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Pediatric Pulmonary Board Review


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Title: Pediatric Pulmonary Board Review

Pediatric Pulmonary Board Review
  • June 10, 2009
  • Siby Moonnumakal, MD Nidhy Paulose, MD
  • Pediatric Pulmonary Medicine Fellows

Question 1
  • You are seeing a 6-year-old boy for a health
    supervision visit. He was born with trisomy 21
    and an atrioventricular septal defect for which
    he underwent complete surgical repair at 2 months
    of age with an excellent result. His mother
    reports that he has not been sleeping well, that
    he snores loudly, and that she believes that he
    fatigues easily with play. On physical
    examination, he appears in no distress, his
    respiratory rate is 24 breaths/min, his heart
    rate is 100 beats/min, and his blood pressure is
    110/70 mm Hg. His precordial impulse is
    prominent, and there is a loud second heart sound
    and a 2/6 holosystolic murmur at the cardiac apex
    with radiation to the left axilla. There is mild
    hepatomegaly and jugular venous congestion. Of
    the following, the MOST likely cause of this
    patient's findings is
  • A dehiscence of the ventricular septal patch
  • B dilated cardiomyopathy
  • C residual atrial septal defect
  • D right heart failure
  • E supraventricular tachycardia

D. right heart failure
  • Obstructive sleep apnea syndrome is when the
    upper airway is obstructed completely or
    partially during sleep
  • Associated with hypoxemia, hypercapnia, and
    disrupted normal ventilation and sleep patterns.
  • Prevalence of 1 to 2 in children.
  • Risk factors adenotonsillar hypertrophy,
    craniofacial abnormalities, nasal obstruction,
    neuromuscular/hypotonic disorders, and obesity.
  • Pathophysiology mechanical obstruction of the
    upper airway due to tonsillar hypertrophy or
    decreased muscular tone of the airway.
  • Clinical manifestations snoring during sleep,
    mouth breathing, restless sleep patterns, mood
    swings, inattentiveness, learning difficulties,
    and failure to thrive.
  • Diagnosis is confirmed by polysomnography.
  • Complications
  • Obese patients higher incidence of systemic
    hypertension and stroke.
  • Right heart failure may develop due to hypoxemia
    and hypercapnia which are potent
    vasoconstrictors. Over time, the increased
    afterload on the right heart may lead to
    hypertrophy, dilation, and failure of the right
    ventricle (cor pulmonale).

Why the other choices are wrong
  • Dehiscence of the ventricular septal patch would
    lead to pronounced signs and symptoms consistent
    with pulmonary overcirculation. Also unlikely 6
    years post-op.
  • Dilated cardiomyopathy would be referable to the
    systemic circulation, and tachycardia probably
    would develop as a compensatory mechanism for the
    poor cardiac output.
  • Residual atrial septal defect would not be
    expected to cause significant symptoms in a
    6-year-old child and would not explain the
    findings during sleep.
  • Supraventricular tachycardia normal heart rate
    reported in this child.

Question 2
  • You are examining a 4-year-old child who has
    just undergone tonsillectomy and adenoidectomy
    for obstructive sleep apnea syndrome. He has just
    been extubated. While talking with his parents,
    you notice that his breathing is becoming more
    labored. On chest auscultation, you hear equal
    but poor air entry, no wheezes, and some crackles
    bilaterally. There are no murmurs, but a loud and
    palpable second heart sound is evident. You
    increase the oxygen level and reintubate the
    patient. After intubation, you obtain a chest
    radiograph. Of the following, the MOST likely
    finding on the chest radiograph is
  • A bilateral perihilar infiltrates
  • B hemothorax
  • C normal pulmonary findings
  • D pulmonary edema
  • E tension pneumothorax

D. pulmonary edema
  • Complications of tonsillectomy and adenoidectomy
    hemorrhage, dehydration, and velopharyngeal
  • Reported to occur with a frequency of 3 or less.
  • Respiratory complications are less frequent
  • Laryngospasm, apnea, pulmonary hypertension,
    pulmonary hypertensive crisis, and pneumonia.
  • Subgroup of OSA patients who should receive
    particularly intensive perioperative
    management/monitoring younger than 3 years of
    age those who have medical comorbidities,
    neuromuscular disease, bleeding diatheses, or
    syndromes such as trisomy 21 and those who have
    severe OSAS demonstrated on polysomnography.
  • Postobstructive pulmonary edema (POPE)
  • Large negative intrathoracic pressures are
    required to overcome the obstruction ? effects on
    the left ventricle similar to afterlord, raising
    pressures higher than pleural pressures.

Why the other choices are wrong
  • Bilateral perihilar infiltrates and hemothorax
    are possible findings on chest xray but would not
    explain all the clinical findings.
  • Normal pulmonary findings unlikely given the
    degree of respiratory distress.
  • Tension pneumothorax equal breath sounds
    bilaterally so this is also unlikely.

Question 3
  • A 3-year-old boy who was involved in a motor
    vehicle crash sustained a significant head injury
    and had a Glasgow Coma Scale score of 8 on
    arrival in the emergency department. An
    endotracheal tube was placed shortly after his
    arrival, and the respiratory therapist is
    providing ventilation at 12 breaths/min with 100
    oxygen via a bag-valve mask until a ventilator
    can be brought to the emergency department. Ten
    minutes later, the child becomes more restless
    and agitated. His heart rate has increased from
    an initial 110 beats/min to about 150 beats/min,
    and he appears flushed. The monitor indicates
    that his oxygen saturation is 96. Of the
    following, a TRUE statement about this child's
    ventilatory status is that
  • A a patient who has acute carbon dioxide
    retention due to hypoventilation still can have
    near-normal blood oxygen saturation
  • B an arterial blood gas determination likely
    would reveal a pH of approximately 7.45
  • C his agitation is due to relative hypoxemia
  • D his agitation is not related to his
    ventilatory status it is due to tracheal
    irritation from the endotracheal tube
  • E the oxygen saturation of 96 indicates that he
    is being ventilated adequately

A. Patient who has acute carbon dioxide retention
due to hypoventilation still can have near-normal
blood oxygen saturation
  • Acute hypercapnia due to the relatively low
    number of ventilations that are being provided
    per minute.
  • The observed near-normal oxygen saturation is a
    reflection of the high inspired oxygen
  • Even minimal oxygen therapy can maintain normal
    oxygenation in patients who are experiencing
    severe hypoventilation.
  • For this reason, an oxygen saturation monitor is
    not an accurate means to assess the adequacy of
  • Flushing is a clinical manifestation of acute

