chronic rhinosinusitis helps differentiate PCD from asthma and immunodeficiency disorders.
Clinical Importance of Differential Diagnosis
Because PCD frequently presents with chronic respiratory symptoms common to more prevalent diseases such as asthma, CF, and immunodeficiencies, misdiagnosis or delayed diagnosis is frequent. Identifying PCD early improves management focused on preventing bronchiectasis and long-term lung damage. Misdiagnosing PCD can lead to inappropriate treatments or missed interventions for fertility, hearing, and laterality anomalies.
Bronchopulmonary Dysplasia (BPD)
Clinical Terminologies & Codes
ICD-10 Code: P27.1 – Bronchopulmonary dysplasia originating in the perinatal period
SNOMED CT: 5692000 – Bronchopulmonary dysplasia
Related Terminology: Chronic lung disease of prematurity (CLD), Neonatal chronic lung disease
Disease Description/Overview:
Bronchopulmonary dysplasia (BPD) is a breathing disorder in premature infants where the infants' lungs become irritated and do not develop normally. It occurs most often in low-weight infants born more than two months early. Premature (preterm) infants who require treatment with supplemental oxygen or require long-term oxygen are at a higher risk. The alveoli that are present tend to not be mature enough to function normally. It is also more common in infants with low birth weight (LBW) and those who receive prolonged mechanical ventilation to treat respiratory distress syndrome. It results in significant morbidity and mortality. The definition of bronchopulmonary dysplasia has continued to evolve primarily due to changes in the population, such as more survivors at earlier gestational ages, and improved neonatal management including surfactant, antenatal glucocorticoid therapy, and less aggressive mechanical ventilation.
Bronchopulmonary dysplasia is also known as:
Chronic lung disease of prematurity
Chronic lung disease
Neonatal chronic lung disease
Respiratory insufficiency
BPD is often seen in infants with respiratory distress syndrome (RDS). This breathing disorder is common in babies, born too early, whose lungs have not fully grown. As many as 10,000 babies each year in the United States could develop BPD.
Bronchopulmonary dysplasia can be mild, moderate or severe. While breathing difficulties improve, babies with BPD are often in the hospital and need a lot of care. Many children struggle with illnesses, especially during the first two years of life. BPD may also cause asthma-like symptoms like cough and wheezing throughout childhood.
What are the Causes of Bronchopulmonary Dysplasia?
BPD develops because babies born too early have lungs that are not fully developed and are at risk of damage and swelling. Premature babies often need oxygen and/or other types of breathing support, such as ventilators, which can cause damage such as scarring. Some babies may get infections such as pneumonia, Congenital (present at birth) malformations of the lung, which can worsen swelling as well. The blood vessels in the lungs may also be underdeveloped, which can cause issues with the heart. All of these things can lead to BPD. Your baby’s lungs are vulnerable right after birth, and the delivery of oxygen and pressure can overstretch their fragile air sacs (alveoli). This can lead to inflammation and damage to their lung tissue over time.
Risk factors
Newborns who are especially at risk of developing bronchopulmonary dysplasia include:
Babies born more than 10 weeks early
Babies who weigh less than 2 pounds at birth
Babies with underdeveloped lungs or breathing problems
It’s rare for babies born after 32 weeks to develop BPD.
Signs and Symptoms of Bronchopulmonary Dysplasia
The pathogenesis of bronchopulmonary dysplasia (BPD) remains complex and poorly understood. BPD results from various factors that can injure small airways and that can interfere with alveolarization (alveolar septation), leading to alveolar simplification with a reduction in the overall surface area for gas exchange. The developing pulmonary microvasculature can also be injured. Alveolar and lung vascular development are intimately related, and injury to one may impair development of the other. Damage to the lung during a critical stage of lung growth can result in clinically significant pulmonary dysfunction.
Key mediators of BPD are fetal and neonatal exposures to perinatal oxidative stress. In a study that analyzed data of cord blood adducts (electrophilic addition products in blood protein) from 205 preterm infants (≤28 weeks) and 51 full-term infants in which infant plasma was collected at birth, 1 week, 1-month, and 36 weeks postmenstrual age, investigators found an increase of 51 of 105 adducts in cord and postnatal blood with BPD (small thiols, direct oxidation products, reactive aldehydes).  Moreover, there was a direct correlation of serial concentrations of several known products of oxidative stress with exposure to supplemental oxygen. The identification of oxidative stress-related exposure biomarkers may potentially aid antioxidant strategies in protecting the health of infants.
Premature birth and subsequent events (eg, exposure to oxygen, mechanical ventilation, inflammatory agents, infection) likely shifts the balance from lung development consisting of lung alveolar and vascular growth to one of premature maturation, which is associated with an arrest in development and a loss of future gas exchange area; however, alveolar maturation might facilitate gas exchange in the short-term.
While inflammation is associated with development of BPD, the role of chorioamnionitis in development of BPD after adjustment for prematurity is uncertain.  Relatively recent large studies indicate that chorioamnionitis is not associated with BPD.  However, alterations in the airway microbiome at birth have been noted in infants exposed to chorioamnionitis, and these alterations have been found to be associated with BPD. 
Signs and symptoms of bronchopulmonary dysplasia include:
Breathing that is fast or difficult
Shortness of breath
Pauses in breathing that last for a few seconds (apnea)
Nostrils flare while breathing
Grunting while breathing
Wheezing
Skin pulling in between the ribs or collar bones (retractions)
Bluish color of the skin (cyanosis) – due to low oxygen levels in the blood
How is Bronchopulmonary Dysplasia Diagnosed?
Almost half of all premature (born at 32 weeks gestation or before) and low birth weight infants (those that weigh less than 4.5 lbs.) require breathing assistance. BPD is typically not diagnosed until a baby is 28 days of age (36 weeks post-conception) and usually only if they are still requiring additional oxygen and/or continuing to show signs of respiratory distress. Other factors that are considered in making a diagnosis include:,,,
Your baby’s symptoms (Infection)
How premature your baby is
Ventilator dependence and Oxygen exposure
They may also use the following tests:
Laboratory tests
Laboratory studies used to evaluate and monitor infants with bronchopulmonary dysplasia include:
Arterial blood gas (ABG) levels: To assess for acidosis, hypercarbia, and hypoxia (with increased oxygen requirements)
Transcutaneous or end-tidal carbon dioxide levels: To evaluate trends, especially if the results are correlated with ABG levels
Pulmonary function tests
Continuously monitor oxygenation by using pulse oximetry because of frequent desaturations. In addition, routinely monitor blood pressure, as infants with bronchopulmonary dysplasia can also develop systemic hypertension.
Imaging studies
Radiologic studies used to evaluate infants with suspected bronchopulmonary dysplasia include:
Chest radiography: To determine the severity of bronchopulmonary dysplasia; to differentiate bronchopulmonary dysplasia from atelectasis, pneumonia, and air leak syndrome; to demonstrate decreased lung volumes, areas of atelectasis and hyperinflation, pulmonary edema, and pulmonary interstitial emphysema
High-resolution chest CT scanning
Chest MRI
Echocardiogram (echo) – an ultrasound test to view the heart and find out if a heart problem is causing your baby’s breathing trouble
Treatment
There is no specific cure for BPD, but treatment focuses on minimizing further lung damage and providing support for the infant’s lungs, allowing them to heal and grow. Newborns suffering from BPS are frequently treated in a hospital setting, where they can be continuously monitored. Types of drug therapies that may be used are:
Diuretics: This class of drugs helps to decrease the amount of fluid in and around the alveoli. They are usually given by mouth one to four times per day.
Bronchodilators: These medications help relax the muscles around the air passages, which makes breathing easier by widening the airway openings. They are usually given as an aerosol by a mask over the infant's face and using a nebulizer or an inhaler with a spacer.
Corticosteroids: These drugs reduce and/or prevent inflammation within the lungs. They help reduce swelling in the windpipe and decrease the amount of mucus that is produced. Like bronchodilators, they are also usually given as an aerosol with a mask, either with the use of a nebulizer or an inhaler with a spacer.
Viral immunization: Children with BPD are at increased risk for respiratory tract infections especially respiratory syncytial virus (RSV). Infants with moderate or severe BPD receive monthly injections with a medication that helps prevent the infection during the RSV season.
Cardiac medications: A few infants with BPD may require special medications that help relax the muscles around the blood vessels in the lung, allowing the blood to pass more freely and reduce the strain on the heart.
Patients with more severe disease may need oxygen for several months. They may also need some form of support with a machine that delivers pressure through the nose through special prongs or a mask like:
Mechanical ventilation: In most cases of bronchopulmonary dysplasia, respiratory distress syndrome is diagnosed and treated. The mainstay for treating RDS has been surfactant replacement with oxygen supplementation, continuous positive airway pressure (CPAP), and mechanical ventilation. The treatment necessary to recruit alveoli and prevent atelectasis in the immature lung may cause lung injury and activate the inflammatory cascade.
Trauma secondary to positive pressure ventilation (PPV) is generally referred to as barotrauma. With the relatively recent focus on a ventilation strategy involving low versus high tidal volume, some investigators have adopted the term volutrauma. Volutrauma suggests the occurrence of lung injury secondary to excessive tidal volume from PPV.
The severity of lung immaturity, the fetal milieu, and the effects of surfactant deficiency determine the need for PPV, surfactant supplementation, and resultant barotrauma or volutrauma. With severe lung immaturity, the total number of alveoli is reduced, increasing the positive pressure transmitted to distal terminal bronchioles. In the presence of surfactant deficiency, surface tension forces are increased. Some compliant alveoli may become hyperinflated, whereas other saccules with increased surface tension remain collapsed. With increasing PPV to recruit alveoli and improve gas exchange, the compliant terminal bronchiole and alveolar ducts may rupture, leaking air into the interstitium, with resultant pulmonary interstitial emphysema. The occurrence of pulmonary interstitial emphysema greatly increases the risk of bronchopulmonary dysplasia.
Many modes of ventilation and many ventilator strategies have been studied to potentially reduce lung injury, such as synchronized intermittent mechanical ventilation, high-frequency jet ventilation, and high-frequency oscillatory ventilation. Results have been mixed, although some theoretical benefits are associated with these alternative modes of ventilation. Although shorter duration of mechanical ventilation has been demonstrated in some trials of synchronized intermittent mechanical ventilation, most trials have not had a large enough sample size to demonstrate a reduction in bronchopulmonary dysplasia. Systematic reviews suggest that optimal use of conventional ventilation may be as effective as high-frequency oscillatory ventilation in improving pulmonary outcomes. Regardless of the high-frequency strategy used, avoidance of hypocarbia and optimization of alveolar recruitment may decrease the risk of bronchopulmonary dysplasia and associated neurodevelopmental abnormalities.
PPV with various forms of nasal CPAP has been reported to decrease injury to the developing lung and may reduce the development of bronchopulmonary dysplasia. In general, centers that use "gentler ventilation" with more CPAP and less intubation, surfactant, and indomethacin had the lowest rates of bronchopulmonary dysplasia.
Oxygen therapy:Oxygen can accept electrons in its outer ring to form free radicals. Oxygen free radicals can cause cell-membrane destruction, protein modification, and DNA abnormalities. Compared with fetuses, neonates live in a relatively oxygen-rich environment. Oxygen is ubiquitous and necessary for extrauterine survival. All mammals have antioxidant defenses to mitigate injury due to oxygen free radicals. However, neonates have a relative deficiency in antioxidant enzymes.
The major antioxidant enzymes in humans are superoxide dismutase, glutathione peroxidase, and catalase. Activity of antioxidant enzymes tends to increase during the last trimester of pregnancy, similar to surfactant production, alveolarization, and development of the pulmonary vasculature. Increases in alveolar size and number, surfactant production, and antioxidant enzymes prepare the fetus for transition from a relatively hypoxic intrauterine environment to a relatively hyperoxic extrauterine environment. Preterm birth exposes the neonate to high oxygen concentrations, increasing the risk of injury due to oxygen free radicals.
Animal and human studies of supplemental superoxide dismutase and catalase supplementation have shown reduced cell damage, increased survival, and possible prevention of lung injury. Evidence of oxidation of lipids and proteins has been found in neonates who develop bronchopulmonary dysplasia. Supplementation with superoxide dismutase in ventilated preterm infants with RDS substantially reduced in readmissions compared with placebo-treated control subjects. Further trials are currently under way to examine the effects of supplementation with superoxide dismutase in preterm infants at high risk for bronchopulmonary dysplasia.
Ideal oxygen saturation for term or preterm neonates of various gestational and postnatal ages has not been definitively determined. Many clinicians have adopted oxygen saturation target ranges of 90-95% following results of the SUPPORT trial [31] and more recent similar trials, which indicate an increased risk of mortality in infants with target oxygen saturation of 85-89% compared with 91-95%.
In SUPPORT, the rate of oxygen use at 36 weeks was reduced in the lower-oxygen-saturation group compared with the higher-oxygen-saturation group (P = 0.002), but the rates of bronchopulmonary dysplasia among survivors, as determined by the physiological test of oxygen saturation at 36 weeks, and the composite outcome of bronchopulmonary dysplasia or death by 36 weeks did not differ significantly between the treatment groups. A delicate balance to optimally promote neonatal pulmonary (alveolar and vascular) and retinal vascular homeostasis is noted.
In the STOP-ROP trial to reduce severe retinopathy of prematurity, oxygen saturations of more than 95% minimally affected retinopathy but increased the risk for pneumonia or bronchopulmonary dysplasia.
The normal oxygen requirement of a preterm infant is unknown. Pulmonary hypertension and cor pulmonale may result from chronic hypoxia and lead to airway remodeling in infants with severe bronchopulmonary dysplasia. Oxygen is a potent pulmonary vasodilator that stimulates the production of nitric oxide (NO). NO causes smooth muscle cells to relax by activating cyclic guanosine monophosphate. Currently, pulse oximetry is the mainstay of noninvasive monitoring of oxygenation.
Prevention
The multifactorial etiology of bronchopulmonary dysplasia complicates its prevention. Note the following:
Prenatal steroid therapy and postnatal surfactant has improved survival and mitigated the severity of bronchopulmonary dysplasia. Prevention of preterm birth and chorioamnionitis should reduce the incidence of bronchopulmonary dysplasia.
Meticulous attention to optimal oxygenation, ventilation (early extubation, increased use of CPAP), and fluid management may decrease the incidence and severity of bronchopulmonary dysplasia.
Maximizing nutritional support, careful monitoring of fluid intake, and judicious use of diuretics promote lung healing.
