Harmful alcohol use among acutely ill hospitalized medical patients in Oslo and Moscow: A cross-sectional study.

BACKGROUND: The aim was to estimate the prevalence of harmful alcohol use in relation to socio-demographic characteristics among acutely ill medical patients, and examine identification measures of alcohol use, including the alcohol biomarker phosphatidylethanol 16:0/18:1 (PEth). METHODS: A cross-sectional study, lasting one year at one hospital in Oslo, Norway and one in Moscow, Russia recruiting acute medically ill patients (≥ 18 years), able to give informed consent. Self-reported data on socio-demographics, mental distress (Symptom Check List-5), alcohol use (Alcohol Use Disorder Identification Test-4 (AUDIT-4) and alcohol consumption past 24 h were collected. PEth and alcohol concentration were measured in whole blood. RESULTS: Of 5883 participating patients, 19.2% in Moscow and 21.1% in Oslo were harmful alcohol users, measured by AUDIT-4, while the prevalence of PEth-positive patients was lower: 11.4% in Oslo, 14.3% in Moscow. Men in Moscow were more likely to be harmful users by AUDIT-4 and PEth compared to men in Oslo, except of those being ≥ 71 years. Women in Oslo were more likely to be harmful users compared to those in Moscow by AUDIT-4, but not by PEth for those aged < 61 years. CONCLUSIONS: The prevalence of harmful alcohol use was high at both study sites. The prevalence of harmful alcohol use was lower when assessed by PEth compared to AUDIT-4. Thus, self-reporting was the most sensitive measure in revealing harmful alcohol use among all groups except for women in Moscow. Hence, screening and identification with objective biomarkers and self-reporting might be a method for early intervention.

Validation of the Haemophilia & Exercise Project-Test-Questionnaire (HEP-Test-Q)-An instrument for the assessment of subjective physical functioning in children with haemophilia.

INTRODUCTION: Contemporary haemophilia management recommends sport and physical activity in children with haemophilia. Assessment of subjective physical functioning requires standardized and validated instruments. AIMS: To adapt and psychometrically test the adult Haemophilia & Exercise Project-Test-Questionnaire (HEP-Test-Q) for children (aged 6-17 years). METHODS: In discussion rounds with children, single items of the adult HEP-Test-Q were reformulated to make them understandable without changing the item concept. The validation of the child-adapted version in children with haemophilia (n = 228) included pre-testing with feasibility testing, cognitive interviewing (n = 34), pilot-testing of the revised version in the EIS Study (n = 67) and field-testing in the SO-FIT Study (n = 127). RESULTS: Pre-testing revealed a completion time of 8.2 ± 4.1 minutes and children liked the instrument. Cognitive interviews demonstrated that most items were easy to understand; 9 items were reformulated. Pilot-testing demonstrated good psychometric characteristics in terms of reliability (α = .94 Total Score) and validity. Convergent validity testing showed moderate correlations with the Haemo-QoL (r = -.491), but low correlations with the Petrini Score (r = -.293). Known groups' validity revealed significant differences in clinical subgroups; chronic pain (P < .002) and target joints (P < .021). Field-testing confirmed psychometric characteristics; Cronbach's alpha ranged from α = .80 ("endurance") to α = .94 (Total Score). The child-adapted HEP-Test-Q showed moderate correlations with the PedHAL (r = .634, P < .0001) and the Haemo-QoL SF (r = -.575, P < .0001). Known groups' validity testing proved that the HEP-Test-Q could discriminate between clinical subgroups. CONCLUSION: The child-adapted HEP-Test-Q is a short, practical and acceptable instrument for the assessment of subjective physical functioning. Outcomes can be compared to adults because item concepts are identical to the adult version.

Pain and structural alterations in knee joints in patients with haemophilia.

