Background: Haemophilia is a congenital bleeding disorder going along with diseasespecific joint complications and general health implications attributed to the lack of daily movement. Recent guidelines recommend physical activity for people with haemophilia (PwH). Yet, aerobic exercise in PwH is little studied and seldomly prescribed by clinicans. Aim:The aim of this systematic review is to investigate the safety, feasibility and health-related efficiency of AE in PwH. Methods:A systematic literature search according to the PRISMA guidelines was conducted (PubMed, Web of Science). Inclusion criteria were defined using PICOS.Methodological quality was assessed via TESTEX. Results:Out of 789 studies identified, seven studies (three randomized controlled, two controlled, two single-group prospective trails) were included. The TESTEX mean score was 8.1 (±3.8). AE was realized as aquatic exercise, nordic walking, treadmill running, bicycle riding and swimming. Neither bleeding rates nor the factor amount increased and AE led to diverse health-related improvements.Conclusions: Little research has been conducted evaluating AE in PwH. Yet, AE can be considered as safe and feasible when being supervised by experts. However, diseasespecific recommendations for AE are difficult to provide. Therefore, experts can currently only back AE recommendations on experience and nonhaemophilia-specific general guidelines.
Background:In patients with haemophilia (PwH), most frequently affected joints are the ankle, knee and elbow. Due to improved factor therapy in the last decades, these previous findings have to be verified in Germany. Aim:The aim of this study is to detect the most affected joint, evaluate the significance of the source of pain and determine the point prevalence of back pain in Germany today. Patients and methods:In a retrospective study, data of n = 300 patients with severe moderate and mild haemophilia were evaluated regarding the most affected joint, the most common source of pain, and the point prevalence of back pain. An anamnesis questionnaire and the German Pain Questionnaire were used for this assessment. Results:The most affected joint in German PwH is still the ankle (41%), followed by the knee (27%) and the elbow (11%). The most common source of pain is also the ankle joint (32%). Back pain was also identified as one of the most common sources of pain, which is comparable to the elbow (elbow:15%; back:13%). The point prevalence in PwH for back pain was significantly higher compared to the general German population (P = .031). Conclusion:Our data showed that the ankle is still the most affected joint and the most common source of pain in Germany. These results also showed the relevance of back pain as a pain source. The evaluations also demonstrated the high point prevalence of back pain in PwH. Future therapies should also focus on the spine because joint changes affect posture.
Objectives Patients with haemophilia (PwH) often suffer from joint pain due to repetitive haemarthroses and resulting arthropathy. Literature focuses so far on pain causes, diagnosis or treatment. A summary of prevalence rates, providing facts on the absolute occurrence of pain, is not sufficiently described so far. This review aimed to explore and systematically review different pain conditions, focussing on prevalence rates of pain in adult PwH. Methods A review of English articles using PubMed and Web of Science was conducted in February 2020. The search strategy included patients with haemophilia A or B suffering from pain. The articles were selected based on defined PICOS-selection criteria. Results Out of 606 identified articles, 13 studies matched the given eligibility criteria and indicated pain prevalence rates. The weighted mean (WM) for the prevalence rate (varying timeframes) for chronic pain was 40% whereas for point prevalence the rate was WM=75%. Regarding pain intensity, findings of the EQ-5D-3L revealed moderate pain to be more present (61.0%) compared to extreme (11.6%). The main problem was the inconsistency of the definition of both acute and chronic pain as well as for prevalence types. Conclusions Pain is a major problem in patients with haemophilia. Pain therapy should be carried out taking into account the difference between bleeding-related or arthropathy-related causes of pain. In addition, the intensity and duration of pain should be recorded consistently to better monitor therapy and allow comparison with existing data.
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