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The article discusses various consumer wearable activity trackers like triaxial accelerometer, heart rate (HR) sensors and global positioning systems (GPS) that can monitor physical activity (PA) in adult patients with haemophilia. According to recommendations given by the World Health Organization (WHO), sufficient physical activity with an adequate adjustment of prophylaxis treatment decreases the risk of bleeding in haemophiliacs.

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 (90Y in Knees and 186Re 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. [ABSTRACT FROM AUTHOR]

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]

A letter to the editor is presented on haemarthrosis home treatment in patients with haemophilia A through home delivered ultrasound monitoring.

The HERO (Haemophilia Experiences, Results and Opportunities) quantitative surveys collected information on characteristics and perceptions of adult persons with haemophilia ( PWH) and parents of children with haemophilia. The aim of this article is to describe the perceptions of PWH and parents on psychosocial aspects related to treatment. Two online surveys (one for PWH, one for parents) were conducted in 10 countries. Among 675 PWH respondents, 77% reported having responsibility for their own care; 72% of 561 parent respondents had the main responsibility for their son. PWH were most commonly treated on demand (45% of 648 adults using factor concentrate), with 32% on regular prophylaxis and 23% treated on demand with short-term prophylaxis (e.g. for sports/physiotherapy). Children were most often treated with prophylaxis (65% of 549 children using factor concentrate), with 26% treated on demand and 8% treated on demand with short-term prophylaxis. Factor was generally used as instructed at home. Some respondents (41% PWH; 30% parents) had difficulties/concerns with factor availability/affordability. PWH reported more bleeds in the last 12 months than parents reporting their son's bleeds (mean 17.8 vs. 8.7). Both PWH and parents generally perceived that overall, their (their son's) haemophilia was well controlled. Results differed by country. The HERO study captured new, patient-based data regarding many facets of life relevant to PWH, including treatment. The information conveyed in this article largely represents new insights regarding perceptions of treatment and provides initial benchmark statistics for further research. [ABSTRACT FROM AUTHOR]

Background Retrospective publications show a decrease in the bleeding frequency and an improvement in the quality of life (QoL) in severe adult haemophilia A ( SAHA) after switching from the on-demand treatment ( DT) to secondary prophylaxis ( SP). But there are no prospective studies which demonstrate, using a haemophilia-specific questionnaire, an improvement in the QoL after such treatment change. The main objective of this study is to prospectively compare the QoL and the musculoskeletal assessment after switching from DT to SP in SAHA using the A36 Hemofilia-QoL®. As secondary objective, we compare the haemarthrosis frequency and factor VIII consumption in DT and SP during a similar period of time (12 months) after switching. Materials and Methods We have designed a prospective study including SAHA who have been under DT and were changed to a protocol, which combines SP (biweekly administration of factor VIII) with individualized physiotherapy programme. Results Twelve months after switching to SP, the QoL was significantly improved ( P = 0·005). Musculoskeletal assessment of pathologic irreversible joints and joints with a reversible alteration was generally improved, although in only a few joints, this improvement was statistically significant. Haemarthrosis was strongly reduced (12·60-1·42, P < 0·001). Conclusions This prospective study has demonstrated a statistically significant improvement in the QoL after 1 year from switching patients from DT to SP. The musculoskeletal assessment after 1 year was maintained similar or slightly improved. When we compared retrospective DT and prospective SP, haemarthrosis where strongly reduced requiring a slight increase in the consumption of factor VIII concentrates. [ABSTRACT FROM AUTHOR]

. 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. [ABSTRACT FROM AUTHOR]

. Psychosocial factors have a significant impact on quality of life for patients with chronic diseases such as haemophilia. Interventions to support the psychosocial needs of patients and their families, such as offering information and assistance, clarifying doubts, and teaching coping strategies to minimize the impact of disabilities, may help to maximize patient outcomes and improve quality of life for their families. The aim of this study was to evaluate the current literature on psychosocial aspects of haemophilia. Literature searches were performed using the PubMed database to identify studies evaluating psychosocial stressors in persons with haemophilia. Articles pertaining to the HIV epidemic were excluded from the analysis, as were those published before 1997. The literature reviews identified 24 studies, covering a range of different populations, generally with small cohorts ( n < 100). Most studies were questionnaire based, with almost no overlap in terms of the instruments used. Only one study combined questionnaire techniques with qualitative methods. Except for two European studies, all publications reported data from a single country. Overall, studies tended to show that quality of life is reduced in persons with haemophilia, with a potential impact on education and employment, particularly when prophylactic treatment is not available. Carrier status in women may have a psychosocial impact and affect reproductive choices. Data on psychosocial aspects of the haemophilia life cycle are lacking in the published literature, along with data from developing countries. There is a need for more international, multifaceted research to explore and quantify the social and psychological aspects of life with haemophilia. [ABSTRACT FROM AUTHOR]