Looking at the other choices
  • An arterial blood gas determination likely would
    reveal a pH of approximately 7.45
    Hypoventilation leading to hypercapnia results in
    a pH well below 7.4 due to carbon dioxide
  • Agitation is due to relative hypoxemia The
    oxygen saturation of 96 reported for the child
    in the vignette is not consistent with hypoxemia.
  • Agitation is not related to his ventilatory
    status it is due to tracheal irritation from the
    endotracheal tube less likely if adequate
    sedation has been given.
  • The oxygen saturation of 96 indicates that he is
    being ventilated adequately

Question 4
  • A frail 6-year-old child who has cystic fibrosis
    is transported by ambulance to the emergency
    department. She has had hemoptysis for the past 4
    hours, yielding approximately 10 mL of bright red
    blood. She has had increased cough over the past
    3 days. Physical examination findings include a
    respiratory rate of 38 breaths/min, heart rate of
    90 beats/min, oxygen saturation of 92 on room
    air, blood pressure of 100/70 mm Hg, and
    temperature of 98.6F (37C). She is awake and
    alert but seems breathless when she tries to
    speak. On auscultation, you note diffuse crackles
    throughout her lung fields. Of the following,
    the BEST next step in the management of this
    patient is to
  • A administer methylprednisolone
  • B begin therapy with ceftriaxone
  • C insert an endotracheal tube and begin positive
    pressure ventilation
  • D obtain blood for determination of prothrombin
    and partial thromboplastin time
  • E transfuse with O-negative blood

D. Obtain blood for determination of prothrombin
and partial thromboplastin time
  • Acute pulmonary hemorrhage, or hemoptysis, is an
    uncommon problem in pediatrics.
  • Causes
  • Acute lower respiratory tract infection is the
    leading cause today, accounting for 40 or more
    of cases.
  • Cystic fibrosis and congenital heart disease
  • In children younger than 4 years of age, foreign
    body aspiration should be considered.
  • Other, less common causes include bronchiectasis,
    clotting disorders, and trauma.
  • Unlike in adults, neoplasm is an uncommon cause
    of hemoptysis in children.
  • Massive hemoptysis is defined as acute bleeding
    of more than 240 mL in 24 hours.
  • Evaluation
  • Determine the source of the bleeding. Blood
    produced via hemoptysis usually is bright red and
    frothy, in contrast to the dark or "coffee
    ground" material produced in hematemesis.
  • The pH of the material also can be helpful an
    alkaline pH is found in hemoptysis, and an acidic
    pH is found in hematemesis. Epistaxis generally
    can be established after careful examination of
    the oropharynx and nasopharynx.
  • Lab tests CBC, coagulation studies, chemistries
    to assess for renal pathology, urinalysis, and a
    sputum sample for Gram stain.
  • A chest radiograph also should be ordered to aid
    in localizing the bleeding, although films appear
    normal in up to one third of patients
  • Vitamin K deficiency or liver disease in CF may
    prevent clot formation.
  • The source of the bleeding for the child in the
    vignette likely is either pulmonary infection or
    erosion of a blood vessel due to bronchiectasis.

The castaways
  • Administer methylprednisolone no benefit in
    bronchiectasis-related bleed or infection-related
  • Begin therapy with ceftriaxone not effective
    therapy against the expected pathogens.
  • Insert an endotracheal tube and begin positive
    pressure ventilation Most hemoptysis in children
    resolves spontaneously without the need for
    invasive measures. The girl in the vignette has
    not had massive hemoptysis and has adequate
    oxygen saturation on room air, so endotracheal
    intubation is not indicated.
  • Transfuse with O-negative blood indicated only
    for hypotension due to acute massive blood loss.

Question 5
  • You are seeing a 2-month-old male infant who has
    trisomy 21 for a health supervision visit. The
    boy's mother expresses concern that the infant
    has been having "noisy breathing" during the past
    2 to 3 weeks. The infant has been exclusively
    formula-fed and has had no choking or difficulty
    feeding. According to the mother, the noise,
    which occurs on inspiration, is louder when the
    infant is supine and when crying. She has not
    noticed any rhinorrhea, cough, or other upper
    respiratory viral illness symptoms. The infant
    was born via an uneventful vaginal delivery that
    did not require forceps. Apgar scores were 8 and
    9 at 1 and 5 minutes, respectively. On physical
    examination, the infant, whose physical
    appearance is consistent with trisomy 21, is
    resting comfortably. His vital signs are
    appropriate for age, but you hear an audible
    noise during inspiration. Of the following, the
    MOST likely explanation for the infant's
    respiratory symptoms is
  • A laryngomalacia
  • B subglottic tracheal web
  • C tracheomalacia
  • D vascular ring
  • E vocal cord paralysis

A. Laryngomalacia
  • Evaluation
  • Thorough birth history and current medical
  • Observe breathing patterns in different positions
  • Auscultate the airway
  • Secondary evaluation tools include radiography,
    spirometry, and direct airway visualization.
  • Classification of breathing noises
  • Inspiratory at the level of and superior to the
    vocal cords result in a harsh, inspiratory noise
    called stridor
  • Expiratory inferior to the vocal cords typically
    result in wheezing, a high-pitched expiratory
  • Biphasic fixed lesion
  • Laryngomalacia is the most common congenital
    laryngeal abnormality resulting in stridor.
  • Symptoms may begin shortly after birth, although
    they typically occur between 1 and 2 months after
  • Usually happy, thriving, and not having
    difficulty during feedings, but stridor usually
    worsens during supine positioning, increased
    crying or agitation, or a viral illness.
  • Direct visualization of an omega-shaped
    epiglottis that prolapses during inspiration
  • Severe cases may require surgical correction,
    although laryngomalacia usually improves
    spontaneously by 2 years of age.

