Document Detail


Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations.
MedLine Citation:
PMID:  20203157     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In contrast to pregnancy-associated thrombotic thrombocytopenic purpura, the pathogenesis and presentation of pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) remain ill-defined. We conducted a retrospective study to assess the presentation and outcomes of patients presenting with P-aHUS and the prevalence of alternative C3 convertase dysregulation. P-aHUS occurred in 21 of the 100 adult female patients with atypical HUS, with 79% presenting postpartum. We detected complement abnormalities in 18 of the 21 patients. The outcomes were poor: 62% reached ESRD by 1 month and 76% by last follow-up. The risk for P-aHUS was highest during a second pregnancy. Thirty-five women, 26 (74%) of whom had complement abnormalities, had at least one pregnancy before the onset of a non-pregnancy-related aHUS. Outcomes did not differ between patients with pregnancy-related and non-pregnancy-related aHUS. Mutations in the SCR19-20 domains of factor H were less frequent in P-aHUS patients compared with non-pregnancy-related aHUS. Pregnancies in female patients with complement abnormalities (n = 44) were complicated by fetal loss and preeclampsia in 4.8% and 7.7%, respectively. Better understanding of complement dysregulation in pregnancy complications is essential, especially to guide development of pharmacologic agents to modulate this system.
Authors:
Fadi Fakhouri; Lubka Roumenina; François Provot; Marion Sallée; Sophie Caillard; Lionel Couzi; Marie Essig; David Ribes; Marie-Agnès Dragon-Durey; Frank Bridoux; Eric Rondeau; Veronique Frémeaux-Bacchi
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-03-04
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 May 
Date Detail:
Created Date:  2010-05-03     Completed Date:  2010-06-21     Revised Date:  2013-05-30    
Medline Journal Info:
Nlm Unique ID:  9013836     Medline TA:  J Am Soc Nephrol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  859-67     Citation Subset:  IM    
Affiliation:
Department of Nephrology and UMR 643, CHU de Nantes, Paris, France.
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MeSH Terms
Descriptor/Qualifier:
Adult
Complement C3 Convertase, Alternative Pathway / genetics,  metabolism*
Female
Hemolytic-Uremic Syndrome / enzymology*,  genetics
Humans
Pregnancy
Pregnancy Complications, Hematologic / enzymology*,  genetics
Pregnancy Outcome
Retrospective Studies
Young Adult
Chemical
Reg. No./Substance:
EC 3.4.21.47/Complement C3 Convertase, Alternative Pathway
Comments/Corrections
Comment In:
J Am Soc Nephrol. 2010 May;21(5):731-2   [PMID:  20395375 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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