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A 12 year old boy has a productive cough characterized by large volumes of mucoid, foul smelling sputum. He has a history of recurrent pneumonia, epigastric pain and increased stool frequency since age 2. His mother states he was delivered via a normal vaginal delivery with meconium ileus noted at birth. His vitals are normal but his temp is 100.4 f. Physical exam shows height and weight in 39th percentile, a few small nasal polyps bilaterally, diffuse crackles on lung auscultation, hyperresonace with chest percussion and scattered petechiae and purpura. Cultures of sputum grow oxidase positive colonies with a fruity aroma on blood agar. 1. What underlying condition does this boy have? Why do you think this? 2. What is he likely infected with and why? 3. What is the most likely mechanism of action of the exotoxin produced by the causal organism?

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The General Principles section of the USMLE covers these topics:
Biochemistry
Genetics
Microbiology
Pathology
Pharmacology


1. A 12 year old boy has a productive cough characterized by large volumes of mucoid, foul smelling sputum. He has a history of recurrent pneumonia, epigastric pain and increased stool frequency since age 2. His mother states he was delivered via a normal vaginal delivery with meconium ileus noted at birth. His vitals are normal but his temp is 100.4 f. Physical exam shows height and weight in 39th percentile, a few small nasal polyps bilaterally, diffuse crackles on lung auscultation, hyperresonace with chest percussion and scattered petechiae and purpura. Cultures of sputum grow oxidase positive colonies with a fruity aroma on blood agar. 1. What underlying condition does this boy have? Why do you think this? 2. What is he likely infected with and why? 3. What is the most likely mechanism of action of the exotoxin produced by the causal organism?





 
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