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Background: Haemophilic arthropathy is one of the main causes of morbidity in people with haemophilia (PWH), inducing pain and reduced functionality. Therefore, PWH are complex patients and must be approached from a multidisciplinary perspective., Objectives: To evaluate the effectiveness of a therapeutic exercise and cognitive-behavioural therapy (CBT) combined protocol on functionality, pain, and joint health of PWH, arthropathy and chronic pain. Treatment satisfaction was also evaluated., Methods: A single-blinded clinical trial with 21 PWH in prophylactic regimen was carried out. Participants were divided into an experimental group (EG, n = 11) and a control group (CG, n = 10). The EG underwent a 4-month programme of home-based therapeutic exercise plus CBT, whilst CG performed their daily activities. Patients were evaluated at baseline, post-intervention and after 12 additional weeks. Measures of functionality (Haemophilia Activities List, Timed Up and Go Test, 2-Minutes-Walking-Test and Sit-to-Stand Test), pain (PainDETECT and Visual Analogue Scale) and joint health (Haemophilia Joint Health Score) were taken. Related dimensions of the A36 Haemophilia Quality of Life Questionnaire were calculated. Effects were calculated using a two-factor ANOVA., Results: The EG showed significant improvements in function (p < .001), pain (p < .001), joint damage (p = .006), and satisfaction with the treatment (p = .006) dimensions of the A36 Haemophilia Quality of Life Questionnaire, as well as in pain measured with the Visual Analogue Scale (p = .008) and PainDETECT (p = .035)., Conclusions: The combined physiotherapy and CBT protocol showed a partial improvement in functionality, pain and joint health of PWH, arthropathy and chronic pain. In addition, participants were satisfied with the treatment., (© 2022 John Wiley & Sons Ltd.)

Introduction: The Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) system and scoring scale has proven to be an accurate and time-efficient imaging method for identifying joint damage in patients with haemophilia., Aim: Observational, multicentre, cross-sectional study conducted in 8 centres in Spain that assessed the joint status of adult patients with severe haemophilia A (SHA) using HEAD-US., Methods: Joint status of the elbow, knee and ankle was evaluated in adults with SHA receiving on-demand (OD) treatment, or primary (PP), secondary (SP), tertiary (TP) or intermittent (IP) prophylaxis., Results: Of the 95 patients enrolled, 87 received prophylaxis (6.3% PP, 38.9% SP, 43.2% TP and 3.2% IP). Mean age was 35.2 years, and 59% of patients had not undergone image testing in the last year. The HEAD-US score was 0 in all joints in 6.3% of patients. The ankle was the most affected joint, regardless of treatment regimen. Patients receiving OD treatment, TP or IP had the overall worst scores, mainly in the ankles and elbows; a similar but milder profile was observed in patients on SP; and patients on PP had the best score in all joints., Conclusion: Joint function may be effectively preserved in patients with SHA on PP, but OD treatment or later initiation of prophylaxis does not seem to prevent progression of arthropathy. Disease worsening was observed in patients OD, TP or IP, most often affecting ankles and elbows. Closer ultrasound imaging monitoring may improve management of these patients., (© 2021 John Wiley & Sons Ltd.)

Introduction: People with haemophilic arthropathy (PWHA) have impairments in postural control. However, little is known about the effects of demanding conditions, including the unipedal stance and dual tasks, on postural control in PWHA., Aim: Determine the effects of performing dual tasks while in the one-leg stance on postural sway and postural control complexity in PWHA vs. healthy active (HAG) and non-active (HNAG) groups of individuals., Methods: Fifteen PWHA and 34 healthy subjects (18 active and 16 non-active) were recruited. Vertical (V), mediolateral (ML) and anteroposterior (AP) centre of mass signals were acquired using a 3-axis accelerometer placed at the L3/L4 vertebrae of subjects as they performed the one-leg stance under single and dual-task conditions. Sway balance and the complexity of postural control were studied via root mean square (RMS) acceleration and sample entropy, respectively. Increased complexity of postural sway was attributed to increased automatism of postural control., Results: RMS values for PWHA were higher than HAG under both conditions for the V and ML axes, and higher than HNAG under the dual-task condition for the ML axis. Sample entropy was lower in PWHA than healthy individuals under the dual-task condition for V and ML axes, and the single-task condition for the ML axis (P < .05)., Conclusion: PWHA had poorer postural sway and decreased postural control complexity when performing a one-leg stance than healthy people, especially when the dual-task condition was applied. These results may help to design new approaches to assess and improve postural control in PWHA., (© 2020 John Wiley & Sons Ltd.)

