Management of muscle haematomas in patients with severe haemophilia in an evidence-poor world

Treatment studies in haemophilia focus on joint bleeds; however, some 10-25% of bleeds occur in muscles. This review addresses management of muscle haematoma in severe haemophilia, defines gaps in the published evidence, and presents a combined clinician and physiotherapist perspective of treatment modalities. The following grade 2C recommendations were synthesized: (i) Sport and activity should be based on individual factor levels, bleeding history and physical characteristics, (ii) Musculoskeletal review aids the management of children and adults, (iii) 'Time to full recovery' should be realistic and based on known timelines from the healthy population, (iv) Diagnosis should be carried out by both a clinician and physiotherapist, (v) Severe muscle bleeds should be treated similarly to surgical patients: a 50% trough for 10-14 days followed by high-level prophylaxis, (vi) Protection, rest, ice, compression and elevation should be implemented in the acute stage, and (vii) Physiotherapy and rehabilitation should be divided into: control of haemorrhage (phase 1); restoration of Range of Movement (ROM) and strength (phase 2); functional rehabilitation and return to normal living (phase 3). Recommendations specifically for inhibitor patients include: (i) Minor to moderate bleeds should be managed by home-treatment within 1 h of bleed onset using either one injection of rFVIIa 270 μg kg(-1), or two to three injections of rFVIIa 90 μg kg(-1) (2-3 h intervals), or FEIBA 50-100 U kg(-1) (repeated at 12-hourly intervals, if necessary) and (ii) Severe muscle bleeds should be supervised by the treatment centre and include bypassing agents until clinical improvement is observed.     

Common mental disorders and long‐term sickness absence in a general working population. The Hordaland Health Study

Objective: To examine and compare the prospective effect of the common mental disorders (CMD) anxiety and depression on duration and recurrence of sickness absence (SA), and to investigate whether the effect of CMD on SA is detectable over time. Method: Information from a large epidemiological health study (N = 13 436) was linked with official records of SA episodes lasting ≥16 days up to 6 years after participation. Common mental disorders were assessed with the Hospital Anxiety and Depression Scale (HADS). Associations were analysed with Cox regression and multinomial logistic regression models controlling for potential covariates. Results: Comorbid anxiety and depression, and anxiety only were significant risk factors for SA after adjusting for covariates, whilst depression only was not. Anxiety and depression were stronger predictors for longer duration of SA episodes compared with shorter duration and associated with more frequent recurrence of SA. There was a general trend toward the effect of CMD on SA becoming weaker over time; however, the effect of anxiety only on SA remained stable throughout the follow‐up. Conclusion: Common mental disorders are long‐lasting predictors of onset, duration and recurrence of SA. Anxiety appears to be a more important contributor to long‐term SA than previously described in the literature.     

Muscle phenotype in patients with myotonic dystrophy type 1

Introduction: The pathogenesis of muscle involvement in patients with myotonic dystrophy type 1 (DM1) is not well understood. In this study, we characterized the muscle phenotype in patients with confirmed DM1. Methods: In 38 patients, muscle strength was tested by hand‐held dynamometry. Myotonia was evaluated by a handgrip test and by analyzing the decrement of the compound muscle action potential. Muscle biopsies were assessed for morphological changes and Na⁺‐K⁺ pump content. Results: Muscle strength correlated with a decline in Na⁺‐K⁺ pump content (r = 0.60, P < 0.001) and with CTG expansion. CTG expansion did not correlate with severity of myotonia, proximal histopathological changes, or Na⁺‐K⁺ pump content. Histopathologically, we found few centrally placed nuclei (range 0.2–6.9%). Conclusions: The main findings of this study are that muscle weakness correlated inversely with CTG expansion and that central nuclei are not a prominent feature of proximal muscles in DM1. Muscle Nerve 47:409‐415, 2013     

