| Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome. | |
| | |
MedLine Citation:
|
PMID: 21051740 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy that associates, in 70% of cases, with genetic or acquired disorders leading to dysregulation of the alternative pathway of complement. Autoantibody directed against Factor H causes at least 6% to 10% of aHUS cases, but only a few clinical reports are available. Here, we describe the clinical, biologic, genetic features, treatment, and outcome of 45 patients who presented with aHUS associated with anti-FH autoantibody. We found that this form of aHUS primarily affects children between 9 and 13 years old but it also affects adults. It presents with a high frequency of gastrointestinal symptoms and with extrarenal complications and has a relapsing course. Activation of the alternative pathway of complement at the onset of disease portends a poor prognosis. Early specific treatment may lead to favorable outcomes. These data should improve the recognition and diagnosis of this form of aHUS and help identify patients at high risk of a poor outcome. |
| | |
Authors:
|
Marie-Agnès Dragon-Durey; Sidharth Kumar Sethi; Arvind Bagga; Caroline Blanc; Jacques Blouin; Bruno Ranchin; Jean-Luc André; Nobuaki Takagi; Hae Il Cheong; Pankaj Hari; Moglie Le Quintrec; Patrick Niaudet; Chantal Loirat; Wolf Herman Fridman; Véronique Frémeaux-Bacchi |
Publication Detail:
|
Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-11-04 |
Journal Detail:
|
Title: Journal of the American Society of Nephrology : JASN Volume: 21 ISSN: 1533-3450 ISO Abbreviation: J. Am. Soc. Nephrol. Publication Date: 2010 Dec |
Date Detail:
|
Created Date: 2010-12-01 Completed Date: 2011-01-07 Revised Date: 2011-12-21 |
Medline Journal Info:
|
Nlm Unique ID: 9013836 Medline TA: J Am Soc Nephrol Country: United States |
Other Details:
|
Languages: eng Pagination: 2180-7 Citation Subset: IM |
Affiliation:
|
Service d'Immunologie Biologique, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France. marie-agnes.durey@egp.aphp.fr |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Age Factors Autoantibodies / immunology* Child Child, Preschool Cohort Studies Complement Activation / immunology Complement Factor H / immunology* Female Follow-Up Studies Hemolytic-Uremic Syndrome / diagnosis, epidemiology, immunology*, therapy* Humans Immunoglobulins, Intravenous / administration & dosage Infant Kidney Transplantation Male Middle Aged Plasma Exchange / methods Risk Assessment Severity of Illness Index Sex Factors Stem Cell Transplantation / methods Time Factors Treatment Outcome |
| Chemical | |
Reg. No./Substance:
|
0/Autoantibodies; 0/Immunoglobulins, Intravenous; 80295-65-4/Complement Factor H |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Lack of renal dopamine D5 receptors promotes hypertension.
Next Document: A new role for charge of the glomerular capillary membrane.