Aim: The use of musculoskeletal ultrasound (MSK-US) following protocols for haemophilic arthropathy and the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score can help standardize monitoring in haemophilia. This study evaluated the joint status (elbows, knees and ankles) of patients with haemophilia B (HB) in Spain using MSK-US and the HEAD-US score., Methods: Haemophilia B patients ≥14 years old were included in this observational, multicentre, cross-sectional study, regardless of their clinical condition, HB severity and treatment received. Two blinded observers were involved in image acquisition and scoring in each centre., Results: Eighty-two patients from 12 centres were enrolled: 27% mild HB, 23% moderate, 50% severe HB. Mean age was 38.9 ± 16.4 years, 60% were treated on demand (OD) and 40% were on prophylaxis. HEAD-US was zero in all joints in 28.6% OD patients and 36.4% on prophylaxis. Mean scores significantly worsened with HB severity, except for the left knee. Patients on primary and secondary prophylaxis had significantly better joint health vs OD patients in all joints, except the right ankle. Among OD patients, those with severe disease presented significantly worse scores in all HEAD-US items related to permanent damage., Conclusion: Joint status of HB patients in Spain is influenced by severity and treatment modality, related to the development of arthropathy, which appears prevalent in OD patients with severe HB. Routine assessment with an imaging tool such as ultrasound and HEAD-US system may help to improve joint health by personalizing and adjusting treatment in this population., (© 2018 John Wiley & Sons Ltd.)

Introduction: Repeated haemarthrosis is widely accepted as the triggering cause of synovitis and haemophilic arthropathy. A first-line treatment of chronic synovitis is radiosynoviorthesis (RS). The aim of this study was to evaluate the RS effects on the progression of arthropathy and on a reduction in bleeding in patients with haemophilia., Methods: An observational-retrospective study was performed. Bleeding episodes in the 12 months following and in the 12 months preceding RS was compared. The arthropathy was clinically and radiologically analysed by age range, joint and subject, comparing those undergoing RS (Radiosynoviorthesis Group, RSG) against those not undergoing this treatment (Non-Radiosynoviorthesis Group, Non-RSG)., Results: One hundred and seventy-four RS were performed in 71 patients ( 90 Y in Knees and 186 Re in elbows/ankles/shoulder). RS resulted in significant reduction in bleeding (582 preintervention and 168 postintervention, P < .001). In general, the level of arthropathy measured clinically and radiologically was greater with age increase in both groups (RSG and Non-RSG), especially in the 25-40 age range. A significant increase (P < .05) in the progression of arthropathy was also observed, both globally by patient and specifically for each joint, in non-RSG and RSG group., Conclusion: RS is an effective method to reduce the number of haemarthrosis episodes in chronic synovitis. Moreover, RS can positively affect arthropathy by slowing down its progression. However, the results obtained suggest that arthropathy may be conditioned by the subject's age, regardless of whether or not the joint has undergone RS., (© 2017 John Wiley & Sons Ltd.)

Recurrent haemarthrosis is the final cause of haemophilic arthrosic disease in haemophilia patients. Therefore, it is essential to diagnose it early, both clinically and by imaging. In addition, haemophilia patients experience chronic synovitis, joint degeneration, muscle haematoma and pseudotumours. The objective of this article is to highlight the value of ultrasounds in the diagnosis and control of the evolution of musculo-skeletal problems in haemophilia patients. To this end, we have performed a literature search in the PubMed, Web of Science(®) (WOS) and SciVerse bases, using the following keywords: hemophilia or haemophilia and ultrasonography (US), ultrasound, echography and sonography. The search was limited to studies published in English between the years 1991 and 2011, finding a total of 221 references. After reviewing the title or abstract for evidence of the use of US for the diagnosis of musculo-skeletal lesions in haemophilia, we selected 24 of these references. We added data collected from our experience to the most important data found in the references. Our main conclusion is that US is highly valuable for the diagnosis of musculo-skeletal diseases in haemophilia. It is a fast, effective, safe, available, comparative, real-time technique that can help us confirm the clinical examination. It is particularly important in acute haemarthrosis, as it can be used to objectively identify the presence of blood in the joints, measure its size, pinpoint its location, assess its evolution and confirm its complete disappearance., (© 2011 Blackwell Publishing Ltd.)

