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Deaths associated with acquired haemophilia in France from 2000 to 2009: multiple cause analysis for best care strategies.
MedLine Citation:
PMID:  21910792     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Summary.  Deaths occurring in the context of acquired haemophilia (AH) may be related to inter-connected causes and mechanisms including bleeding, specific or older patient co-morbidities or iatrogenic complications. However, their magnitude remains unknown. This study aimed to determine the respective weight and frequency of the various causes of death in AH. Multiple-cause analysis based on death certificates data is used in this purpose. Over a 10-year period (2000-2009), 121 deaths with AH as a cause were registered in France. All the deaths were of adults (extremes: 47 and 99 years; mean age: 80.7 years). The average number of causes per death certificate was 4.7. AH was the underlying cause of death (UCD) in 69.4% of the cases, and was more frequent in the older subjects. In contrast, before age of 75 years, AH was more often a contributing cause of death. No postpartum or obvious thromboembolism-related deaths were registered. Haemorrhagic shock was the most frequent direct cause of death (DCD), followed by infectious events, cardiac dysfunction, metabolic and nutritional disorders with muscle wasting and decubitus complications, and cancers (52.9%, 26.4%, 7.5%, 5.8% and 4.1%, respectively). However, when AH was not reported as an UCD, infections become the first DCD (32.4%) followed by bleeding events (16.2%). Best prophylactic and curative strategies for infections are particularly required to improve the prognosis in AH. Moreover, as several of its DCD correspond also to steroids side effects, best tolerated immunosuppressant regimen with steroid-sparing agents adjoining are particularly awaited in AH population.
Authors:
A Aouba; G Rey; G Pavillon; E Jougla; C Rothschild; M-F Torchet; L Guillevin; O Hermine; A Aouba
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-9-13
Journal Detail:
Title:  Haemophilia : the official journal of the World Federation of Hemophilia     Volume:  -     ISSN:  1365-2516     ISO Abbreviation:  -     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-9-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9442916     Medline TA:  Haemophilia     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2011 Blackwell Publishing Ltd.
Affiliation:
Inserm, CépiDc, Le Kremlin-Bicêtre, France Center for Haemophilia Care and Department of Adult Haematology, Hôpital Necker Enfants-Malades, Université Paris-Descartes, Assistance Publique-Hôpitaux de Paris; Department of Internal Medicine, Hôpital Cochin, Université Paris-Descartes, Assistance Publique-Hôpitaux de Paris, Paris, France.
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