A 28-year-old female with a history of progressive hepatosplenomegaly and epistaxis was admitted to the hospital with severe pain in her right thigh of 3 days' duration. A similar episode occurred 5 months earlier. There is no family history of these symptoms, and her 2 siblings are unaffected. A CT scan reveals flaring of the right distal femur. Results of a neurologic examination are normal. A bone marrow biopsy demonstrates the cells characteristic of her disease (shown below - macrophages with crinkled paper appearance). What is the material accumulating abnormally in these cells?

  1. A)Sphingomyelin
  2. B)Dermatan sulfate
  3. C)Fatty acyl carnitines
  4. D)Cholesterol esters
  5. E)GlucocerebrosideGABARITO

Explicação

The correct answer is D. Source: Kaplan USMLE Step 1 QBank — Biochemistry Test 2. Ver explicação completa e trilha adaptativa →

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