Clinical mutations in the TERT and TERC genes coding for telomerase components induced oxidative stress, DNA damage at telomeres and cell apoptosis besides decreased telomerase activity.

Telomeres are nucleoprotein structures at the end of chromosomes that maintain their integrity. Mutations in genes coding for proteins involved in telomere protection and elongation produce diseases such as dyskeratosis congenita or idiopathic pulmonary fibrosis known as telomeropathies. These diseases are characterized by premature telomere shortening, increased DNA damage and oxidative stress. Genetic diagnosis of telomeropathy patients has identified mutations in the genes TERT and TERC coding for telomerase components but the functional consequences of many of these mutations still have to be experimentally demonstrated. The activity of twelve TERT and five TERC mutants, five of them identified in Spanish patients, has been analyzed. TERT and TERC mutants were expressed in VA-13 human cells that express low telomerase levels and the activity induced was analyzed. The production of reactive oxygen species, DNA oxidation and TRF2 association at telomeres, DNA damage response and cell apoptosis were determined. Most mutations presented decreased telomerase activity, as compared to wild-type TERT and TERC. In addition, the expression of several TERT and TERC mutants induced oxidative stress, DNA oxidation, DNA damage, decreased recruitment of the shelterin component TRF2 to telomeres and increased apoptosis. These observations might indicate that the increase in DNA damage and oxidative stress observed in cells from telomeropathy patients is dependent on their TERT or TERC mutations. Therefore, analysis of the effect of TERT and TERC mutations of unknown function on DNA damage and oxidative stress could be of great utility to determine the possible pathogenicity of these variants.

Effects of resistance training on muscle strength in adults with haemophilia: A systematic review and meta-analysis.

INTRODUCTION: Although resistance training is frequently prescribed for people with haemophilia (PWH), no previous meta-analyses have quantified the effect of this intervention on muscle strength, nor the implications of the intervention's modality and duration. AIM: (1) To determine the effects of resistance training on muscle strength in adults with haemophilia; (2) To determine the most effective duration and modality among the exercise protocols. METHODS: A systematic search from inception until 28 November 2023 was conducted in PubMed, Embase, Web of Science, CENTRAL and CINAHL databases. We included randomised controlled trials or before-after studies that involved resistance training without other physiotherapy co-interventions. Study selection, data extraction and risk of bias assessment were independently performed by two reviewers. Disagreements were resolved in consultation with a third author. The level of evidence was determined according to the GRADE methodology. RESULTS: Seven studies were included. Measurements of knee extensor strength and elbow extensor strength were included in the meta-analysis. Subgroup analysis showed significant effects for both elastic resistance protocols (SMD: 0.54; 95% CI: 0.02-1.07) and conventional training (isometric and weight-based equipment) (SMD: 0.88; 95% CI: 0.50-1.25), demonstrating small and moderate effect sizes respectively. Additionally, both protocols of duration 5-7 weeks (SMD: 1.16, 95% CI: 0.63-1.69) as well as those of duration ≥8 weeks (SMD: 0.57, 95% CI: 0.20-0.94) showed a significant difference. CONCLUSION: Resistance training is effective in improving muscle strength of the knee and elbow extensors in PWH. Both elastic resistance and conventional training show benefits.

Acute neuromuscular and perceptual responses to blood flow restriction exercise in adults with severe haemophilia: A pilot study.

INTRODUCTION: No previous studies have implemented a standard blood flow restriction (BFR) training session in people with severe haemophilia (PwH), where this type of training has been contraindicated. AIMS: The purpose of this study was to evaluate the tolerability, adverse events, and neuromuscular and perceptual responses to an acute session of low load (LL) knee extensions with BFR in PwH under prophylaxis. METHODS: Eight PwH performed one LL-BFR session with 40% arterial occlusion pressure (AOP). Perceptual responses and adverse effects were assessed, together with high-density surface electromyography of vastus medialis (VM) and lateralis (VL). RESULTS: Significant normalized root mean square differences were found within each set, but not between sets. Spatial distribution (centroid displacement (p > .05), modified entropy (VM, set two, cycles three and five, p = .032) and coefficient of variation (VM, set two, cycles four and five lower than cycle three (p = .049; p = .036)) showed changes within each set. Median frequency showed a slight increase during cycle four of set four (p = .030). Rate of perceived exertion slightly increased with each set while tolerability slightly decreased in the last set and fear of training with BFR generally decreased after the session. CONCLUSIONS: In PwH, a LL-BFR session at 40% AOP is safe and feasible. Our results suggest that potential muscle impairments may blunt neuromuscular adaptations induced by BFR.