Others explained
  • Subglottic tracheal web results from failure of
    the embryonic airway to recanalize. Most
    laryngeal webs occur at the level of the vocal
    cords and present at birth with stridor. The
    delayed onset of stridor in the infant in the
    vignette makes a laryngeal web unlikely.
  • Tracheomalacia defective cartilaginous rings
    causing flaccidity of the tracheal wall. Both
    tracheomalacia and laryngomalacia present at
    similar ages and are exacerbated by crying, viral
    respiratory infections, and supine positioning.
    However, infants who have tracheomalacia present
    with wheezing instead of stridor.
  • Vascular ring Extrinsic compression of the
    trachea by vascular anomalies such as a vascular
    ring can result in recurrent wheezing that is
    worsened with crying, feeding, or neck flexion.
    The right-sided aortic arch with left ligamentum
    arteriosum and the double aortic arch account for
    most cases.
  • Vocal cord paralysis can be associated with
    inspiratory stridor, but often results in a weak
    cry, coughing, and choking. When vocal cord
    paralysis is suspected, a thorough search for
    underlying causes (eg, congenital central
    lesions, atrial enlargement, birth trauma,
    traumatic forceps delivery, prior surgical
    procedures) should be undertaken.

(No Transcript)
Question 6
  • A 2-year-old boy is brought to your clinic
    because he has a nighttime cough. According to
    his mother, several times over the past few
    months he has awakened with a barking,
    nonproductive cough that improves by the next
    morning. She denies fever and rhinorrhea with the
    episodes. Evaluation of his lungs yields normal
    results. Of the following, the clinical feature
    that is MOST suggestive of spasmodic croup rather
    than recurrent laryngotracheobronchitis in this
    boy is
  • A age of the patient
  • B barking nature of the cough
  • C lack of rhinorrhea and fever
  • D nonproductive nature of the cough
  • E normal findings on physical examination

C. Lack of rhinorrhea and fever
  • Acute laryngotracheobronchitis/croup
  • Most common cause of acute upper airway
    obstruction in children.
  • It usually is caused by a virus (typically
    parainfluenza), and it may affect infants,
    toddlers, and school-age children.
  • Common features include low-grade fever,
    rhinorrhea, and mild cough, followed 1 to 3 days
    later by a harsh, nonproductive, "barking" cough
    and inspiratory stridor.
  • Laryngoscopy rarely is indicated, but if
    performed, reveals erythema and edema of the
    tracheal epithelium. Symptoms usually abate
    within 1 week. Recurrent infections may occur,
    especially in children who attend child care and
    are repeatedly exposed to viral illnesses.
  • Spasmodic croup
  • similar to acute laryngotracheobronchitis in that
    the cough is often "barking" and nonproductive.
  • May affect young children.
  • Allergic and psychological factors are believed
    to be primarily responsible for spasmodic croup.
  • Classic infectious signs of rhinorrhea and fever
    are not seen.
  • Symptoms may last for only a few hours and
    generally not as long as with infectious croup.
    If performed, laryngoscopy reveals boggy, pale
    edema of the epithelium, with little to no
    inflammatory changes

Question 7
  • A 3-year-old boy presents to the emergency
    department following the abrupt onset of coughing
    and wheezing. You order a chest radiograph for
    evaluation of a suspected foreign body
    aspiration. Of the following, the MOST
    appropriate statement regarding foreign body
    aspiration is that
  • A most foreign body aspirations present within
    24 hours
  • B nonfood items (eg, coins, pins, pencaps) are
    the most common items aspirated by infants and
  • C the classic triad of cough, wheeze, and
    decreased breath sounds is present in most cases
  • D the majority of aspirated foreign bodies are
    located in the larynx or trachea
  • E toy balloons are a common cause of foreign
    body aspirations

A. Most foreign body aspirations present within
24 hours
  • Most foreign body aspirations (50 to 75)
    present and are diagnosed within 24 hours.
  • Although an acute choking or coughing episode
    accompanied by wheezing is highly suspicious for
    a foreign body aspiration, some infants and
    children present with few or no symptoms.
  • The classic triad of coughing, wheezing, and
    decreased breath sounds is present in less than
    50 of cases.
  • What do they swallow?
  • Food is the most common item aspirated by infants
    and toddlers (eg, sunflower seeds, nuts, beans,
    carrots, corn)
  • Nonfood items (eg, coins, paper clips, pins, pen
    caps) more commonly are aspirated by older
  • Although a rare cause of foreign body aspiration,
    toy balloon aspiration can be fatal.
  • Most aspirated foreign bodies lodge in the right
    lung rather than the larynx or trachea.
  • Long-term complications of undiagnosed or
    retained foreign bodies include recurrent
    pneumonia, atelectasis, and bronchiectasis.

  • Evaluation
  • Plain radiography of the chest should be the
    initial diagnostic test, keeping in mind that
    many foreign bodies are food particles and,
    therefore, radiolucent.
  • Abnormal findings may include atelectasis,
    consolidation, mediastinal shift, or air
  • Airway fluoroscopy is an appropriate next test.
  • Barium swallow results may be abnormal following
    ingestion of a foreign body, but they will not
    confirm or rule out a diagnosis of aspiration.

Question 8
  • A 4-year-old boy presents with a history of
    chronic upper and lower respiratory tract
    infections. His weight is 15 kg (25th
    percentile), height is 97 cm (10th percentile),
    temperature is 98.1F (36.8C), and pulse
    oximetry is 96 on room air. On physical
    examination, he coughs intermittently and has
    mild clubbing. On nasal examination, you note
    purulent rhinorrhea and nasal polyps.
    Auscultation of the heart reveals a regular rate
    and rhythm, with the point of maximal impulse
    displaced to the right. Of the following, the
    MOST likely diagnosis is
  • A cystic fibrosis
  • B human immunodeficiency virus infection
  • C primary ciliary dyskinesia (Kartagener
  • D severe combined immunodeficiency
  • E X-linked (Bruton) agammaglobulinemia

C. Primary ciliary dyskinesia
  • PCD triad chronic sinopulmonary infections,
    nasal polyps, and right-sided heart
  • Approximately 50 of patients who have PCD have
    situs inversus, a reversal of the visceral organs
    (Kartagener syndrome).
  • The diagnosis can be confirmed by identification
    of abnormal cilia orientation or missing dynein
    arms by electron microscopy.

