Episodic CFC replacement over a large range of doses does not alter the natural course of bleeding in haemophilia or the musculoskeletal deterioration and should not be recommended as a long term option for treatment. Prophylaxis is the only way to preserve musculoskeletal function in haemophilia.

Introduction: Recurrent joint bleeds in haemophilia patients often cause musculoskeletal changes leading to functional capacity impairment. Aim: In this study, we assessed the effects of aquatic activities performed to improve functional capacity in these patients. Methods: The interventional protocol consisted of 24 hydrotherapy sessions during three months, in comparison with 24 swimming sessions. The pre-and postintervention assessment consisted of Functional Independence Score, haemophilia joint health score (HJHS), Pediatric Haemophilia Activities List (PedHAL), surface electromyography (SEMG) of thigh muscles to assess muscle electric activity, and load cell on extensor and flexor thigh muscles to evaluate muscular strength. Results: Forty-seven haemophilia patients were enrolled in this study, and 32 (23 severe haemophilia A, one moderate haemophilia A and 8 severe haemophilia B), median age 12y (6 to 40y), concluded the aquatic intervention. We observed a statistically significant increase with substantial improvement in functional capacity in the pre-and post-intervention evaluation of hydrotherapy in comparison with the swimming protocol, with HJHS (p = .006 and p = .001 respectively), PedHAL (Sum score) (p = .022 and p = .001) and score FISH (p = .021). The swimming group revealed significant improvements in muscular strength, in all muscles tested (p = .005 and p = .001). SEMG signal amplitude reached significantly higher levels in all muscles evaluated after both interventions except for the vastus medialis (right) in the hydrotherapy group. Conclusion: Our results concluded that both swimming and hydrotherapy were associated with physical improvement in haemophilia patients; however, only hydrotherapy lead to a more significant improvement in functional capacity.

No abstract

Objective: Evaluate the effect of radiosynovectomy of the ankle with samarium-153 and yttrium-90 in hemophilic arthropathy through the possible reduction of hemarthrosis, analyzing complications, adverse effects, and the need for complementary therapies. Methods: Radiosynovectomy of the ankle in 15 hemophilic patients was analyzed retrospectively and followed between January 2008 and December 2021. The analysis was performed through patients’ medical records and diaries to quantify hemarthrosis that occurred six months before and six months after the procedure. Clinical follow-up and evaluation of outcomes were also analyzed. Results: Eighteen radiosynovectomies of the ankle were performed in 15 patients with a mean follow-up of 124 months. A reduction in episodes of haemarthrosis has been demonstrated within six months (p<0.001). There were no adverse effects or complications in the short and long term. Approximately 61.1% of the patients did not require complementary therapies afterward. Conclusion: The study demonstrated that radiosynovectomy of the ankle with samarium-153 and yttrium-90 in hemophilic patients is effective and safe. There was a decreased frequency of hemarthrosis, less need for complementary procedures, and no complications and adverse effects. Level of Evidence IV, Case Series.

Joint bleeding is the main clinical manifestation in people with haemophilia (PWH) occurring in approximately 70% to 80% of cases 1 and may result in haemophilic arthropathy, chronic pain, function disorders and decreased quality of life. 2,3 The treatment is based on the replacement of the deficient clotting factor, either on demand or on prophylactically. 4 The primary prophylaxis only began to be used in Brazil at the end of 2011, 4 and

Hemophilia is characterized by recurrent bleeding into the joints leading to irreversible chronic arthropathy with reduced joint range of motion (ROM), which may lead to changes in gait patterns. To analyze the gait pattern in a 35-year-old male with severe hemophilia A, three-dimensional biomechanical analysis was performed during overground walking. The control group data from a public gait dataset of 10 healthy male individuals were used for comparison. The clinical examination was assessed with the Functional Independence Score in Hemophilia (FISH), Haemophilia Activities List (HAL), and Hemophilia Joint Health Score (HJHS). The biomechanical analysis demonstrated a pattern for both left knee and ankle joints with greater similarity to the control group compared to the right knee and ankle joints. ROM based on the HJHS questionnaire also showed greater impairment of the right-side knee joint compared to the left-side knee joint. This unique pattern could be the result of a compensation mechanism due to limited movement during the walking task and the surgical treatment.

To the Editor, People with haemophilia (PWH) usually begin to suffer pain at a young age. Physical and psychological components can accentuate patients' pain. Pain has been identified as the most challenging and prevalent symptom, affecting up to 80% of PWH, and is the leading cause of disability in these patients. 1 Some factors such as cognitive and social behaviour contribute to the impact of pain and are associated with poor prognosis following the onset of musculoskeletal pain. 1,2 There is a complex interaction of factors that influence pain perception, such as mood swings, emotional problems, hypervigilance, kinesiophobia, catastrophic thoughts and negative beliefs. Negative thoughts can affect the sensation of pain. As a result, pain can be interpreted as a threat, and an overreaction can be initiated in response to this threat. 3 Kinesiophobia is defined as an excessive, irrational and debilitating fear to carry out a movement resulting from a feeling of vulnerability due to a painful injury and may be associated with pain and disability. Kinesiophobia alters how people move, causing adjustments in motor behaviour, which affects the performance of actions related to the management and control of pain. 3 This study aimed to measure kinesiophobia and catastrophic thoughts in PWH and analyze their impact on clinical and functional parameters, especially in PWH with chronic pain.In this study, we enrolled patients diagnosed with haemophilia A or B aged over 12 years. The evaluation was performed from August 2018 to July 2019, during PWH routine consultation at the haemophilia unit of Hemocentro UNICAMP, Brazil. Patients were considered eligible for the study when they could perform all clinical and functional assessments and adequately respond to all applied questionnaires.

Esse estudo foi feito com o intuito de avaliar a correlação entre as alterações radiológicas e a clínica de portadores de artropatia hemofílica crônica. No total foram avaliados 103 pacientes com o escore radiográfico de Pettersson, a avaliação de saúde articular (HJHS) e o Escore de Independência Funcional (FISH). Houve uma correlação excelente entre os valores dos escores e correlação boa e excelente quando as articulações foram avaliadas separadamente.