Hemolytic Uremic Syndrome
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چکیده
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 PATHOGENESIS Although many cases of HUS/TTP are idiopathic, several underlying causes have been identified and are listed in Table 1. Most cases of childhood HUS and occasional cases in adults follow infection by enterohemorrhagic E.coli (typically E.coli 0157:H7). A toxin released from these bacteria is responsible for the manifestations and since it is identical to the toxin produced by Shigella dysenteriae, it is called Shiga toxin (Stx). The toxin has a predilection for the renal circulation since receptors for it are preferentially expressed in the kidney. Both endothelial injury and activation of platelets and neutrophils may contribute to Stxinduced HUS. The toxin can also cause apoptosis and significant damage to renal tubular epithelial cells.5 Autosomal recessive and dominant forms of familial HUS comprise less than 5% of all cases of HUS. Mutations in the gene for factor H, a serum complement regulatory protease, account for some of these. This gene is located on chromosome 1q32.
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...
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Background and Objective: Hemolytic Uremic Syndrome (HUS) is a thrombotic microangiopathy that contains hemolytic anemia, thrombocytopenia, renal failure and other organ involvement including central nervous system. Frequency of neurologic complications in patients with HUS is high and important cause of mortality. This study was performed to determine seizure disorders in term of acute neurolo...
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The purpose of this study has been to evaluate various factors influencing prognosis in children with hemolytic-uremic syndrome (HUS). Forty children with classical picture of HUS were seen in 1986-1 991. Boys and girls were equally affected, aged from two months to ten years. In 35 patients (87.5%) there was a history of diarrhea which was bloody in25. All were treated with peritoneal dial...
متن کاملHemolytic uremic syndrome with simultaneous Shiga toxin producing Escherichia coli and complement abnormalities
BACKGROUND Hemolytic uremic syndrome is a common cause of acute kidney injury in children. In children, hemolytic uremic syndrome is most commonly associated with gastrointestinal infections caused by Shiga toxin-producing Escherichia coli or other enteric organisms. Although less common, atypical hemolytic uremic syndrome is triggered by multiple factors and portends a significantly worse prog...
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تاریخ انتشار 2009