Evidence regarding the use of high-frequency ventilation, inhaled nitric oxide, and antioxidants (other than vitamin A) to prevent bronchopulmonary dysplasia is inconclusive.
If there is a risk of delivering your baby prematurely, your doctor may give you injections of a corticosteroid medicine that speeds up the development of the baby’s lung ability to produce surfactant. This will lower the risk of your baby developing respiratory distress syndrome, which can lead to BPD.
Prognosis
Most neonates with bronchopulmonary dysplasia ultimately survive. Note the following:
As infants, patients are at increased risk for repeated and serious pulmonary infections (eg, respiratory syncytial virus, asthma, cardiac dysfunction, and neurologic impairments.
Infants with severe bronchopulmonary dysplasia remain at high risk for pulmonary morbidity and mortality during the first 2 years of life.
Rehospitalization for impaired pulmonary function is most common during the first 2 years of life.
Hakulinen and associates found a gradual decrease in symptom frequency among children aged 6-9 years compared with infants aged 0-2 years.
In children and adults with a history of bronchopulmonary dysplasia, high-resolution chest CT reveals lung abnormalities that are directly correlated with the degree of pulmonary dysfunction.
The infant with severe bronchopulmonary dysplasia is at high risk for long-term pulmonary and neurologic sequelae.
Persistent right ventricular hypertrophy or fixed pulmonary hypertension unresponsive to oxygen supplementation is associated with a poor prognosis.
Northway followed up pediatric patients with bronchopulmonary dysplasia to adulthood and reported that patients had airway hyperreactivity, abnormal pulmonary function, and hyperinflation, as noted on chest radiography. 
Bader et al and Blayney et al found persistence of respiratory symptoms and abnormal pulmonary function in children aged 7 and 10 years. 
Your baby may experience feeding difficulties, which could lead to growth faltering. Taking your baby to all scheduled appointments is very important to address these issues sooner.
Because babies with bronchopulmonary dysplasia are born early, they can experience a delay in their developmental milestones. Most babies will catch up with various therapies, including physical therapy, speech therapy and occupational therapy. Severe cases of bronchopulmonary dysplasia can be life-threatening. But most babies survive, with their health gradually improving as they get older and gain weight.
Complications 
Most infants recover from BPD by the time they’re 5 years old. But they’re at risk of developing other health conditions, including:
Feeding difficulties
Gastroesophageal reflux disease (GERD)
Learning disabilities and disorders
Neurological disorders
Problems with hearing or vision
Pulmonary hypertension
Some children and adults who had the condition as newborns are at risk of developing certain lung complications. These include:
Health problems after leaving the hospital that involve oxygen therapy or breathing support (Asthma)
Bronchitis
Breathing problems as a child and adult (Obstructive sleep apnea)
Higher risk for colds, flu and other infections (Pneumonia)
Reactive airway disease and Trouble swallowing
Delayed growth and development, especially in the first two years after birth
Severe respiratory syncytial virus (RSV) infections
Predefined Q&A Sets
What is bronchopulmonary dysplasia (BPD) in infants?
How common is BPD among premature babies?
What causes bronchopulmonary dysplasia in neonates?
How does BPD differ from other pediatric respiratory disorders?
What are the main risk factors for developing BPD?
How does mechanical ventilation or oxygen therapy lead to BPD?
Is there a genetic component to BPD in preterm infants?
What are the typical symptoms of BPD in newborns or infants?
How does BPD present differently in mild vs severe cases?
Can a full-term infant develop BPD?
How is bronchopulmonary dysplasia diagnosed in neonates?
What role does a chest X-ray or CT scan play in diagnosing BPD?
Are there specific criteria used to classify the severity of BPD?
What are the treatment options for infants with BPD?
How is oxygen therapy used in managing BPD?
What medications (e.g., corticosteroids, diuretics, bronchodilators) are commonly used?
When is home oxygen therapy required?
How can BPD be prevented in high-risk preterm infants?
What neonatal practices reduce the risk of BPD?
Is the use of surfactant therapy effective in preventing BPD?
What is the long-term prognosis for children with BPD?
Do children outgrow bronchopulmonary dysplasia?
What complications can occur later in life (e.g., asthma, pulmonary hypertension)?
How does BPD affect growth and development?
What should parents know about caring for a child with BPD at home?
How often should children with BPD be monitored by a pediatric pulmonologist?
What signs should alert parents to seek urgent medical care?
Here are some likely questions and answer:
Q: "What causes bronchopulmonary dysplasia?"
 A: "BPD is primarily caused by lung injury in premature infants, often due to prolonged mechanical ventilation and oxygen therapy, combined with inflammation and underdeveloped lungs."
Q: "How is BPD treated?"
 A: "Treatment includes oxygen therapy, medications like bronchodilators and steroids, nutritional support, and sometimes ventilation support to help the lungs develop over time."
Q: "Can a baby recover from BPD?"
 A: "Many infants improve as their lungs grow, but severe cases may have long-term breathing issues requiring ongoing care."
Q: "Will my baby outgrow BPD?"
 A: "Most babies improve significantly as they grow, but some may need oxygen or medications longer. We’ll monitor lung function and growth closely."
Q: "Why does my baby need oxygen at home?"
 A: "Even after leaving the NICU, some babies need extra oxygen to support their growing lungs and prevent complications like pulmonary hypertension."
Q: "Is BPD the same as asthma?"
 A: "They’re different, but children with BPD may develop asthma-like symptoms. BPD results from early lung injury, not allergic reactions like asthma."
Learn as much as you can about your baby’s daily care. This will help you to identify questions early on. Talk to your doctor about BPD and become familiar with the various medications and treatments.
How severe is my child’s BPD?
What treatments do you suggest and what are the benefits and the side effects for each?
What is likely to happen in the short-term and as the baby gets older?
How long will he/she need treatment?
What things do I need to be aware of when taking my baby home to prevent complications?
How do I fit the care of my child into my daily routine?
Do I need a follow-up visit for my child and how often?
References/Sources
https://emedicine.medscape.com/article/973717-overview
https://www.cincinnatichildrens.org/health/b/bronchopulmonary-dysplasia
https://www.lung.org/lung-health-diseases/lung-disease-lookup/bronchopulmonary-dysplasia
https://my.clevelandclinic.org/health/diseases/22675-bronchopulmonary-dysplasia
https://www.nhlbi.nih.gov/health/bronchopulmonary-dysplasia
https://www.merckmanuals.com/home/children-s-health-issues/lung-and-breathing-problems-in-newborns/bronchopulmonary-dysplasia-bpd
Foreign Body Aspiration
Clinical Terminologies & Codes
ICD-10 Code: T17.9 – Foreign body in respiratory tract, part unspecified
SNOMED CT: 29725000 – Foreign body in respiratory tract
Related Terms: Airway obstruction, Choking, Aspiration of food/object
Disease Description/Overview
Foreign body aspiration, also known as pulmonary aspiration, occurs when an object is accidentally inhaled into a person’s airways. This usually results in an obstruction of a specific area of the respiratory tract, such as: 
The larynx. Also known as the voice box, the larynx is part of the throat and located in the neck
The trachea. Also known as the windpipe, the trachea connects the larynx to the bronchi
The bronchi. Two passageways, each individually known as a bronchus, which carry air from the trachea into the lungs
Foreign body aspiration can be a life-threatening emergency. An aspirated solid or semisolid object may lodge in the larynx or trachea. If the object is large enough to cause nearly complete obstruction of the airway, asphyxia may rapidly cause death. Lesser degrees of obstruction or passage of the obstructive object beyond the carina can result in less severe signs and symptoms.
Chronic debilitating symptoms with recurrent infections might occur with delayed extraction, or the patient may remain asymptomatic. The actual aspiration event can usually be identified, although it is often not immediately appreciated. The aspirated object might even escape detection. Most often, the aspirated object is food, but a broad spectrum of aspirated items has been documented over the years. Commonly retrieved objects include seeds, nuts, bone fragments, nails, small toys, coins, pins, medical instrument fragments, and dental appliances.
Geographic differences in the spectrum of objects commonly found in a particular environment and variations in dietary and eating habits affect the relative frequency with which various objects are aspirated.
Acute choking, with respiratory failure associated with tracheal or laryngeal foreign body obstruction, may be successfully treated at the scene with the Heimlich maneuver, back blows, and abdominal thrusts. Even in nonemergency situations, expeditious removal of tracheobronchial foreign bodies is recommended.
Causes
A foreign body aspiration is caused when a person inhales a foreign object into their airways. This is most often done by accident, usually while eating or when a non-edible object is placed in the mouth.Children are at risk for putting small toys, candies, or nuts into their mouths. Children aged 1-3 years chew incompletely with incisors before their molars erupt, and objects or fragments may be propelled posteriorly, triggering a reflex inhalation.
Food is the most frequently aspirated type of object, with nuts and seeds being particularly common. Raisins, grapes and sweets are other food items which are often seen aspirated. Liquids, such as water, can also be inhaled into the airways. In addition to edible objects, other non-edible items which are commonly retrieved from the airways include:
Balloons can be particularly dangerous when inhaled and account for roughly 29 percent of aspiration deaths in children, Other small toys, such as marbles, Coins, Pins, Dental appliances, such as crowns, fillings and orthodontic retainers.
Among adults, the following conditions, actions, and procedures facilitate foreign body aspiration:
Impaired swallow reflex
Impaired cough reflex
Intellectual disability
Alcohol or sedative use
General anesthesia
Poor dentition
Dental, pharyngeal, or airway procedures
Altered sensorium
Loss of consciousness
Convulsions
Maxillofacial trauma
Risk factors 
Age is a primary risk factor for foreign body aspiration, and approximately 80 percent of instances occur in young children under the age of three years old.  This is due to a number of reasons, including:   
Children are much more likely to place foreign objects in their mouth
Young children may not yet have their molar teeth, which can result in a reduced ability to chew their food down sufficiently
Young children usually have slightly smaller airways than adults, making them more prone to obstruction
However, foreign body aspiration can occur at any age. According to one study, the median age for foreign body aspiration in adults is 60 years old.  In adults, foreign body aspiration usually occurs while eating. 
Other risk factors for foreign body aspiration include:  
Wearing oral appliances, such as an orthodontic retainer or mouthpiece
Intoxication
Sedation
Neurological disorders, such as dementia or Parkinson’s disease
Psychiatric disorders, such as schizophrenia or bipolar disorder. In these cases, foreign body aspiration may be a deliberate act, although the motivation behind it is often unclear 
Eating high-risk foods, such as nuts, seeds, sweets and small fruits
Medical conditions or procedures that may impair the swallowing reflex, such as throat surgery 
Infants and children
Behavioral factors
Oral exploratory habits
Inability to distinguish between edible/inedible objects
Tendency to perform other activities with objects in their mouth
Anatomical factors
Immature swallowing coordination
Lack of dentition and decreased ability to chew food
Small diameter airway
Less ability to generate forced air through airway and relieve obstruction
Commonly aspirated objects
Round, ovoid, cylindrical shapes with a diameter similar to child’s airway
Most common aspirated foreign bodies include:
Organic objects
Hotdogs (most common food associated with fatal choking among children)
Peanuts
Popcorn
Candy
Seeds
Bones
Inorganic objects
Coins
Toy parts (balloons most commonly associated with fatal choking)
Crayons or pen tops
Tacks, pins, nails, or screws
Bullets and casings
Symptoms:
Near-total obstruction of the larynx or trachea can cause immediate asphyxia and death. Should the object pass beyond the carina, its location would depend on the patient's age and physical position at the time of the aspiration. Because the angles made by the mainstem bronchi with the trachea are identical until age 15 years, foreign bodies are found on either side with equal frequency in persons in this age group. [3] With normal growth and development, the adult right and left mainstem bronchi diverge from the trachea with very different angles, with the right mainstem bronchus being more acute and therefore making a relatively straight path from larynx to bronchus. Objects that descend beyond the trachea are more often found in the right endobronchial tree than in the left.
In the series reported by Debeljak et al, 42 foreign bodies were in the right endobronchial tree, 20 were in the left, and 1 was in the trachea. [4] Once aspirated, objects may subsequently change position or migrate distally, particularly after unsuccessful attempts to remove the object or if the object fragments. The object itself might cause obstruction. Vegetable material may swell over hours or days, worsening the obstruction. Cough, wheeze, stridor, dyspnea, cyanosis, and even asphyxia might ensue.
Most foreign bodies are spontaneously expelled through protective reflexes such as coughing and spitting
Foreign body aspiration may include several clinical phases
Initial stage or impaction phase occurs when a foreign body partially or fully obstructs the airway often resulting in reflexive choking, gagging, and paroxysms of coughing to relieve the obstruction
Second stage or asymptomatic phase may occur hours to weeks following aspiration event, when muscle reflexes fatigue and foreign body becomes lodged in airway
Third stage or complications stage may occur if foreign body impaction causes obstruction, erosion, and/or infection
80%-90% of foreign bodies in the airway are located in the bronchus
Organic material may cause a severe mucosal inflammatory reaction and development of granulation tissue within hours of aspiration
Materials that may absorb water such as beans, seeds, and corn may swell after aspiration and cause more severe airway obstruction
Sudden onset coughing, choking, or gagging
Wheezing, especially unilateral
Stridor (in upper airway obstruction)
Decreased breath sounds on the affected side
Persistent cough or recurrent pneumonia
Cyanosis (in severe obstruction)
Where in the airways the object has settled. Most foreign bodies settle in the bronchi, which is considered a lower airway obstruction. Obstructions higher up in the airways, such as in the larynx or trachea, are typically more severe
The size and nature of the foreign body inhaled. Whether it is big or small, sharp or blunt, hard or soft
The time since the object was inhaled. Whether the aspiration has only just taken place, if it occurred within the last few days or weeks, or further in the past
Any symptoms experienced will typically occur immediately after the foreign body has been inhaled. If the blockage to the airways is significant enough, symptoms may occur in quick succession, become severe and can eventually lead to loss of consciousness and even death, unless the object is dislodged. 
In milder cases, where the obstruction is less significant, the symptoms experienced may be less severe.  However, anyone who is displaying immediate symptoms of having inhaled a foreign body should still seek emergency help.
How to tell the severity of a foreign body aspiration
If the affected person is displaying immediate choking symptoms, it can be useful to distinguish between the signs of a mild or significant obstruction.
If the person is experiencing a mild obstruction, they are often able to: Breathe, Talk, Cough, Be responsive and answer questions
More severe obstructions in the airways may be indicated by:
- An inability to breathe
- An inability to speak or properly vocalise
- Coughs that are particularly quiet or silent
- Blushing tinge to the skin
- Loss of consciousness
Making the Diagnosis
The diagnosis of foreign body aspiration involves:
History and physical examination: The healthcare provider will evaluate the patient's symptoms, medical history, and perform a physical examination, including listening for abnormal lung sounds.
