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Question 1 of 23
1. Question
Investigators study the prevalence of respiratory pathogens in patients with cystic fibrosis across the age spectrum. It is found that age is a strong predictor of colonization by a specific microbe, as shown below.
The pathogen, once acquired, often switches into mucoid phenotype, which is able to produce an alginate-containing biofilm matrix that helps to establish chronic infection. Which of the following bacteria was most likely found in this study?
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Question 2 of 23
2. Question
A 14-year-old boy is brought to the physician by his mother due to chronic diarrhea and failure to gain weight appropriately. He has a history of recurrent respiratory infections, and prior sputum cultures have been positive for Pseudomonas aeruginosa. His younger brother died from a severe respiratory infection at age 9. Which of the following agents is most likely to improve this patient’s condition in the long term?
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Question 3 of 23
3. Question
A 6-year-old boy has a history of 5 bone fractures, 3 of them occurring after only minimal trauma. His birth was unremarkable following a normal pregnancy. The patient has been in the 50th percentile for both height and weight since birth, and he has reached all developmental milestones. On physical examination, he is a pleasant, interactive child with small, malformed teeth. Further evaluation shows the finding in the image below.
This patient’s condition is most likely associated with impairment of which of the following processes?
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Question 4 of 23
4. Question
A 3-year-old boy is brought to the office by his parents. They say that the child cannot keep up with his cousin, who is the same age. The boy climbs up from a squat by using his hands to push off the ground and his lower extremities. His calves appear enlarged on physical examination. This patient’s condition is most likely associated with which of the following?
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Question 5 of 23
5. Question
A 2-day-old boy develops abdominal distension and refuses to breastfeed. He was born via normal spontaneous vaginal delivery at 39 weeks gestation to a primigravid mother. Examination shows significant abdominal distension with palpable intestinal loops. The rectum has no stool and rectal tone is normal. During the examination, the infant has several episodes of dark green emesis. Plain films of the abdomen show air-fluid levels and small bowel dilation. Contrast enema fails to relieve the obstruction, and the patient is taken for emergency laparotomy, which shows an inspissated, green fecal mass obstructing the distal ileum. The infant recovers from the surgery uneventfully. Which of the following will be the most likely cause of mortality in this patient?
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Question 6 of 23
6. Question
A 3-year-old boy is brought to the office with 5 days of productive cough and fever. Examination shows a tachypneic, ill-appearing child with rales over the right lower lung. Chest x-ray reveals right lower lobe pneumonia. The boy is admitted to the hospital for intravenous antibiotics. Review of his medical history shows 4 prior pneumonias, and his weight is at the third percentile. Further testing demonstrates high sweat chloride content, and genetic sequencing shows a mutation in a transmembrane protein. Which of the following best describes the dysfunctional transmembrane protein causing this patient’s disease?
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Question 7 of 23
7. Question
The pancreas of a 22-year-old man with recurrent pulmonary infections and finger clubbing shows extensive exocrine gland atrophy and fibrosis. The pancreatic ducts are lined with squamous epithelium with areas of keratinization. The finding of squamous metaplasia in the pancreatic ducts is most likely related to a deficiency of:
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Question 8 of 23
8. Question
A 1-month-old boy is brought to the office on a hot summer day due to vomiting and excessive sleepiness. He had been exclusively breastfeeding with normal feeding patterns until this afternoon, when he had 2 episodes of emesis and appeared much sleepier than usual. The infant was born at 41 weeks gestation after an unremarkable pregnancy. Newborn screening was positive for a homozygous ΔF508 mutation affecting an epithelial transmembrane protein; further confirmatory testing is pending. Physical examination shows a somnolent boy with a sunken fontanelle and dry mucous membranes. Which of the following electrolyte disturbances is most likely responsible for this patient’s current symptoms?
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Question 9 of 23
9. Question
A 6-year-old boy is brought to the office by his mother due to recurrent episodes of sinusitis. He also has a chronic cough that never seems to go away. The mother says that his previous pediatrician performed a “sweat test” to help determine the cause of his frequent infections, but results were normal. DNA testing was inconclusive. Despite these results, the new physician suspects that the patient has an inherited disorder and orders nasal transepithelial potential difference measurements. In this test, an isotonic solution of sodium chloride is applied to the nasal mucosal surface, and the electrical potential overlying the mucosa is compared with that of the interstitial fluid. The results show a transepithelial potential difference that is more negative than normal. This patient’s nasal mucosa is most likely to demonstrate which of the following physiologic changes?
