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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.
Which statement is true regarding atypical HUS?
- Atypical HUS accounts for 60% of HUS cases in children
- Non-Shigella gastrointestinal illness prodrome is usually present
- Prognosis is better for atypical HUS than classic HUS
- Infection and genetic mutations are both causes of atypical HUS
Medicine
D. Atypical HUS cases account for about 10% ofHUS in children. Although infections can precede atypical HUS, the diarrheal prodrome characteristic of HUS is usually not present. Patients with familial atypical HUS have a poor prognosis, with end-stage renal disease or death in 50% to 80% of patients. Sporadic atypical HUS may be caused by a variety of conditions, including infections, cancer and antineoplastic agents, pregnancy, systemic lupus, organ transplantation, and medications such as cyclosporine and ticlopidine.
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