Pediatric Pulmonology

1. An 8-year-old female is admitted to the hospital for an exacerbation of asthma. She has recently moved to this area and has not seen a primary care provider in more than six months. She reports complaints of dyspnea, cough and wheeze intermittently for the last four months. Initially the symptoms were only related to exercise which forced her to stop playing actively with her friends. Now she has symptoms almost daily and awakens several times each week with wheezing. She lives in a non-smoking environment. She and her parents deny any known environmental factors which contribute to her difficulty in breathing. Prior to the last several months, she has had no respiratory problems. Since admission, she has been stabilized with frequent albuterol treatments and is feeling much better. She is alert and conversive, able to speak and play without restriction when you see her in the emergency room. Physical examination is significant for an only mildly elevated respiratory rate and mild expiratory wheezing with no accessory muscle use/WOB at this time. Spirometry done at this time reveals a reduced FEV1 and FEV1/FVC at 60% of predicted for age. You consider asthma education needs for this patient and begin thinking about appropriate medication regimens for her condition upon hospital discharge. WHICH of the following is the MOST APPROPRIATE choice of medication(s) to recommend to her and her parents for long-term maintenance therapy of her condition?

Inhaled short-acting beta-2 agonist medication (albuterol), every 4-6 hours, as symptoms warrant

Inhaled long-acting beta-2 agonist medication (salmeterol), twice daily

Inhaled corticosteroid twice daily, plus short-acting beta-2 agonist (albuterol) treatment as needed

Oral prednisone tablets, twice daily

Montelukast (Singulair) tablets, once daily

The most appropriate choice of medication for long-term maintenance therapy of this patient's asthma is an inhaled corticosteroid twice daily, along with a short-acting beta-2 agonist (albuterol) treatment as needed. This is because inhaled corticosteroids are the preferred long-term controller medication for asthma, as they help reduce airway inflammation and prevent symptoms. The short-acting beta-2 agonist (albuterol) is used as a rescue medication to relieve acute symptoms as needed. This combination therapy is effective in managing asthma symptoms and preventing exacerbations.

Explanation

The most appropriate choice of medication for long-term maintenance therapy of this patient's asthma is an inhaled corticosteroid twice daily, along with a short-acting beta-2 agonist (albuterol) treatment as needed. This is because inhaled corticosteroids are the preferred long-term controller medication for asthma, as they help reduce airway inflammation and prevent symptoms. The short-acting beta-2 agonist (albuterol) is used as a rescue medication to relieve acute symptoms as needed. This combination therapy is effective in managing asthma symptoms and preventing exacerbations.

2. A 10-month-old child is brought in for urgent evaluation by her worried parents. She has been ill with low-grade fever, cough, and nasal congestion for several days. Last night, her cough sounded "barky" like a seal and her parents are worried about her ability to breathe. She seems to have more difficulty breathing when she becomes upset. On examination, you note the child who is fussy but consolable. She appears well hydrated and in no acute respiratory distress, although audible breath sounds are noted when she begins to cry. You note her high-pitched barking cough. What clinical finding would you expect to hear upon auscultation?

Decreased breath sounds over the right lung field

Prominent expiratory sounds caused by bronchospasm and inflammation of small airways

Prominent inspiratory and expiratory sounds caused by mucous deposition in both large and small airways

Prominent inspiratory sounds caused by re-expansion of collapsed of alveolar air spaces

Prominent inspiratory sounds caused by subglottic airway narrowing

The correct answer is "Prominent inspiratory sounds caused by subglottic airway narrowing." In this scenario, the child's high-pitched barking cough and difficulty breathing when upset are indicative of croup, which is characterized by inflammation and narrowing of the subglottic airway. Auscultation would reveal prominent inspiratory sounds due to the narrowed airway.

Explanation

The correct answer is "Prominent inspiratory sounds caused by subglottic airway narrowing." In this scenario, the child's high-pitched barking cough and difficulty breathing when upset are indicative of croup, which is characterized by inflammation and narrowing of the subglottic airway. Auscultation would reveal prominent inspiratory sounds due to the narrowed airway.