INTRODUCTION: Patients with haemophilia (PwH) suffer from haemophilic arthropathy which leads to an enhanced pain sensitivity. The aim of this study was to determine whether the individual pain condition in terms of pressure pain thresholds (PPT) at the knee joints is linked to changes of underlying anatomical structures in PwH. MATERIAL AND METHODS: Eleven landmarks at both knee joints of 36 PwH and 36 controls were examined in terms of PPT and ultrasound sonography (US). PPT were used to generate four groups: pain sensitive and insensitive knees of PwH and controls. RESULTS: PPT of the knee joints were significantly decreased at all landmarks in PwH when compared to controls (P ≤ .004). US findings revealed that especially osteophytes are more pronounced in pain-sensitive knees of PwH in comparison with pain-insensitive knees of PwH or pain-(in)sensitive knees of controls. The synovia tissue was also thickened in PwH when PPT was altered. In contrast to findings in osteoarthritis-related pain, no differences between the groups were found regarding effusion, whether assessed, for example on the distal edge of m. vastus lateralis (P = .893) or on the lateral joint space (P = .417). CONCLUSION: Particular degenerative changes in terms of osteophytes and thickness of synovial tissue are associated with an enhanced pain sensitivity in PwH. Altered PPT which were not associated with structural findings may be an indicator for a complex peripheral and/or central sensitization of the affected joints in PwH. The role of this mechanism should be clarified in further studies.

Cost-utility of a six-month programmed sports therapy (PST) in patients with haemophilia.

INTRODUCTION: Recurrent musculoskeletal haemorrhages in people with haemophilia (PwH) lead to restrictions in the locomotor system and, as a result, in physical performance, too. Due to its physical and psychological benefits, sport is increasingly re-commended for haemophilic patients. Evidence on the cost-effectiveness of sports therapy is still lacking. AIM: The aim of this study was to determine the cost-effectiveness of a 6-month programmed sports therapy (PST). METHODS: The cost-effectiveness of the 6-month PST was assessed from a societal perspective alongside a RCT using cost-utility analysis. The analysis included 50 PwH with moderate-to-severe haemophilia A and B and a training period over 6 months. The health-related quality of life was measured with the EuroQoL-domain questionnaire. Resource utilization was assessed by questionnaire before and after the intervention. A cost-effectiveness acceptability curve was constructed, and sensitivity analyses were performed. RESULTS: During the 6-month study period, mean adjusted total healthcare costs were lower (mean difference: -22 805 EUR; 95%-CI: -73 944-48 463; P = .59) and the number of QALYs was higher in the intervention group (mean difference: 0.3733; 95%-CI: 0.0014-0.0573; P = .04). The probability of an incremental cost-effectiveness ratio <50 000 EUR per QALY was 71%. The performed sensitivity analysis confirmed these results. CONCLUSION: Results showed that the PST is effective in terms of a significant gain of QALYs. Furthermore, results weakly indicate the potential of the PST to reduce healthcare costs. Future studies should expand the observation period to have a closer look at the influence of PST on lifetime costs.

Somatosensory profile of patients with haemophilia.

INTRODUCTION: Patients with haemophilia (PwH) suffer from an enhanced pain sensitivity due to repetitive joint bleedings. A comprehensive, quantitative examination of the somatosensory system has not been performed in this population to date. MATERIAL AND METHODS: Thirty patients with moderate or severe haemophilia A or B and 30 healthy controls were examined by means of Quantitative Sensory Testing to assess the function of the somatosensory system. Detection (DT) and pain thresholds (PT) were determined, amounting to a total of 13 parameters. Both knee joints and the hand as reference were examined in order to assess both joint-specific as well as general changes in the somatosensory profile. RESULTS: Analysing DT and PT, a significant main effect was found for group × stimulus interaction (P ≤ .001). Post hoc tests revealed significant differences in DT between PwH and controls for thermal stimuli across both knees (cold DT: P < .001; warm DT: P < .01) and the hand (cold DT: P < .01; warm DT: P < .05). Mechanical DT was increased in PwH at both knee joints (left knee: P ≤ .05; right knee: P ≤ .01). Furthermore, pressure PT was decreased in PwH at both knees (P ≤ .001). CONCLUSION: Haemophilic arthropathy leads to alterations of the somatosensory profile in PwH. Our results reveal initial evidence of a combination of peripheral sensitization, indicated by decreased pressure PT and mechanical DT at the knee joints, as well as general changes of the somatosensory system, shown by reduced thermal DT at affected sites and remote from these. Therefore, both mechanisms have to be considered regarding the pain management in PwH.

Trunk muscle activation characteristics in patients with severe haemophilia.

