Pulmonary Medicine

Question 1

A 17-year-old African-American girl presents for evaluation after receiving a CXR in the emergency department for a “possible pneumonia” that turned out to be a viral infection. However, on the CXR, it is noted that she has significant bilateral hilar and mediastinal adenopathy. She is completely asymptomatic and denies fever, night sweats, or weight loss. She has no infiltrates on CXR. Tuberculosis, fungal diseases, and malignancy have been excluded from her differential diagnosis.

What is the most likely diagnosis?

Answer 1

Sarcoidosis

This is a classic presentation for sarcoidosis. She has Stage I, with bilateral hilar adenopathy and no parenchymal infiltrates. Many patients with sarcoidosis are asymptomatic, in which case, there is nothing therapeutically to do. If she had eye or CNS involvement, heart conduction abnormalities, severe pulmonary or skin signs/symptoms, or persistent hypercalcemia, then you would intervene with systemic steroids. Without these findings, just follow her for symptoms, which are unlikely to occur in most patients. Remember that erythema nodosum is associated with this and if found, is a good prognostic indicator.

Question 2

A 19-year-old young man presents with:

• History of hemoptysis for several months
• Iron deficiency anemia
• New onset renal insufficiency
• Biopsy shows linear deposition of IgG and C3 on alveolar and glomerular basement membranes.
• ANCA is negative.

What is the diagnosis?

Answer 2

Antiglomerular Basement Membrane (Anti-GBM) Antibody Disease (Goodpasture Syndrome)

Anti-GBM antibody disease is an autoimmune disease. It tends to present in young adult males with a male-to-female ratio of 3:1. Lung disease is the same as idiopathic pulmonary hemosiderosis (IPH), but Goodpasture syndrome also affects the kidneys. Typically, there is no frank hemorrhage, but often there is hemoptysis that precedes renal abnormalities. Think of this disease if the patient presents with dyspnea, hemoptysis, iron deficiency anemia, and glomerulonephritis but without the upper airway signs that are seen in granulomatosis with polyangiitis (GPA).

Question 3

A 13-year-old girl with known cystic fibrosis presents for followup evaluation. She is noted to have the following:

• Chronic productive cough and wheezing
• Recurrent bacterial pneumonia
• Clubbing of her fingers
• Large total lung capacity
• Large functional residual capacity
• Small vital capacity
• Areas of ventilation-perfusion mismatch

What is likely occurring in her lungs?

Answer 3

Bronchiectasis

The clues here are her known predisposing CF (and most common etiology of bronchiectasis in children) and the finding of clubbing, which is found in 50% of children with bronchiectasis.

Question 4

A 6-month-old presents in January with:

• Temperature of 100.0° F (37.8° C)
• Runny nose
• Poor feeding
• On examination, scattered wheezes and intermittent inspiratory crackles
• CXR shows hyperinflation.

What is the likely diagnosis?

Answer 4

Bronchiolitis

This is a classic presentation for bronchiolitis, which typically occurs in the winter and spring. It is most commonly due to respiratory syncytial virus (RSV).

Question 5

A 4-year-old girl presents with abrupt onset of:

• Fever
• Sore throat
• Drooling
• Stridor

She presents to the office and is sitting on the examination table, leaning forward with her chin extended. She has an “I’m-about-to-die” look on her face.

What is the most likely diagnosis?

Answer 5

Epiglottitis

This is rarely seen anymore thanks to H. influenzae vaccine; however, other bacteria can cause this on occasion.

Question 6

A 15-year-old boy presents with abrupt onset of:

• Fever
• Cough with wheezing
• Joint pains
• Rash

What is the most likely bacterium causing this illness?

Answer 6

Mycoplasma pneumoniae

M. pneumoniae may be associated with extrapulmonary manifestations, including hemolytic anemia, splenomegaly, erythema multiforme (and Stevens-Johnson syndrome), arthritis, myringitis bullosa, pharyngitis, tonsillitis, and neurologic changes—especially confusion.

Question 7

An 11-year-old boy presents with:

• Nasal polyps
• History of asthma
• And _________ sensitivity

What drug sensitivity is this child likely to have with this triad?

