Atypical hemolytic-uremic syndrome associated with antiphospholipid antibodies and antiphospholipid syndrome; a novel presentation
نویسندگان
چکیده
Introduction Atypical hemolytic-uremic syndrome (HUS) is defined by the presence of microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia without a diarrheal prodrome. It is responsible for only ten percent of cases in children. The role of genetic deficiencies of complement regulation, Von Willebrand factor cleaving protease (ADAMTS 13), and intracellular defects of vitamin B12 metabolism has been known in pathogenesis of disease. Antiphospholipid antibodies (aPLs) are autoantibodies against negatively charged phospholipids or phospholipid binding plasma proteins. It is clear that their presence is associated with thrombosis, pregnancy morbidity, hematologic, skin, neurological conditions, and microangiopathy.
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