Study design:A clinical blind study was conducted to determine the efficacy of transdermal nitroglycerine treatment on the awareness of shoulder pain.Objective:This study aims to determine the effects of transdermal nitroglycerine on shoulder pain and on functional shoulder movement in patients with spinal cord injuries and shoulder tendinopathies. A second aim is to establish the side effects of the treatment.Setting:Hospital 'La Fe' in Valencia, Spain.Methods:A total of 45 spinal cord injury patients, all of whom are wheelchair users with shoulder tendinopathy, were randomly divided into two groups: placebo (n=12) and experimental (n=33). The experimental group (EG) received transdermal treatment for 6 months in the form of a 1.25 mg nitroglycerine (NT) patch on the greater tubercle. A placebo patch was used with the placebo subjects. Functional shoulder movements were assessed by articular range of motion and pain, using a visual analogical scale.Results:NT treatment reduced the awareness of shoulder pain and increased the functional movement and range of articular motion in the shoulders of the EG members (P<0.05).Conclusions:We conclude that transdermal NT is an efficient method for treating shoulder tendinopathies in wheelchair users with spinal cord injuries. [ABSTRACT FROM AUTHOR]

In recent studies, adolescent haemophilia A patients and healthy adolescents have been encouraged to participate in physical activity (PA) based on its many established health benefits. However, none of the studies to date has used objective measures of PA and sedentary behaviour. The aims of the current study included: (i) to determine the amount and intensity of habitual PA among haemophilia A and healthy adolescents, and in haemophilia A patients with and without bleeding episodes in the previous year, and (ii) to identify the type and determine the time spent in sedentary activities in which both groups participate to obtain a broadened view of their daily activities. A total of 41 adolescent haemophiliacs and 25 healthy adolescents, between the ages of 8 and 18 years, participated in this cross-sectional study. A triaxial accelerometer was used to measure PA and the Adolescent Sedentary Activity Questionnaire to assess sedentary behaviours among members of both groups. Adolescent haemophilia A patients showed a higher daily mean time engaged in light, moderate and moderate-to-vigorous PAs relative to their healthy counterparts ( P < 0.001). Patients who had experienced bleeding episodes during the previous year also spent more time participating in vigorous PAs than healthy adolescents ( P = 0.002). With regard to sedentary behaviours, healthy adolescents spent more time listening to music than haemophilia A adolescents ( P = 0.003), whereas haemophilia A adolescents spent more time watching TV ( P < 0.001) and playing videogames ( P = 0.003) than healthy counterparts. Findings suggest that increased participation in moderate intensity PAs and reduced sedentary behaviours should be recommended among adolescents with haemophilia A. [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 article presents a case study on using augmented visual-stimulus feedback to describe the sensorimotor learning process over three consecutive days in two haemophiliacs with different degrees of joint damage while performing a unipedal balance task. Topics include both subjects stated to have the same percentage of errors in the first trial, haemophilic arthropathy found to affect performance during a sensorimotor stability task and subsequent learning.

In haemophiliacs, the physical condition, muscular strength, aerobic resistance, anaerobic resistance and proprioception have all diminished. Muscle atrophy and instability, being more vulnerable to stressful motor demands, increase the risk of lesion and establish a vicious circle that is hard to break: pain, immobility, atrophy, articular instability and repeated bleeding episodes. In haemophilia, physical and/or sporting activities were not recommended until the seventies. Nowadays, the overall policy is to recommend certain physical activities, especially swimming, to improve the patient’s quality of life, thanks to prophylaxis programmes. The objective of this study is to perform a systematic review of the exercise and sporting activities recommended for haemophiliacs. Experimental and observational studies and clinical assays about rehabilitation for haemophiliacs with exercise and sporting activities have been included. The relevant studies were identified in Medline, Cinahl, Embase and SportDiscus, and key words were: haemophilia, exercise and sport (with no language restrictions). Works were independently analysed by reviewers and the following were identified: of 3603 studies, 103 were included in this review: 29 (28.15%) were experimental, 27 (26.21%) were observational and 47 (45.63%) were clinical. Physiotherapy, physical activity and sport are basic elements to improve quality of life and the physical condition, increase strength and resistance and to reduce the risk of musculoskeletal lesions and to prevent haemophilic atrophy. In general, professionals in haemophilia believe that regular exercise and rehabilitation with physiotherapy is fundamental, particularly in countries where replacement therapy is not readily available. [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]