3. A 6-year old child presents to the emergency room with a four-day history of fever and cough. He is otherwise healthy, although he did have a flu-like illness about 2 weeks ago. On further questioning, his mother tells you that he has been having a lot of cough. She can t tell you if its productive or not, but the child says he thinks that he swallows stuff after coughing. Physical examination is significant for temperature of 39.3 C (102.7 F), and respiratory rate in the 40 s. Oxygen saturation is normal. On exam, the patient is using accessory muscles of breathing. There are decreased breath sounds over the right base, and dullness to percussion in the same area. You obtain a PA and lateral CXR which reveals a lobar consolidation in the right lower lobe. What is the MOST likely etiology of this patient s pulmonary process?

Mycoplasma pnemoniae

Streptococcus pneumoniae

Respiratory syncytial virus

Bordatella pertussis

Pseudomonas aeruginosa

The most likely etiology of this patient's pulmonary process is Streptococcus pneumoniae. This is suggested by the history of fever and cough, as well as the physical examination findings of decreased breath sounds and dullness to percussion in the right lower lobe. Streptococcus pneumoniae is a common cause of lobar consolidation, especially in children.

Explanation

The most likely etiology of this patient's pulmonary process is Streptococcus pneumoniae. This is suggested by the history of fever and cough, as well as the physical examination findings of decreased breath sounds and dullness to percussion in the right lower lobe. Streptococcus pneumoniae is a common cause of lobar consolidation, especially in children.

4. A 16-month-old child is evaluated for respiratory distress in the middle of winter. His anxious mother reports that he has had a few days of nasal congestion and drainage. She also reports that the child has has felt warm to her, although she did not measure his temperature. He started coughing earlier today and his mother reports that the quality of his cough has recently changed, in that it is now becoming more high-pitched and "barky" in nature. He has been otherwise healthy and has no chronic illness. His mother thinks that his breathing has become much more labored over the past several hours. Your examination reveals a child who appears to be in mild respiratory distress with an elevated respiratory rate of 36. Other vital signs, including oxygen saturation, are within normal parameters. There is no accessory muscle use or work of breathing noted. The child is not posturing in an unusual position and has a non-toxic appearance. You note that most of the child's work of breathing appears to be upon inspiration. WHICH of the following findings are you MOST likely to appreciate upon auscultation of this child's lung fields?

Expiratory wheezes

Fine crackles in bilateral lung fields

Inspiratory stridor

Rhonchi in bilateral lung fields

Whooping sound on inspiration

The child's symptoms, including nasal congestion, drainage, barky cough, and labored breathing, along with the finding of most of the work of breathing being upon inspiration, suggest that the child may have croup. Croup is a viral infection that causes inflammation of the upper airway, leading to the characteristic "barky" cough and inspiratory stridor. Inspiratory stridor is a high-pitched sound that occurs during inspiration and is caused by narrowing of the upper airway. Therefore, upon auscultation of this child's lung fields, the most likely finding would be inspiratory stridor.

Explanation

The child's symptoms, including nasal congestion, drainage, barky cough, and labored breathing, along with the finding of most of the work of breathing being upon inspiration, suggest that the child may have croup. Croup is a viral infection that causes inflammation of the upper airway, leading to the characteristic "barky" cough and inspiratory stridor. Inspiratory stridor is a high-pitched sound that occurs during inspiration and is caused by narrowing of the upper airway. Therefore, upon auscultation of this child's lung fields, the most likely finding would be inspiratory stridor.

5. A 3-week-old infant is brought to the emergency room by his parents. They report that he seems to be breathing hard and had a couple of episodes where it looked like he stopped breathing. They deny cyanosis or fever. When you ask his mother about her pregnancy, she reports that it was uneventful. She had prenatal care. She had no perinatal infections, and she was GBS negative. The patient was born at full term via spontaneous vaginal delivery. His nursery course was uneventful and he went home at approximately 36 hours of life. He established care with his pediatrician at 2 weeks of life, and his mother proudly reports that he had already surpassed his birth weight. He received his first vaccination, and his mother reports that his pediatrician said he was in excellent health. He is exclusively breast fed, and had been eating well (approximately 15 minutes per breast every 1-2 hours) until today. Of note, the patient s 4-year-old brother has a cold. Physical examination reveals the following: Temperature: 37.7 C (100 F), respiratory rate 65, blood pressure 73/45, heart rate 168, oxygen saturation is 90% on room air. The infant appears to be in respiratory distress. There are deep subcostal retractions with inspiration. Exam of the lungs reveals diffuse wheezing and poor air movement. Cardiovascular exam reveals tachycardia, but no murmurs. Capillary refill is normal. After placing the infant on supplemental oxygen, he appears much more comfortable and O2 sat increases to 95%. You obtain a PA and lateral CXR which reveals hyperinflation and interstitial infiltrates. You obtain appropriate laboratory studies to hopefully identify the organism causing this infant s distress. Based upon the MOST LIKELY etiology for this infant s respiratory difficulty, initial management should include WHICH of the following measures:

Broad spectrum antibiotic therapy to cover most likely organisms

Inhaled corticosteroid therapy along with antibiotics

Systemic corticosteroid therapy along with antibiotics

Supportive care, including oxygen, hydration and bulb syringe suction as needed

Ventilatory management as the infant is in significant respiratory distress

Based on the information provided, the infant is presenting with respiratory distress, wheezing, and poor air movement. The physical examination and imaging findings suggest hyperinflation and interstitial infiltrates. The most likely etiology for this infant's respiratory difficulty is bronchiolitis, which is commonly caused by respiratory syncytial virus (RSV) infection. The initial management for bronchiolitis is supportive care, including oxygen to improve oxygen saturation, hydration to prevent dehydration, and bulb syringe suction as needed to clear airway secretions. Antibiotics are not necessary as bronchiolitis is usually caused by a viral infection. Inhaled or systemic corticosteroids are not recommended for the treatment of bronchiolitis. Ventilatory management may be considered if the infant's respiratory distress worsens.

Explanation

Based on the information provided, the infant is presenting with respiratory distress, wheezing, and poor air movement. The physical examination and imaging findings suggest hyperinflation and interstitial infiltrates. The most likely etiology for this infant's respiratory difficulty is bronchiolitis, which is commonly caused by respiratory syncytial virus (RSV) infection. The initial management for bronchiolitis is supportive care, including oxygen to improve oxygen saturation, hydration to prevent dehydration, and bulb syringe suction as needed to clear airway secretions. Antibiotics are not necessary as bronchiolitis is usually caused by a viral infection. Inhaled or systemic corticosteroids are not recommended for the treatment of bronchiolitis. Ventilatory management may be considered if the infant's respiratory distress worsens.

6. You see a 10-year-old boy in the emergency room with a 1 1/2 week history of cough. He reports that his symptoms started with sore throat, headache, malaise, and cough. He feels better overall, but his cough hasn t gone away. In addition, he just started the little league season, and he notices that he gets really out of breath when he s running the bases. On exam, the patient is afebrile, but his respiratory rate is slightly increased. The patient appears comfortable at rest. Auscultation of the lungs reveals diffuse rales. A PA and lateral CXR shows diffuse fine interstitial infiltrates, and small bilateral pleural effusions. Heart size is normal. WHICH of the following organisms is the MOST LIKELY cause of this patient s pulmonary process?

Mycoplasma pneumoniae

Streptococcus pneumoniae

Respiratory syncytial virus

Bordatella pertussis

Pseudomonas aeruginosa

The most likely cause of this patient's pulmonary process is Mycoplasma pneumoniae. The patient's symptoms of sore throat, headache, malaise, and cough are consistent with a respiratory infection. The presence of diffuse rales on lung auscultation and the findings of diffuse fine interstitial infiltrates and bilateral pleural effusions on CXR suggest a lower respiratory tract infection. Mycoplasma pneumoniae is a common cause of atypical pneumonia, which can present with these clinical and radiographic findings. Streptococcus pneumoniae is a common cause of community-acquired pneumonia, but the absence of fever and the presence of diffuse rales make it less likely in this case. Respiratory syncytial virus is more commonly associated with bronchiolitis in infants. Bordatella pertussis is the causative agent of pertussis, which typically presents with paroxysmal cough and post-tussive vomiting, but not with diffuse rales or pleural effusions. Pseudomonas aeruginosa is more commonly associated with nosocomial pneumonia and would be less likely in a 10-year-old boy with community-acquired infection.

Explanation

The most likely cause of this patient's pulmonary process is Mycoplasma pneumoniae. The patient's symptoms of sore throat, headache, malaise, and cough are consistent with a respiratory infection. The presence of diffuse rales on lung auscultation and the findings of diffuse fine interstitial infiltrates and bilateral pleural effusions on CXR suggest a lower respiratory tract infection. Mycoplasma pneumoniae is a common cause of atypical pneumonia, which can present with these clinical and radiographic findings. Streptococcus pneumoniae is a common cause of community-acquired pneumonia, but the absence of fever and the presence of diffuse rales make it less likely in this case. Respiratory syncytial virus is more commonly associated with bronchiolitis in infants. Bordatella pertussis is the causative agent of pertussis, which typically presents with paroxysmal cough and post-tussive vomiting, but not with diffuse rales or pleural effusions. Pseudomonas aeruginosa is more commonly associated with nosocomial pneumonia and would be less likely in a 10-year-old boy with community-acquired infection.