Imaging studies: Imaging tests such as chest X-ray, computed tomography (CT) scan, or bronchoscopy may be performed to visualize the presence and location of the foreign body.
Diagnosis usually presumed based on choking or coughing in otherwise healthy children and recent history of eating/playing with small objects
Testing to confirm suspected foreign body aspiration
Radiograph
Obtain posteroanterior and lateral chest x-rays 
May not detect radiolucent foreign bodies or any foreign body within 24 hours of aspiration
Findings may include
Atelectasis
Air trapping
Pulmonary infiltrates
Mediastinal shift
Presence of double contour image which may suggest button battery foreign body (batteries require urgent removal)
Rarely, chest computed tomography (CT) and magnetic resonance imaging (MRI) may also be used
Bronchoscopy may be used for both diagnosis and treatment
Most definitive method for diagnosis 
Rigid bronchoscopy under general anesthesia is recommended method in children
Flexible and fiberoptic bronchoscopy is more often used in adults
Diagnosis may also be made after successful removal of foreign body by Heimlich maneuver or by endoscopy
Rapid diagnosis is extremely important for foreign body aspiration, because a significant obstruction in the airways that is restricting breathing can quickly become life-threatening. After assessing the symptoms, if a doctor suspects a significant airway obstruction, emergency treatment will likely be sought to remove the object, without any further need for imaging tests. However, if the affected person is stable and displaying symptoms of a mild or historic foreign body aspiration, further tests may be recommended before treatment.
Physical examination
A doctor might choose to perform a physical examination first, which can include:  
Checking breathing function
Checking speech and voice function
Checking physical appearance, including for signs of any bluish tinge to the skin
Vital signs tests, such as pulse, blood pressure and temperature readings
Pulse oximetry, a non-invasive test which monitors the levels of oxygen in the blood
Imaging tests
If foreign body aspiration is still suspected, a doctor may recommend an imaging test in order to gain an internal view of the body. An X-ray is usually the first imaging test suggested.  This non-invasive method uses electromagnetic radiation and can often detect the presence of foreign bodies in the airways. However, not all objects are visible on radiograph tests such as X-rays.
If an X-ray is inconclusive, a CT scan may then be recommended.  This test is also non-invasive and uses powerful X-rays to generate a more detailed image of the inside of the body.
As well as detecting the presence of a foreign body in the airways, imaging tests may also be able to identify certain possible complications of the aspiration, such as any collapse of a lung. 
Diagnostic bronchoscopy
If imaging tests are inconclusive, a bronchoscopy might be suggested to help diagnose a foreign body aspiration. During a bronchoscopy, a long, thin tube called a bronchoscope is typically inserted through the mouth to gain an internal view of the airways. The bronchoscope has a light source and camera on one end, which allows the doctor to see areas such as the larynx, trachea and bronchi up close. 
In the case of foreign body aspiration, a bronchoscopy can either be used diagnostically, to confirm the presence and location of a foreign body in the airways, or as a treatment method to physically remove the item.
Treatment of Foreign Body Aspiration
The treatment approach for foreign body aspiration depends on various factors, including the type, size, and location of the foreign object or substance. Treatment options may include:
Observation and supportive care: In some cases, smaller or less obstructive foreign bodies may be managed with observation and supportive care. The healthcare provider will closely monitor the patient's symptoms and provide interventions as needed.
Removal by bronchoscopy: Bronchoscopy is a procedure in which a flexible tube with a camera is inserted into the airway to visualize and remove the foreign body. It allows for direct visualization and precise removal under controlled conditions.
Surgical intervention: In complex cases or when other methods are unsuccessful, surgical intervention may be necessary to remove the foreign body. This typically involves a more invasive procedure, such as a thoracotomy or bronchotomy.
Treatment of foreign body aspiration involves managing the person's ability to breathe and removing the foreign body. It is extremely important to respond quickly if the affected person has a significant obstruction that is affecting their ability to breathe, as this can quickly become life-threatening.
Emergency first aid treatment for choking
If an airway obstruction is suspected, it is important to deliver first aid quickly. Both mild and severe choking can be successfully treated at the scene, and this should take precedence over immediately calling for emergency medical care.  
When someone is displaying signs of choking, the following steps can be used to help remove the object:   
Encourage the person to keep coughing. If the obstruction is mild, they are usually able to cough and clear the blockage themselves.
Back blows. If the person is unable to cough, or coughing is unsuccessful, lean the person slightly forward and give up to five sharp blows to their back, between the shoulder blades. Deliver these blows with the heel of one hand, while supporting the person’s chest with the other hand.
Abdominal thrusts/Heimlich Maneuver. If back blows are unsuccessful, give up to five abdominal thrusts. Stand behind and slightly to the side of the person and wrap both arms around their waist. Clench a fist with one hand, cover it with the other hand, and pull sharply inwards and upwards just above their navel.
If the above measures are unsuccessful, call for emergency medical help and then continue alternating between back blows and abdominal thrusts until assistance arrives. If the person becomes unconscious, lay them on a flat surface and begin to deliver cardiopulmonary resuscitation (CPR), even if a pulse is present.
Emergency medical treatment for choking
If further attempts at first aid are unsuccessful and once medical help arrives, an emergency endotracheal intubation may be attempted. This process involves passing a flexible tube, known as an endotracheal tube, through the person’s mouth and into the airways. The tube can assist in opening the airways to provide oxygen and can also be used to remove blockages.  
In the most severe cases of choking, an emergency tracheotomy may be performed. A tracheotomy involves creating a small opening at the front of the neck. A tube is then inserted through this opening into the trachea, helping the person to breathe.  
Treatment for non-emergency foreign body aspiration
People who have milder cases of foreign body aspiration may not require emergency medical treatment. However, prompt treatment is usually still necessary, because a foreign body in the airways can quickly cause other health complications, such as a respiratory infection like pneumonia.
Bronchoscopy treatment
Removal of the foreign body during a bronchoscopy is a common, and usually successful, treatment method for inhaled objects located in the trachea or bronchi. A bronchoscope is a long, thin tube with a camera and light source on one end. This tube can be flexible or rigid and is inserted into a person’s airways to access and look into the respiratory tract. A bronchoscopy is typically performed under general anesthesia, although local anesthesia and/or sedatives may be used for more simple procedures. 
As well as helping to locate the foreign body, certain surgical instruments, such as forceps or a suction pad can be attached to the bronchoscope to enable removal of the object. Once the item is removed, the doctor will usually return the bronchoscope to the airway to ensure no fragments of the foreign body remain. 
If the object is located in the larynx, a similar treatment method known as a laryngoscopy may be used instead. 
Surgery
Surgical removal is rarely necessary for foreign body aspiration.  However, if the inhaled item is particularly large, sharp or difficult to remove, surgery may be required. 
A tracheotomy is a surgical procedure that involves making a small opening in the front of a person’s neck, known as a tracheostomy, in order to access the trachea. Removal of an aspirated foreign body located in this region can then be attempted through the surgical opening. Tracheotomies are usually performed under general anesthetic, meaning the patient is unconscious.  
A thoracotomy is a surgical procedure that involves making an incision between a person’s ribs, in order to open the chest and access the lungs. This is also performed under general anesthesia and is usually only suggested if the foreign body is located in the bronchi, and bronchoscopic attempts to remove the foreign body have been unsuccessful.   
Endoscopic removal by bronchoscopy indicated in cases of suspected foreign body aspiration
If patient is not in respiratory distress, they should fast prior to procedure to prevent aspiration
Perform procedure under general anesthesia
In children, a rigid bronchoscope with ventilation recommended to perform extraction
Tracheotomy and bronchotomy may be indicated in emergency situations such as
Complete airway obstruction
Suspected battery ingestion
Failed attempts at endoscopic removal
Postoperative management
Antibiotic and steroids may be indicated for patients with significant airway damage
Chest physical therapy may be used to clear secretions in patients with chronic pneumonia, atelectasis, or purulent bronchitis
Routine follow-up chest x-ray not needed unless symptoms persist/progress
Medication
Medication is typically not prescribed to treat foreign body aspirations. However, antibiotics may be prescribed to target any bacterial infections that arise as a result of the condition. 
Prevention
Because foreign body aspiration occurs most commonly in children under the age of three, it is important to educate caregivers about the condition, so they can help prevent the children in their care from inhaling small objects. Preventive suggestions include:    
Keep small objects that may be a choking hazard, such as coins, buttons and marbles, away from children
Teach children not to place foreign objects in their mouth, nose or other body openings
Avoid giving children under three years of age high-risk foods, such as nuts, seeds, small fruits and sweets
Avoid talking, laughing or playing while eating
Avoid running or exercising while eating
Although common in children, foreign body inhalation can occur to anyone at any age. Avoid putting non-edible objects into the mouth, and always take care to eat slowly.
Prevention of choking in children should include education for parents and caretakers on how to
Avoid high-risk objects and foods
Check for warning labels and recalls on children’s toys
Recognize acute pulmonary obstruction
Perform cardiopulmonary resuscitation or choking first aid
Food safety education for parents and caregivers regarding selection, processing, and supervising appropriate foods for children < 3 years old to make them safer for this highest-risk population
Prognosis
According to the National Safety Council, choking remained the fourth leading cause of unintentional injury death in the United States as of 2021. In 2021, a total of 5325 deaths from unintentional ingestion or inhalation of food or other objects resulting in airway obstruction was reported.  Death from choking is more common among the elderly. Living alone, having dentures or difficulty swallowing increases the risk of choking in older adults.  The overall risk of death from choking is estimated to be 1.6 deaths per 100,000 people. Even if the patient does not die, symptoms often develop immediately. Morbidity increases if extraction of the object is delayed beyond 24 hours.
Complications of foreign body aspiration
In the most severe cases of foreign body aspiration, the inhaled object can cause choking, and impaired breathing function. Unless the object is urgently removed, the condition may become fatal. If a person is suspected to be choking, urgent treatment such as back blows and abdominal thrusts are required to help remove the foreign body and prevent any further complications. 
For foreign bodies that remain in the airways, urgent removal is usually necessary as most complications arise as a result of delayed diagnosis. Approximately 67 percent of people with a foreign body obstruction in the larynx or trachea who do not undergo removal within 24 hours experience complications. 
The appearance and severity of any complications depend on the size, nature and location of the foreign object inhaled.
Aspiration Pneumonia
Pneumonia is a potentially severe infection that causes swelling to the tissue in one or both of the lungs. There are various types of pneumonia, and the term <em>aspiration pneumonia</em> specifically relates to bacterial pneumonia caused by breathing food or stomach contents into the lungs. 
The most common symptom of pneumonia is a wet cough that often produces mucus. Other symptoms can include shortness of breath, chest pain and a fever. Aspiration pneumonia is usually treated with antibiotics. 
Aspiration Pneumonitis
Aspiration pneumonitis is a chemical injury to the lungs that occurs when food, stomach contents or a foreign body is breathed all the way into the lungs. Various substances, including gastric acid, can cause a chemical burn to the airways and/or lungs, resulting in swelling and symptoms such as sudden shortness of breath and coughing. A fever may also be present.  If severe, aspiration pneumonitis can potentially lead to acute respiratory distress syndrome. 
Because the injury is chemical rather than bacterial, antibiotics are usually not effective at treating aspiration pneumonitis. Instead, doctors may suggest oxygen therapy, assistance from a breathing machine or clearing the foreign body from the airways using a bronchoscope. 
Other complications of foreign body aspiration
Other potential complications that can occur as a result of foreign body aspiration include:     
Atelectasis, where the air sacs in the lung, also known as alveoli, collapse and cannot expand properly
Pneumothorax, where air accumulates in the area between the lung and chest wall, causing a partial or full collapse of the lung. This condition is sometimes known as a collapsed lung
Pneumomediastinum, which is the abnormal presence of air in the mediastinum, the space between the two lungs
Bronchiectasis, a long-term condition that leads to excess mucus in the lungs
Lung abscess, a pus-filled cavity in the lungs surrounded by inflamed tissue, often caused by a bacterial infection
Emphysema, a lung condition that can cause shortness of breath
Brain damage, resulting from lack of oxygen to the brain
Injury to the abdomen and ribs is sometimes caused by the delivery of abdominal thrusts. People who receive abdominal thrusts may require further examination by a doctor to rule out any internal injuries, such as rib fractures or tears to the stomach. 
 Predefined Q&A Sets
What is foreign body aspiration in children?
Why are children more prone to aspirating foreign bodies than adults?
At what age is foreign body aspiration most common?
Are certain foods more likely to cause aspiration in young children?
What behavioral or developmental factors increase the risk of aspiration?
What are the early signs and symptoms of foreign body aspiration in a child?
Can foreign body aspiration present without choking or coughing?
How does partial airway obstruction differ from complete obstruction in symptoms?
What are the delayed symptoms that may suggest an undiagnosed aspiration?
What should be done immediately if a child is choking on a foreign object?
How do you perform back blows and chest thrusts on an infant with airway obstruction?
What is the role of the Heimlich maneuver in children?
How is foreign body aspiration diagnosed in children?
What imaging tests are used to detect aspirated foreign bodies (e.g., X-ray, CT scan)?
Can a chest X-ray be normal even if a foreign body is present?
When is bronchoscopy indicated for suspected FBA?
How is an aspirated foreign body removed from a child’s airway?
What are the complications of delayed removal of an aspirated object?
Can FBA lead to long-term lung damage or infection?
What steps can parents take to prevent foreign body aspiration in children?
At what age should parents begin avoiding foods like grapes, nuts, or popcorn?
What toys or household items pose the highest choking risk?
What is the prognosis for a child after successful foreign body removal?
How long should a child be monitored after aspiration?
Are antibiotics required after foreign body aspiration?
Here are some likely questions and answers:
Q: What are the main symptoms of foreign body aspiration?
A: When a person experiences an obstructed airway due to inhaling a foreign object, they may experience some or all of the following symptoms:
- Choking
- Coughing
- Difficulty breathing and/or abnormal breath sounds such as wheezing
- Difficulty speaking
- Bluish tinge to the skin
The severity of the symptoms depends on the size, nature and location of the inhaled object. In mild cases, the affected person may move into an asymptomatic phase, where no symptoms are present despite the object still being settled in the airways. However, this stage is usually temporary, and symptoms that may arise after a period of time include recurrent coughing, fever and difficulty swallowing.