INTRODUCTION: Recurrent bleeding episodes in patients with haemophilia (PWH) lead to joint alterations and therewith disturbed muscle coordination patterns. Major weight-bearing joints are affected most. However, possible effects on trunk muscle activity have not been examined so far. The objective of this work was to study consequences of haemarthropathy on characteristics of trunk muscles in PWH while standing on surfaces with different mechanical properties. METHODS: Surface EMG of internal oblique (IO) and multifidus (MF) muscles were bilaterally recorded during a natural bilateral stance in 20 PWH with severe haemophilia A [age: 42 years (SD: 10)] and 25 non-haemophilic controls [NHC, 43 (12)]. Amplitude ratios, a symmetry index between sides and the co-activation ratio of IO over MF served as outcome measures and compared standing on three different surfaces (stable, soft, unsteady). RESULTS: PWH revealed markedly restricted lower extremity joints (P < 0.001), but without any hint of back pain. Neither result revealed significant main or interaction effects of 'group' (P > 0.24). Group-independent analyses showed amplitude ratios (MF: P < 0.05) as well as symmetry indices (MF: P < 0.02) significantly altered by 'surface' in NHC only. Effects of utilizing soft vs. unsteady surfaces were not detectable (P > 0.77). CONCLUSION: Utilizing unstable surfaces does not lead to altered trunk muscle activity in PWH. Differently than expected, a quite similar behaviour of lower trunk muscles in terms of applied indices can be found in PWH and NHC. Ascending alterations of muscle coordination in PWH could not be verified.

RCT - subjective physical performance and quality of life after a 6-month programmed sports therapy (PST) in patients with haemophilia.

INTRODUCTION: Musculoskeletal bleedings lead to limitations in the locomotor system and consequently, in health-related quality of life (HRQoL) in patients with haemophilia (PwH). Sports therapy is increasingly recommended to improve their physical performance. Until today, randomised controlled studies investigating changes in physical performance in PwH are rare. AIM: This study investigates the impact of programmed sports therapy on the subjective physical performance and the HRQoL in PwH. METHODS: A randomised controlled trial (RCT) was conducted with a training intervention for over 6 months. For this purpose, 64 PwH with moderate (n = 5) or severe (n = 59) haemophilia A (n = 57) or B (n = 7) were randomised into two groups - intervention (IG) or control group (CG). The HRQoL was assessed with the SF-36 questionnaire and the disease-specific Haem-A-QoL before and after the intervention. The subjective physical performance was tested by the HEP-Test-Q. RESULTS: After the 6-month training intervention, PwH in the IG subjectively reported significant better 'endurance' (P = 0.000) in the HEP-Test-Q compared to the CG. In the SF-36, a significant difference in the domains 'general health perceptions' (P = 0.005) and 'mental health' (P = 0.001) was detected. The haemophilia-specific HRQoL questionnaire showed a significant improvement in the dimensions 'feeling' (P = 0.049), 'work' (P = 0.046) and 'family' (P = 0.040). CONCLUSION: In the first RCT evaluating the impact of a 6-month training intervention on the subjective perception of PwH, an increase in subjective physical performance and some domains of HRQoL was demonstrated in the IG. Specific sports therapy should be included into the comprehensive treatment under supervision and monitoring by experienced staff.

RCT of a 6-month programmed sports therapy (PST) in patients with haemophilia - Improvement of physical fitness.

UNLABELLED: Physical fitness is of major importance for patients with haemophilia (PwH) but is highly influenced by bleeding episodes. Although some cohort studies describe an improvement of physical fitness after training intervention, randomized controlled studies (RCT) in PwH are still rare. AIM: The aim of this study was to prove the enhancement of physical work capacity in PwH by programmed sports therapy (PST). METHODS: This RCT includes a training period over six months. Sixty-four PwH with moderate (n = 5) to severe (n = 59) haemophilia A (n = 57) and B (n = 7) were randomized into two groups - training intervention group (IG) and control group (CG) by block randomization. Complex strength measurements, joint score, coordination check and a 12-min walking test were carried out before and after training intervention. RESULTS: Significant differences were tested in M. triceps brachii (Δ+0.62 N kg(-1) ), biceps brachii (Δ+1.25 N kg(-1) ), latissimus dorsi (Δ+0.59 N kg(-1) ), rectus abdominis (Δ+0.51 N kg(-1) ), biceps femoris (right: Δ+0.68 N kg(-1) ; left: Δ+0.59 N kg(-1) ) and the quadriceps femoris (right: Δ+0.71 N kg(-1) ; left: Δ+0.55 N kg(-1) ) after intervention between the two groups (all p ≤ 0.003). Furthermore, an increase in distance (Δ+171.5 m) covered in the 12-min walking test (P = 0.011) was observed. Regarding one-leg stand, a significant improvement (P = 0.037) in the IG (Δ+2.2 s right leg) after intervention could be determined. CONCLUSION: For the first time, a study with a corresponding number of adult PwH in a RCT-design showed that programmed sport therapy with specific instructions over 6 months has a positive effect on physical performance of PwH, independent of constitution and disease process.

