Macrovascular involvement in a child with atypical hemolytic uremic syndrome

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F14%3A10293028" target="_blank" >RIV/00064203:_____/14:10293028 - isvavai.cz</a>

Alternative codes found

RIV/00216208:11130/14:10293028

Result on the web

<a href="http://dx.doi.org/10.1007/s00467-013-2713-3" target="_blank" >http://dx.doi.org/10.1007/s00467-013-2713-3</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1007/s00467-013-2713-3" target="_blank" >10.1007/s00467-013-2713-3</a>

Result language

angličtina

Original language name

Macrovascular involvement in a child with atypical hemolytic uremic syndrome

Original language description

Atypical hemolytic uremic syndrome (aHUS) is a disorder of the complement system which leads to thrombotic microangiopathy. It is caused by either acquired or hereditary defects in the activation or regulation of the alternative complement pathway and istherefore considered to be a disease of local complement dysregulation in microvasculature with predominantly renal involvement. However, extrarenal manifestations are observed in approximately one-fifth of aHUS patients, with the myocardium and centralnervous system (CNS) being involved most often. Additionally, there have been a few reports of aHUS with cerebral artery stenoses or periphereal gangrene, suggesting the possibility of 'macrovascular' involvement in aHUS. We present a child with early onset aHUS and a C3 gain-of-function mutation who developed cerebral artery stenoses, leading ultimately to death due to a massive stroke 9 days after successful renal transplantation under prophylactic eculizumab treatment. Similar cases

Czech name

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Czech description

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Type

J_x - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

CEP classification

FG - Paediatrics

OECD FORD branch

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Project

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Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2014

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Pediatric Nephrology

ISSN

0931-041X

e-ISSN

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Volume of the periodical

29

Issue of the periodical within the volume

7

Country of publishing house

US - UNITED STATES

Number of pages

5

Pages from-to

1273-1277

UT code for WoS article

000336434600020

EID of the result in the Scopus database

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