La sindrome emolitico uremica.

Translated title of the contribution: Hemolytic uremic syndrome

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

Research output: Contribution to journalArticle

1 Citation (Scopus)

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.

Original languageItalian
JournalGiornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia
Volume22 Suppl 33
Publication statusPublished - Nov 2005

Fingerprint

Hemolytic-Uremic Syndrome
Chronic Kidney Failure
Glycoproteins
Shiga Toxins
Shiga-Toxigenic Escherichia coli
Alternative Complement Pathway
Kidney
Complement Factor H
Mutation
Hemolytic Anemia
Acute Disease
Platelet Count
Complement System Proteins
Mortality
Brain
Genes

ASJC Scopus subject areas

  • Nephrology

Cite this

La sindrome emolitico uremica. / Rota, S.; Cravedi, P.; Remuzzi, G.; Ruggenenti, P.

In: Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia, Vol. 22 Suppl 33, 11.2005.

Research output: Contribution to journalArticle

@article{db84017312594c6cb350753a6f076756,
title = "La sindrome emolitico uremica.",
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.",
author = "S. Rota and P. Cravedi and G. Remuzzi and P. Ruggenenti",
year = "2005",
month = "11",
language = "Italian",
volume = "22 Suppl 33",
journal = "Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia",
issn = "0393-5590",
publisher = "Wichtig Publishing Srl",

}

TY - JOUR

T1 - La sindrome emolitico uremica.

AU - Rota, S.

AU - Cravedi, P.

AU - Remuzzi, G.

AU - Ruggenenti, P.

PY - 2005/11

Y1 - 2005/11

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=33645957965&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=33645957965&partnerID=8YFLogxK

M3 - Articolo

VL - 22 Suppl 33

JO - Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia

JF - Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia

SN - 0393-5590

ER -