Objective: To determine changes occurring in the cross-sectional area, electromyography (EMG) activity, and the strength of the biceps brachii after an 8-week period of bilateral training with surface muscle electrical stimulation in patients with hemophilic arthropathy., Design: Controlled trial., Setting: Coagulopathy unit, university hospital., Participants: Volunteer subjects (N=30) participated in this study: 15 with severe hemophilia A (hemophilic group) and 15 nonhemophilic control subjects (control group)., Interventions: The hemophilic group followed a surface electrical stimulation program (frequency 45 Hz, impulse 200 micros, 10s on/10s off) over an 8-week period on the biceps brachii of both arms. The control group did no training of any kind., Main Outcome Measures: The cross-sectional area, maximum voluntary isometric contraction, and EMG activity of the biceps brachii in both arms were determined before and after the 8-week-long task., Results: The results of the hemophilic group showed significant increases in the diameter (15.8%, P<.001), isometric force (4.6%, P<.05), and EMG activity (37.6%, P<.05) of the biceps brachii muscles in both arms. No significant changes were observed for the control group., Conclusions: Our findings confirm the efficacy of muscle electrical stimulation in causing muscles to hypertrophy in patients with hemophilia, thereby improving their muscular strength. In addition, these results may also be clinically applicable in the rehabilitation of patients who have similar deficiencies in the locomotor system.

This paper provides an outline of the results obtained in a cross-sectional study conducted primarily with the aim of ascertaining orthopaedic complications in a group of young severe A and B haemophiliacs, the effects which these complications have on the medical resources used with these patients, and the impact of severe haemophilia on their quality of life. Its secondary aim was to link their current orthopaedic status to the type of treatment they had received prior to the study. Eleven Spanish hospitals took part in this study, monitoring 70 severe haemophiliacs (FVIII:C <2%) without inhibitors who had a mean age of 21.6 years and a median age of 22. Retrospective data collected from birth to the conclusion of the study were used and, for certain variables, data from the last 12 months. The type of treatment given had been on-demand treatment, together with prophylaxis of variable time periods, which in 32 cases (45.7%) were prolonged (>6 months). In 40 cases (57.1%) the patients underwent one or more periods of prophylaxis. Thirty-three patients (47.8%) had over 1000 days of administration of factors VIII and IX. The analysis of the total study group reveal an average of 348 bleeding episodes per patient. The findings of this study revealed that 84.3% of these patients suffer from articular complaints on the Gilbert scale, and 85.7% on the Pettersson scale. In addition, pain was reported in 16.1% of the joints, the most frequently affected being the ankle joints. Twenty-six patients (37%) had undergone orthopaedic surgery from the time of birth to the conclusion of the study. The quality of life of the severe haemophiliacs reviewed seems to have been affected. During the last 12 months, there were 216 outpatient haematological visits and 176 orthopaedic-rehabilitation visits, as well as 12 radiological explorations and two hospitalizations. During these 12 months, medical expenditure totalled $55 473 per patient per year, the most important item being factor concentrates VIII/IX ($54 119 per patient per year). The type of treatment given to these patients (on-demand and/or as secondary prophylaxis) was found to be incapable of preventing haemophilic arthropathy or guaranteeing an acceptable quality of life, although both the administration of coagulant factor concentrate to such patients and the financial resources dedicated to their treatment was very high. Consequently, a strategic change is to be considered for the prevention of haemophilic arthropathy, based on the introduction of replacement treatment involving continuous administration of factors VIII and IX in primary prophylaxis regimens from the first years of life.