Answer 7

Aspirin Sensitivity in the “Aspirin Triad”

Question 8

In October, a 2-year-old boy presents with:

• A high-pitched, barking cough
• Inspiratory stridor
• An AP neck x-ray shows subglottic narrowing.

What is the diagnosis?

Answer 8

Laryngotracheobronchitis (Croup)

This is the classic presentation for viral laryngotracheobronchitis (croup). It commonly occurs in children between the ages of 3 months and 3 years, with an average age of 2. Boys are more likely to be affected than girls. Most incidents occur in fall and early winter. Parainfluenza viruses cause most cases of croup.

Question 9

A 2-week-old infant presents with inspirational stridor. It is much worse when the child is upset.

What is the most likely diagnosis?

Answer 9

Laryngomalacia

Laryngomalacia is the most common cause of stridor in the newborn period. The laryngeal cartilage is just not stiff enough, and inspiration causes significant luminal narrowing. The stridor can occur at birth, but it most commonly presents at 2 weeks of age. Most children outgrow the disorder by 12–24 months of age.

Question 10

A 5-year-old boy presents with:

• An initial history of abdominal pain, headache, and vomiting
• This is followed the next day by development of:
  
  • Fever
  • Moderate-to-severe sore throat pain
  • Diffuse erythema of the tonsils and tonsillar pillars
  • Petechiae of the soft palate

What is the most likely disgnosis?

Answer 10

Streptococcus pyogenes Pharyngitis

(Strep Throat)

This is the classic presentation. He has no URI symptoms.

Question 11

A 15-year-old boy presents with abrupt onset of:

• Fever
• Chills
• Chest pain with dyspnea
• Blood-tinged sputum
• A pleural friction rub

What is the most likely bacterium causing this illness?

Answer 11

Streptococcus pneumoniae

Streptococcus pneumoniae classically presents with an abrupt onset of fever, chills, and chest pain with dyspnea. Blood-tinged or “rust-colored” sputum is also common. In some cases, you can detect a pleural friction rub. Children with pneumococcal pneumonia appear clinically ill and have tachypnea and tachycardia.

Question 12

A 16-year-old girl presents with:

• Worsening muscle weakness that is exacerbated by repetitive muscle use
• Respiratory compromise
• Ocular muscles are also involved

What is the diagnosis?

Answer 12

Juvenile Myasthenia Gravis

It is an acquired autoimmune disorder with development of autoantibodies to acetylcholine receptors. The disease progresses gradually with worsening muscle weakness and respiratory compromise. Ocular muscles are commonly involved.

Question 13

A 5-year-old boy presents with a 2-day history of:

• Low-grade fever
• Malaise
• Runny nose
• Congestion
• Thick, copious, green nasal discharge

What is the likely diagnosis?

Answer 13

Viral Upper Respiratory Infection (URI)

Recall that for the diagnosis of sinusitis you need to have symptoms at least 7 to 10 days! The green “snot” is just an indication that WBCs are being called in to help the infection - in case it was a bacterial infection.

Question 14

A 4-year-old boy with a 1-day history of “acute pharyngitis” presents with:

• Abrupt fever if 104.2 F
• Difficulty swallowing
• Refusing to eat
• Severe throat pain
• Drooling
• In examination, he has:
  
  • Hypertension of the head
  • “Bulge” in the posterior pharyngeal wall

What is the most likely diagnosis?

Answer 14

Retropharyngeal Abscess

It most commonly presents in children 2 to 4 of age with an abrupt onset if high fever and difficulty swallowing. They commonly refuse to eat, have severe throat pain, and may present with hyperextension if the head. Drooling is common. In exam, many children have a “bulge” on the posterior pharyngeal wall. If you took a lateral x-ray, you would see the mass and the retropharyngeal soft tissue will be more than 50% if the width if the adjacent vertebral body. This is a medical emergency requiring emergent antibiotics, and, if the mass is fluctuant, drainage is necessary. This differs from peritonsillar abscess, which presents with “hot potato” voice and a displaced uvula (to the opposite side). Epiglottitis could be confused here with the drooling and fever. Commonly, children with epiglottitis will sit forward with the chin extended, and they will not have the “bulge” described here.

Question 15

A newborn presents at day 2 of life with:

• Difficulty with breastfeeding
• On examination, you note that the infant has difficulty extending the tongue past the alveolar ridge.

What is the diagnosis?

