Cystic fibrosis Quiz: Ace Your Exams

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A 14-year-old boy is brought to the physician for a routine checkup by his parents. The patient has a past medical history of recurrent pneumonia requiring multiple hospitalizations for intravenous antibiotic therapy. The patient was adopted at a young age and his parents lack information on his biological family history. They state that the patient has had episodes of chronic, foul-smelling diarrhea for the past several years. Vitals are within normal limits. Lung auscultation reveals occasional crackles. The liver is palpated 3 cm below the costal margin and is not tender. Laboratory results are as follows:  
 
 Laboratory value  Result 
 Aspartate aminotransferase   67 U/L 
 Alanine aminotransferase   72 U/L 
 Alkaline phosphatase  142 U/L 
 Total bilirubin  1.0 mg/dL 
 Direct bilirubin   0.3 mg/dL 
 p-ANCA  Negative 

Which of the following is the most likely cause for this patient’s abnormal laboratory results?

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