We recommend prophylaxis in haemophilic children with an inhibitor as a way of preventing the musculoskeletal impairment that is likely to affect them. This approach has been used for children without inhibitors with excellent results. If prophylaxis is not feasible, we suggest that intensive on-demand treatment should be given. Two agents, recombinant activated FVII (rFVIIa) and activated prothrombin complex concentrates (aPCC), are currently used to control haemostasis either for prophylaxis or intensive on-demand treatment. As it is recombinant, rFVIIa would seem more appropriate to be employed in children. aPCC could be used in adults, or in the event of an unsatisfactory response to rFVIIa. We recommend prophylaxis or, at least, intensive on-demand treatment in haemophilia children with inhibitors. Both rFVIIa and aPCC are being used for this purpose. It would seem that rFVIIa might be more appropriate for children as it is a recombinant product. Nevertheless, after skeletal maturity (in adults), both agents could be used indistinctively (taking into consideration that FEIBA is a plasma-derived product). We still need more well-designed comparative studies in order to be able to assert that our consensus-based conclusion is evidence based. In orthopaedic surgery, both aPCC and rFVIIa have been reported to be effective in controlling perioperative haemostasis, although in practice most centres have so far used rFVIIa for their orthopaedic procedures. We recommend rehabilitation programmes for all patients with inhibitors in order to mitigate the disabling and handicapping impact of their condition and thereby enable them to achieve social integration. Programmes for haemophilic children without inhibitors can be applied to children with inhibitors but should be individually tailored. [ABSTRACT FROM AUTHOR]

Musculoskeletal disorders in haemophiliacs represent the highest percentage of lesions, giving rise to haemophilic arthropathy (HA) which predominantly affects lower limbs, influencing postural control, standing and walking. Leading a sedentary lifestyle seems to influence strength and muscular resistance in haemophiliacs which, in turn, are related to articular stability and the prevention of articular degenerative processes. The objective of this work was to study alterations in balance to subsequently evaluate the appropriate therapeutics and how this influences the development of arthropathy. Twenty-five haemophiliacs with HA, 25 haemophiliacs without HA (NHA) and 25 healthy control subjects (CTL) took part in this study. Tests were performed on a force platform and the subjects remained as still as possible for 30 s under different conditions: (i) bilateral stance with eyes open; (ii) bilateral stance with eyes closed; (iii) right unilateral stance; and (iv) left unilateral stance. The results of these tests indicated significant differences ( P < 0.05) between the groups HA, NHA and CTL. The HA group presented worse results for both unilateral and bilateral stance when compared with the other two cohorts. Surprisingly, the NHA group displayed a worse balance than the controls. A single calculated parameter (mean frequency) did not show significant differences. This apparently indicates the absence of pathology in the nervous system in relation to postural control. The results suggest that our patients should participate in physical exercise programmes, rehabilitation and physiotherapy to improve their postural control. [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]

Eighteen sessions of surface electrical stimulation was applied to the quadriceps femoris of the left leg of ten male subjects affected by severe haemophilia A, while ten healthy subjects constituted the control group. The isometric strength, the electromyographic activity and the diameter of the rectus femoris were measured in both legs before and after a six-week treatment period. After the treatment, the people affected by haemophilia showed a gain in strength by 13.8% in the stimulated leg and by 17.1% in the non-stimulated one. No changes were detected in the electromyographic activity. On the contrary, the diameter of the rectus femoris of the stimulated leg increased in 24.34%, while no significant change was found in the nonelectrically stimulated leg. These results show for the first time that the application of electrical stimulation in haemophilic patients contributes to the gain and development of strength and trophism. The results also show that the surface electrical stimulation does not represent a threat to the patients' health, and that can be used for therapeutic purposes. [ABSTRACT FROM AUTHOR]

