Hemolytic uremic syndrome: sound minds, sick kidneys

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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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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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Hemolytic Uremic Syndrome

below), the emphasis for differentiating HUS from TTP has shifted from the pattern of clinical involvement to the presence or absence of the enzyme deficiency. It is now suggested that the diagnosis of TTP should be used to describe only those patients who have this enzyme deficiency; while patients without the enzyme deficiency should be labeled as suffering from HUS.4 ETIOLOGY AND PATHOGENESI...

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ژورنال

عنوان ژورنال: Developmental Medicine & Child Neurology

سال: 2013

ISSN: 0012-1622

DOI: 10.1111/dmcn.12155