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Pediatric Nephrology
Published in: Pediatric Nephrology 8/2019

01-08-2019 | Educational Review

Extra-renal manifestations of atypical hemolytic uremic syndrome

Authors: Cassandra Formeck, Agnieszka Swiatecka-Urban

Published in: Pediatric Nephrology | Issue 8/2019

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Abstract

Atypical hemolytic uremic syndrome (aHUS) is a rare and complex disease resulting from abnormal alternative complement activation with a wide range of clinical presentations. Extra-renal manifestations of aHUS can involve many organ systems, including the peripheral and central nervous, gastrointestinal, cardiovascular, integumentary, pulmonary, as well as the eye. While some of these extra-renal manifestations occur in the acute phase of aHUS, some can also occur as long-term sequelae of unopposed complement activation. Extra-renal symptoms are observed in approximately 20% of patients with aHUS, with the incidence of specific organ system complications ranging from a few case reports to 50% of described patients. Careful monitoring for extra-renal involvement is critical in patients with aHUS, as prompt evaluation and management may decrease the risk of high morbidity and mortality associated with aHUS.
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Metadata
Title
Extra-renal manifestations of atypical hemolytic uremic syndrome
Authors
Cassandra Formeck
Agnieszka Swiatecka-Urban
Publication date
01-08-2019
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Nephrology / Issue 8/2019
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI
https://doi.org/10.1007/s00467-018-4039-7

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