Complement disorders and hemolytic uremic syndrome.

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.

journal_name

Curr Opin Pediatr

authors

Joseph C,Gattineni J

doi

10.1097/MOP.0b013e32835df48a

subject

Has Abstract

pub_date

2013-04-01 00:00:00

pages

209-15

issue

2

eissn

1040-8703

issn

1531-698X

journal_volume

25

pub_type

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