Answer 15

Lingual Ankyloglossia (tongue-tie)

The lingual frenulum limits the movement of the anterior tongue tip. Most patients do well and can adjust, but some require a frenulectomy.

Question 16

A 3-year-old boy presents with a history of:

• Waking up in the middle of the night with a barking cough
• Mild stridor

The next day, the child is perfectly healthy. That night, the symptoms reoccur. The next day, the child is again healthy. The family says this has occurred for 3 nights.

What is the most likely diagnosis?

Answer 16

Spasmodic Croup (non-infectious)

This is a classic cycle of waking up with croupy cough and mild stridor and then being normal the next day without issues. Then, that night, the croupy cough returns and resolves the next day with a normal-appearing child. This is noninfectious croup and may be related to gastrointestinal reflux.

Question 17

An 8-year-old with a 2-day history of acute pharyngotonsillitis presents with:

• High, abrupt fever to 104.5 F
• Severe sore throat pain
• Trismus
• Refusing to speak or swallow
• “Hot potato” voice
• Uvula is displaced to the opposite side

What is the diagnosis?

Answer 17

Peritonsillar Abscess

It occurs after or with an acute pharyngotonsillitis. Fever can be very high. Severe pain, Trismus, and refusing to speak or swallow are common. A “hot potato” voice is classic as is displacement of the uvula to the opposite side - these 2 symptoms help distinguish this entity from retropharyngeal abscess or epiglottitis.

Question 18

A 2-year-old girl presents with:

• High fever
• Brassy cough
• Stridor

She is given treatment for croup but does not respond, and she deteriorates rapidly. She is intubated in the emergency department.

What is the most likely diagnosis?

Answer 18

Bacterial Tracheitis

This is the classic presentation for bacterial tracheitis. Initially, it appears that the patient has laryngotracheobronchitis (viral croup), but after receiving appropriate therapy, the child continues to deteriorate rapidly and may require intubation or tracheostomy.

Question 19

A newborn presents with the following:

• Short ribs
• Small rib cage
• Renal disease
• + Short limb dwarfism
• + Pelvic anomalies
• + Polydactyly
• + Hepatic involvement

What is this syndrome?

Answer 19

Jejune Syndrome (Asphyxiating Thoracic Dystrophy)

It is an autosomal recessive disorder. Restrictive lung disease is common due to the small rib cage.

Question 20

A 6-hour-old newborn presents with the following:

• Respiratory failure
• Heart failure
• On evaluation, you find that pulmonary venous blood from all or part of the right lung returns to the inferior vena cava, just above or below the diaphragm.

What is the diagnosis?

Answer 20

Scimitar Syndrome

Congenital pulmonary venolobar syndrome (“Scimitar syndrome”) is a rare disorder in which the pulmonary venous blood from all or part of the right lung returns to the inferior vena cava (IVC) just above or below the diaphragm. CXR may show the shadow of the veins involved as they course, giving a “scimitar-like” (Turkish sword) appearance.

Question 21

A 3-hour-old infant of a mother with a neurologic disease presents with:

• Hypotonia
• Weak cry
• Difficulty feeding
• Facial weakness
• Palpebral ptosis
• Respiratory distress

What is the likely cause of the infant’s problems?

Answer 21

Neonatal Myasthenia Gravis from Transplacental Transmission of Maternal Acetylcholine Receptor Antibodies

The Palpebral ptosis and facial weakness should clue you in, because nothing else is going to cause this with respiratory distress. Neonatal myasthenia Gravis resolves in 2 to 6 weeks after the maternal antibodies have cleared. Note: This differs from congenital myasthenia gravis, which is an autosomal recessive disorder and does not have circulating antibodies to acetylcholine receptors.

Question 22

A 4-year-old boy presents with:

• Frequent falling
• Waddling gait
• Toe walking
• Calf muscles appear to be hypertrophied
• You observe him getting up off the floor by first pushing on his shins, then his knees, and finally his thighs
• Elevated CPK

What is the most likely diagnosis?

Answer 22

Duchenne Muscular Dystrophy

It is the most common muscular dystrophy of childhood. It is an X-linked recessive disorder and thus only affects boys. The observed “climbing up his legs” to get off the floor describes Gower sign. The calf muscles are hypertrophied compared to the rest of his leg muscles. CPK is elevated.

