Haemophilia is a genetic X-linked congenital haemorrhagic disorder characterized by a deficiency in the production of coagulation factors VIII and IX corresponding to haemophilia types A and B, respectively. 1,2 Although rare, with a frequency of 1 in 10 000 births, haemophilia can be found in about 400 000 people worldwide. 3 Approximately 85% of haemorrhagic episodes occur in the musculoskeletal system, hemarthroses and intramuscular haematomas, resulting in the main complications of haemophilia: pain, muscle atrophy and functional limitations that, in the long term, directly affect the performance of activities of daily living. 4,5 As a result, people with haemophilia (PWH) experience a more sedentary lifestyle, with lower muscle strength, aerobic and anaerobic endurance, and proprioception compared with healthy individuals. 6,7 A few decades ago, exercise was contraindicated for PWH due to low clotting factor concentrations and the high risk of bleeding.However, with the advent of concentrates for coagulation factor replacement as a form of treatment and prophylaxis to haemorrhages, Abstract Introduction: There is considerable evidence to indicate that exercise can have a positive impact on the treatment of people with haemophilia (PWH). However, there is a requirement for in-depth and comprehensive studies.Aim: This study aimed to analyse the evidence regarding the effects of exercise in PWH through an umbrella review of existing systematic reviews and meta-analyses.The secondary objective was to analyse the quality of the evidence.Methods: This umbrella review followed the PRISMA guidelines and was documented in the PROSPERO registry (CRD42019140785). We searched the PubMed, Web of Science, SPORTDiscus, Scopus, CINAHL and Cochrane Library databases.The methodological quality of the systematic reviews was assessed using AMSTAR 2.Results: Out of a total of 1030 systematic reviews, 10 fulfilled the criteria. Only one study was classified as high quality, and half of the selected studies were classified as low or critically low quality according to AMSTAR 2. Furthermore, most reviews investigated the effects of strength training and aquatic training, with positive results associated with low adverse events. Range of motion, strength and pain were the most investigated variables. All reviews showed overlapping studies.
Conclusion:Exercise is an effective way to treat haemophilia and has a low incidence of related adverse events. However, caution is needed in the interpretation of the results due to half of the selected reviews showed low or critically low quality and only one have high quality.
K E Y W O R D Sbleeding disorders, evidence-based practice, exercise, haemophilia, haemophilic arthropathy | 929 SIQUEIRA Et Al.