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Question 10 of 23
10. Question
A 35-year-old woman, gravida 1 para 0, comes to the office for an initial prenatal visit. The patient has had no vaginal bleeding or abdominal pain and has not yet felt fetal movement. Menarche occurred at age 13. She sometimes has heavy menstrual bleeding with passage of clots, and other times she skips her menses. The patient’s estimated gestational age based on her last menstrual period is 16 weeks. She has type 1 diabetes mellitus that is controlled with insulin. She smokes a pack of cigarettes daily. As part of her prenatal laboratory screening, a second-trimester maternal serum quadruple screen is performed and reveals an elevated alpha-fetoprotein level. Which of the following is the most likely etiology of this patient’s abnormal screening result?
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Question 11 of 23
11. Question
A 21-year-old man comes to the clinic due to weakness and gait disturbance. For the past few months, he has noticed difficulty opening the lids on jars, along with trouble releasing his grip afterward. The patient has also tripped and fallen several times, particularly when walking up stairs. He has no known chronic medical conditions and takes no medications. Vital signs are normal. The patient has male pattern baldness. Cardiopulmonary examination is unremarkable. The abdomen is soft with no organomegaly. A muscle biopsy shows muscle atrophy involving mostly type 1 fibers. This patient most likely has a form of which of the following neuromuscular diseases?
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Question 12 of 23
12. Question
A 10-year-old boy is brought to the clinic due to stiffness and weakness in his hands. A few weeks ago, the parents first noticed that the child was having trouble letting go of his toothbrush after brushing his teeth. The patient’s teacher has also mentioned that the boy seems to have difficulty maintaining a firm grip on his pencil when doing schoolwork. He has a history of intellectual disability and takes no medications. Examination shows bilateral cataracts. Laboratory evaluation shows unstable cytosine-thymine-guanine trinucleotide repeats, resulting in the transcription and accumulation of untranslatable mutant mRNAs, which negatively affect cellular function. Which of the following cell types is likely to be most affected by this patient’s condition?
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Question 13 of 23
13. Question
A 7-year-old girl is brought to the emergency department due to 2 days of fever, productive cough, and shortness of breath. Medical history is significant for recurrent lung infections and cystic fibrosis. Weight is at the 5th percentile. Temperature is 38.1 C (100.6 F), pulse is 104/min, and respirations are 30/min. Pulse oximetry is 86% on room air. Examination shows a thin girl with digital clubbing and a hyperexpanded chest. Diffuse wheezes and crackles are present bilaterally. The rest of the examination is unremarkable. Chest x-ray reveals hyperinflation, chronic interstitial changes, and an infiltrate in the right lower lobe. Gram stain of sputum shows gram-negative rods. Which of the following is the most likely cause of this patient’s recurrent infections?
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Question 14 of 23
14. Question
A 7-year-old girl is brought to the clinic due to nasal congestion with facial pain. The patient first developed congestion over a year ago. Her parents have been treating it with over-the-counter allergy medications, but there has been no improvement. Over the past 4 months, the congestion has worsened, and now the patient has difficulty breathing through her nose. She also developed a dry cough and a constant, dull pain over her cheeks. The patient’s stools have been loose. Temperature is 37.8 C (100 F). Weight is at <1st percentile at 18 kg (39.7 lb), decreased from 19 kg (41.9 lb) 3 months ago. Examination reveals copious yellow mucus within both nares. Translucent, gray, shiny masses obscure the middle turbinates bilaterally. Lymphadenopathy is not present. The lungs have coarse breath sounds bilaterally. The abdomen is soft with no organomegaly. Which of the following is the most likely diagnosis?
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Question 15 of 23
15. Question
A 15-month-old boy is brought to the office by his parents for poor weight gain. The parents have also noticed frequent, large, foul-smelling stools. He was admitted to the hospital for a prolonged bout of bacterial pneumonia 6 months ago. Both parents are healthy and have no similar symptoms. The child was born at term at home. He is at the 20th percentile for length and 3rd percentile for weight. Vital signs are normal. On examination, the child appears thin and has a nontender, nondistended abdomen. Which of the following is most likely present in this patient?
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Question 16 of 23
16. Question
A 20-year-old woman with cystic fibrosis comes to the clinic for routine follow-up. She has no concerns since her last visit 3 months ago. Vital signs are normal. Examination shows a hyperexpanded chest with equal aeration bilaterally. Fasting glucose level is 90 mg/dL. Plasma glucose level 2 hours after oral glucose tolerance testing is 185 mg/dL (normal: <140). Which of the following is the primary mechanism responsible for this patient’s abnormal test result?
