While appropriate haemostasis will stop the bleeding within the muscular compartment, the latency between the correction of the coagulopathy and the decrease in compartment pressures that will allow perfusion of the muscle may be long enough to lead to muscular death by isquemia and future contracture [19]. To challenge the paradigm, Selleckchem MK-2206 Caviglia et al. have developed an algorithm that requires optimal correction of the coagulopathy and

compartment pressure monitoring such that pressures above the 45 mmHg threshold would require mandatory fasciotomy [18]. Implementation of this algorithm may help prevent the severe neuromuscular lesions leading to contractures and loss of function that are often seen in haemophilia centres around the world [20]. Due to the fear of articular and muscular haemorrhages, there is a strong tendency among parents of boys with haemophilia to prevent them from engaging in exercise programmes. Visible signs of sedentarism are muscle atrophy, instability and restriction of motion [21]. These are more present in adults than in the younger patients [22]. First subclinical symptoms like tender ligaments are found even in the clinically healthy young

group [23]. This leads to a lack of physical activity and exercise that results in a poor physical condition with diminished muscle strength, aerobic/anaerobic power, proprioception and flexibility [24]. Interestingly, increasing amount of evidence indicates that biological changes induced selleck by physical activity produce a transient hypercoagulability

state. This is mostly due to increased thrombin generation, platelet hyperactivity and increased activity of several coagulation factors, especially factor VIII and von Willebrand′s [25,26]. These findings strengthen the foundations for the recommendation of exercise in persons with haemophilia. Regular and controlled exercise that significantly improves click here the physical condition are: training with light [21], medium or in special cases also heavy weights [27], dynamic [28], isokinetic [29] and isometric [30] or electrically stimulated strength exercise [31]. Proprioception is the second important exercise field, performed alone or in combination with strength training [27]. The fitness of 255 children and young adults (8–25 years) was tested for proprioception, strength, flexibility, endurance and body fat. In a comparison between the very active and the non-active group, proprioception and the total fitness were significantly better in the active group [32]. Thus, exercise should be carried out regularly, 30–120 min for 2–4 times a week, as recommended in the literature. All these interventions from 2 weeks up to 2 years showed first and last positive results.