Question 23

A 15-year-old boy with cerebral palsy and seizure disorder with recurrent choking presents with:

• Fever
• Tachypnea
• Tachycardia
• Cyanosis
• CXR shows diffuse infiltrates.

What is the most likely diagnosis?

Answer 23

Aspiration Pneumonia

When there is a predisposition to aspiration, such as a history of seizure, neurologic disease, or dysphagia, pneumonia may be caused by anaerobic oral flora and gram-negative organisms.

Question 24

A 17-year-old boy from Northwest Arkansas, who hunts frequently, presents with a 1-month history of:

• Low-grade fever
• Cough with occasional hemoptysis
• Chest pain
• 10-lb weight loss
• Several verrucous lesions with irregular borders and microabscess formation at the periphery on the left arm
• CXR shows upper lobe infiltrates with a cavitary lesion.

What is the most likely diagnosis?

Answer 24

Blastomycosis

This is a classic description of blastomycosis that causes chronic pneumonia with dissemination to the skin. Most cases occur in Arkansas, Mississippi, Illinois, Wisconsin, and the states bordering these areas.

Question 25

A 5-year-old boy presents with:

• Fever
• Rhinitis
• Moderate-to-severe sore throat pain
• On exam, the pharynx is bright red with petechiae and erythema of the tonsils; exudates are noted on the posterior pillars.

What is the most likely etiology of his sore throat?

Answer 25

Viral Infection

Some of you went right for Strep pyogenes (GAS) because of the petechiae and exudates in the throat - these are very nonspecific. The clue here, that this is viral and not bacterial, is the rhinitis. Viral pharyngitis is accompanied by URI symptoms and can include conjunctivitis, rhinitis, cough, hoarseness, coryza, ulcerative lesions, or viral rashes.

Question 26

An 8-year-old girl presents with:

• Iron deficiency anemia that was initially diagnosed a year ago
• Progressive dyspnea
• Fatigue
• Recurrent cough with new onset hemoptysis
• Sputum shows hemosiderin-laden alveolar macrophages.

What is the most likely diagnosis?

Answer 26

Idiopathic Pulmonary Hemosiderosis (IPH)

Even though this disease is very rare, it still appears in the content specifications of the ABP. Typically, IPH presents before 10 years of age and may be progressive or abrupt in presentation. The progressive form is like this vignette with iron deficiency anemia being the first abnormality noted, followed by the cough and hemoptysis. The key to making this diagnosis is having sputum or bronchoalveolar lavage that shows hemosiderin-laden alveolar macrophages.

Question 27

A 17-year-old girl known to have asthma presents with:

• Cough, wheezing, and chest tightness with URI and exercise
• Emergency department visit 2 years ago for an asthma exacerbation
• Spirometry pulmonary function test (PFT), showed:
  
  • Forced expiratory volume in 1 second (FEV1) = 74%
  • FEV1/forced vital capacity (FVC) = 79%
  • Change in FEV1 post-bronchodilator = 35%

What is the severity of her asthma based on PFT? Is there reversibility post-bronchodilator?

Answer 27

Moderate Persistent Asthma with Reversibility

For pediatric patients ≥ 12 years of age, FEV1 > 60% but < 80% predicted is the defined range for moderate persistent asthma. For a 17-year-old patient, normal FEV1/FVC is 85%; that figure reduced by 5% is consistent with moderate persistent asthma. Reversibility is defined as an increase in FEV1 ≥ 12% after a bronchodilator. Therefore, this case is consistent with moderate persistent asthma with airway reversibility.

Question 28

A 13-year-old girl presents with an initial history of:

• Sore throat that is negative for group A streptococcus by culture
• Hoarseness

Now, 2-3 weeks later, she develops pneumonia.

What is the most likely etiology of her biphasic disease?

Answer 28

Chlamydophila pneumoniae

Chlamydophila pneumoniae causes epidemic pneumonia in older children and adolescents. It is the cause of up to 10% of community-acquired pneumonias. This biphasic course is classic for Chlamydophila pneumoniaeinfection.

Question 29

A 2-year-old boy presents acutely with:

• Choking
• Coughing
• Wheezing
• Diminished breath sounds on the right
• CXR shows nothing

What is the most likely etiology of his symptoms?

