La sindrome emolitico uremica.

Translated title of the contribution: Hemolytic uremic syndrome

S. Rota, P. Cravedi, G. Remuzzi, P. Ruggenenti

Research output: Contribution to journalArticlepeer-review

Abstract

Hemolytic uremic syndrome (HUS) is a disease characterized by non immune hemolytic anemia, low platelet count and renal impairment. In children, the disease is most commonly triggered by Shiga-like toxin (Stx)-producing Escherichia coli (Stx-E. Coli): however, renal function recovers in up to 70% of patients. Plasma infusion or exchange reduces mortality and the risk of end-stage renal disease (ESRD) in adult patients. Non-Shiga toxin-associated HUS (non-Stx-HUS), accounting for only 5-10% of all disease cases, can be sporadic or familial. Collectively, non-Stx-HUS forms have a poor outcome. Up to 50% of cases progress to ESRD or have irreversible brain damage, and 25% can die during the acute phase of the disease. Genetic studies have recently documented that the familial form is associated with genetic abnormalities of complement regulatory proteins, and evidence is now emerging that similar genetic alterations can predispose to sporadic cases of non-Stx-HUS as well. Mutations of genes encoding for factor H, a glycoprotein that plays an important role in the regulation of the alternative pathway of complement and for MCP, a widely expressed transmembrane glycoprotein with an inhibitory role of activated C3, are reported in familial HUS. These mutations are more likely to predispose rather than to cause the disease directly.

Translated title of the contributionHemolytic uremic syndrome
Original languageItalian
JournalGiornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia
Volume22 Suppl 33
Publication statusPublished - Nov 2005

ASJC Scopus subject areas

  • Nephrology

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