Remaining choices
  • Cystic fibrosis neither dextrocardia nor situs
    inversus is a typical finding.
  • Human immunodeficiency virus infection Clinical
    manifestations vary widely. Initial symptoms can
    include lymphadenopathy, hepatosplenomegaly,
    failure to thrive, diarrhea, and thrush.
    Recurrent otitis media, sinusitis, and
    lymphocytic interstitial pneumonia are common,
    but bronchiectasis and nasal polyps are rare.
  • Severe combined immunodeficiency and X-linked
    (Bruton) agammaglobulinemia present with
    recurrent bacterial and viral infections. Nasal
    polyps may result from chronic sinusitis,
    although digital clubbing and dextrocardia are
    not observed

Question 9
  • During the health supervision visit of a
    2-year-old boy who is new to your practice, you
    note a productive cough that his mother says has
    been present for several months. His weight is
    below the 5th percentile. Fine crackles are
    present in his lungs bilaterally, and he has mild
    clubbing of the fingers. The remainder of his
    physical examination findings are unremarkable. A
    chest radiograph reveals poorly defined
    bronchovascular markings and a few cystic spaces
    bilaterally, prompting you to order computed
    tomography scan of the chest, which reveals
    diffuse bronchiectasis. Of the following, the
    MOST likely diagnosis is
  • A allergic bronchopulmonary aspergillosis
  • B cystic fibrosis
  • C foreign body aspiration
  • D Mycobacterium tuberculosis infection
  • E primary ciliary dyskinesia

B. Cystic Fibrosis
  • Bronchiectasis
  • Irreversible, abnormal dilatation of the bronchi
    and bronchioles.
  • It may be localized or diffuse.
  • Diffuse causes most common cause is cystic
    fibrosis, and affected children also may exhibit
    chronic diarrhea and failure to thrive. Less
    common causes of diffuse bronchiectasis include
    allergic bronchopulmonary aspergillosis, primary
    ciliary dyskinesia, immunodeficiency, and
    infections such as tuberculosis, pertussis, and
  • Local causes foreign body aspiration, lobar
    pneumonia, right middle lobe syndrome, and
    extrinsic compression by enlarged lymph nodes.
  • Clinical symptoms of bronchiectasis cough
    productive of copious sputum, hemoptysis, and
    anorexia. Localized rales or wheezing may be
    present on physical examination.
  • Chest radiographs typically reveal nonspecific
    findings such as atelectasis, focal pneumonitis,
    and increased bronchovascular markings.
  • The diagnosis may be made by high-resolution
    computed tomography (CT) scan of the chest, which
    reveals enlargement of the bronchial tree.
  • CT scan is used to differentiate between the
    types of bronchiectasis, which are cylindrical,
    varicose, and saccular (the most severe type).
  • If bronchiectasis is diagnosed, an evaluation for
    cystic fibrosis, immunodeficiencies, and
    infections is warranted.
  • Treatment consists of chest physiotherapy,
    bronchodilators, and antibiotics.

(No Transcript)
Question 10
  • A 16-year-old girl who has moderate persistent
    asthma presents to the emergency department with
    coughing, wheezing, and increasing dyspnea. She
    states that she was feeling fine until she was
    exposed to cologne that one of her classmates was
    wearing. An ambulance was called after her
    symptoms did not improve following administration
    of two puffs of her beta-2 agonist inhaler. On
    physical examination, the teenager has a
    respiratory rate of 30 breaths/min, heart rate of
    90 beats/min, and pulse oximetry of 98 on room
    air. She has difficulty completing a sentence and
    points to her neck, saying it is "hard to get air
    in." Her lungs are clear to auscultation, and
    rhinolaryngoscopy demonstrates adduction of one
    of the vocal cords during inspiration with a
    posterior glottic "chink." Pulmonary function
    testing shows a blunted inspiratory loop. Of the
    following, the MOST likely cause for this
    patient's symptoms is
  • A asthma exacerbation
  • B subglottic stenosis
  • C vocal cord dysfunction
  • D vocal cord nodule
  • E vocal cord paralysis

C Vocal Cord Dysfunction
  • VCD can mimic and coexist with asthma.
  • Key features normal room air pulse oximetry
    readings, failure to improve with the beta-2
    agonist inhaler, clear lungs on examination,
    difficulty with inspiration instead of
    expiration, and a normal expiratory flow loop
    without signs of obstruction.
  • The hallmark finding on rhinolaryngoscopy is
    paradoxic adduction of the vocal cords during

(No Transcript)
Other choices
  • Subglottic stenosis and vocal cord paralysis
    usually present with stridor and primarily affect
    infants and younger children. Subglottic stenosis
    can result from direct laryngotracheal trauma,
    intubation, or infection (eg, tuberculosis,
    diphtheria). Flexible or rigid laryngoscopy can
    be used to assess the degree of subglottic
    stenosis. Vocal cord paralysis can occur in the
    context of congenital central nervous system
    lesions, surgical correction of congenital
    cardiac anomalies or tracheoesophageal fistula,
    or inflammatory processes of the larynx, or it
    may be idiopathic. Direct visualization of the
    vocal cords demonstrates the absence of vocal
    cord motion during inspiration and expiration.
  • Vocal cord nodule also called singer's nodules,
    usually occur after repetitive vocal misuse or
    abuse (eg, excessive loudness, inappropriate
    pitch, excessive coughing, or throat clearing).
    On flexible laryngoscopy, nodules appear as
    superficial growths on the medial surface of the
    true vocal folds.

(No Transcript)
Question 11
  • A 17-year-old boy is applying for entry into
    military service and requires a complete history
    and physical examination. During the interview,
    he states that he is healthy, although he admits
    to being treated for three cases of pneumonia
    over the past 10 years. A chest radiograph
    performed during the last infection showed a left
    lower lobe pneumonia, and the patient states that
    the infection is " always on that side." The only
    finding of note on the physical examination today
    is slightly diminished breath sounds over the
    left lower lobe. Of the following, the MOST
    likely cause for this boy's recurrent pneumonias
  • A bronchogenic cyst
  • B congenital cystic adenomatoid malformation
  • C congenital lobar emphysema
  • D extrapulmonary sequestration
  • E intrapulmonary sequestration

E Intrapulmonary Sequestration
  • Suspect whenever there is recurrent unilateral
  • Intrapulmonary sequestrations account for 75 to
    90 of all sequestrations.
  • Present in adolescence or adulthood with cough,
    wheezing, fever, and recurrent pulmonary
  • Surgical lobectomy generally is curative.