Some questions and possible answers:
Q: Can a foreign body be aspirated into the lungs?
A: Yes. An object inhaled into the airways can cause an obstruction in the bronchi, which are the two passageways that deliver air into the lungs. Foreign bodies can also become lodged in the larynx/voice box and the trachea/windpipe.
Q: What treatment options are there for removal of a foreign body?
A: The treatment method used to remove a foreign body depends on the object’s size, nature and exact location in the respiratory tract. First aid can often be administered during a choking episode, by encouraging the affected person to cough or by delivering back blows and abdominal thrusts.
If medical help is required to remove the foreign body, the most common treatment method is a bronchoscopy. This is usually performed under general anesthetic and entails a long, thin tube called a bronchoscope, being inserted into the airways to access the respiratory tract and remove the foreign body. In rare cases, surgery may be necessary.
Q: What are the after effects of choking?
A: Choking is a serious and potentially life-threatening condition that requires immediate intervention. However, if the foreign object is successfully removed from the airways, usually no further medical attention is needed. If any signs of further irritation do occur, such as persistent coughing, pain or discomfort, medical attention should be sought.
Q: Does foreign body aspiration happen to both children and adults?
A: Yes, anyone at any age can accidentally inhale a foreign body into the airways. However, the condition is most common in children under the age of three years old.
Q: What is the difference between foreign body aspiration and foreign body ingestion?
A: Foreign body aspiration occurs when a foreign body is inhaled into the airways, whereas a foreign body ingestion occurs when a foreign body is swallowed into the digestive tract. The two conditions have completely different symptoms, treatments and complications. 
Pediatric Tuberculosis (TB)
Clinical Terminologies & Codes for Common Respiratory Diseases
ICD-10 Code: A15–A19 – Respiratory tuberculosis
SNOMED CT: 56717001 – Tuberculosis
MeSH: D014376 – Tuberculosis
LOINC: 5792-7 (Tuberculosis NAA with probe detection)
Disease Description/Overview
Tuberculosis (TB) is an ancient infectious disease with many varied presentations. Although prevention and treatment are available, between 8 and 10 million people still develop TB, and 2 to 3 million people die from it globally, according to the 2022 estimates from the World Health Organization (WHO). For years, children have been thought to contribute little to the global epidemic as they are rarely infectious, and they often clear the infection without treatment. However, without testing and treatment for those children with exposure, a potential reservoir is created from which new future cases will develop, and global efforts at control and eradication will fail. Significant gaps are present between the recognition of children at risk for acquiring TB and their subsequent diagnosis and management. Children, especially those younger than 5, develop TB more readily and in more severe forms than older children and adults. According to the most recent WHO data for children younger than 15, 1.3 million cases of TB were diagnosed, and nearly a quarter million TB-related deaths were reported.
The developed world has seen some improvement in diagnosing and preventing TB cases, but no evidence of a decline in high-burden nations has been seen.  In fact, high-burden countries have seen significant increases in not only infection and disease but also major increases in multidrug-resistant TB. Poverty, lack of access to adequate medical care, malnutrition, and concurrent infections such as human immunodeficiency virus continue to fuel the infection rates in high-burden countries and pockets throughout the developed world. Pediatric TB case rates are likely underreported in many high-burden countries, and study results have suggested that children may represent as many as 50% of TB cases worldwideMuch work is needed in the diagnosis and management of pediatric TB, as well as addressing the underlying social causes that create the perfect environment for the transmission of TB and the comorbidities that facilitate worse outcomes following infection.
Tuberculosis (TB) is an ongoing (chronic) infection caused by bacteria. It usually infects the lungs. But other organs such as the kidneys, spine, or brain may be affected. TB is most often spread through droplets breathed or coughed into the air. A child can be infected with the TB bacteria and not have active disease.
The stages of TB are:
- Exposure: This occurs when a child has been in contact with a person who may have or does have TB. The child will have a negative skin test, a normal chest X-ray, and no symptoms.
- Latent TB infection: This occurs when a child has TB bacteria in his or her body, but does not have symptoms. The infected child’s immune system causes the TB bacteria to be inactive. For most people who are infected, the TB will be latent for life. This child would have a positive skin test but a normal chest X-ray. He or she can't spread the infection to others.
- TB disease: This is when a child has signs and symptoms of an active infection. This child would have a positive skin test and a positive chest X-ray. He or she can spread the disease if untreated.
Inactive TB
TB germs can live in the body without making you sick. This is called inactive TB, or latent TB infection. Children with inactive TB are infected with TB germs, but they do not have active TB disease. They do not feel sick, do not have any symptoms of TB disease, and cannot spread TB to others. Children diagnosed with inactive TB can take medicine to prevent the development of TB disease.
Active TB disease
TB germs become active if the immune system can't stop them from growing. When TB germs are active (multiplying in your body), this is called active TB disease. Children with active TB disease feel sick. They may also be able to spread the germs to people they spend time with every day.
Once infected with TB germs, children, especially children younger than 5 years of age, are more likely to get sick with active TB disease and to get sick more quickly than adults.
Infants and young children are more likely than older children and adults to develop severe forms of TB disease [such as TB disease of the brain (TB meningitis) or miliary TB disease (TB disease in multiple parts of the body)]. Without treatment, TB disease can be fatal. Children diagnosed with active TB disease can take medicine to treat the disease.
TB has a wide variety of presentations in children and can produce disease in nearly all organ systems. Most TB disease in children takes a pulmonary form, with only approximately 20% manifesting as extrathoracic disease. An overlap is often seen between pulmonary disease and extrapulmonary manifestations, especially in children younger than 2, primarily including the following areas:
Intrathoracic
Pulmonary
Pleural
Cardiac (1% to 4%)
Extrathoracic
Disseminated (lymphohematogenous)
Lymphatic
Central nervous system
Osteoarticular
Abdominal and gastrointestinal
Genitourinary
Cutaneous
Congenital 
Other
Intrathoracic Manifestations
Pulmonary disease
The pulmonary system is the most affected in children with TB. After exposure, silent pulmonary infection without any obvious signs, symptoms, or imaging abnormalities ensues for many children. Some may have a prodrome consisting of several days of low-grade fever and cough resembling a viral illness, but these symptoms will resolve over several weeks. Ninety percent of newly infected older children will have an asymptomatic infection, whereas up to 50% of infected infants develop respiratory or constitutional symptoms with radiographic abnormalities during primary infection. Clinicians must be aware of the age discrepancy in developing progressive severe disease.
Primary pulmonary infection in children includes infection of the lung tissue coupled with hyperplasia of regional lymph nodes, called the primary pulmonary complex. Unlike adults, all lung lobes are equally affected in children, and a quarter of the cases can have multiple parenchymal foci. Radiographic findings may note disproportionately enlarged regional lymph nodes compared with little to no parenchymal focus (see Image. Hilar Adenopathy).
Radiographic changes often resolve quickly, but in some children, particularly those younger than 2, lymph nodes will continue to enlarge, resulting in partial or complete bronchial obstruction from external compression. Such children may present with wheezing and shortness of breath due to the small caliber of their airways, similar to a foreign body aspiration or other obstructive disorders. Chest radiographs may demonstrate localized hyperinflation with atelectasis of contiguous parenchyma areas known as segmental lesions (see Image. Lymph Node Disease With Airway Compression). Clinicians and radiologists must know that this finding is not typical of other types of bacterial pneumonia but can be M tuberculosis-related. Adolescents are frequently more symptomatic from pulmonary TB disease than school-aged children, especially when the disease is acquired near puberty. Adolescents may also present with reactivation disease like that seen in adults, with a classical picture consisting of progressive fatigue, loss of appetite, night sweats, weight loss, and fevers.Chest pain and productive or nonproductive cough may occasionally be accompanied by hemoptysis. Physical findings may be absent, but chest radiographs demonstrate extensive upper lobe infiltrates and possibly even cavities (see Image. Adult-Type Cavitary Tuberculosis Disease). Fortunately, M tuberculosis is localized well by the immune system if the teen is not immunocompromised, and anti-tuberculous treatment clears the signs and symptoms of infection rapidly if diagnosed and treated promptly.
Pleural disease
Occasionally, pleural disease is seen in pediatric TB and may happen without segmental or miliary lesions in the lung parenchyma. This occurs when bacilli form a subpleural focus and erode into the pleural space. A hypersensitivity response ensues, stimulating the formation of a pleural effusion. Due to the brisk immune response, these collections may become large enough to be associated with abrupt onset of fever, chest pain, and shortness of breath with rapid respiratory compromise depending on the child's size and relative pleural effusion size. The physical examination will reveal dullness to percussion and diminished breath sounds on the affected side. Despite appropriate treatment, fevers are often very high and may persist for several weeks. Microbiologic confirmation is often difficult due to low numbers of bacteria.Pleural biopsy may demonstrate granuloma formation but requires surgical intervention or a fine needle biopsy. Newer techniques, such as polymerase chain reaction, may prove useful in such situations, but TB detection rates still fall below 50%. Because true empyema rarely forms, it must be considered in the differential diagnosis of any culture-negative pleural specimen, especially in high-burden countries.
Cardiac disease
Infection involving the cardiac system is unusual, occurring in less than 4% of all cases of pediatric TB.The most common site of infection is pericardial tissue, which results in pericarditis and pericardial effusion. The presentation of pericardial involvement is similar to other forms of pericarditis with symptoms of fever, malaise, and weight loss; children are less likely to complain of chest pain than adults with TB pericarditis. Generally, other organ systems are involved, and these features may overshadow the pericardial disease. A physical examination may demonstrate a pericardial friction rub, distant heart sounds, and pulsus paradoxus, especially if the disease has progressed to the fibrinous stage. Chest radiography reveals a large globular heart, and ultrasonography demonstrates fluid collection in the pericardial space. When aspirated, pericardial fluid is serofibrinous or mildly hemorrhagic but rarely is acid-fast bacillus smear positive. Nonetheless, cultures are positive in 30% to 70% of cases, and a biopsy of the pericardium reveals caseating granulomas in 50% to 75% of the cases. Without early recognition and treatment of this condition, constrictive pericarditis from fibrosis over months to years will develop. In contrast to the outcomes of TB pericardial disease in adults, results from a recent study in South Africa found that the mortality was low and the residual morbidity among children in their cohort primarily resulted more from the concurrent disease at the time of presentation, particularly that of TB meningitis, than the pericardial involvement.
Extrathoracic Manifestations
Disseminated disease
TB can disseminate to distant anatomic sites following the establishment of primary infection in young children or during reactivation disease in older children and adolescents, often triggered by the erosion of a previously healed primary pulmonary lesion. Depending upon the host's age and immune function, infection from this seeding to distant sites may become readily apparent or quiescent for years. Miliary TB is a highly lethal form of disseminated TB that results from a massive lymphohematogenous dissemination of M tuberculosis from an infected focus. The presentation varies depending on the organs involved and may resemble other common childhood diseases. Fortunately, pediatric miliary TB is rare, comprising less than 1% of all pediatric TB cases, but this may be underestimated due to lack of recognition. A recent case report from Tunisia highlighted the complexities of miliary disease masquerading as multisystem inflammatory disease secondary to COVID-19 yet found to be caused by M tuberculosis.
During early hematogenous dissemination of M tuberculosis, there may be an acute onset of high-spiking fevers, but a prolonged and intermittent fever is also possible. Classic features of TB infection may develop, including chronic weight loss, hepatosplenomegaly, and superficial or deep node lymphadenitis. Other unusual features, such as crops of skin lesions called papulonecrotic tuberculids and choroid tubercles seen on ophthalmological evaluation, are possible. Later in the clinical course, meningitis may also develop. Pulmonary disease, which may have been mild initially, can become more severe over time (see Image. Chest Radiograph and Computed Tomography, Tuberculosis). Due to the atypical multiple-organ involvement, a high index of suspicion is required for early recognition, followed by comprehensive investigation and prompt initiation of appropriate treatment for disseminated TB to mitigate the potential for severe complications and to reduce morbidity and mortality associated with this complex disease.
Lymphatic disease
Tuberculous lymphadenitis (previously known as scrofula) is the most common form of extrapulmonary TB disease in children and constitutes nearly 60% of extrapulmonary cases. In the past, tuberculous lymphadenitis was often caused by drinking unpasteurized cow's milk contaminated by M bovis; pasteurization of milk and milk products has nearly eliminated M bovis as a cause of adenitis in low-burden countries. However, in high-burden regions of the world, particularly in pastoral areas, M bovis and other members of the M tuberculosis complex still warrant consideration. Now, most cases of lymphadenitis are caused by M tuberculosis primary pulmonary infection acquired by aerosol or droplet contact.
Lymph nodes of the anterior cervical, supraclavicular, tonsillar, and submandibular areas are commonly involved due to extension from the primary lesion in the upper lung fields or the abdomen (see Image. Tuberculosis Disease, Cervical Lymph Node). Other areas of lymph node involvement include the inguinal, epitrochlear, and axillary chains. These generally are associated with skin lesions secondary to the M tuberculosis complex. Involved nodes often enlarge slowly but can become massive in size. They are discrete and firm but not hard or tender to touch. However, these affected nodes can feel fixed to adjacent tissue, suggesting a malignancy. Even though a primary pulmonary focus is invariably present, it is often asymptomatic and may be radiographically inapparent in as many as 30% of cases. Untreated lymphadenitis may resolve spontaneously or progress with fluctuance and an overlying purplish red hue indistinguishable from nontuberculous mycobacterial infections in children (see Image. Lymph Node Disease). A chest radiograph may be negative in tuberculous and nontuberculous lymphadenitis, but an interferon-gamma release assay will be unequivocally positive. If untreated, the node may rupture and create a draining sinus tract that will require surgical resection. Clinicians need to maintain a high index of suspicion in low-burden countries to arrive at the proper diagnosis and treatment, especially for children of parents from high-burden nations.
Central nervous system disease
The most deadly and debilitating form of TB is meningitis, which disproportionately affects young children. Without antibiotic therapy, corticosteroids, and critical care management of fluids, electrolytes, and elevated intracranial pressure, tuberculous meningitis is rapidly fatal within several weeks. Despite advances in the care of children with tubercular meningitis, mortality is still high; more than 50% of survivors have neurodevelopmental disabilities.Areas of the world with a high burden of TB may be more likely to recognize the disease than others but lack some tools for fully successful diagnosis and treatment. In contrast, high-resource areas lack knowledge and experience with the disease to recognize signs and symptoms early on.
