Atypical Hemolytic Uremic Syndrome: Atypical Course and Atypical Mutations Combination
نویسندگان
چکیده
منابع مشابه
Atypical 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
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
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 کاملAn Atypical Cause Of Atypical Hemolytic Uremic Syndrome
Atypical hemolytic syndrome is an extremely rare, life threatening, progressive disease. Approximately one to two cases per million are seen annually in the US. It is known to be associated with variety of conditions including infections, drugs, autoimmune conditions, vaccination, malignancy, organ transplantation, pregnancy, and metabolic conditions. We hereby describe a case of atypical hemol...
متن کاملHow atypical can Atypical Hemolytic Uremic Syndrome be?
KEY CLINICAL MESSAGE A 24-year-old man with diarrhea found to have acute renal failure with microangiopathic hemolytic anemia (MAHA). A diagnosis of hemolytic uraemic syndrome (HUS) was made. He was initiated on plasma exchange and hemodialysis. On day 6, he was started on eculizumab. His renal functions progressively improved. His main complication during eculizumab therapy was hypertension-re...
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: Open Access Journal of Urology & Nephrology
سال: 2017
ISSN: 2578-4676
DOI: 10.23880/oajun-16000114