Remaining choices
  • Bronchogenic cyst most common cause of a cyst in
    the lung. Most commonly located near central
    airway structures, bronchogenic cysts may present
    with symptoms of airway compression or infection,
    but they frequently are asymptomatic and
    discovered incidentally on chest radiography
  • Congenital cystic adenomatoid malformation
    typically is identified on prenatal
    ultrasonography. Most CCAMs present in the
    newborn period with respiratory distress and,
    depending on the type, may involve an entire
    lung, be associated with congenital anomalies, or
    result in fetal hydrops and pulmonary hypoplasia.
    Affected patients can present during childhood
    with recurrent pneumonia, but CCAM is less common
    than intrapulmonary sequestration for adolescent
  • Congenital lobar emphysema the most common
    neonatal cause of cystic malformation of the lung
    and similar to CCAM, typically presents in the
    neonatal period with respiratory distress and
    airway obstruction.
  • Extrapulmonary sequestration Usually also
    left-sided, present prior to 6 months of age and
    often occur in conjunction with other congenital
    anomalies such as colonic duplication, pulmonary
    hypoplasia, or vertebral anomalies. can present
    similarly to intrapulmonary sequestrations, with
    cough, dyspnea, and infection, but also can
    result in feeding difficulty and, in rare cases,
    congestive heart failure due to increased

Bronchogenic cyst
(No Transcript)
Intralobar sequestration
Question 12
  • A 4-year-old boy presents with a 2-year history
    of persistent bilateral nasal congestion. His
    parents are worried because at night he snores
    loudly and has had pauses in his breathing. His
    symptoms occur daily and have not improved with
    the administration of oral decongestants, nasal
    corticosteroids, oral antihistamines, or
    antibiotics. The boy denies ocular pruritus,
    sneezing, or rhinorrhea. On physical examination,
    a low-pitched inspiratory noise is audible, and
    there is " cobblestoning " of his posterior
    pharynx. Findings on the remainder of the
    physical examination, including the tonsils, nose
    (by nasal speculum examination), and neck, are
    unremarkable. Of the following, the MOST likely
    diagnosis is
  • A adenoidal hypertrophy
  • B allergic rhinitis
  • C choanal atresia
  • D chronic sinusitis
  • E juvenile nasopharyngeal angiofibroma

A. Adenoidal hypertrophy
  • Suspect in children 2 to 5 years of age
  • Typical symptoms of adenoidal hypertrophy
    chronic mouth breathing, snoring, nasal
    obstruction, and hyponasal speech
  • More severe symptoms include obstructive sleep
    apnea syndrome and recurrent infections (eg,
    sinusitis, otitis media).
  • Because the adenoidal tissue is located at the
    posterior aspect of the nasopharynx, it cannot be
    seen in the usual nasal speculum examination
    nasal rhinoscopy or a lateral neck radiograph is
  • Although enlarged tonsillar tissue may indicate
    enlarged adenoid tissue, a direct correlation is
    not always true.

Why not these?
  • B allergic rhinitis Allergic rhinitis may be
    seasonal or perennial and can produce symptoms of
    rhinorrhea and nasal obstruction. Allergic
    rhinitis usually does not occur until 5 to 10
    years of age. The absence of sneezing or
    rhinorrhea, and the lack of improvement with
    usual allergy medications (eg, nasal
    corticosteroids, oral antihistamines) described
    for the boy in the vignette also make allergic
    rhinitis unlikely.
  • C choanal atresia rare congenital structural
    malformations of the nose that can result in
    nasal congestion and obstructive sleep apnea
    syndrome. Obstruction may be unilateral or
    bilateral and may not present until later in
    infancy if not complete or bilateral. However,
    symptoms often are noted at birth because of
    difficulty during feedings.
  • D chronic sinusitis can present at any age
    because the maxillary and ethmoid sinuses are
    present at birth. Typical symptoms may include
    chronic rhinorrhea (clear or discolored) and
    nasal obstruction. The lack of improvement with
    oral antibiotics for the boy in the vignette
    suggests that sinusitis is unlikely, but if the
    evaluation for adenoidal hypertrophy was
    negative, sinus imaging should be considered.
  • E juvenile nasopharyngeal angiofibroma has been
    described in children as young as 2 years, but
    typically it presents with profuse epistaxis and
    a nasal mass during puberty.

Question 13
  • The parents of a 6-month-old boy call you in the
    middle of the night because he is coughing and
    has nasal congestion. You had diagnosed a viral
    upper respiratory tract infection when you saw
    him earlier today. The parents are frustrated
    that the cough is persistent and request medicine
    for their boy so they can sleep. Of the
    following, the MOST appropriate recommendation is
  • A chlorpheniramine/pseudoephedrine combination
  • B codeine
  • C dextromethorphan
  • D guaifenesin
  • E saline nasal drops

E. saline nasal drops
  • Cough is an important reflex that helps to clear
    excessive secretions from the airway.
  • Many of the studies on the efficacy of cough and
    cold preparations have been conducted in adults
    only a few have been performed in children.
  • Randomized, placebo-controlled, blinded trials
    have failed to show any benefit in children for
    medications such as antihistamines and
    decongestants (alone and in combination),
    antitussives, and narcotics.
  • No studies have shown that the use of
    expectorants, such as guaifenesin, provides any
  • Several adverse effects, including sedation,
    irritability, and tachycardia, may be seen with
    the use of these medications.
  • In addition, a recent study attributed three
    infant deaths to cough and cold medications.
  • When using saline nasal drops, proper technique
    is essential for maximum improvement in
    congestion, and suctioning should be performed at
    times when it is most important to relieve
    congestion, such as before sleeping and eating.
  • Parents also should be educated on the cause and
    expected duration of their child's illness as
    well as concerning symptoms that require further
    investigation, such as continuation of symptoms
    beyond 10 to 14 days and dehydration.