Pain sensitivity in patients with haemophilia following moderate aerobic exercise intervention.

BACKGROUND: Physical activity is influenced by pain and vice versa. Although studies recommend exercise therapy for patients with haemophilia (PwH), the influence of physical activity on the pain condition in PwH has not been investigated so far. AIM: Aim of this study was to examine the effect of a treadmill intervention with self-chosen velocity on the acute pain sensitivity in PwH. PATIENTS AND METHODS: Twenty PwH [aged 24-58 years, moderate (n = 3) to severe (n = 17) haemophilia A (n = 17) or B (n = 3)] and 20 control subjects (aged 26-61 years) were included in this study. Eighteen PwH and all controls completed a treadmill intervention for 30 min. Pressure pain thresholds (PPT) in Newton (N) were measured at both the knees, ankles and elbows, sternum and forehead before (pre) and immediately after walking (post). RESULTS: PwH and controls walked with comparable speed (mean speed in km h-1 ; PwH: 3.5, controls: 3.8), resulting in significantly different values of performance-related parameters such as heart rate (mean heart rate per minute; PwH: 102, controls: 86; P ≤ 0.01). Compared to baseline values, PPT remained unaltered at all landmarks in both groups after walking (e.g. pre/post in Newton; knee right: PwH: 63.1/63.0, controls: 93.8/93.8; left knee: PwH: 62.1/62.7, controls: 90.0/93.4), indicating a non-increasing pain condition. CONCLUSION: Findings of unaltered PPT following moderate aerobic exercise showed initial evidence that PwH are able to perform an endurance exercise with self-chosen velocity for 30 min as recommended, without increasing the acute pain condition. By doing so, PwH can benefit from the positive effects of endurance exercise.

Complex strength performance in patients with haemophilia A. Method development and testing.

UNLABELLED: The aim of this study was to develop a complex strength measurement method and to apply this new method for the first time in patients with haemophilia (PwH). 20 PwH with severe haemophilia A and 20 controls were included into the study. All subjects completed ten measurements of maximum isometric strength. Furthermore, the 20 control subjects completed re-test-measurements to evaluate the method. As a result, the method showed a high reliability (ICC 0.764 to 0.934). Between the two groups significant reductions in PwH between -(19-35%) were detected, regarding the relative force of the M. triceps brachii (-19%; p = 0.008), M. biceps brachii (-19%; p = 0.031), M. latissimus dorsi (-17%; p = 0.019), M. biceps femoris right (-20%; p = 0.036) and M. quadriceps femoris (right: -29%; p = 0.004; left: -35%; p = 0.002). No differences were found for M. rectus abdominis and in the hand strength. Thus, there is no general deficit in the muscle strength in PwH. The most obvious deficits exist in the upper and lower extremities and in the back muscles. CONCLUSION: PwH should carry out complex muscle strength training and integrate it early into a comprehensive treatment concept.

Neuropathic pain in patients with haemophilia, that is the question.

Chronic pain caused by recurrent joint bleedings affects a large number of patients with haemophilia (PwH). The basis of this pain, nociceptive or neuropathic, has not been investigated so far. In other pain-related chronic disorders such as osteoarthritis or rheumatoid arthritis, initial studies showed nociceptive but also neuropathic pain features. 137 PwH and 33 controls (C) completed the painDETECT-questionnaire (pDq), which identifies neuropathic components in a person´s pain profile. Based on the pDq results, a neuropathic pain component is classified as positive, negative or unclear. A positive neuropathic pain component was found in nine PwH, but not in C. In 20 PwH an unclear pDq result was observed. In comparison to C the allocation of pDq results is statistically significant (p≤0.001). Despite various pDq results in PwH and C a similar appraisal pain quality, but on a different level, was determined. Summarising the results, there is a potential risk to misunderstand underlying pain mechanisms in PwH. In chronic pain conditions based on haemophilic arthopathy, a differential diagnosis seems to be unalterable for comprehensive and individualised pain management in PwH.

