Atypical Hemolytic Uremia Syndrome in A Young Adult Male
نویسندگان
چکیده
Atypical hemolytic uremia syndrome (aHUS) is a rare, life-threatening disease, characterized by the same triad of anemia, thrombocytopenia and renal failure as seen in (HUS). It differs its etiology, being caused dysregulation complement pathway rather than Shiga-like toxin-producing Escherichia coli (STEC). Diagnosis usually after exclusion STEC infection also thrombotic thrombocytopenic purpura (TTP). The prognosis aHUS poor, with 50% cases progressing to end-stage disease 25% resulting mortality acute phase. treatment choice plasma exchange which can lower mortality. Monoclonal antibody drugs such eculizumab, suppress dysregulated help prevent mediated kidney injury, but remain prohibitively expensive.
منابع مشابه
Concurrent Atypical Hemolytic Uremic Syndrome and Autoimmune Hemolytic Anemia: a case report
Background: Atypical hemolytic uremic syndrome (aHUS) is a life-threatening and scarce disorder characterized by acute renal failure and disease, non-immune microangiopathic hemolytic anemia and thrombocytopenia, leading to end-stage renal failure or death, and consequently maybe accompanying by extra renal manifestations. Case report: We reported aHUS accompanied by autoimmune hemolytic anemi...
متن کاملAtypical hemolytic uremic syndrome
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Most cases of aHUS are caused by uncontrolled complement activation due to genetic mutations in the alternative pathway of complement. More recently, mutations in the gene of coagulation system have also been identified in...
متن کاملAtypical hemolytic uremic syndrome
Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5 -...
متن کاملAtypical Hemolytic Uremic Syndrome
Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulatory proteins have been described. Incomplete penetrance of mutations in all predisposing genes is r...
متن کاملAtypical Hemolytic Uremic Syndrome: A Brief Review
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majo...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Indian Journal of Case Reports
سال: 2022
ISSN: ['2454-129X', '2454-1303']
DOI: https://doi.org/10.32677/ijcr.v8i10.3498