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PubMed · 11045177

[Hemophilic arthropathy as a multidisciplinary problem].

V Krislo·O Masek·M Sorf·P Dubac·A Slávková·L Klubertová·P Muth·Z Macejková
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Abstract

The authors have at present on their records in Slovakia 422 patients with haemophilia type A, i.e. 7.9 per 100,000 population, 76 cases of haemophilia type B, i.e. an incidence of 1.4, 189 patients with von Willebrand's disease with an incidence of 3.6 and 215 patients with hypoconvertinaemia with an incidence of 3.98 per 100,000 population. The authors present an account on the most frequent complications of haemophilia with special attention to haemorrhage into the joints and the development of serious haemophilic arthropathies. They emphasize the great contribution of team work of different medical specialists who participate in the diagnosis and treatment of these diseases.

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HemarthrosisHemophilia AHumansEnglish AbstractJournal Article

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V Krislo, O Masek, M Sorf, P Dubac, A Slávková, L Klubertová, P Muth, Z Macejková. 1999. [Hemophilic arthropathy as a multidisciplinary problem].. https://pubmed.ncbi.nlm.nih.gov/11045177/

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PubMed2006

Prevention and treatment of musculoskeletal disease in the haemophilia population: role of prophylaxis and synovectomy.

Prophylaxis is defined as primary (started before the onset of joint damage) or secondary (started after the onset of joint damage). The aim of primary prophylaxis is to prevent recurrent bleeding into joints and the development of chronic arthropathy in later life. When started early, and at most after two joint bleeds, the result is predictably excellent if there is compliance with the primary prophylaxis regimen. In order to decrease the need for central venous access devices to assure reliable venous access, a number of centres start primary prophylaxis with once weekly infusions with dose-escalation based on frequency of joint bleeding. A major unanswered question is whether primary prophylaxis can be safely discontinued in adolescents/young adults and if so, when. A promising predictor for the milder bleeding phenotype in persons with severe haemophilia is a later onset of joint bleeding. Once joint damage has occurred as a result of recurrent bleeding, secondary prophylaxis can only retard, but not prevent, ongoing joint damage. Other strategies to decrease recurrent bleeding from target joints include surgical synovectomy (ideally performed using an arthroscopic technique), radionuclide synovectomy and chemical synovectomy. These interventions have very good outcomes when performed by an experienced team. Given the very high cost of factor concentrates required for programmes of prophylaxis prospective studies that document benefits to the child and family, e.g. quality of life are to be encouraged.

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