Answer 29

Aspiration of Foreign Body

Promptly consider aspiration of a foreign body when a child ≤ 2 years of age acutely presents with choking, particularly if this is followed by the classic triad of cough, wheezing, and diminished breath sounds on physical exam. CXR may show asymmetric hyperinflation but is commonly normal. The most commonly aspirated objects are seeds, nuts, coins, hot dogs, balloons, jewelry, batteries, and firm vegetables.

Question 30

A 13-month-old girl presents with:

• Coryza
• Postnasal discharge
• High fever (103.9 F)
• Poor appetite
• Cervical lymphadenitis

What is a possible bacterial diagnosis?

Answer 30

Streptococcus

This is a classic presentation for streptococcal disease in a child under the age of 2 years. Granted, this is difficult to tease out without the pharyngitis, but how can you really assess pharyngitis in a 13-month-old? On the Boards, look out for a child under the age of 2 with high fever, poor appetite, coryza, and cervical lymphadenitis. Usually, they will have very crusted and thick nasal discharge described as well.

Question 31

A 17-year-old boy has the following:

• Sinusitis
• Bronchiectasis
• Situs inversus
• Reduced male fertility

What is the syndrome?

Answer 31

Primary Ciliary Dyskinesia (PCD)

Kartagener Syndrome

PCD is a rare autosomal recessive disease that can be sporadic or familial. There are numerous mutations that can cause the disorder, but half of the patients with PCD have Kartagener syndrome. It is associated with the 3 listed findings, as well as reduced male fertility.

Question 32

A 15-year-old girl with asthma presents with recurrent episodes of:

• Malaise
• Coughing up brownish mucous plugs
• Occasional hemoptysis
• Peripheral eosinophilia
• High IgE

She improves with corticosteroid therapy, but 2–3 months later, the signs and symptoms recur.

What is likely causing the recurrent episodes?

Answer 32

Allergic Bronchopulmonary Aspergillosis (ABPA)

This is a classic presentation for ABPA. In addition to patients with asthma, it commonly occurs in patients with cystic fibrosis. Major clues here are the recurrent nature with the associated eosinophilia and very high IgE.

Question 33

A 10-year-old boy confirmed group A streptococcal pharyngitis. He is given an IM injection of penicillin G benzathine 1.2 million U.

When may he return to school?

Answer 33

The next day

Children become noninfectious within a few hours after penicillin therapy; therefore, if clinically improved, the child may return to school the next day.

Question 34

A 5-year-old girl presents with:

• A 12-day history of URI
• Worsening cough over the last few days, worse at night when she is supine
• Sore throat worsening the last few days
• Nasal discharge is clear

What is the most likely diagnosis?

Answer 34

Acute Bacterial Sinusitis

For children < 6 years of age, who have persistent respiratory symptoms that have not improved for > 10 but < 30 days, the diagnosis is acute bacterial sinusitis can be made on clinical grounds without imaging. Again, the nasal discharge “color” does lot matter; the key is the duration of the symptoms. Cough is much more common in the younger children than in adults with sinusitis, and, commonly, it will be worse at night in the supine position.

Question 35

Two weeks ago, a 17-year-old Filipino girl was visiting her grandfather in Bakersfield, CA and now presents with:

• Fever
• Cough
• Several pounds weight loss
• Chest pain
• Marked fatigue
• Erythema nodosum

What is the likely etiology for her symptoms?

Answer 35

Coccidioidomycosis (a.k.a. “Valley Fever”)

The girl has the classic presentation of coccidioidomycosis. Symptoms generally occur 1–3 weeks after visiting an endemic area and are very nonspecific. The finding of erythema nodosum or erythema multiforme makes this diagnosis highly likely in someone who has visited the desert southwest of Arizona or Southern California.

Question 36

A newborn presents with:

• Intermittent cyanosis especially when being fed
• Inability to pass a firm catheter through either nostril to a depth of 3 cm

What is the diagnosis?

Answer 36

Choanal Atresia

It is the most common congenital anomaly of the nose. Classically, infants present with cyanosis especially when feeding. Some infants will suck in their lips when they inspire. Failure to pass a firm catheter confirms the diagnosis. CT scan will also confirm the abnormality and location.