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Question 17 of 23
17. Question
A 17-year-old girl is brought to the emergency department due to hemoptysis and severe respiratory distress. The patient has been on several oral antibiotics for pneumonia over the past week and has required numerous similar treatments in the past. She has been coughing up blood for the past day, which has become progressively worse. Temperature is 37.0 C (98.6 F), blood pressure is 90/60 mm Hg, pulse is 120/min, and respirations are 42/min. Pulmonary examination reveals diffusely reduced air flow, rales, and intercostal retractions. Copious red blood is produced while coughing during the examination and progresses rapidly. Despite aggressive management, the patient ultimately expires. An autopsy is performed and a gross lung specimen is shown in the image below:
Which of the following is the most likely etiology of this patient’s hemoptysis?
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Question 18 of 23
18. Question
A 19-year-old man with a history of chronic lung problems dies due to respiratory distress after a prolonged episode of pneumonia. Autopsy shows dilation of the bronchial tree, which extends almost to the pleural surface. Many of the small airways are filled with mucin plugs containing abundant inflammatory cells and debris. Which of the following is the most likely cause of this patient’s autopsy findings?
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Question 19 of 23
19. Question
A 25-year-old nulligravid woman comes with her husband to the clinic for preconception genetic counseling. She has oculocutaneous albinism due to a homozygous OCA2 gene mutation within the region of chromosome 15q12-q13. Examination shows pale hypopigmented skin with blonde hair. Eye examination shows faint brown irises. Her husband is 26 years old and has oculocutaneous albinism due to a biallelic TYR gene mutation at position 11q14.3. Examination of the husband shows complete absence of pigmentation in the skin, hair, and irises. The couple asks about their chance of having a child with oculocutaneous albinism and are told that the chance is 0%. Which of the following is the best explanation for this?
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Question 20 of 23
20. Question
A 50-year-old previously healthy man is evaluated for progressive fatigue, weakness, and recurrent gingival bleeding. Laboratory studies reveal normocytic normochromic anemia, thrombocytopenia, and leukocytosis with circulating myeloblasts. Bone marrow biopsy establishes a diagnosis of acute myeloid leukemia. Induction chemotherapy followed by allogeneic hematopoietic cell transplantation (HCT) is planned. Molecular typing of human leukocyte antigen (HLA) -A, -B, -C, -DP, -DQ, and -DR is performed. The patient’s biological sister, with whom he shares both parents, is eligible for stem cell donation and undergoes HLA typing. Which of the following is the most likely probability that the sibling will be an identical HLA match with this patient?
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Question 21 of 23
21. Question
An 8-year-old boy is brought to the clinic due to behavioral problems. The patient has a history of intellectual disability and is in special education classes. For the last few months, his teachers have reported that he has had increasing difficulty following directions and maintaining his attention. The parents have recently noticed that the child seems to have muscle weakness in his hands. The patient’s mother developed a similar weakness several years ago. Vital signs are normal. Eye examination shows bilateral lenticular opacities. Firm percussion over the thenar eminence results in prolonged thumb abduction with slow muscle relaxation. Further workup would most likely reveal which of the following?
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Question 22 of 23
22. Question
A 27-year-old man comes to the office due to dyspnea on exertion and intermittent episodes of palpitations. Blood pressure is 130/80 mm Hg and pulse is 78/min. On examination, there is no peripheral edema or jugular venous distension. The lungs are clear to auscultation. There is a 3/6 ejection-type systolic murmur along the left sternal border that decreases in intensity when the patient moves from the standing to the squatting position. Which of the following is the most likely form of inheritance of this patient’s condition?
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Question 23 of 23
23. Question
A 1-day-old boy is evaluated in the neonatal intensive care unit due to severe hypotonia, poor feeding, and respiratory distress. The patient was born to a 30-year-old woman via vaginal delivery; the pregnancy was complicated by polyhydramnios. The neonate’s mother has a history of recurrent muscle cramps, mostly in her hands; her face is long and narrow and lacks expression. She is otherwise healthy. The patient’s length, weight, and head circumference are at the 30th percentile. Examination shows profound hypotonia, truncal and appendicular weakness, and marked hyporeflexia. Flexion deformities and clubfoot are present bilaterally. Assuming that the patient and his mother have the same inheritable condition, which of the following mechanisms best explains their different phenotypic presentations?
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