[Food consumption and dietary behaviour in haemophilia patients before and after a nutrition consultation]. / Lebensmittelkonsum und Ernährungsverhalten bei Patienten mit Hämophilie vor und nach Ernährungsberatung.

UNLABELLED: In over two-thirds of deaths, nutrition is a determining factor. Nutritional condition and dietary recommandations are unspecified in the haemophilia treatment. Aim of this study was to examine the food consumption and dietary behaviour in affected patients before and after a nutrition consultation. PATIENTS, METHODS: Data were assessed via questionnaires and compared between 38 patients with haemophilia (PwH) and 20 controls without haemophilia. Furthermore, in a randomised controlled trial 11 patients with haemophilia (PwH-I) took part in an adapted nutrition consultation and were supervised over six months as opposed to 12 patients with haemophilia (PwH-O) without intervention. PwH were compared to controls more pleased with their weight (53 vs. 40%), used more nutrition consultations in the past (53 vs. 15%) and consumed more dairy products (40 vs. 15%) and fruits (45 vs. 30%). RESULTS: After nutrition consultation PwH-I were better informed about their own blood values than PwH-O. The nutrition rated high in both groups, but PwH-I were more mindful of the feeling of satiety (9 vs. 36%) compared to PwH-O (33 vs. 17%). Moreover, PwH-I ate less under stress and/or out of boredom, showed a higher satisfaction regarding their weight and increased the liquid intake (55 vs. 73%), which remained unchanged in PwH-O with 42%. Compared to PwH-O, PwH-I ate more roughage, low-fat food, fish and fruits, therefore consuming less coffee/tea. CONCLUSION: Food consumption and dietary behaviour are similar between patients with haemophilia and controls without haemophilia. A nutrition consultation affected the food consumption and dietary behaviour in patients with haemophilia positively and can consequently contribute to preservation of health and prevention of nutritional diseases.

[Age-related activation of the muscles during upright standing in patients with haemophilia]. / Altersabhängige Ansteuerung der Muskulatur im Stand bei Patienten mit Hämophilie.

Thehaemophilicarthropathyaffects thefunction of theknee joint muscles. The aim of thisstudywas to investigatethe myoelectrical signal ofknee jointmusclesin different agestages during upright standing. Surface EMG (SEMG) amplitudes of quadriceps, hamstrings and gastrocnemii were measured in 191 patients with severe haemophilia A (n=164) and B (n=27) while standing on an even surface. After an age-based classification of patients into the subgroups H(A): 17-29 (n = 37), H(B): 30-39 (n = 50), HC: 40-49 (n = 61), H(D): 50-70 in years (n = 43) the clinical WFH score for the ankle and knee joint was determined. To normalize the SEMG values amplitude ratios (percentage of cumulated activity) were calculated with respect to the specific limb. With increasing age, the patient showed descriptively a deterioration of the joint situation. The extensors of the knee joint reached significantly higher absolute and percentage levels in the muscle activity with increasing age (p < 0.05). The absolute amplitude values of the Mm. gastrocnemii showed no differences in the age groups while the relative levels were decreased. The present study shows that patients with increasing age and degree of haemophilic arthropathy develop a modified control strategy during upright standing, in the form of a shift from the plantar flexors to the extensors of the knee joint.

Impact of joint status on contraction steadiness of m. quadriceps femoris in people with severe haemophilia.

Impaired contraction steadiness of lower limb muscles affects functional performance and may increase injury risk. We hypothesize that haemophilic arthropathy of the knee and the strength status of quadriceps are relevant factors which compromise a steady contraction. This study addresses the questions if impaired steadiness of the quadriceps is verifiable in people with haemophilia (PWH) and whether a connection between the status of the knee joint and quadriceps strength exists. A total of 157 PWH and 85 controls (C) performed a strength test with a knee extensor device to evaluate their bilateral and unilateral maximal quadriceps strength and steadiness. Isometric steadiness was measured by the coefficient of variation of maximum peak torque (CV-MVIC in %). For classification of the knee joint status the World Federation of Haemophilia (WFH) score was used. Lower steadiness (higher CV values) was found in PWH compared with C during bilateral [PWH vs. C; 0.63 (0.36/1.13) vs. 0.35 (0.15/0.72), median (Q25/Q75) P < 0.001] and unilateral trials [left leg: 0.70 (0.32/1.64) vs. 0.50 (0.23/1.04), P < 0.05; right leg: 0.68 (0.29/1.51) vs. 0.39 (0.18/0.68), P < 0.001]. PWH with a WFH score difference (≥1) between their extremities showed a less steady contraction in the more affected extremity (P < 0.05). More unsteady contractions have also been found in extremities with lower quadriceps strength compared with the contralateral stronger extremities (P < 0.001), whereby the weaker extremities were associated with a worse joint status (P < 0.001). The results of this study verify an impaired ability to realize a steady contraction of quadriceps in PWH and the influence of joint damage and strength on its manifestation.