Question 37

A 3-year-old girl presents with:

• Rectal prolapse
• Nasal polyps

What is the most likely diagnosis?

Answer 37

Cystic Fibrosis

Nasal polyps occur in about 25% of patients with CF and finding them in a child < 12 years of age guides you toward CF as a possible diagnosis. About 20% of patients with CF have rectal prolapse during early childhood. Remember, at birth about 10–20% of CF patients present with bowel obstruction, which is manifested by meconium ileus.

Question 38

A 10-year-old girl presents with:

• Nasal stuffiness
• Mouth breathing
• A “nasal” voice
• History of recurrent pneumonia
• On physical exam, you note nasal polyps

For what diagnosis should she be screened and/or tested?

Answer 38

Cystic Fibrosis

Any child under the age of 12, who presents with nasal polyps, should be screened and/or tested for CF. CF is the most common cause of nasal polyps in children. Other things that can cause nasal polyps include chronic sinusitis and allergic rhinitis (for which this script does not give you clinical clues).

Question 39

A 13-year-old boy presents with an initial history of:

• Headache
• Fever
• Pharyngitis

This is followed in about a week with a cough. And, on lab testing, you find a positive cold agglutinin titer.

What is the most likely diagnosis?

Answer 39

Mycoplasma pneumoniae

Classically, it presents with a prodrome of headache, fever and pharyngitis, which then progresses to cough and clinical pneumonia. Cold agglutinins are classically found and elevated with infection due to this organism.

Question 40

A 3-month-old girl presents with:

• Hypotonia
• Symmetric proximal muscle weakness
• Difficulty feeding
• Tongue fasciculations

What is the diagnosis, and what gene carries this anomaly?

Answer 40

Spinal Muscular Atrophy Type I (Werdnig-Hoffman Syndrome); SMN1 gene found in chromosome 5q13

SMA type 1 presents before 6 months of age - if u see tongue fasciculations in a question in a child this age, look for SMA as an answer! SMA is the 2nd most common lethal autosomal recessive disorder; cystic fibrosis is # 1.

Question 41

A 17-year-old boy was visiting his grandparents in Indiana over the summer. Two months after cleaning out his grandfather’s chicken coop, he presents with:

• Mild respiratory symptoms
• Low grade fever
• CXR shows a few focal infiltrates with hilar adenopathy.

What is the most likely etiology of his symptoms?

Answer 41

Histoplasmosis

Most cases of histoplasmosis are asymptomatic or only mild symptoms like our patient here, although some patients have more severe respiratory symptoms. Classically, this is seen in the Mississippi and Ohio River valleys. It is associated with the droppings of chickens and bats.

Question 42

A 15-year-old boy with a current history of active influenza A presents with abrupt onset of:

• Fever
• Tachypnea
• Tachycardia
• Cyanosis
• CXR shows distinct pneumatoceles.

What is the most likely bacterium causing this illness?

Answer 42

Staphylococcus aureus

Classically, look for this organism to cause pneumonia in a patient with recent or current URI or influenza infection. They may be doing better and then suddenly develop abrupt new symptoms of fever, tachypnea, tachycardia, and cyanosis. Pneumatoceles are classic for S. aureus, but can also be seen in Streptococcus pyogenes pneumonia—the influenza history here helps you separate these two.

Question 43

A 3-year-old girl presents with abrupt onset of:

• High fever
• Sore throat
• Drooling
• Stridor

She is given treatment for croup but does not respond. She deteriorates rapidly and is intubated in the emergency department. Her parents don’t believe in immunizations.

What is the most likely diagnosis?

Answer 43

Epiglottitis

This is a classic presentation for epiglottitis on the background of no Haemophilus influenzae type b (Hib) immunization. Initially, it appears that the patient has laryngotracheobronchitis (viral croup), but after receiving appropriate therapy, the child continues to deteriorate rapidly and requires intubation (or tracheostomy).

Question 44

A 2-year-old boy presents with:

• Unilateral nasal discharge
• The mother has noted that the drainage seems to have a bad odor.

What is the most likely diagnosis?

Answer 44

Foreign Body

Various items can end up in a child’s nostril without anyone’s knowledge of how they got there. These items may include crayons, toys, erasers, paper, beads, beans, stones, pencils, and various foods.