The clinical onset of tuberculous meningitis can be acute or gradual in the early stages. Nonspecific symptoms, such as flu-like illness, low-grade fever, malaise, and pulmonary symptoms, may overshadow central nervous system findings. Many children are initially seen with pneumonia that will not resolve, poor appetite, and vomiting before more serious signs of central nervous system disease become manifest. Advanced disease may demonstrate signs of meningeal irritation (eg, elevated intracranial pressure with a bulging fontanelle, sunsetting sign, and papilledema), cranial nerve palsies, neurologic deficits and loss of milestones, altered sensorium, and seizures. The Glasgow Coma Scale can help assess the child's consciousness level and aid in determining the ultimate prognosis.
Urgent evaluation of the central nervous system with head imaging is crucial, followed by lumbar puncture for cultures and fluid analysis. Brain imaging commonly shows basilar meningeal enhancement, hydrocephalus, enhancement of basal ganglion, infarcts, or a combination of these. Magnetic resonance imaging (MRI) is preferred over computed tomography for its improved ability to identify early infarcts, basal ganglia enhancement, and exudates in the basal cisterns. However, MRI is not widely available in low-resource countries (see Image. Tuberculosis Meningitis). 
The treatment of tubercular meningitis requires a regimen of prolonged antituberculosis therapy, initially with 4 drugs. (Please refer to the Treatment section for more information on antituberculosis regimens). Management of increased intracranial pressure may require additional medications, including steroids, mannitol, and acetazolamide. The placement of a temporary or permanent ventriculoperitoneal shunt may be needed. Close outpatient follow-up is required to manage adherence and potential adverse events from non TB medications, particularly electrolyte disturbances with the use of acetazolamide.
Skeletal disease
Osteoarticular TB also behaves differently in pediatrics than in adults. The primary focus of infection in children is generally the pulmonary system with dissemination to the osteoarticular system, but extension from either a caseous regional lymph node or an adjacent bone is also possible. Metaphyseal involvement develops initially, followed by necrosis from the pressure of granulation tissue and caseation. Extension into the joint space and adjacent soft tissues can complicate established bone infection, but the swollen joint makes the disease clinically apparent.
Skeletal TB mainly involves weight-bearing bones and joints; the knee, hip, spine, elbow, and ankle are the most affected. The spectrum of presentation ranges from mild joint effusion without any bony destruction to significant destruction of bone and restriction of joint mobility caused by chronic synovial fibrosis. A single joint or many may be involved This slow process may evolve over months to years, causing mild pain, stiffness, limping, and restricted movement. A tuberculin skin test or interferon-gamma release assay is often reactive, and joint fluid culture or bone biopsy will usually be positive for M tuberculosis.
Dactylitis is more common in young infants and toddlers but is occasionally seen in older children and adults. Distal endarteritis from M tuberculosis dissemination through the bloodstream leads to painless swelling of the hands or feet. In the case of digital involvement, a typical lesion involves the proximal phalanx of the index and middle fingers. Fusiform swelling of the digits may resemble juvenile idiopathic arthritis of the hands or feet but is painless. Radiographs showing lytic lesions in the involved digits, called spina ventosa, are typical for M tuberculosis dactylitis.
The vertebral bodies are a particular target of TB (Pott disease), and multiple vertebral bodies may become involved. Infection in the lower thoracic or the upper lumbar vertebrae can lead to bone necrosis, with wedging and collapse; this can lead to gibbous or kyphotic spinal deformity (see Image. Vertebral Osteomyelitis Due to Tuberculosis). Rupture of the infection into adjacent soft tissue may lead to local abscess formation in the psoas muscle, paraspinal muscles, or retropharyngeal space. Paraspinal muscle spasms in children can be associated with low-grade fever, irritability, back pain, abnormal positioning of the back, refusal to bear weight, and movement difficulty. If significant bone destruction and collapse ensues, neurological complications are highly likely to occur, and neurologic impairment may be permanent.
Radiographs of the involved bones generally reveal a lytic lesion, but an MRI should be performed to obtain further information. Several characteristic MRI findings are often present, including synovial thickening, fluid collections, and bony erosions. T1 images show a low signal on the thickened synovium, and T2 images show a hypointense synovium. A confirmatory biopsy should be performed by either needle or open methods, but often, the bony cavity requires debridement of the necrotic bone and caseous material.
Cultures are frequently negative, as in other pediatric TB infections elsewhere in the body. However, biopsy material is critical to the diagnosis since caseating giant cell granulomas seen on histopathology are highly specific for TB and should be treated as such. A history of TB contact is critical when evaluating a child with skeletal disease from a high-burden country or a child with HIV or other immunocompromising conditions who may have had an infectious contact. History is critical in younger age groups presenting with dactylitis.
Abdominal and gastrointestinal disease
Abdominal TB in children may be more common than previously appreciated since it has nonspecific abdominal signs and symptoms, and the lack of available diagnostic tools makes the diagnosis difficult. Abdominal TB tends to occur in slightly older children, usually older than 5 years. Abdominal TB can involve the mesenteric and intra-abdominal lymph nodes, the peritoneum, the intestines, and the intra-abdominal and pelvic organs. Lymphatic or hematogenous seeding, spread from adjacent lymph nodes, and the introduction of mycobacteria orally through M tuberculosis-contaminated animal milk, breast milk, or swallowed pulmonary secretions are the most common acquisition routes. The presentation may resemble other abdominal diseases such as appendicitis, intestinal perforation, and inflammatory bowel disease.
TB enteritis generally results from hematogenous spread to the gut or from swallowing mycobacteria in contaminated milk or respiratory secretions. The jejunum and the ileum are the most common sites involved and may present with shallow ulcers that cause localized pain, diarrhea or constipation, and weight loss. A diagnosis of Crohn disease may be entertained before TB testing is completed as the signs and symptoms are similar. Mesenteric lymphadenitis is often present but generally not palpable on abdominal exams. Enlarged nodes can obstruct the intestines or erode through the omentum, resulting in generalized peritonitis. Tuberculous peritonitis can also result from TB of the genitourinary tract with extension, especially from infection in the fallopian tubes. Intestinal obstruction is a common complication of intestinal TB resulting from mural thickening, ileal stricture, or adhesions. 
Abdominal ultrasound may aid in identifying enlarged lymph nodes, visceral disease with abscess formation, and the presence or absence of ascites.Peritoneal fluid may demonstrate neutrophil or lymphocyte predominance. In a South African series, aspirated peritoneal fluid with an elevated adenosine deaminase level was present in three-quarters of the children with TB peritonitis; the children in this study were significantly more ill.
Genitourinary disease
The genitourinary tract can be seeded by M tuberculosis during direct hematogenous or lymphatic spread from primary M tuberculosis lesions or reactivation disease elsewhere in the body. M tuberculosis organisms can lodge in any portion of the genitourinary tract in both sexes. The disease progression is slow and insidious and is often missed clinically until the disease has caused significant organ damage.
Renal TB is the most common clinical presentation of urogenital TB. In the past, renal TB was believed to spare young children as the progression of the disease was slow. However, currently, several case series underscore the fact that renal TB may not be clinically obvious, though slow destruction of the organ is ongoing, even in infants and younger children.
The intense vascularity of the kidney makes it an easy target for hematogenous spread, and M tuberculosis establishes foci with a granulomatous response. Small caseous tubercles develop and shed M tuberculosis into the tubules and, hence, into the urine. Large caseous masses can also develop and erode into the renal pelvis. Infection can readily spread to the ureters, prostate, epididymis, and adjacent organs like the uterus and fallopian tubes. Untreated, renal TB can lead to the destruction of the renal parenchyma, obstructive uropathy, and end-stage renal failure.
The genital tract can be infected with M tuberculosis in both sexes. In young women of reproductive age (15 and older), infertility is the most common presentation of TB disease involving the genital tract in high-burden areas of the world. In vitro fertilization for a woman with unrecognized genital tract TB can have severe consequences for the outcome of the procedure. Adolescent females may complain of lower abdominal pain and experience dysmenorrhea or amenorrhea. Adolescent males may present with unilateral, nodular, and painless scrotal swelling due to epididymitis or orchitis. All parts of the male genital tract, including the prostate, seminal vesicles, bladder, urethra, and the penis, can be infected with M tuberculosis. Fortunately, this is a rare situation for children.
Congenital and perinatal disease
Congenital TB is rare, but clinicians must always maintain a high index of suspicion when faced with a patient diagnosed with active TB during pregnancy. In-utero infections can occur following maternal bacteremia and have also been reported following in vitro fertilization of women from high-burden countries who likely had unrecognized or subclinical genital TB infection without treatment. Perinatal TB carries up to a 50% mortality since many of the typical features, such as fever, respiratory distress, pulmonary infiltration, lethargy, enlarged liver and spleen, and hematologic abnormalities, cannot distinguish TB from other types of serious systemic infections in a newborn. Further complicating the problem is that tuberculin skin test results are usually negative in infants with congenital or perinatally acquired infection, and the sensitivity of interferon-gamma release assay testing is unknown but also likely to be low due to the infant's immune response to M tuberculosis. If M tuberculosis is suspected, regardless of the tuberculin skin test or interferon-gamma release assay results, therapy for the infant should be initiated immediately with a 4-drug regimen. The placenta should be evaluated for granulomas and acid-fast bacilli and cultured for M tuberculosis. The parent should also have testing for tuberculosis as well as HIV since the risk of coinfection with HIV and TB in high-burden countries is significant.
For patients with known or suspected TB, the following management of the parent-infant dyad is recommended depending on whether the parent has latent or active TB:
The maternal parent has been diagnosed with TB infection in pregnancy (positive tuberculin skin test or interferon-gamma release assay with negative chest radiograph): 
This scenario suggests that the pregnant individual has TB infection (formerly latent TB) as opposed to TB disease. The pregnant patient does not have active TB and is noninfectious. Generally, the pregnant patient will be treated after the postpartum period for latent TB.
In this scenario, the infant needs no special testing and need not be separated from the maternal parent, who may breastfeed if she desires. However, the entire household and other potential contacts of the infant should be questioned and tested, especially if the index case for the maternal parent's positive TB test is not known since it will place the infant at risk from exposure.
The maternal parent has been diagnosed or treated for active TB disease during pregnancy (positive TST or IGRA with an abnormal chest radiograph consistent with tuberculosis): 
This is a high-risk situation for the infant to acquire TB disease through respiratory droplets or airborne transmission. Unfortunately, the infant and maternal parent need to be separated until both have been evaluated thoroughly for TB disease and placed on therapy.
The postpartum patient should begin antituberculosis therapy targeting her specific disease location. She needs to wear a mask and adhere to infection control measures. She may not breastfeed, but the infant could be fed expressed milk if the mother has no evidence of TB mastitis. Once the mother has been on therapy for 2 or more weeks AND she is no longer considered contagious, ie, has smear-negative sputum, she may breastfeed.
The infant requires evaluation for congenital tuberculosis, and if excluded, the infant should be started on isoniazid (INH) unless the M tuberculosis isolate is known or suspected to be INH resistant until the infant is 3 or 4 months of age when the TST should be performed. If this 3- to 4-month TST is negative AND the child has not developed any signs or symptoms of TB disease, INH can be discontinued as long as the postpartum parent has been adherent to medication and remains noninfectious. However, if the infant's 3-month TST is positive, they should be reassessed for M tuberculosis disease. Even if TB disease is excluded, the infant still requires further treatment for the positive TST. A full 9 months of INH, or 4 months of rifampin, is warranted.
Consultation with a specialist in pediatric infectious disease or a clinician with the local TB branch of the health department is recommended to ensure proper care.
Other disease manifestations
TB can also present with rare and varied features that are often part of disseminated disease but may occasionally be seen in isolation. Otitis media and mastoiditis, cutaneous lesions called papulonecrotic tuberculids, and ophthalmologic findings of vitritis, keratitis, choroid tuberculids are the result of dissemination of M tuberculosis to distant sites
Causes
Mycobacterium tuberculosis is an aerobic, slow-growing, nonmotile, acid-fast bacillus. Unlike other aerobes, M tuberculosis has a cell wall containing an elevated amount of high molecular weight lipids. M tuberculosis is part of a group of closely related organisms (including Mycobacterium bovis, Mycobacterium africanum, and Mycobacterium canetti) associated with human disease. M tuberculosis is primarily found in the United States, while M bovis, M africanum, and M canetti are rare in the United States and are not routinely identified by clinical laboratories. If necessary, reference laboratories can distinguish M bovis from M tuberculosis. This differentiation may be relevant for a given case presentation since the epidemiology, treatment, and prevention are different, even though the clinical presentation is similar to M tuberculosis. Other mycobacteria in the M tuberculosis complex primarily cause animal disease.
TB is spread through the air from one person to another. The TB germs are put into the air when a person with active TB disease of the lungs or throat coughs, speaks, or sings.
These germs can stay in the air for several hours, depending on the environment. TB germs are more likely to spread in indoor areas or other places with poor air circulation (such as a closed vehicle) than in outdoor areas. Children who breathe in the air become infected with TB.
Young children are less likely to spread TB germs to others. Health care providers and public health workers will help decide if someone who has been around a child with active TB disease should get tested for TB.
Most children exposed to TB do not get sick. Those most at risk include children who:
Are under 5-years-old
Have weakened immune systems
Live in communities that receive inadequate medical care
Live in a household with an adult who has active tuberculosis or has a high risk of contracting TB
Live in a shelter or with someone who has been in jail
Were born in a country or visit a country with a high prevalence of TB
Risk factor
Most M tuberculosis infections and disease in children are found in areas of the world where adults have the greatest incidence of TB infection. The highest M tuberculosis disease burden is borne in countries in sub-Saharan Africa and Asia, particularly India and the Western Pacific. In the United States, which is a low-incidence country, children who develop TB are often either born outside the United States or have been exposed to close contact who are from outside the United States, particularly from a high-burden country. Another small percentage of cases result from travel to high-burden areas. Drug resistance to first- and even second-line medications is an emerging public health problem globally. While data collection is variable, children with multidrug-resistant TB in some areas of the world reach as high as 13%.
The 2023 WHO Global TB report indicated that 1.3 million children younger than 14  contracted TB in 2022, about 12% of the global total TB disease burden, while a quarter million have succumbed to TB-related disease. Most likely, this is an underrepresentation of the actual number of pediatric cases and deaths, as childhood TB is often misdiagnosed or miscategorized. Deaths from TB in many high-burden regions are frequently ascribed to pneumonia or meningitis without a specific diagnosis.