Managing haemophilia for life: 4th Haemophilia Global Summit.

The 4th Haemophilia Global Summit was held in Potsdam, Germany, in September 2013 and brought together an international faculty of haemophilia experts and delegates from multidisciplinary backgrounds. The programme was designed by an independent Scientific Steering Committee of haemophilia experts and explored global perspectives in haemophilia care, discussing practical approaches to the optimal management of haemophilia now and in the future. The topics outlined in this supplement were selected by the Scientific Steering Committee for their relevance and potential to influence haemophilia care globally. In this supplement from the meeting, Jan Astermark reviews current understanding of risk factors for the development of inhibitory antibodies and discusses whether this risk can be modulated and minimized. Factors key to the improvement of joint health in people with haemophilia are explored, with Carlo Martinoli and Víctor Jiménez-Yuste discussing the utility of ultrasound for the early detection of haemophilic arthropathy. Other aspects of care necessary for the prevention and management of joint disease in people with haemophilia are outlined by Thomas Hilberg and Sébastian Lobet, who highlight the therapeutic benefits of physiotherapy and sports therapy. Riitta Lassila and Carlo-Federico Perno describe current knowledge surrounding the risk of transmission of infectious agents via clotting factor concentrates. Finally, different types of extended half-life technology are evaluated by Mike Laffan, with a focus on the practicalities and challenges associated with these products.

[Development and evaluation of a dance-based exercise therapy for patients with haemophilia]. / Entwicklung und Evaluierung einer tanzbasierten Bewegungstherapie für Patienten mit Hämophilie.

UNLABELLED: So far, the use of methods derived from creative arts has not been considered in the haemophilia treatment. The AIM was to investigate the expectations for a dance-based exercise therapy for patients with haemophilia and the extent of its acceptance. PATIENTS, METHOD: The one-hour dance-based exercise therapy was offered to 30 haemophilia patients (HI30) (49 ± 11, 30-67 years). For the evaluation of expectations, questionnaires were created and filled out by participants before and after the intervention. Additionally, 19 haemophilia patients (HF) and 20 controls without haemophilia (KF) who did not participate in the intervention were also questioned. The RESULTS show that haemophilia patients have more experience in dance than controls (HI30:62%, HF:74%, KF:45%). In contrast, the proportion of those who are currently dancing is higher in controls without haemophilia (HI30: 17%, HF: 10%, KF:26%). The termination of dance activity in patients with haemophilia who were part of the intervention was mainly due to pain (HI30: 40%, HF: 29%, KF: 0%), whereby controls without intervention terminated the dance activity mainly due to lack of time (HI30: 30%, HF: 57%, KF: 56%). Ultimately, 24 out of 30 patients with haemophilia (HI24) completed the intervention. All HI24 met their expectations. 38% felt limited by haemophilia while carrying out the exercises. The majority of the participants were able to follow the exercises well (96%) and were did not overstrain physically (92%) nor mentally (87%), also 79% did not have pain. 23 of HI24 (96%) can envision a continuation of the dance-based exercise therapy. CONCLUSION: The experience with the dance-based exercise therapy was predominantly positive. It represents an alternative sports therapy programme for patients with haemophilia. Further studies are needed in order to make statements concerning the long-term use of such training.

Quadriceps strength, inter-extremity difference (IED) and joint status in adult persons with severe haemophilia in different age stages.