Introduction The sensory strategies of postural control in adult haemophilic arthropathy patients are still poorly understood. Aim To determine sensorial posture-control strategies through postural sway frequency analysis when in a bipedal quiet stance with and without visual stimulus deprivation in healthy subjects and patients with haemophilic arthropathy. Secondarily, to determine the irregularity of postural balance control through sample entropy (SampEn). Methods A triaxial accelerometer attached at the L3 level determined the displacement and acceleration of the centre of mass (DCoM and ACoM, respectively) under open- and closed-eyes conditions. Sensorial strategies were studied by spectral analysis of the DCoM signal, divided into low, medium and high frequencies for visual/vestibular, cerebellum and somatosensory strategies respectively. DCoM irregularity was also analysed by SampEn. Results Fifteen young, healthy subjects and fifteen young, haemophilia patients were included. The mediolateal DCoM and anteroposterior ACoM differed between groups. During the open-eyes condition, haemophiliacs presented limited high and medium frequencies, and more low frequency bands as compared to non-haemophiliacs ( P<.05). In the closed-eyes condition, haemophiliacs had a minor percentage of high frequencies but an elevated percentage of low frequencies as compared to non-haemophiliacs ( P<.05). Non-haemophiliacs had higher SampEn than haemophiliacs in the mediolateral axis with open- and closed-eyes ( P<.05 and <.001, respectively). Conclusions The presented results indicate that patients with haemophilic arthropathy, as compared to healthy subjects, have less postural control irregularity and poor somatosensory system contributions that are compensated by more vestibular inputs. [ABSTRACT FROM AUTHOR]

Introduction Alterations in the musculoskeletal system, especially in the lower limbs, limit physical activity and affect balance and walking. Postural impairments in haemophilic preteens could increase the risk of bleeding events and deteriorate the physical condition, promoting the progression of haemophilic arthropathy. Aim This study aims to evaluate static postural balance in haemophilic children, assessed by means of the Wii Balance Board® ( WBB). Methods Nineteen children with haemophilia and 19 without haemophilia aged 9-10 years, have participated in this study. Postural balance was assessed by performing four tests, each one lasting 15 s: bipodal eyes open ( BEO), bipodal eyes closed ( BEC), monopodal dominant leg ( MD) and monopodal non-dominant leg ( MND). Two balance indices, standard deviation of amplitude ( SDA) and standard deviation of velocity ( SDV) were calculated in the anterior-posterior ( AP) and medial-lateral ( ML) directions. Results Index values were higher in haemophilic group and the differences were statistically significant ( P < 0.05) in only six ( SDAAP in BEO, BEC and MD conditions, SDAML in BEO, SDVAP in BEO and SDVML in MND condition) of 16 parameters analysed. Conclusion Tests performed indicate a poorer static postural balance in the haemophilic cohort compared to the control group. Accordingly, physiotherapy programmes, physical activity and sports should be designed to improve the postural balance with the aim of preventing joint deterioration and improving quality of life. [ABSTRACT FROM AUTHOR]

Thirteen patients with haemophilia A took part in this study voluntarily. They underwent an aquatic training programme over a 9-week period (27 sessions; three sessions per week; 1 h per session). Their motor performance was assessed by the following cardio-respiratory and mechanical variables before and after the training programme: oxygen uptake (VO2, mL min−1), relative oxygen uptake (rel VO2, mL min−1·kg−1), carbon dioxide (CO2, mL min−1), respiratory quotient (R), heart rate (bpm) and the distance covered in 12 min (the Cooper test, m). Nine patients successfully completed the intervention and measurement protocols without bleeding or other adverse events. After the proposed training programme, significant differences between the pre-test and post-test were observed. Patients’ aerobic capacity increased considerably, and their oxygen uptake improved by 51.51% ( P < 0.05), while their relative oxygen uptake went up by 37.73% ( P < 0.05). Their mechanical capacity also increased considerably (14.68%, P < 0.01). Our results suggest that 27 specially designed aquatic training sessions for our patients with haemophilia A had a positive effect on their motor performance and considerably improved their aerobic and mechanical capacity without causing adverse effects. [ABSTRACT FROM AUTHOR]