Aerobic training in persons who have recovered from juvenile dermatomyositis

A recent study has shown that 36 persons who had recovered from juvenile dermatomyositis (JDM) have on average an 18% decrease in maximal oxygen uptake. The objective of this study was to investigate the effect of a 12-week aerobic training program in this group, and assess whether aerobic training can normalize aerobic capacity to the expected level for age and gender.The patients participating in the study, one male and nine females (16–42 years of age), were in remission from JDM, defined as no clinical or biochemical evidence of disease activity and no medical treatment for 1 year. The patients had a median disease duration of 3.4 years (1.4–10.3), a median treatment duration of 2.4 years (0.4–9.3) and a median duration of remission of 7.0 years (1.2–30.0).Patients trained at home on a cycle ergometer for 12 weeks at a heart rate interval corresponding to 65% of their maximal oxygen uptake (VO_2max)_. VO_2max and maximal workload (W_max) were determined before and after the 12-week training period through an incremental cycling test to exhaustion. The patients served as their own controls.Eight patients with JDM in remission completed the 12-week exercise program; one patient completed 9 weeks out of the 12-week program and one dropped out of the study. Training increased VO_2max and W_max by 26% and 30% (P < 0.001). Creatine kinase (CK) levels were normal pre-training and did not change with training, reflecting no muscle damage. We also found that at a given workload, heart rate was lowered significantly after the 12-week training period, indicating an improvement in cardiovascular fitness.This study shows that 12 weeks of moderate-intensity aerobic training is an effective and safe method to increase oxidative capacity and fitness in persons who have recovered from JDM. The results indicate that the low oxidative capacity in JDM patients in remission is reversible and can be improved. Thus, we recommend frequent aerobic training to be incorporated into supervised physiotherapy sessions in the treatment of JDM patients in remission.     

Brief checklists for assessing violence risk among patients discharged from acute psychiatric facilities: A preliminary study

Research evidence indicates that approximately 10 h a week is a sufficient intensity for short-term day treatment programmes for patients with personality disorders. In this article, we discuss which therapeutic components should be included in such a programme. Relevant research and clinical literature are reviewed. The fit between the therapeutic components and the programme as a whole is discussed according to: 1) scientific evidence of the effectiveness of the therapeutic components, 2) a sound theoretical rationale, 3) evidence of user satisfaction among patients, 4) clinical experiences of staff, 5) comprehensiveness and consistency, and 6) available therapeutic skills and resources. We advocate an 11-h treatment programme comprising small group psychotherapy, art group therapy, large group psychotherapy, cognitive group therapy, problem-solving group therapy and optional adjuncts (cognitive behavioural group therapy) for patients with additional anxiety and eating disorders.     

Health-related quality of life in refugee minors from Syria,Iraq and Afghanistan resettled in Sweden: a nation-wide,cross-sectional study

Purpose

To examine health-related quality of life (HRQoL) in refugee minors resettled in Sweden and compare results to a European reference population, while exploring associations between sociodemographic factors and HRQoL dimensions.

Methods

A cross-sectional, nation-wide study was conducted with a stratified sample of refugee minors ages 12–15 and 16–18 from Afghanistan, Iraq and Syria, resettled in Sweden between 2014 and 2018. HRQoL was measured using KIDSCREEN-27. HRQoL dimension scores of the sample were compared to mean scores of European age and gender-matched reference population. Associations between sociodemographic factors and HRQoL dimensions were investigated with independent t tests and ANOVA. A multivariable regression analysis was performed to identify the sociodemographic factors associated with HRQoL.

Results

The questionnaire was sent to 10,000 potential respondents. The response rate was 26%, yielding n = 2559 refugee minors (boys 55%, girls 45%) in the study sample. Compared to European references, minors in the present study had significantly lower scores of HRQoL within psychological wellbeing and peers and social support, whereas levels for autonomy and parent/guardian relations and school environment were higher. Several sociodemographic factors were significantly associated with all HRQoL dimensions, with those 16–18 years old, having average or poor family economy, and living with an unrelated adult or family reporting lower levels of HRQoL. Minors from Afghanistan had significantly lower scores of HRQoL for all dimensions compared to those from Iraq and Syria.

Conclusion

Refugee minors had significantly lower levels of HRQoL for psychological wellbeing and peers and social support compared to European references. Future research should further investigate this potential HRQoL gap further.