Quadriceps weakness seems to be a hallmark in adult persons with severe haemophilia (PWH). The purpose of this study was to compare PWH and non-haemophilic controls in different age stages with reference to joint status and quadriceps strength. Further aims were to examine the extent of strength-specific inter-extremity-difference (IED) and the prevalence of abnormal IED (AIED). A total of 106 adults with severe haemophilia (H) and 80 controls (C) had undergone an orthopaedic examination for classification of knee and ankle status using the WFH score. Quadriceps strength was evaluated unilaterally as well as bilaterally with a knee extensor device. Each group was divided into four age-related subgroups (HA/CA: 18-29, HB/CB: 30-39, HC/CC: 40-49, HD/CD: 50-70; in years). H presented a worse knee and ankle status than C indicated by higher WFH scores (P < 0.01). Regarding the age-matched subgroups only HB showed higher knee scores than CB (P < 0.05). The ankles were clinically more affected in HB-HD compared with those in age-matched controls (P < 0.05). H showed lower quadriceps strength than C (P < 0.05). In addition, all subgroups of H presented lower strength (HA: 10-17, HB: 19-23, HC: 35-36, HD: 53-61; in%, P < 0.05). IED was higher in H than in C [H: 12.0 (5.3/32.2) vs. C: 7.1 (2.9/10.9); Median (quartiles) in%, P < 0.001] and increased with age in H. We discovered an AIED in 35% of H. These findings highlight the importance for the early implementation of preventive and rehabilitative muscle training programmes in the comprehensive treatment of PWH.

Haemophilia & Exercise Project (HEP): the impact of 1-year sports therapy programme on physical performance in adult haemophilia patients.

Episodes of bleeding in people with haemophilia (PWH) are associated with reduced activity and limitations in physical performance. Within the scope of the 'Haemophilia & Exercise Project' (HEP) PWH were trained in a sports therapy programme. Aim of this study was to investigate subjective and objective physical performance in HEP-participants after 1 year training. Physical performance of 48 adult PWH was compared before and after sports therapy subjectively (HEP-Test-Q) and objectively regarding mobility (range of motion), strength and coordination (one-leg-stand) and endurance (12-min walk test). Sports therapy included an independent home training that had previously been trained in several collective sports camps. Forty-three controls without haemophilia and without training were compared to PWH. Of 48 PWH, 13 performed a regular training (active PWH); 12 HEP-participants were constantly passive (passive PWH). Twenty-three PWH and 24 controls dropped out because of incomplete data. The activity level increased by 100% in active PWH and remained constant in passive PWH, and in controls (P ≤ 0.05). Only mobility of the right knee was significantly improved in active PWH (+5.8 ± 5.3°) compared to passive PWH (-1.3 ± 8.6°). The 12-min walk test proved a longer walking distance for active PWH (+217 ± 199 m) compared to controls (-32 ± 217 m). Active PWH reported a better subjective physical performance in the HEP-Test-Q domains 'strength & coordination', 'endurance' and in the total score (+9.4 ± 13.8) compared to passive PWH (-5.3 ± 13.5) and controls (+3.7 ± 7.5). The 'mobility'-scale and one-leg-stand remained unchanged. Sports therapy increases the activity level and physical performance of PWH, whereby objective effects do not always correspond with subjective assessments.

[Severe and moderate haemophilia under prophylactic replacement treatment--maximal knee extensor and flexor torque of children and adolescents]. / Schwere und mittelschwere Hämophilie unter prophylaktischer Substitutionstherapie. Maximale Drehmomente der Knieextensoren und Knieflexoren bei Kindern und Jugendlichen.

UNLABELLED: Due to its influence on haemophilic arthropathy, the evaluation of knee extensor (K(Ext)) and flexor (K(Flex)) torques plays an important role in the preventive and rehabilitative context of haemophilia. Thus, the present study aimed at investigating maximal static torque (M(Max)) of K(Ext) and K(Flex). 14 boys with haemophilia (8 severe, 6 moderate; age: 11.7 ± 2.8 years; prophylactic treatment > 5 years) and 14 healthy carefully pair-matched controls (age: 11.5 ± 2.7 years) were separately measured for the left and right leg for M(Max). Furthermore, the ratio K(Flex)/K(Ext )was calculated and the joint situation assessed using the Haemophilia Joint Health Score. RESULTS: No significant group-effect was observed for M(Max) of the K(Ext) and K(Flex) as well as for the ratio K(Flex)/K(Ext) (p>0.05). Despite significant higher joint scores in haemophilic children compared to their healthy controls (p<0.01), patients merely showed minor joint impairments. CONCLUSION: Children and adolescents with severe and moderate haemophilia under prophylactic replacement treatment with a good joint status showed comparable maximal strength performance of relevant knee muscles compared to their healthy peers.