Question 14
  • An infant who was born at 26 weeks' gestation,
    weighing 700 g, is nearing 37 weeks corrected
    age. He is receiving nasal cannula oxygen (0.2
    L/min) and being treated with diuretics for
    chronic lung disease. He has no intracranial
    hemorrhage and is growing well on enteral
    feedings via a nasogastric tube. Attempts at oral
    feeding have been unsuccessful because of
    frequent oxygen desaturation, bradycardia, and
    concerns over the inability to coordinate
    sucking-swallowing. The mother asks why you are
    continuing to feed her son via a feeding
    tube. Of the following, the condition that BEST
    explains why her son has feeding problems is
  • A apnea of prematurity
  • B chronic lung disease
  • C gastroesophageal reflux
  • D necrotizing enterocolitis
  • E tracheoesophageal fistula

B. chronic lung disease
  • For preterm infants who have chronic lung disease
    (CLD), potential intermittent or chronic hypoxia
    and impaired feeding and caloric intake may have
    negative effects on their growth.
  • Oral aversion following prolonged oral
    endotracheal intubation and delayed oromotor
    development and function.
  • Swallowing dysfunction (dysphagia) may be noted
  • Radiographic imaging using a fluoroscopic
    modified barium swallow study can reveal problems
    with hypopharyngeal pooling, laryngeal
    penetration, or frank aspiration of milk.

Other choices
  • A apnea of prematurityApnea of prematurity is
    not present the infant has passed the
    gestational age equivalent of prematurity.
  • C gastroesophageal reflux does not occur during
    a feeding but typically presents with
    postprandial emesis.
  • D necrotizing enterocolitis associated with
    systemic decompensation and an acute abdomen.
  • E tracheoesophageal fistula respiratory distress
    and secretion control problems during intervals
    between feeding the rare H-type fistula with an
    intact esophagus (accounting for lt5 of all TEFs)
    typically does not present in the newborn period.

Question 15
  • You are asked to consult on a 9-month-old boy
    who has been hospitalized five times for
    wheezing. His history reveals occasional coughing
    with feedings, but results of a pH probe
    performed during his last admission were normal.
    His weight and height are at the 50th percentile.
    Except for scattered wheezes with good aeration
    bilaterally, results of his physical examination
    are normal. Of the following, the test that is
    MOST likely to reveal the cause of his recurrent
    wheezing is
  • A chest computed tomography scan
  • B immunoglobulin panel
  • C inspiratory and expiratory chest radiographs
  • D pulmonary function testing
  • E videofluoroscopic swallow study

E. videofluoroscopic swallow study
  • Recurrent wheezing can be caused by many
    diseases, including reactive airway disease,
    cystic fibrosis, extrinsic airway compression,
    and aspiration with and without gastroesophageal
  • Gastroesophageal reflux is a common cause of
    recurrent aspiration, but swallowing dysfunction
    without gastroesophageal reflux also can occur.
  • Several types of swallowing dysfunction are seen
    in infants.
  • Laryngeal penetration without aspiration
    describes the entry of food particles into the
    airway down to the level of the vocal cords.
  • Aspiration is defined as the entry of food below
    the level of the vocal cords
  • Nasopharyngeal backflow or reflux is the entry of
    food posterior or superior to the soft palate.
  • Absence of cough with feedings does not eliminate
    the possibility of silent aspiration.
  • If a fluoroscopic swallow study reveals
    swallowing dysfunction, thickening formula or
    human milk and feeding in the upright position
    may improve symptoms.
  • In some cases, cessation of oral feedings and
    placement of a nasojejunal or gastrostomy tube
    may be indicated for a period of time.

Question 16
  • An 18-month-old girl has been having an
    intermittent nonproductive cough for the past 6
    months. Her parents state that the cough awakens
    the toddler at night a few times a month and
    occurs when playing vigorously. During a recent
    upper respiratory tract illness, her cough
    worsened and occurred daily for 3 weeks. On
    physical examination, there is no nasal
    discharge, and the toddler appears healthy. Of
    the following, the MOST likely diagnosis is
  • A asthma
  • B atypical pneumonia
  • C gastroesophageal reflux
  • D sinusitis
  • E upper airway cough syndrome

A. asthma
  • Chronic cough typically is defined as one that
    persists for more than 8 weeks.
  • With a normal CXR, three causes account for 95
    of chronic coughs
  • asthma
  • gastroesophageal reflux (GER)
  • upper airway cough syndrome (aka postnasal drip
  • Asthma usually develops in early childhood, with
    80 of patients reporting symptoms prior to age 6
  • Symptoms may include cough, wheezing, shortness
    of breath, and chest tightness.
  • The most common trigger for infants and toddlers
    is a viral upper respiratory tract infection
  • Fortunately, URI-induced wheezing resolves in
    most infants by age 6 years (so-called "transient
    or viral-induced wheezers").
  • Those who continue to have asthma symptoms after
    age 6 are at greater risk for persistent asthma.

Explanation of other choices
  • B atypical pneumonia caused by Mycoplasma
    pneumoniae and Chlamydophila pneumoniae
    (previously termed Chlamydia pneumoniae) may
    present at any age, although it is unusual prior
    to age 3 years. Characteristic constitutional
    symptoms include fever, malaise, and headache.
  • C gastroesophageal reflux Cough can represent
    the sole manifestation of GER, but GER usually
    becomes symptomatic during the first few
    postnatal months, improving by 12 months of age.
    GER may worsen at night during supine
    positioning, but exercise and URIs are uncommon
    precipitating factors for GER symptoms.
  • D sinusitis characterized by rhinorrhea and
    postnasal symptoms.
  • E upper airway cough syndrome encompasses
    allergic rhinitis, nonallergic rhinitis, and
    sinusitis. Allergic rhinitis typically occurs in
    children older than 3 years of age and is
    associated with other ocular and nasal symptoms,
    such as pruritus, sneezing, and rhinorrhea.

Question 17
  • A 10-year-old boy presents with a 2-month
    history of chronic cough. His parents are unsure
    of a specific preceding trigger. They are
    concerned because the school nurse has called on
    multiple occasions requesting that the boy be
    taken home due to his persistent cough. The boy
    denies any chest pain, dyspnea, or syncope. Use
    of a sedating antihistamine and over-the-counter
    cold and cough liquid has not alleviated his
    symptoms. On physical examination, the boy has
    vital signs within the normal range and appears
    healthy. A thorough examination reveals no
    abnormalities. During the encounter, the boy
    repeatedly exhibits a harsh, "barky" cough that
    resolves when you leave the examination room,
    only to recur when you return. You suspect he has
    a psychogenic cough. Of the following, the MOST
    accurate statement regarding psychogenic cough is
  • A most cases are associated with underlying
    psychological illness
  • B most cases are preceded by an upper
    respiratory tract infection
  • C resolution of the cough often is followed by
    recurrent wheezing
  • D symptoms persist during the day and while the
    child is asleep
  • E the cough noise often is dramatically
    different from the postnasal drip syndrome cough

B. most cases are preceded by an upper
respiratory tract infection
  • Psychogenic cough, sometimes called habit-cough
    syndrome, is an uncommon cause of chronic cough
    in children and adolescents.
  • Most cases of psychogenic cough begin with an
    uncomplicated upper respiratory tract viral
    illness, but the cough lingers for months to
  • The classic triad of symptoms
  • repetitive cough
  • cessation during sleep
  • characteristic hacking, barky cough.
  • Successful therapies for habit-cough include
    behavioral therapy, suggestion therapy, and
  • Once resolved, most cases of habit-cough do not
  • Unlike some cases of viral upper respiratory
    tract infections, habit-cough resolution is not
    followed by recurrent wheezing.

