Document Detail

Exploring the biological basis of haemophilic joint disease: experimental studies.
MedLine Citation:
PMID:  22044636     Owner:  NLM     Status:  Publisher    
Summary.  Haemophilia has been recognized as the most severe among the inherited disorders of blood coagulation since the beginning of the first millennium. Joint damage is the hallmark of the disease. Despite its frequency and severity, the pathobiology of blood-induced joint disease remains obscure. Although bleeding into the joint is the ultimate provocation, the stimulus within the blood inciting the process and the mechanisms by which bleeding into a joint results in synovial inflammation (synovitis) and cartilage and bone destruction (arthropathy) is unknown. Clues from careful observation of patient material, supplemented with data from animal models of joint disease provide some clues as to the pathogenesis of the process. Among the questions that remain to be answered are the following: (i) What underlies the phenotypic variability in bleeding patterns of patients with severe disease and the development of arthropathy in some but not all patients with joint bleeding? (ii) What is the molecular basis underlying the variability? (iii) Are there strategies that can be developed to counter the deleterious effects of joint bleeding and prevent blood-induced joint disease? Understanding the key elements, genetic and/or environmental, that are necessary for the development of synovitis and arthropathy may lead to rational design of therapy for the targeted prevention and treatment of blood-induced joint disease.
L A Valentino; N Hakobyan; C Enockson; M L Simpson; N C Kakodkar; L Cong; X Song
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-11-2
Journal Detail:
Title:  Haemophilia : the official journal of the World Federation of Hemophilia     Volume:  -     ISSN:  1365-2516     ISO Abbreviation:  -     Publication Date:  2011 Nov 
Date Detail:
Created Date:  2011-11-2     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.
Department of Pediatrics, RUSH Hemophilia and Thrombophilia Center, Rush Children's Hospital and Rush University Medical Center, Chicago, IL, USA.
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