Q:

What treatment option is not part of the standard management of atypical HUS?

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A 38-year-old male patien t is evaluated for fatigue. He has no other specific com-plaints; he den ies fever. chills. abdomi nal pain. and urinary symptoms. Family history includes a brother who died at age of 8 years of renal failure. Complete blood count shows leukocyte count of 7 . 800/ J.LL. hemoglobin 8.9 g/dl. and platelet count 9.000/ J.LL. Creatinine is 7.9 mg/dl. LDH is 2200 U/L. The peripheral blood smear is shown below.

What treatment option is not part of the standard management of atypical HUS?


  1. Cyclosporine
  2. Combined kidney-liver transplantation
  3. Eculizumab
  4. Plasma exchange

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A. Cyclosporine is not recommended in the treatment of typical or atypical HUS. Com-bined kidney-liver transplant is recommended for a subset of patients with high-risk genetic muta-tions. Eculizumab is a monoclonal antibody to human CS and has been approved for use in atypical HUS. Plasma exchange may be utilized in the acute treatment of atypical HUS.

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