Question 18
  • An 8-year-old girl presents with multiple
    episodes of "bronchitis." For the past 2 years,
    she has had problems with coughing, wheezing, and
    difficulty catching her breath during vigorous
    exercise. Treatment with a metered dose beta2
    agonist inhaler has improved her symptoms. In
    your office, you discuss the different tests to
    assess lung function. Of the following, the BEST
    test to measure lung function for this girl is
  • A arterial blood gas
  • B exhaled breath condensate
  • C exhaled nitric oxide
  • D pulse oximetry
  • E spirometry

E. spirometry
  • Established pretest norms are based on the
    patient's height and ethnicity.
  • Measurements of lung function that can be
    obtained with spirometry include the forced vital
    capacity (FVC), volume of air exhaled during the
    first second (FEV1), FEV1/FVC ratio, peak
    expiratory flow (PEF), and airflow during the
    middle half of the effort (forced expiratory
    flow) (FEF25-75).
  • Does not measure total lung capacity (TLC) or
    residual volume (RV).
  • Ideal test conditions require a patient to exhale
    for 6 seconds, children younger than 6 to 7 years
    of age often are unable to complete the test.

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Why not the other choices?
  • A arterial blood gas This is a gas diffusion
    measurement that provides insight into
    oxygenation (PO2) and ventilation (PCO2), but it
    does not measure lung function.
  • B exhaled breath condensate Similar to eNO,
    exhaled breath condensate does not measure lung
    function but is being developed as another tool
    to assess airway inflammation and assist with
    asthma management.
  • C exhaled nitric oxide approved by the United
    States Food and Drug Administration in 2003 for
    children ages 4 years and older. Although not a
    measure of lung function, eNO is a useful
    noninvasive tool to measure nitric oxide, a
    marker of airway inflammation.
  • D pulse oximetry simple, noninvasive method to
    measure oxygenation, but it also does not measure
    ventilation or lung function.

Question 19
  • You are evaluating a 2-month-old girl who has
    suspected infantile botulism. On physical
    examination, she has a weak cry, poor head
    control, dilated pupils, and a markedly decreased
    gag reflex. Her respiratory rate is 30
    breaths/min, and she has decreased breath sounds
    bilaterally at her lung bases. Her oxygen
    saturation on room air is 85, but has increased
    to 90 on 3 L/min of oxygen administered by nasal
    cannula. An arterial blood gas evaluation on 3
    L/min oxygen shows a pH of 7.24, a Paco2 of 60 mm
    Hg, and a Pao2 of 70 mm Hg. Of the following,
    the MOST appropriate immediate step is
  • A intubation with mechanical ventilation
  • B oxygen administration via a nonrebreathing
    face mask
  • C oxygen administration via a high-flow nasal
  • D placement of a nasal trumpet
  • E serial measurements of negative inspiratory

A. intubation with mechanical ventilation
  • Respiratory failure inability to maintain
    adequate oxygenation (hypoxemia, as defined by a
    Pao2 less than 60 mm Hg) and ventilation
    (hypercarbia, as defined by a Paco2 greater than
    50 mm Hg).
  • Impending respiratory failure in pediatric
    patients typically is characterized by tachypnea,
    increased use of accessory muscles, or
  • Hypoxemia, grunting, and fatigue are late signs
    and worrisome for respiratory collapse.

What about the other choices?
  • B oxygen administration via a nonrebreathing face
    mask and C oxygen administration via a high-flow
    nasal cannula Administration of higher
    concentrations of oxygen (eg, by high-flow nasal
    cannula or nonrebreathing face mask) has the
    potential of increasing the patient's oxygenation
    but would not improve the ventilation.
  • D placement of a nasal trumpet Placement of a
    nasal trumpet is indicated for patients who have
    an intact respiratory drive and evidence of upper
    airway obstruction.
  • E serial measurements of negative inspiratory
    force excellent method of following respiratory
    effort in patients who have underlying
    neuromuscular disorders, but they normally
    require a cooperative patient of at least 5 years
    of age.

Question 20
  • During the health supervision visit of a term
    newborn boy, his mother relates that a cousin's
    child died at age 4 months from sudden infant
    death syndrome. She asks what she can do to
    prevent such an occurrence in her son. Of the
    following, the single MOST important preventive
    measure is to
  • A avoid use of a pacifier
  • B cosleep in the parental bed for close
  • C ensure side sleep positioning to prevent
  • D ensure supine sleep positioning
  • E use home apnea and bradycardia monitoring

D. ensure supine sleep positioning
  • A avoid use of a pacifier may reduce the
    incidence of SIDS but is not without controversy
    due to possible interference with breastfeeding
    in the early weeks after birth.
  • B cosleep in the parental bed for close
    observation In the United States, infant
    cosleeping with parents increases the risk of
    SIDS, possibly related to frank suffocation.
    However, the recommendation to avoid cosleeping
    remains controversial when providing support for
    breastfeeding and considering the prevalence of
    cosleeping worldwide. Further risk factors
    associated with cosleeping include parental
    obesity and sleeping on a very soft surface, such
    as a waterbed.
  • C ensure side sleep positioning to prevent
    aspiration can increase the risk of SIDS,
    possibly due to the propensity for a bundled
    infant placed on his or her side to roll forward
    into the prone position.
  • E use home apnea and bradycardia monitoring not
    been shown to reduce the risk of SIDS, even in
    families where a previous child has died from
    SIDS. Its use is limited to preterm infants who
    have apnea of prematurity and infants in whom
    central apnea, known cardiac arrhythmia, or other
    identifiable cause of events that may respond to
    monitoring and cardiorespiratory resuscitation is
    a distinct possibility.

