Exploring the biological basis of haemophilic joint disease: experimental studies.

摘要: Haemophilia has been recognized as the most severe among inherited disorders of blood coagulation since beginning first millennium. Joint damage is hallmark disease. Despite its frequency and severity, pathobiology blood-induced joint disease remains obscure. Although bleeding into ultimate provocation, stimulus within inciting process mechanisms by which a results in synovial inflammation (synovitis) cartilage bone destruction (arthropathy) unknown. Clues from careful observation patient material, supplemented with data animal models provide some clues to pathogenesis process. Among questions that remain be answered are following: (i) What underlies phenotypic variability patterns patients development arthropathy but not all bleeding? (ii) molecular basis underlying variability? (iii) Are there strategies can developed counter deleterious effects prevent disease? Understanding key elements, genetic and/or environmental, necessary for synovitis may lead rational design therapy targeted prevention treatment