| Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. | |
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MedLine Citation:
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PMID: 20203157 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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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:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2010-03-04 |
Journal Detail:
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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:
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Created Date: 2010-05-03 Completed Date: 2010-06-21 Revised Date: 2011-07-28 |
Medline Journal Info:
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Nlm Unique ID: 9013836 Medline TA: J Am Soc Nephrol Country: United States |
Other Details:
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Languages: eng Pagination: 859-67 Citation Subset: IM |
Affiliation:
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Department of Nephrology and UMR 643, CHU de Nantes, Paris, France. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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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:
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EC 3.4.21.47/Complement C3 Convertase, Alternative Pathway |
| Comments/Corrections | |
Comment In:
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J Am Soc Nephrol. 2010 May;21(5):731-2
[PMID:
20395375
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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