Summary. We report on a series of 108 elective orthopaedic surgical procedures. It includes 88 radiosynoviortheses and 20 major orthopaedic procedures, performed on 51 patients at nine centres worldwide. The average age of patients was 28.5 years (range 5–40 years), and the average follow-up time was 2 years (range 1–5 years). There were 82 good results, 15 fair and 11 poor. In the synoviorthesis group (41 patients, 88 synoviortheses) the average age was 14.3 years (range 5–40 years) and the average follow-up was 6.5 years (range 1–10 years). There were 66 good results, 14 fair and eight poor. There were no complications. In the group of major orthopaedic procedures, the average age of the 10 patients was 32.5 years (range 27–40 years), and the average follow-up was 2.3 years (range 1–5 years). There were 16 good results, one fair and three poor. Postoperative bleeding complications occurred in three of the 20 major orthopaedic procedures performed (15% complications rate). They occurred in three patients treated with insufficient doses of recombinant activated factor VII. Despite such complications, the study has shown that haemophilic patients with inhibitors requiring elective orthopaedic surgery (EOS) can undergo such procedures with a high expectation of success. In other words, EOS is now possible in haemophilic patients with inhibitors, leading to an improved quality of life for these patients. Thorough analysis of each case as part of a multidisciplinary team will allow us to perform elective orthopaedic procedures in patients with inhibitors. [ABSTRACT FROM AUTHOR]

In haemophilia patients, orthoses are used for the prevention and management of bleeds and their effects on the musculoskeletal system. The characteristics of these devices vary according to the joint to which they are applied, although two basic types are distinguished: static and functional. The upper limbs make up a functional unit that is adapted to prehension, or the action of grasping or seizing objects. The most affected joints of this unit in haemophilia are the elbow and shoulder, which require immobilization, followed by stabilization and protection and in the case of disability, assistance in the performance of function. The lower limbs, on the other hand, are adapted to the action of ambulation. The knee, ankle and hip joints frequently sustain damage in haemophilia, and in the acute phase they should be immobilized and the patient discouraged from walking. Once bleeding has ceased, and motion and muscle balance improved, a gradual return to normal activity may commence. The principle aim of orthotics in these cases is to immobilize and unburden the affected body segment, and to provide stability and assistance for ambulation. Shoe insoles cushion and modify stance, and therefore may help prevent lesions. Crutches and walking frames are used for restoring normal gait. Orthotic devices improve the quality of life of haemophilia patients with musculoskeletal lesions and are useful for preventing lesions resulting from strenuous activities, whether performed in the course of daily living or in the pursuit of games and sports. [ABSTRACT FROM AUTHOR]

The article focuses on the study that evaluates the usefulness of ultrasonography in assessing the evolution and monitoring treatment of haemarthrosis. It outlines the advantages of ultrasonography such as yielding objective measurements of echo-structure and requiring no sedation of paediatric patients. It suggests that systematic ultrasound explorations are performed with own resources at the Haemophilia Treatment Centres in all muscle-skeletal haemorrhages.

Summary. This paper outlines the results obtained in a cross-sectional study of a group of young patients with severe haemophilia A and B. The primary aim of the study was to ascertain the level of orthopaedic complications in the group, the effects that these complications have on quality of life, and the medical resources used on these patients. The secondary aim was to relate their current orthopaedic state to the type of treatment received before the study. The study was carried out in 11 hospitals in Spain, where 70 severe haemophilia patients (factor VIII [FVIII] < 2%), with an average age of 21.6 and a median age of 22, and no inhibitors, were monitored. The percentage of patients suffering from articular complaints was 84.3% and 85.7% according to the Gilbert and Petterson scales, respectively. Furthermore, pain was reported in 16.1% of joints, most frequently in ankles. Using the SF-36 Health Survey, patients were observed to have a poorer quality of life relative to healthy controls. Despite high levels of both the coagulant factor given to patients and the financial resources dedicated to their treatment, the type of treatment (on demand and/or as secondary prophylaxis) provided to the young adults was found to be incapable of preventing haemophilic arthropathy and subsequent negative quality of life consequences. [ABSTRACT FROM AUTHOR]