The human immunodeficiency virus (HIV) epidemic has also affected the epidemiology of M tuberculosis, as coinfection with HIV is not an uncommon occurrence.Although many children in the developing world have access to antiretroviral therapy (ART), children with HIV have a higher risk for M tuberculosis-associated disease even with a normal cluster of differentiation 4 lymphocyte counts and strong viral suppression compared with their non-HIV-infected cohort. Several studies conducted in African nations documented that even though ART reduces TB risk and improves outcomes among children with HIV in sub-Saharan Africa, the risk of TB remains highly elevated compared to children without HIV in the same settings.Children younger than 14 represent the lowest percentage of HIV-infected persons on ART (57% vs 77% for those older than 14). This discrepancy also occurs at a time when both international and domestic funding for HIV initiatives has declined significantly and will impact not only HIV cases but also coinfections, including M tuberculosis that can accompany severe HIV disease.
Any child can develop TB after being exposed. A child is more at risk for TB if they:
Live with someone who has TB
Are homeless
Come from a country where TB is common
Have a weak immune system, including from diabetes, HIV, or medicines that can weaken the immune system.
Very young children are more likely than older children to have TB spread through their bloodstream and cause complications, such as meningitis.
 Signs and Symptoms
The pathophysiology of TB is well-documented. Please see StatPearls' companion resource, "Tuberculosis," for an overview of TB. However, clinicians should note that differences exist between children and adults with TB in terms of host control of the disease. Healthy adults with TB infection have only a 5% to 10% risk of developing TB disease during their lifetime and tend to develop their disease in the first 1 to 2 years after infection is established. However, infants and toddler-age children have a much higher risk of disease progression after exposure than school-age children if left untreated (40% to 50%, 25%, and 10% to 15%, respectively).
Infants and young children have different immunologic responses to TB exposure than adults An infant's immune system has altered inflammatory responses to accommodate the transition to an extrauterine environment with inherent contact with beneficial and virulent pathogens.They typically have lower numbers of macrophages, neutrophils, and tissue dendritic cells, and the function of these cells is different compared to that of adult cells. Production of proinflammatory cytokines, such as tumor necrosis factor and interleukins 1 and 12, is reduced, while anti-inflammatory cytokines are increased, including interleukin 10. Infant T cells are less able to differentiate into interferon gamma-producing T cells, a key factor in M tuberculosis control. 
Therefore, infants are 5 to 10 times more likely to develop active TB than adults, and they have significantly higher rates of severe disseminated disease, including miliary TB and meningitis. Lack of T-cell responses with interferon-gamma release also interferes with tuberculin diagnostic testing for interferon-gamma release assay and tuberculin skin tests, even in the face of overwhelming disease. Children with immune deficiencies, particularly HIV, are at even greater risk for TB progression and severe disease.
Symptoms can occur a bit differently in each child, and they depend on the child's age.
A skin test or blood test reveals a tuberculosis infection. Even if it’s not an active infection, doctors may still prescribe medicine to keep children from developing active TB.
Active TB symptoms include
Constant fatigue (tiredness)
Coughing up blood
Fatigue
Fever
Irritability
Heavy and fast breathing
Loss of appetite
Night sweats
Persistent cough
Swollen glands
Weakness
Weight loss 
Poor growth
 Diagnosis
The healthcare provider will ask about your child’s symptoms and health history. They may also ask about your family’s health history. They will give your child a physical exam.
One way of diagnosing TB is with a TB skin or blood test. In the skin test, a small amount of testing material is injected into the top layer of the skin. If a certain size bump develops within 2 or 3 days, the test may be positive for TB infection. For the TB blood test, a small amount of blood is taken from the child’s arm or hand. It takes a few days for results to come back.Your child may also need a chest X-ray, sputum testing, or a biopsy of abnormal glands or other body tissue.
A TB skin or blood test is advised for children who:
May have been exposed to TB in the last 5 years
Has an X-ray that looks like TB
Has any symptoms of TB
Comes from a country where TB is common
Yearly TB skin or blood testing should be done on children who:
Have HIV
Are in a detention facility
A child who is exposed to high-risk people should be tested every 2 to 3 years.
Differential Diagnosis
Unfortunately, the differential diagnosis of TB is highly dependent upon the location of the disease. As noted in the clinical description of TB, many more common childhood diseases may be mistakenly diagnosed in a child with TB. Other viral, bacterial, and fungal types of pneumonia can present similarly, and M tuberculosis should always be considered in these cases. Cervical lymphadenitis can be viral, especially if multiple nodes are involved. Bacterial pathogens, eg, Bartonella and atypical mycobacteria, can also cause cervical, axillary, and inguinal lymphadenitis; atypical mycobacteria, like M tuberculosis, may produce purplish-red skin discoloration during the progression of the infection. M tuberculosis meningitis is often missed until late as it can mimic other more common childhood viral and bacterial infections that affect the central nervous system.
Furthermore, in high-burden countries, numerous vector-borne diseases (eg, malaria and encephalitis viruses) are present, in addition to parasites that infect the central nervous system. Disseminated TB, abdominal TB disease, and genitourinary tract TB disease can mimic inflammatory bowel disease, severe systemic viral, bacterial, and fungal infections, including multisystem inflammatory disease secondary to COVID-19, and even malignancy. Additionally, HIV should always be tested for in a patient diagnosed with M tuberculosis, and all persons with HIV should have yearly testing for M tuberculosis.
 Treatment
Treatment may include a short-term hospital stay to be treated with medicine. For latent TB, several medicine options are available. Children over 2 years old can be treated with once-weekly medicine for 12 weeks or several months of daily medicine. For active TB, a child will be given 2 to 4 medicines for 6 months or more. With active TB, children usually start to get better within a few weeks of starting treatment. After 2 weeks of treatment with medicine, a child is usually not contagious. Treatment must be fully finished as prescribed. It is important that your child take all of the medicines for the entire time period. Talk with your child’s healthcare provider about the risks, benefits, and possible side effects of all medicines.
Treatment decisions regarding TB management are made based on a detailed medical history and a comprehensive physical examination. After careful consideration for TB in the differential diagnosis of a presenting child, one of the first tests that should be performed is a tuberculin test, either a purified protein derivative (PPD)/TST or a NAAT (eg, IGRA).Information from this test will guide subsequent testing.
A chest x-ray is warranted for evaluation following a positive PPD/TST or NAAT test to determine if pulmonary involvement is present; in cases of high suspicion for M tuberculosis in the absence of a positive screening test, imaging may be performed to assess for silent intraparenchymal disease or adenopathy. Other studies depend upon the location of potential infectious foci and can include ultrasonography of enlarged lymph nodes, imaging of the central nervous system followed by lumbar puncture, skeletal x-rays, imaging of the gastrointestinal or genitourinary tract, or a combination of these tests. Unfortunately, the lack of a positive reaction to a tuberculin test does not always exclude TB infection or TB disease, nor does a positive reaction distinguish between latent TB and actual disease. Up to 40% of immunocompetent children with culture-positive TB disease did not initially react to the tuberculin test. Host factors, particularly young age (younger than 2 years), poor nutrition, immunosuppression, and concurrent viral infections, can adversely affect a positive result. Recent TB infection and overwhelming disease (eg, those seen in disseminated disease) may also result in a negative reaction. Immunosuppression, particularly in children with advanced HIV disease, can also lead to a false negative PPD/TST result.
Treatment protocols for children parallel those of adults, except weight-based dosing is employed for all the needed medications. If directly observed therapy (DOT) is available or is required, as is the case for some regimens, it can be a helpful adjunct to adherence when children transition from the intensive phase with 3 or 4 drugs to the continuation phase with 2 drugs that can be administered several times per week rather than daily (see Tables. Recommendations for Regimens to Treat Latent TB Infection, Recommended Drug Regimens and Therapy Length for Drug-Susceptible Tuberculosis Treatment, and Pediatric Drugs and Dosing for Drug Susceptible Tuberculosis Infection and Disease).
Treatment of DR-Mtb in children is more difficult as some of the medications are not well-studied in children. Children are more likely to experience possible DR-Mtb if they or a close contact fit the following criteria:
Reside in or emigrate from a country with high rates of resistance (eg, Russia and former Soviet bloc countries, Asia, Africa, and Latin America)
Personal or contact history of prior incomplete drug treatment
Personal or contact history of treatment for DR-Mtb
Personal or contact history of persistently positive smear 2 months into therapy
The attached references can guide the treatment of DR-Mtb therapy, but consultation with an expert experienced in the treatment of DR-Mtb is highly recommended to prevent inadequate or partial treatment.
Adherence can be significantly aided by home health nursing assistance where available. A care team consisting of the prescribing physicians, social workers, mental health clinicians, and local health department personnel is often needed to aid the family in completing successful treatment for a young child. Home visits for DOT and intermittent visits to the office for the child and family to assess adverse effects, adherence, potential therapeutic barriers, and response to therapy may be needed for some types of TB treatment protocols. Unlike adults, children do not necessarily require monitoring of laboratory values, such as liver enzymes, unless they initially had abnormal testing or underlying conditions that may make tolerance of treatment regimens difficult without adverse effects.
There are several treatments available, and health care providers will consider a child's age, weight, and other factors when prescribing treatment.
Treating inactive TB in children
Treatment is recommended for children with inactive TB to prevent them from developing TB disease. Depending on the TB treatment regimen a health care provider prescribes, treatment for inactive TB may take three months, four months, or longer.
Treating active TB disease in children
Children with active TB disease will need to take several different TB medicines. This is because there are many TB germs to be killed. Taking several TB medicines will do a better job of killing all the TB germs and prevent them from becoming resistant to the medicines. Depending on the TB treatment regimen a health care provider prescribes, treatment for TB disease may take four months, six months, or longer.
Importance of completing treatment
It is very important that children or anyone being treated for inactive TB or active TB disease finish the medicine and take the drugs exactly as instructed. If a child stops taking the TB medicines before completion, the child can become sick again. If medicines are taken incorrectly, the TB germs that are still alive may become resistant to those drugs. TB that is resistant to drugs is harder and more costly to treat, and treatment can take a long time.
Directly Observed Therapy (DOT)
The best way to take medicines for active TB disease (and in some cases, inactive TB) is by receiving directly observed therapy (DOT). Through DOT, a health care worker will visit the child every day or several times a week. These visits may be in-person or virtual (through a smartphone, tablet, or computer). The health care worker will watch the child take their TB medicines and make sure that the TB medicines are working as they should.
Vaccine
Bacille Calmette-Guérin (BCG) is a vaccine for TB disease. The vaccine is not generally used in the United States. It is given to infants and small children in countries where TB is common. It protects children from getting severe forms of active TB disease, such as TB meningitis (TB disease of the brain). The vaccine can cause a false positive TB skin test reaction. TB blood tests are the preferred tests for people 5 years of age and older who have received the BCG TB vaccine. Tell your child's health care provider if they have received the BCG TB vaccine.
Prognosis
Tuberculosis is both curable and preventable. The overall prognosis for TB should be excellent for a healthy child with an early diagnosis and adequate treatment. Sadly, that is not often the case; morbidity and mortality from tuberculosis take their toll. Many complications, noted below, of late diagnosis or inadequate treatment occur worldwide, but especially in high-burden countries. This is especially true for children with disseminated disease with or without meningitis and for tuberculosis when coinfecting a child with impaired immunity, particularly HIV. Overall mortality in children with HIV as a subset of high-risk children who are coinfected with TB have a disproportionate share of the overall TB mortality, estimated to be as high as 17%.
For children lacking access to early diagnosis and treatment, mortality can reach 21%, rivaling the M tuberculosis pretreatment era before 1946. Mortality in tuberculous meningitis, even with treatment, can reach as high as 19%, and neurodisabilities are seen in >50% of survivors. Survivors may suffer from significant complications related to their disease, the stage at which they started treatment, and any underlying conditions that compromise their immune system. Delays in diagnosis of other organ-specific involvement, eg, renal disease, can progress slowly with significant damage once the disease manifests.
Complications
Most children with proper diagnosis and treatment will experience no complications related to their disease. However, children with immunodeficiencies, particularly HIV, children whose diagnosis was delayed, or children with poor adherence to therapy may lead to the following complications: 
Pulmonary
Extensive lung damage with an increased risk of subsequent wheezing
Reduced lung function
Cavitary disease
Lymphadenitis
Development of chronic fistula
Central nervous system:
Hydrocephalus
Cerebrovascular diseases such as stroke and vasculitis
Tuberculoma
Coma
Skeletal
Pott disease
Paraplegia related to spinal disease 
Epiphyseal invasion, deformity, and bone shortening
Deformities of the affected limb
Spinal deformities
Abdominal
Generalized peritonitis
Intestinal obstruction (from enlarged nodes and erosions)
Genitourinary
Infertility in females
Hydronephrosis
Ureteral strictures
Miliary spread (development of disseminated disease)
Meningitis
Rapid progression to death 
Prevention
The prevention of TB disease has been available for 100 years using the Mycobacterium bovis bacille Calmette-Guérin (BCG) vaccine. This vaccine remains the first and only licensed vaccine against tuberculosis and is utilized in over 180 countries worldwide. The BCG vaccine is administered in infancy and can provide newborns with nearly 90% protection against severe disseminated tuberculosis disease in the first year of life. Although neonates generate a poor interferon-gamma response to M tuberculosis in vivo, BCG vaccination activates CD4 T-cell responses to produce TH1 cytokines, including interferon-gamma, which is lacking in a newborn’s responses to natural tuberculosis infection. BCG provides excellent protection against TB meningitis and miliary TB but cannot prevent pulmonary disease. However, the BCG vaccine is also a live attenuated vaccine and, therefore, is contraindicated in immunocompromised individuals, especially those with untreated HIV.
Over a dozen new candidate vaccines are currently in clinical trials. A complete discussion is beyond the scope of this course; the new candidate vaccine platforms include:
Recombinant fusion proteins delivered with an adjuvant
Viral vectors that express M tuberculosis antigens
A new live BCG vaccine replacement
Strategies for infants have included providing an initial BCG vaccination as a priming dose with a subunit vaccine boost or the reverse strategy with the subunit vaccine first followed by BCG. Unfortunately, this model has only produced modest immunogenicity in infants compared with parallel trials in adults. Some were concerned that the coadministration of the vaccine with the routine infant vaccination series might blunt responses to the M tuberculosis vaccine. However, while lower immunogenicity to the M tuberculosis vaccine was seen in vaccinated infants, no effect on the responses to routine infant vaccines was noted.
