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Blood, 1 December 2008, Vol. 112, No. 12, pp. 4542-4545. Prepublished online as a Blood First Edition Paper on July 24, 2008; DOI 10.1182/blood-2008-03-144691. This Article Full Text (PDF) All Versions of this Article: blood-2008-03-144691v1 112/12/4542    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Fakhouri, F. Articles by Fremeaux-Bacchi, V. Search for Related Content PubMed PubMed Citation Articles by Fakhouri, F. Articles by Fremeaux-Bacchi, V. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted March 19, 2008 Accepted June 29, 2008 Factor H, membrane cofactor protein and Factor I mutations in patients with HELLP syndrome Fadi Fakhouri*, Mathieu Jablonski, Jacques Lepercq, Jacques Blouin, Alexandra Benachi, Maryvonne Hourmant, Yves Pirson, Antoine Durrbach, Jean-Pierre Grunfeld, Bertrand Knebelmann, and Veronique Fremeaux-Bacchi Nephrology, Hopital Necker, Paris, France Departments of gynecology and obstetrics, Hopital Saint-Vincent de Paul, Paris, France Department of immunology, Hopital Europeen Georges Pompidou, Paris, France Departments of gynecology and obstetrics, Hopital Necker, Paris, France Department of nephrology, CHU Nantes, Nantes, France Department of nephrology, Universite catholique de Louvain, Brussels, Belgium Department of nephrology, CHU Bicetre, Bicetre, France Centre de Recherche des Cordeliers, INSERM UMRS 872, Paris, France * Corresponding author; email: fadi.fakhouri{at}nck.aphp.fr. The HELLP syndrome, defined by the existence of Haemolysis, Elevated Liver enzymes and Low Platelets count is a serious complication of pregnancy-related hypertensive disorders and shares several clinical and biological features with thrombotic microangiopathy (TMA). Several recent studies have clearly showed that an abnormal control of the complement alternative pathway is a major risk for the occurrence of a peculiar type of TMA involving mainly the kidney. The aim of this study was to screen for complement abnormalities in 11 patients with HELLP syndrome and renal involvement. We identified four patients with a mutation in one of the genes coding for proteins involved in the regulation of the alternative pathway of complement. Our results suggest that an abnormal control of the complement alternative pathway is a risk factor for the occurrence of a HELLP syndrome. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. Noris and G. Remuzzi Atypical Hemolytic-Uremic Syndrome N. Engl. J. Med., October 22, 2009; 361(17): 1676 - 1687. [Full Text] [PDF]

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