Question 21
  • A 3-year-old boy is admitted to the hospital for
    fever, cough and increasing respiratory
    insufficiency of 2 days' duration. Chest
    radiography demonstrates a right middle lobe and
    lower lobe pneumonia with a significant pleural
    effusion (Item Q185). You aspirate a sample of
    pleural fluid and send it to the laboratory for
    analysis. Of the following, the MOST likely
    expected laboratory findings are
  • A Moderate to high white blood cells, lactate
    dehydrogenase (U/L) 2 to 3 times the serum
    concentration, lt3 protein (g/dL), and
    Gram-positive cocci seen in the Gram stain
  • B Moderate to high white blood cells, lactate
    dehydrogenase (U/L) equal to the serum
    concentration, lt3 protein (g/dL), and negative
    Gram stain
  • C Moderate to high white blood cells, lactate
    dehydrogenase (U/L) 2 to 3 times the serum
    concentration, gt3 protein (g/dL), and
    Gram-positive cocci seen in the Gram stain
  • D Rare white blood cells, lactate dehydrogenase
    (U/L) equal to the serum concentration, gt3
    protein (g/dL), and negative Gram stain
  • E Rare white blood cells, lactate dehydrogenase
    (U/L) equal to the serum concentration, lt3 protein
    (g/dL), and Gram-positive cocci seen in the Gram

C. Moderate to high white blood cells, lactate
dehydrogenase (U/L) 2 to 3 times the serum
concentration, gt3 protein (g/dL), and
Gram-positive cocci seen in the Gram stain
  • Pleural effusions in pediatric patients have a
    variety of causes, including pneumonia, trauma,
    malignancy, renal disease, liver failure, and
    congestive heart disease.
  • Parapneumonic effusions (those associated with an
    underlying pneumonia) account for up to 70 of
    pediatric pleural effusions.
  • Pleural effusions may be seen in up to 40 of
    bacterial pneumonias progression to pus
    (empyema) occurs in more than 50 of these cases
    and is increasing in both incidence and
    associated complications.
  • Transudates are plasma ultrafiltrates and usually
    result from renal and liver disease or congestive
    heart failure.
  • Exudates arise from inflammatory processes (such
    as pneumonia, malignancies, trauma, or systemic
    inflammatory diseases) or impaired lymphatic
    drainage, often following thoracic surgery (eg,
  • Empyema, a type of exudate, is characterized by
    the presence of white blood cells, a positive
    Gram stain, or frank pus.

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Question 22
  • You are called to the neonatal intensive care
    unit to examine a newborn who has abdominal
    distention and respiratory distress. She was born
    at 38 weeks' gestation and weighs 4 kg. Apgar
    scores were 3 and 6 at 1 and 5 minutes,
    respectively. She required tracheal intubation
    and assisted ventilation. On physical
    examination, she has a large, distended, and
    tense abdomen without bowel sounds. The abdominal
    wall is not erythematous, and there is no clearly
    palpable mass. She does not display other
    evidence of body wall or scalp edema. The breath
    sounds are coarse and equal bilaterally. There is
    no heart murmur. Radiograph of the chest appears
    normal, but abdominal radiography shows
    background granular density, paucity of
    intraluminal bowel gas, and a calcified mass in
    the left lower quadrant (Item Q210). Of the
    following, the BEST explanation for this infant's
    abdominal findings is
  • A congenital lymphangioma
  • B erythroblastosis fetalis
  • C meconium peritonitis
  • D ovarian cyst
  • E urinary ascites

C. meconium peritonitis
  • Meconium ileus thick meconium obstruction of the
    distal ileum characteristically presenting
    clinically as a small bowel obstruction.
  • This condition occurs in approximately 17 of
    infants who have CF.
  • History may indicate polyhydramnios, a prenatal
    ultrasonographically observed bowel dilation, or
    delayed postnatal passage of stool.
  • Evaluation Physical examination may reveal
    abdominal distention and bile-stained emesis. A
    plain abdominal radiograph may show stacked loops
    of variably dilated bowel, soap-suds bubbly-like
    appearance of meconium stool (most often in the
    right lower quadrant), and a ground-glass
    character of the distal bowel with a relative
    paucity of distal bowel gas. A contrast enema may
    reveal a microcolon and failure to pass contrast
    beyond the ileocecal valve
  • Surgical exploration and removal of inspissated
    meconium is generally necessary, as may be a
    temporizing ileostomy and later reanastamosis.
  • Meconium peritonitis associated with bowel
    obstruction leading to perforation and spillage
    of meconium into the peritoneal cavity,
    occasionally involving a walled-off calcified
    meconium "pseudocyst" apparent on plain abdominal
    radiograph or abdominal ultrasonography.
  • The perforation may have occurred in utero and
    may be associated with fetal ascites or hydrops,
    pulmonary hypoplasia and respiratory failure, and
    impaired bowel or liver function.
  • The newborn may have ascites or anasarca on
    physical examination, with a tense, distended
  • Plain abdominal radiography may reveal a diffuse
    ground-glass density and little intraluminal
    bowel gas there also may be diffuse or focal
  • Medical management is directed at stabilizing
    pulmonary function, fluid and electrolyte
    balance, and hepatic function before addressing
    the surgical condition.
  • Prolonged jaundice with a predominance of
    conjugated hyperbilirubinemia also is seen in
    infants who have CF and may result from
    inspissated bile or prolonged use of parenteral

Meconium ileus Meconium peritonitis
Other choices
  • A congenital lymphangioma may present with
    ascites and hydrothoraces requiring drainage
    analysis of the fluid confirms the diagnosis.
  • B erythroblastosis fetalis isoimmune hemolytic
    condition that involves fetal hydrops (neonatal
    anasarca) and profound anemia, but it is not
    related to bowel obstruction and does not result
    in meconium peritonitis.
  • D ovarian cyst may be massive, occasionally
    resulting in bowel compression and extrinsic
    obstruction that can require surgical excision.
    They are discernible by abdominal and pelvic
    ultrasonography as being distinct from the bowel
  • E urinary ascites develops following urinary
    tract obstruction, with perforation and leakage
    of urine into the peritoneal space. It occurs
    most commonly in boys who have posterior urethral
    valves. Analysis of the ascites fluid reveals an
    elevated creatinine concentration.

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