Another target group for vaccination is the adolescent and young adult population since this age group (15 to 25 years) has had a sharp increase in the incidence of tuberculosis disease worldwide. Many of these individuals present with cavitary disease and are highly infectious. Therefore, mathematical modeling suggests that the prevention of this condition potentially has a significant impact on the TB epidemic. In adults, an adenovirus vector vaccine produced excellent responses and is felt to be sufficient for protective immunity. Safety, efficacy, and immunogenicity trials are ongoing in infants, adolescents, and adults focused on ending the TB pandemic with new preventative strategies.
Predefined Q&A Sets
What are the common signs and symptoms of tuberculosis in children?
How does pediatric TB differ from adult TB?
What are the most common forms of TB seen in children?
At what age are children most at risk of contracting TB?
How is TB transmitted to children?
What are the diagnostic challenges of TB in children?
Which diagnostic tests are most reliable for pediatric TB?
When should a child be screened for TB?
How accurate is the Mantoux (tuberculin skin) test in children?
What role do chest X-rays and gastric aspirates play in diagnosing pediatric TB?
What is the standard treatment regimen for TB in children?
Are TB medications safe for infants and toddlers?
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Pediatric Chronic Cough
Clinical Terminologies & Codes
ICD-10 Code: R05.3 – Chronic cough
SNOMED CT: 11833005 – Chronic cough
Related Terms: Persistent cough, Long-term cough, Post-infectious cough
Disease Description/Overview
Chronic cough is one of the five most common reasons children are taken to the doctor, affecting 5% to 10% of children in the U.S. every year and accounting for 30 million office visits annually. And that's not surprising, given that coughing is a symptom of a host of health conditions. An acute cough due to a cold or respiratory virus usually goes away within a month's time. When coughing becomes regular and persists longer, however, it may mean there's a more serious culprit behind it.
A chronic cough is a cough that lasts eight weeks or longer in adults, or four weeks in children. A chronic cough is more than just annoying. It can interrupt your sleep and leave you feeling very tired. Severe cases of chronic cough can cause vomiting and lightheadedness, and even break a rib.The most common causes are tobacco use and asthma. Other common causes include fluid that drips from the nose down the back of the throat, called postnasal drip, and the backward flow of stomach acid into the tube that connects the throat to the stomach, called acid reflux. Fortunately, chronic cough usually goes away once the underlying issue is treated.
Pediatric chronic cough can occur due to infection, respiratory disease or environmental exposure to various pollutants. Symptoms can include a dry cough, hoarse cough, or productive cough with mucus. Nasal irrigation serves as effective symptom management because it clears secretions in the nasal passages, thereby reducing the likelihood of cough. Additionally, the consistent use of medications prescribed by a doctor should be employed. Furthermore, being aware of and avoiding triggering factors is essential in order to prevent the exacerbation or recurrence of symptoms.
A pediatric chronic cough can disrupt sleep, disturb those nearby, and interfere with daily activities. Parents may be concerned when their child continuously coughs, even during sleep, and may wonder if it is due to a lung abnormality.
Coughing is the body's natural and normal reaction to an irritant in the airway (the nose and nasal passages, pharynx, larynx, trachea and lungs). When the nerves in the airway sense an irritant — for instance, mucus, a foreign particle or even perfume — the nerves send a message to the brain to clear the breathing passages.
Sounds fine, right? But, what if the coughing does not stop after the airway has been cleared? A daily cough that lasts more than four weeks is considered chronic and should be checked out by a doctor.
The good news is that pediatricians can typically address the common causes of a cough. If the cough persists after traditional treatment or if there are other complicating medical conditions, the pediatrician may refer the child to a specialist.
Here, experts from the program shed light on the causes — both common and rare — of chronic coughing in children.
1. Allergies and sinusitis: Because allergic rhinitis and chronic sinusitis are two of the most common causes of a child's cough, they are often considered first.
Your pediatric ENT specialist will ask detailed questions about the patient's nasal symptoms to help determine whether allergies or a chronic infection may be the source of the problem.
Children with allergic rhinitis sneeze frequently, tend to have clear mucus drainage from the nose, and may have itchy eyes.
Chronic sinusitis, by definition, involves more than 12 weeks of symptoms. Children may complain of pain or pressure in the face, and always have thick, yellow-green nasal drainage.
Both allergic rhinitis and chronic sinusitis often lead to post-nasal drip, and the secretions that drip down the back of the throat as the child changes position can cause a cough. So often parents will describe a cough that is worse when the child first lies down at night.
The doctor also will listen to the cough for clues. In many cases, a dry cough can suggest that it's related to an allergy or asthma. A wet or productive (phlegmy) cough can sometimes indicate a problem other than asthma, like pertussis, mycoplasma or pneumonia.
2. Asthma: Wheezing, or breathing with a whistling or rattling sound in the chest, it what most people think of when they hear asthma.
Asthma can present with a chronic cough as the only symptom however. When doing diagnostic investigations for a chronic cough reversible airflow obstruction on a pulmonary function test can be used to diagnose asthma that presents with cough as the only symptom.A cough that is present after a child falls asleep is suggestive of asthma.
With asthma, the body's levels of cortisol — a key hormone — naturally decrease during the night. This can trigger asthmatic bronchospasm, where the air passages become inflamed and narrow.
3. Whooping cough: Pertussis, better known as whooping cough, is caused by a bacterial infection. Pertussis can cause people to cough so uncontrollably that they have to catch their breath by inhaling so deeply they make a "whooping" sound.
A cough from pertussis could last for months, and complications can be serious, including apnea (not breathing), decreased oxygen, pneumonia, seizures and death. In some cases, complications may require hospitalization for supportive care (possibly including mechanical ventilation in serious cases). Because complications can be life-threatening in children, it is recommended that adults protect their children by getting vaccinated (in addition, of course, to making sure children get the vaccine as part of the recommended vaccination schedule).
4. Cystic fibrosis: In rare cases, a continued cough may be a sign of a more serious condition such as cystic fibrosis, a progressive genetic disease that causes persistent lung infections.
"A cough that doesn't go away can be cause for concern especially when a child also is eating voraciously and not gaining weight," says Jenifer Burke, RN, MSN, a pediatric pulmonary nurse practitioner.
Other symptoms of cystic fibrosis may include loose stools, persistent coughing, recurrent respiratory infections, prolonged symptoms of bronchiolitis (inflammation of the smallest air passages of the lungs), and recurrent/chronic rhinosinusitis (swelling and irritation of the sinus lining).
5. Aspiration: Coughing that occurs while eating or drinking — especially if the child a history of recurrent pneumonia — could be a sign of aspiration.
Aspiration occurs when swallowed food or liquids pass below the level of the vocal cords and into the lungs. "Aspiration can be due to vocal cord paralysis, or other neurologic conditions that cause decreased sensation in the upper airway," says Erin Miller, a speech-language pathologist at Rush.
If your doctor suspects aspiration as the cause of chronic cough, your child may be referred to see a speech-language pathologist. The speech-language pathologist will perform an instrumental swallow assessment, such as a video fluoroscopic swallow study (VFSS) or a flexible endoscopic evaluation of swallowing (FEES). 
6. Acid reflux: While acid reflux is typically associated with gastrointestinal symptoms such as stomach aches, heartburn and vomiting, it can also contribute to the development of chronic cough in children, according to Anil A. Kesavan, MD, a pediatric gastroenterologist at Rush.
"Acid reflux does not cause chronic cough by itself, but it can exacerbate and worsen cough in patients with underlying respiratory disease," he says.
Stomach acids are produced to help digest food and are not meant to move upward from the stomach into the esophagus (the canal that connects the throat to the stomach), but when it happens it can trigger a cough reflex.
Reflux-related cough is typically a dry cough that happens more during the daytime when a child is in an upright position. It occurs commonly after eating and with excessive phonation (i.e., laughing, singing, talking).
Certain foods (caffeine, citrus fruits/juices, foods high in fat, tomatoes, pickled vegetables, carbonated beverages) can trigger acid reflux, which can worsen reflux-related cough.
7. A blockage in the airway: In some cases, chronic coughing could be a sign that a foreign object has become stuck in the child’s airway.
After a choking event, there is an asymptomatic period that can last up to two weeks before complications, such as pneumonia, begin to appear. If your child has a chronic cough that develops after a choking event, seek medical care immediately.
8. Habit cough: Sometimes a cough will develop in response to an irritant in the airway, but persist after the original cause has resolved. Habit coughing is typically "honking" in nature, and it's distractible — if the child is preoccupied, the cough will disappear. The cough is also not present once the child has fallen asleep.
If other causes of chronic cough have been ruled out and habit cough is suspected, speech and behavioral therapy can be helpful in retraining the child's abnormal reflex. Giving the child sips of water when he or she feels the urge to cough can also be a helpful therapy.
Causes
A cough that happens once in a while is common. It helps clear irritants and mucus from your lungs and prevents infection. But a cough that lasts for weeks is usually due to a health concern. Many times, more than one health concern causes the cough.
Most cases of chronic cough are due to these causes, which can occur alone or together:
Postnasal drip. When your nose or sinuses produce extra mucus, it can drip down the back of your throat and cause you to cough. This condition also is called upper airway cough syndrome.
Asthma. An asthma-related cough may come and go with the seasons. It may appear after an upper respiratory tract infection. Or it can get worse when you're exposed to cold air or certain chemicals or fragrances. In one type of asthma known as cough-variant asthma, a cough is the main symptom.
Allergies: Seasonal or environmental allergies can lead to a chronic cough, particularly in children with allergic rhinitis. Common allergens, such as pollen, dust mites, pet dander, and mold, can irritate the airways and cause persistent coughing, especially at night or in the morning
Gastroesophageal reflux disease. In this common condition, also called GERD, stomach acid flows back into the tube that connects your stomach and throat. This tube is also known as your esophagus. The constant irritation can lead to chronic coughing. Then the coughing can make GERD worse, creating a vicious cycle.
Abnormalities of pharyngeal structures: This can cause a chronic cough. Tonsils have been associated with a persistent cough when they impinge upon the epiglottis. The uvula also has been reported to cause a cough when of sufficient length or positioned in such a way that it comes in contact with the epiglottis. A nocturnal cough is common with both of these causes of cough. When a chronic cough is not associated with any apparent lung disease and does not fit the pattern of a habit cough, flexible bronchoscopy through the nose permits visualization of the upper airway prior to progression to the lower airway. While the tonsils or uvula are not ordinarily a cause of coughing, visualizing their atypical contact with the epiglottis is sufficient to suspect that those observations are the cause of chronic coughing in these children. Treatment includes tonsillectomy or uvulectomy to eliminate the stimulation on the epiglottis. Postoperative cessation of coughing supports this diagnosis.
Infections. A cough can last long after other symptoms of pneumonia, flu, a cold or another infection of the upper respiratory tract have gone away. A common cause of a chronic cough in adults — but one that often isn't recognized — is whooping cough, also known as pertussis. Chronic cough also can occur with fungal infections of the lung, as well as tuberculosis infection, also called TB, or lung infection with nontuberculous mycobacteria, also called NTM. NTM is found in soil, water and dust.
Chronic obstructive pulmonary disease (COPD). Also called COPD, this is a lifelong inflammatory lung disease that limits airflow from the lungs. COPD includes chronic bronchitis and emphysema. Chronic bronchitis can cause a cough that brings up colored sputum. Emphysema causes shortness of breath and damages the air sacs in the lungs, also known as alveoli. Most people with COPD are current or former smokers.
Blood pressure drugs. Angiotensin-converting enzyme inhibitors, also called ACE inhibitors, which are commonly prescribed for high blood pressure and heart failure, are known to cause chronic cough in some people.
Age - The age of the child also plays a role in the diagnosis and cause. Younger children are more prone to foreign body aspiration and abnormalities in the respiratory tract. For older children, cough is more likely to be caused by asthma, post-nasal drip and GERD. Nonetheless, viral infections are the most common cause of cough in all ages.
Habit cough - a cough without any infectious cause or other explanation, which typically starts when a child is sick but then persists after the illness resolves. The cough occurs when the child is thinking about or focusing on the cough, but goes away when he is distracted or asleep. This diagnosis is fairly common but can only be made by ruling out other causes of cough.
Other illnesses - In some cases, a cough can indicate a more serious illness, such as cystic fibrosis (CF) or other genetic conditions that affect the respiratory system. However as of 2009, CF is on the newborn screen in all 50 states, so if your child was born after that and had a normal newborn screen, CF is much less likely.
Less commonly, chronic cough may be caused by:
Aspiration — when food or other items are swallowed or inhaled and go into the lungs.
Bronchiectasis — widened and damaged airways that slowly lose the ability to clear out mucus.
Bronchiolitis — an infection that causes swelling, irritation and buildup of mucus in the small airways of the lung.
Cystic fibrosis — a genetic disorder that affects the lungs, digestive system and other organs.
Idiopathic pulmonary fibrosis — gradual damage and scarring of the lungs due to a cause that isn't known.
Lung cancer — cancer that starts in the lungs, including non-small cell lung cancer and small cell lung cancer.
Nonasthmatic eosinophilic bronchitis — when airways are inflamed but asthma is not the cause.
Sarcoidosis — groups of inflamed cells that form lumps or nodules in different parts of the body but most often in the lungs.
Risk Factor
Being a current or former smoker is one of the leading risk factors for chronic cough. Exposure to a lot of secondhand smoke also can lead to coughing and lung damage.
Signs and Symptoms
Normally, when children have an infection in the respiratory system they can exhibit coughing for several weeks following the infection. The cough typically subsides within a month after the infection has completely cleared. However, if the cough persists for more than one month parents should take their child to see a healthcare professional to identify the underlying cause and receive appropriate treatment. For example, if the pediatrician finds signs of sinusitis, they may prescribe antibiotics, decongestants, or anti-inflammatory nasal sprays, or they may recommend nasal saline irrigation. In the case of asthma, pediatricians may prescribe inhaled corticosteroids to control airway inflammation and inhaled bronchodilators to manage airway constriction.
A chronic cough can occur with other symptoms, including:
A runny or stuffy nose.
A feeling of liquid running down the back of your throat, also known as postnasal drip.
Clearing your throat a lot.
Sore throat.
Hoarseness.
Wheezing and shortness of breath.
Heartburn or a sour taste in your mouth.
In rare cases, coughing up blood.
DIAGNOSIS
Respiratory Exam
During this exam, a doctor reviews your child’s medical history with you and asks about any respiratory symptoms or previous illnesses. Our doctors are highly skilled in techniques such as observing respiratory movements during breathing and listening with a stethoscope for unusual breathing sounds, such as wheezing or crackling. They may also feel or tap your child’s chest to see if some areas of the lungs are working better than others.