The intention of the ‘clinical and echographical protocol of evaluation the efficacy of recombinant activated factor VII in the haemarthrosis’ (EFFISEVEN protocol) was to provide an extensive study of the evolution of haemarthrosis, and second, of its repercussions on the degenerative process of joints. The clinical evaluation of haemarthroses and their evolution is based on a well-established methodology, although very few studies have sought to determine the correlation between pain, mobility and the objective data regarding the haemorrhage. We believe that it is necessary to unify criteria and that the EFFISEVEN protocol may contribute data that improve standards which, in turn, will influence the degenerative process of joints, and consequently affect the quality of life of haemophilia patients with inhibitors. Echographical control of haemarthrosis is an objective method that allows control over how the haemorrhage evolves and also helps in the identification of rebleeding. Therefore, its use adds a new dimension to patient management strategies. Techniques used to monitor recombinant activated factor VII (rFVIIa) treatment require further study, although preliminary results guarantee their efficacy. [ABSTRACT FROM AUTHOR]

In the general population, the degenerative processes in joints are directly related to adult age, and osteoarthrosis represents the most frequent musculoskeletal alteration. In the haemophilic patient, the degenerative processes in the joint begin at very early ages, and are directly related to musculoskeletal bleeding episodes, which are occasionally subclinical and constitute haemophilic arthropathy. In the haemophilic patient, arthropathy constitutes the most frequent, severe and disabling pathology, and its assessment includes muscular force-related parameters. We have studied the value of Maximum Isometric Voluntary Contraction in the quadriceps femoris of 46 subjects, 28 haemophiliacs (16 severe, eight moderate and four mild) and 18 healthy individuals with a view to establishing appropriate values of force and to restoring physical therapy recommendations. The maximum force values were significantly greater ( P < 0.001) in the healthy individuals group. The mild haemophiliacs group also presented significant differences of force ( P < 0.05) in relation to the severe and moderate haemophilic patient groups. The mild and severe haemophilia patients presented greater fluctuations of force ( P < 0.001) than the control group, the haemophilia group have a minor skill to produce constant force. The seriousness of the arthropathy in the knee is directly related to diminished values of maximum force. Our work evidences that patients with severe haemophilia present a greater degree of arthropathy in relation to moderate and mild haemophilia patients. Haemophilic arthropathy is associated with muscular atrophy and strength deficit. In haemophilic patients, the deficit of maximum force and the presence of fluctuations may suggest an increased risk of bleeding during physical activities and the need to programme specific physical therapy guidelines which increase muscular power through resistance training. [ABSTRACT FROM AUTHOR]

Introduction Alterations in the musculoskeletal system, especially in the lower limbs, limit physical activity and affect balance and walking. Postural impairments in haemophilic preteens could increase the risk of bleeding events and deteriorate the physical condition, promoting the progression of haemophilic arthropathy. Aim This study aims to evaluate static postural balance in haemophilic children, assessed by means of the Wii Balance Board® ( WBB). Methods Nineteen children with haemophilia and 19 without haemophilia aged 9-10 years, have participated in this study. Postural balance was assessed by performing four tests, each one lasting 15 s: bipodal eyes open ( BEO), bipodal eyes closed ( BEC), monopodal dominant leg ( MD) and monopodal non-dominant leg ( MND). Two balance indices, standard deviation of amplitude ( SDA) and standard deviation of velocity ( SDV) were calculated in the anterior-posterior ( AP) and medial-lateral ( ML) directions. Results Index values were higher in haemophilic group and the differences were statistically significant ( P < 0.05) in only six ( SDAAP in BEO, BEC and MD conditions, SDAML in BEO, SDVAP in BEO and SDVML in MND condition) of 16 parameters analysed. Conclusion Tests performed indicate a poorer static postural balance in the haemophilic cohort compared to the control group. Accordingly, physiotherapy programmes, physical activity and sports should be designed to improve the postural balance with the aim of preventing joint deterioration and improving quality of life. [ABSTRACT FROM AUTHOR]