Complement disorders and hemolytic uremic syndrome

Catherine Joseph; Jyothsna Gattineni

doi:10.1097/MOP.0b013e32835df48a

Complement disorders and hemolytic uremic syndrome

Catherine Joseph, Jyothsna Gattineni

Research output: Contribution to journal › Review article › peer-review

36 Scopus citations

Abstract

PURPOSE OF REVIEW: Complement mediated hemolytic uremic syndrome (aHUS) accounts for a significant proportion of non-shiga toxin HUS. The purpose of this review is to outline the pathophysiology, clinical features and therapeutic options for aHUS. RECENT FINDINGS: In the last decade, strides have been made in identifying several new disease-causing mutations in complement-regulating proteins. SUMMARY: Complement mediated HUS (aHUS) has a worse prognosis compared with shiga toxin mediated HUS, often resulting in end stage renal disease. Early identification of aHUS is crucial so that plasma therapy can be initiated. After renal transplantation, there is very high risk of disease recurrence and graft loss. Eculizumab and combined liver-kidney transplantation offer promise for improved prognosis.

Original language	English (US)
Pages (from-to)	209-215
Number of pages	7
Journal	Current opinion in pediatrics
Volume	25
Issue number	2
DOIs	https://doi.org/10.1097/MOP.0b013e32835df48a
State	Published - Apr 2013

Keywords

Factor H
Factor I
Membrane cofactor protein
Plasma exchange

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health

Access to Document

10.1097/MOP.0b013e32835df48a

Cite this

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abstract = "PURPOSE OF REVIEW: Complement mediated hemolytic uremic syndrome (aHUS) accounts for a significant proportion of non-shiga toxin HUS. The purpose of this review is to outline the pathophysiology, clinical features and therapeutic options for aHUS. RECENT FINDINGS: In the last decade, strides have been made in identifying several new disease-causing mutations in complement-regulating proteins. SUMMARY: Complement mediated HUS (aHUS) has a worse prognosis compared with shiga toxin mediated HUS, often resulting in end stage renal disease. Early identification of aHUS is crucial so that plasma therapy can be initiated. After renal transplantation, there is very high risk of disease recurrence and graft loss. Eculizumab and combined liver-kidney transplantation offer promise for improved prognosis.",

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AB - PURPOSE OF REVIEW: Complement mediated hemolytic uremic syndrome (aHUS) accounts for a significant proportion of non-shiga toxin HUS. The purpose of this review is to outline the pathophysiology, clinical features and therapeutic options for aHUS. RECENT FINDINGS: In the last decade, strides have been made in identifying several new disease-causing mutations in complement-regulating proteins. SUMMARY: Complement mediated HUS (aHUS) has a worse prognosis compared with shiga toxin mediated HUS, often resulting in end stage renal disease. Early identification of aHUS is crucial so that plasma therapy can be initiated. After renal transplantation, there is very high risk of disease recurrence and graft loss. Eculizumab and combined liver-kidney transplantation offer promise for improved prognosis.

KW - Factor H

KW - Factor I

KW - Membrane cofactor protein

KW - Plasma exchange

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Complement disorders and hemolytic uremic syndrome

Abstract

Keywords

ASJC Scopus subject areas

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