German Federal Ministry of Education and Research and the German Society for Aphasia Research and Treatment.

Introduction Haemophilia is a congenital bleeding disorder with severe musculoskeletal complications. Resistance exercise is important to increase joint stability and to improve physical performance. Aim This review aimed to investigate the safety and efficacy of resistance exercise interventions on people with haemophilia (PwH) and evaluate whether the American College of Sports Medicine resistance exercise criteria for healthy adults are valid for this population. Methods A systematic search in literature was conducted, using the databases PubMed, MEDLiNE, CINAHL, SCOPUS, PEDro and Cochrane Library. Out of 2.440 studies published between 1960 and November 2019, 14 studies (9 randomized controlled trials, 1 controlled trial, 4 single‐group prospective studies) applying resistance exercise in juvenile and adult PwH corresponded to the inclusion criteria. Results Studies performed dynamic, isokinetic or a combination of isometric and dynamic resistance training. Most interventions were carried out in the context of a multimodal training. Resistance was provided using fixed and free weights, body weight, resistance bands and water resistance. Study protocols included clinical and home‐based settings. Several studies suggest that training intensities lower than those known to increase the strength of healthy people are effective in increasing the strength of PwH. Resistance exercise seems to be a safe intervention if it is adequately monitored, individually adapted and applied with sufficient factor therapy. Due to the heterogeneity of study designs, training interventions and outcome measures a meta‐analysis could not be performed. Conclusions Further studies of higher methodological quality are needed to determine the optimal types of exercise, optimal dosage and timing.

Findings of unaltered PPT following moderate aerobic exercise showed initial evidence that PwH are able to perform an endurance exercise with self-chosen velocity for 30 min as recommended, without increasing the acute pain condition. By doing so, PwH can benefit from the positive effects of endurance exercise.

Summary Quadriceps weakness seems to be a hallmark in adult persons with severe haemophilia (PWH). The purpose of this study was to compare PWH and non‐haemophilic controls in different age stages with reference to joint status and quadriceps strength. Further aims were to examine the extent of strength‐specific inter‐extremity‐difference (IED) and the prevalence of abnormal IED (AIED). A total of 106 adults with severe haemophilia (H) and 80 controls (C) had undergone an orthopaedic examination for classification of knee and ankle status using the WFH score. Quadriceps strength was evaluated unilaterally as well as bilaterally with a knee extensor device. Each group was divided into four age‐related subgroups (HA/CA: 18–29, HB/CB: 30–39, HC/CC: 40–49, HD/CD: 50–70; in years). H presented a worse knee and ankle status than C indicated by higher WFH scores (P < 0.01). Regarding the age‐matched subgroups only HB showed higher knee scores than CB (P < 0.05). The ankles were clinically more affected in HB‐HD compared with those in age‐matched controls (P < 0.05). H showed lower quadriceps strength than C (P < 0.05). In addition, all subgroups of H presented lower strength (HA: 10–17, HB: 19–23, HC: 35–36, HD: 53–61; in%, P < 0.05). IED was higher in H than in C [H: 12.0 (5.3/32.2) vs. C: 7.1 (2.9/10.9); Median (quartiles) in%, P < 0.001] and increased with age in H. We discovered an AIED in 35% of H. These findings highlight the importance for the early implementation of preventive and rehabilitative muscle training programmes in the comprehensive treatment of PWH.

Particular degenerative changes in terms of osteophytes and thickness of synovial tissue are associated with an enhanced pain sensitivity in PwH. Altered PPT which were not associated with structural findings may be an indicator for a complex peripheral and/or central sensitization of the affected joints in PwH. The role of this mechanism should be clarified in further studies.