Sputum Culture
In a sputum culture, a doctor takes a sample of your child’s mucus, if enough can be produced from coughing, to look for the presence of a bacterial infection. If bacteria are found in the sputum sample, our pathologists identify the exact type, so that they can select the most effective antibiotic treatment.
Lung Function Tests
Lung function tests play a major role in identifying the cause of a chronic cough and can be performed in children as young as age 5. Depending on the possible cause of the cough, our doctors may perform one or more of the following tests:
Spirometry
Spirometry measures how much and how quickly your child can blow air out of the lungs by breathing into a special mouthpiece. This test may be used to look for signs of asthma.
Bronchodilator Challenge Test
In a bronchodilator challenge test, your child breathes into a spirometer before and after being given a medication, such as albuterol, to open up the airways. The results of this test may be used to confirm a diagnosis of asthma.
Impulse Oscillometry
Impulse oscillometry is used to diagnose asthma in younger children who have difficulty forcing air into a spirometer. In this test, the child breathes normally into a tube that measures resistance to pressure in the airways.
Exercise Challenge
An exercise challenge test, which uses spirometry to measure lung function before and after riding on a stationary bicycle, may be used to look for signs of exercise-induced asthma, which can trigger a chronic cough.
Bronchoscopy
This test may be used when the cause of a chronic cough cannot be determined with a physical exam and sputum culture, or if the cough remains after treatment.
Bronchoscopy is performed in the hospital while your child is under anesthesia. In this procedure, the pulmonologist inserts a bronchoscope—a flexible tube with a camera at its tip—through your child’s nose or mouth and into the airways. Bronchoscopy allows the doctor to see inside your child’s airways and to identify any blockages or damage to the tissue, such as scarring.
Chest X-ray
X-rays provide a picture of the lungs and may be used to rule out unusual causes of a chronic cough, including lung abnormalities that are present at birth. A chest X-ray and spirometry, at a minimum, are usually ordered to find the cause of a chronic cough in children.
TREATMENT
How are coughs and colds treated?
Most coughs and colds are caused by viruses and get better on their own within a week.
The best treatment for most coughs and colds is rest.
If your child is unwell, keep them at home to rest and recover. This way, their immune system can fight the virus.
Keeping fluids up
Making sure your child drinks plenty of fluids will:
help ease a sore throat
keep them hydrated if they have a fever, vomiting or diarrhoea
Managing runny noses
Saline nose drops or spray can help thin mucus and make it easier to blow out.
Medicines
Paracetamol or ibuprofen can be used to ease the pain of a sore throat or headache. The strengths of these over-the-counter medicines differ, so be sure to check the dose instructions on the pack. Give your child medicines only as directed.
For children older than 12 months of age, honey can help ease their cough. Give your child a teaspoon or 2 of honey before bed.
There are some medicines you should not give to your child, such as:
decongestants
cough syrups
These medicines have not been shown to help children recover from coughs and colds and may be harmful to them.
Sedating antihistamines should not be used in children under 6 years old to treat colds or coughs, as they can cause serious harm.
Vitamins
Vitamin supplements are not necessary if your child has a cold. You may wish to speak to your doctor if you are concerned or want more information.
Special diets
You may have heard that special diets, or 'feeding a fever' and 'starving a cold' can treat colds or coughs. There is no evidence to support this.
Antibiotics
Antibiotics are only used to treat bacterial infections. Most coughs are caused by cold viruses, so antibiotics will not help your child get better any faster.
Finding out what's causing a chronic cough is very important to effective treatment. In many cases, more than one underlying condition may be causing your chronic cough.
If you smoke, your healthcare professional likely will talk with you about your readiness to quit and give you advice on how to achieve this goal. If you're taking an ACE inhibitor medicine, your health professional may switch you to another medicine that doesn't have cough as a side effect.
Medicines used to treat chronic cough may include:
Antihistamines, corticosteroids and decongestants. These medicines are standard treatment for allergies and postnasal drip.
Inhaled asthma medicines. The most effective treatments for asthma-related cough are corticosteroids and bronchodilators. They reduce inflammation and open up your airways.
Antibiotics. If a bacterial, fungal or mycobacterial infection is causing your chronic cough, your healthcare professional may prescribe antibiotic medicines for the infection.
Acid blockers. When lifestyle changes don't take care of acid reflux, you may be treated with medicines that block acid production. Some people need surgery to resolve the problem.
Medicine to reduce coughing
Your healthcare professional works to find the cause of your cough and the best treatment for you. During that time, your healthcare professional also may prescribe a medicine to reduce coughing, called a cough suppressant. Cough suppressants are not recommended for children.
Cough and cold medicines available without a prescription treat the symptoms of coughs and colds — not the underlying disease. Research suggests that these medicines don't work any better than no medicine at all. These medicines are not recommended for children because of potentially serious side effects, including fatal overdoses in children younger than 2 years old.
Don't use over-the-counter cough and cold medicines, except for fever reducers and pain relievers, to treat coughs and colds in children younger than 6 years old. Also, avoid use of these medicines for children younger than 12 years old. Check with your healthcare professional for guidance.
PREVENTION
How can I prevent my child from getting a cough or cold?
It's not possible to prevent all coughs and colds. But there are things you can do to reduce the chance of your kids getting sick.
These include:
hand washing often, especially after coughing, sneezing or blowing your nose
coughing or sneezing into your elbow
avoiding sharing utensils and cups with others
using tissues instead of hankies and throwing them out straight away after use
Eating a balanced diet and getting enough sleep will also help keep your children healthy.
Keeping your child home from school and other activities when they are sick with a cold can stop them spreading their illness to others.
Some children continue to cough for many weeks after a cold — this is known as a post-viral cough. If your child has a cough but is otherwise well, check with your doctor if they can attend school.
The primary factors leading to a cough in children often involve the dripping of nasal secretions into the bronchus. Therefore, performing nasal irrigation is advisable in order to prevent the accumulation of mucus in the nasal passages, thereby reducing the likelihood of cough. Additionally, consistent use of symptom-controlling medications should be employed. Furthermore, being aware of and avoiding triggering factors is essential in order to prevent the exacerbation or recurrence of symptoms. 
Nasal Irrigation to Prevent a Chronic Cough
For nasal irrigation in infants and young children, use 1-2 drops of saline solution in each nostril while elevating their head while they are lying on their back. Gently hold their face still as the saline solution flows, preventing the mucus from becoming sticky and helping it flow out naturally. If there is a significant amount of mucus, you may use a red rubber bulb syringe size 0-1 to gently suction mucus from the nostrils, inserting it approximately 1-1.5 cm. deep. In some cases, it may be necessary to go as deep as 3-4 cm., but this should only be done with caution. 
For nasal irrigation in older children, if there is not a significant amount of mucus you can perform nasal irrigation while the child is sitting. Slightly tilt their head upward, and use 3-4 drops of saline solution in each nostril. Let it sit for a moment, then bend their head forward, open their mouth, and instruct them to blow their nose gently. Repeat this process several times until the nasal passages are clean. If there is a substantial amount of mucus, you can use a 10 ml syringe (without the needle). Gently push 5-10 ml of saline solution into the nostril while the child’s head is bent forward, making sure to have a receptacle ready to collect the drainage. There is no need to push strongly; the mucus will come out. Afterward, have the child blow their nose gently and repeat the process several times on each side until the nasal passages are clear. It is advisable that this procedure be performed in the morning and before bedtime at a minimum. If there is nasal congestion or excessive mucus during the day, you can repeat the nasal irrigation process during periods when the child’s stomach is empty. 
Is Frequent Nasal Irrigation Dangerous?
Most parents, along with the child, often report improved comfort, nasal clearance, better sleep quality, reduced coughing, and improved intake of milk and food after receiving regular nasal irrigation. For infants and young children, the gentle suctioning of nasal mucus is recommended. Older children do not require forceful nasal suctioning but can be instructed to blow their noses gently. This process can be repeated several times until the nasal passages are clear and there is no more secretion. As the saline solution closely resembles bodily substances, it is not harmful. Swallowing a small amount of the saline solution is also safe.
If a child experiences persistent coughing, even during sleep, that is disrupting their daily routine, it is advisable to seek medical attention promptly from a doctor in order to undergo a thorough examination to identify the underlying cause. Prolonged chronic coughing should not be left untreated, as it can impact a child’s learning and overall well-being. 
PROGNOSIS
Prognosis of Pediatric Chronic Cough
The prognosis for chronic cough in children varies significantly depending on the underlying cause, the timeliness of diagnosis, and the appropriateness of treatment.
General Outlook
Favorable Prognosis for Most: Many cases of pediatric chronic cough are due to transient or treatable conditions such as post-infectious cough, mild asthma, or allergic rhinitis, and resolve once the underlying cause is identified and managed appropriately.
Recovery Timeframe: With effective treatment, chronic cough in children typically resolves within weeks. Failure to improve should prompt re-evaluation for less common or more serious diagnoses.
Impact of Underlying Etiology
Infectious Causes: Chronic cough from infections (e.g., prolonged bronchitis, pertussis) generally has a good prognosis with proper antimicrobial therapy, though pertussis may lead to several weeks of lingering cough even after infection is controlled.
Asthma & Allergic Conditions: Children with asthma-related cough can achieve good symptom control with regular medication and trigger avoidance, though some may continue to have intermittent symptoms. Chronic cough may occasionally herald the onset of persistent asthma in susceptible individuals.
Serious Disorders: For chronic coughs due to conditions like cystic fibrosis, bronchiectasis, or anatomical anomalies, prognosis depends on ongoing disease management, early intervention, and access to specialty care. These cases may require long-term treatment and monitoring.
Psychogenic (Habit) Cough: This often resolves with reassurance, counseling, or addressing underlying psychological stressors and rarely persists into adolescence.
Potential for Complications
Undiagnosed or Untreated Cases: Prolonged, unexplained, or untreated chronic cough can result in complications such as poor sleep, fatigue, chest pain, vomiting, or—rarely—rib fractures. It may also signal a serious underlying disease that requires timely intervention.
Recurrent or Persistent Cases: Chronic cough that repeatedly recurs may be associated with a higher risk of respiratory morbidity if the underlying problem is not addressed.
Long-Term Outlook
Most children experience full recovery once the cause is identified and treated.
Persistent cough despite treatment, or cough associated with warning signs (weight loss, poor growth, hemoptysis, severe wheezing), requires urgent specialist assessment to rule out chronic lung disease or systemic conditions.
Key Points
Early identification and targeted treatment yield the best outcomes.
Prognosis hinges on the underlying cause; most are benign, but some require lifelong management.
Children with unexplained chronic cough benefit from pediatric pulmonology evaluation to avoid missed diagnoses and prevent morbidity.
POSSIBLE COMPLICATIONS
Most coughs and colds pass quickly with no medical treatment needed and no complications.
In some cases, your child may develop a bacterial infection after being sick with a cold virus. A bacterial infection may need treatment with antibiotics.
Chronic cough can lead to complications such as disturbed sleep, dizziness, headaches, vomiting, rib fractures in severe cases, and may be a sign of serious respiratory conditions like pneumonia or cystic fibrosis.
DIFFERENTIAL DIAGNOSIS
The differential diagnosis of pediatric chronic cough involves identifying the many possible underlying causes, which can range from common and benign to rare and serious conditions. A detailed clinical history, symptom quality, associated signs, and specialized investigations are essential for accurate diagnosis.
Common and Important Causes
Asthma (including cough-variant asthma): The most frequent cause, often presenting with dry cough, wheezing, or cough triggered by exertion or allergens.
Protracted Bacterial Bronchitis (PBB): A condition in infants and toddlers caused by persistent bacterial infection (e.g., Haemophilus influenzae, Streptococcus pneumoniae) leading to a chronic phlegmy cough without other major symptoms.
Postnasal Drip and Sinusitis: Commonly due to allergies, causing throat clearing and a persistent cough.
Pertussis (Whooping Cough): Caused by Bordetella pertussis infection; characterized by intense coughing fits with possible vomiting, lasting weeks.
Foreign Body Aspiration: Especially in young children, sudden cough onset with possible localized breath sounds; may require bronchoscopy.
Gastroesophageal Reflux Disease (GERD): Less common in children than adults but can contribute to a chronic cough.
Cystic Fibrosis: Genetic disorder causing thick mucus, leading to persistent cough with frequent infections and poor growth.
Primary Ciliary Dyskinesia (PCD): A genetic disorder causing impaired mucociliary clearance, chronic wet cough, recurrent respiratory infections, and sometimes situs inversus.
Tracheomalacia or Tracheobronchomalacia: Weakness and collapse of airway walls causing cough, diagnosed by bronchoscopy.
Chronic Lung Diseases: Including bronchiectasis, asthma, and interstitial lung disease.
Habit (Psychogenic) Cough: Usually dry, repetitive cough that disappears during sleep or distraction.
Diagnostic Clues from History and Symptoms
Quality of cough: Wet/productive vs dry; barking vs staccato.
Associated symptoms: Wheezing, recurrent infections, growth failure, failure to thrive, nasal symptoms, reflux signs.
Onset: Sudden onset may suggest foreign body aspiration; gradual may indicate chronic disease.
Risk factors: Family history, prematurity, exposure to smoke or allergens.
Diagnostic Approach
Physical examination, chest X-rays.
Pulmonary function tests (spirometry) for asthma.
Sweat chloride test and genetic testing for cystic fibrosis.
Bronchoscopy to diagnose airway anomalies or foreign bodies.
Nasal nitric oxide and electron microscopy for primary ciliary dyskinesia.
Trial of antibiotics for suspected protracted bacterial bronchitis.
WHEN TO SEE A DOCTOR
Seek prompt medical attention if the child:
Has a cough lasting more than 4 weeks (considered chronic).
Exhibits signs such as difficulty breathing, wheezing, or exercise intolerance.
Shows associated symptoms like high or persistent fever, poor appetite, fatigue, or weight loss.
Develops warning signs such as coughing up blood, stridor (noisy breathing), or cyanosis (bluish lips or face).
Has a history of lung disease (e.g., asthma, cystic fibrosis) and worsening cough.
Parents suspect the child has inhaled a foreign body.
Experiences worsening or persistent symptoms after initial treatment.
Less urgent but still recommended:
Children with frequent harsh or barking cough without other warning signs should be seen by a doctor within a day or so.
Children with chronic cough but no warning signs should be evaluated by a doctor within a reasonable timeframe, as a delay of a few days to a week is usually not harmful.
Emergency care is needed if:
The child has difficulty breathing or is working hard to breathe.
There is a blue or dusky color to the lips, face, or tongue.
The child is younger than 3 months old with fever or persistent cough.
The child appears lethargic, dehydrated, or very irritable.