7. A 15-year-old girl with cystic fibrosis presents to the emergency room with fever and worsening dyspnea. She has been admitted to the hospital several times this year with pneumonia, and she just completed a course of antibiotics as an outpatient about 2 weeks ago. She admits she may not have taken all the doses as prescribed. In addition to bronchodilator therapy, she reports she had been on inhaled tobramycin, but admits she hasn t taken it for awhile. She says she has been having cough productive of yellowish-green sputum. Physical examination is notable for vital signs as follows: temperature 38.5 C (101.3 F), respiratory rate 28, blood pressure 105/67, heart rate 92, and oxygen saturation 92% on 2L via nasal cannula. She is very thin and appears younger than her stated age. She is barrel-chested (increased AP diameter of the thorax). There is diffuse wheezing on auscultation of the lungs, and a markedly prolonged expiratory phase. A CXR shows marked hyperinflation and lobar consolidation in the right middle lobe. WHICH of following statements regarding cystic fibrosis is true?

A finding of bronchiectasis is inconsistent with a diagnosis of cystic fibrosis

Cystic fibrosis is an autosomal recessive condition inherited through gene expression on chromosome number 5

Exacerbation of illness due to serious infection (as in the vignette above) is most often caused by gram positive organisms

Maintenance therapy of cystic fibrosis includes bronchodilators, airway clearance and DNAse

The underlying defect of cystic fibrosis is in sodium and potassium transport channels in the lungs and other organs

Maintenance therapy of cystic fibrosis includes bronchodilators, airway clearance, and DNAse. This is supported by the information provided in the vignette. The patient is experiencing exacerbation of her illness, which is characterized by fever, worsening dyspnea, and productive cough with yellowish-green sputum. These symptoms indicate the presence of infection and inflammation in the airways. Maintenance therapy aims to manage these symptoms and prevent further complications. Bronchodilators help to open up the airways and improve breathing. Airway clearance techniques, such as chest physiotherapy, help to remove mucus and improve lung function. DNAse is an enzyme that helps to break down thick mucus in the airways. Therefore, the statement that maintenance therapy of cystic fibrosis includes bronchodilators, airway clearance, and DNAse is true.

Explanation

Maintenance therapy of cystic fibrosis includes bronchodilators, airway clearance, and DNAse. This is supported by the information provided in the vignette. The patient is experiencing exacerbation of her illness, which is characterized by fever, worsening dyspnea, and productive cough with yellowish-green sputum. These symptoms indicate the presence of infection and inflammation in the airways. Maintenance therapy aims to manage these symptoms and prevent further complications. Bronchodilators help to open up the airways and improve breathing. Airway clearance techniques, such as chest physiotherapy, help to remove mucus and improve lung function. DNAse is an enzyme that helps to break down thick mucus in the airways. Therefore, the statement that maintenance therapy of cystic fibrosis includes bronchodilators, airway clearance, and DNAse is true.

8. Which of the following is the most specific diagnostic test for cystic fibrosis in pediatric patients?

Chest X-ray

Sweat chloride test

Pulmonary function test (PFT)

Genetic testing for CFTR mutations

The most specific diagnostic test for cystic fibrosis is genetic testing for mutations in the CFTR gene. While the sweat chloride test is commonly used as an initial diagnostic tool due to its high sensitivity, genetic testing provides definitive confirmation by identifying specific mutations in the CFTR gene responsible for the condition. Chest X-rays and pulmonary function tests are useful in assessing lung involvement and disease progression but are not specific for diagnosing cystic fibrosis.

Explanation

The most specific diagnostic test for cystic fibrosis is genetic testing for mutations in the CFTR gene. While the sweat chloride test is commonly used as an initial diagnostic tool due to its high sensitivity, genetic testing provides definitive confirmation by identifying specific mutations in the CFTR gene responsible for the condition. Chest X-rays and pulmonary function tests are useful in assessing lung involvement and disease progression but are not specific for diagnosing cystic fibrosis.