Nephrotic syndrome associated with primary atypical hemolytic uremic syndrome

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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...

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Atypical 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...

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Atypical 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 -...

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Pregnancy-Associated Atypical Hemolytic-Uremic Syndrome.

Introduction Early diagnosis of atypical uremic-hemolytic syndrome may be challenging during the puerperium period. Correct diagnosis and timely management are crucial to improve outcomes. Background Pregnancy-associated atypical hemolytic-uremic syndrome (p-aHUS) is a rare condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Triggered by preg...

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Atypical hemolytic uremic syndrome (aHUS) associated with membranoproliferative glomerulonephritis (MPGN) is an uncommon clinical presentation, especially in children. We report a 8-year-old-boy who presented like aHUS but the kidney biopsy showed MPGN type 1.

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ژورنال

عنوان ژورنال: Brazilian Journal of Nephrology

سال: 2020

ISSN: 2175-8239,0101-2800

DOI: 10.1590/2175-8239-jbn-2020-0050