The importance of a healthy lifestyle despite chronic pain: Prospective cohort with 11-year register follow-up.

The purpose of the study was to investigate to which extent a healthy lifestyle in female healthcare workers with chronic pain contributes to reducing the risk of disability pension. We conducted a prospective cohort study with an 11-year registry follow-up. Overall, 2386 Danish female healthcare workers with chronic pain completed a questionnaire about work and lifestyle (leisure-time physical activity, smoking, and body mass index (BMI)). Data on disability benefit payments were obtained from the Danish Register for Evaluation of Marginalization. Two models (minimally and fully adjusted for different potential confounders) were tested using the Cox proportional hazards model. During the follow-up period, 17.9% of the healthcare workers obtained disability pension. Low levels of leisure time physical activity (reference: moderate level) increased the risk of disability pension in the minimally (Hazard Ratio: 1.38 (95% CI: 1.14-1.69)) and fully adjusted models (Hazard Ratio: 1.27 (95% CI: 1.04-1.56)). Being highly physically active, as opposed to being moderately active, did not confer additional protection. Additionally, a positive association was observed between smoking and disability pension in the minimally adjusted model (Hazard Ratio: 1.27 (95% CI: 1.05-1.54)). BMI was not an influential factor. In female healthcare workers with chronic pain, at least moderate levels of physical activity is a protective factor for disability pension. Effective promotion strategies should be designed for both workplace and non-workplace settings.

Risk profile and mode of transmission of Mpox: A rapid review and individual patient data meta-analysis of case studies.

Since May 2022, an outbreak of Mpox in non-endemic countries has become a potential public health threat. The objective of this rapid review was to examine the risk profile and modes of transmission of Mpox. PubMed, Web of Science, and Scopus were searched from inception through July 30 to collect case reports/series on patients with Mpox infection. For meta-analysis, data on the total number of participants and deaths by binary categories of exposure (age, sex, country, other co-infections or existing conditions, and mode of contagion) were used. A total of 62 studies (4659 cases) were included. Most cases came from Africa (84.3%), followed by Europe (13.9%). In 63.6% of the cases, the mode of contagion was human contact, while 22.8% of the cases were by animal contact, and 13.5% were unknown or not reported. The mortality rate was 6.5% throughout these studies. The risk of mortality was higher in the younger age group (risk difference: 0.19; 95% CI: 0.02-0.36), in cases with other co-infections or current chronic conditions (risk difference: 0.03; 95% CI: 0.01-0.05) and in the category of low- and middle-income countries (risk difference: 0.06; 95% CI: 0.05-0.08). There were no significant differences with respect to sex or mode of contagion. These results help to understand the major infection pathways and mortality risk profiles of Mpox and underscores the importance of preventing outbreaks in specific settings, especially in settings densely populated by children, such as day care centres and schools.

Is the frequency of breakfast consumption associated with life satisfaction in children and adolescents? A cross-sectional study with 154,151 participants from 42 countries.

BACKGROUND: The promotion of daily breakfast consumption and the importance of making appropriate breakfast choices have been underscored as significant public health messages. The aim of this study was to examine the relationship between breakfast frequency and life satisfaction in large and representative samples of school-going children and adolescents aged 10-17 years from 42 different countries. METHODS: This study used information from the 2017/2018 Health Behavior in School-aged Children study, comprising nationally representative samples of children and adolescents aged 10-17 years who were attending school. The total number of participants from the 42 countries included in the study was 155,451 (51.3% girls). The evaluation of breakfast consumption in this study involved a specific question: "How often do you typically have breakfast (more than a glass of milk or fruit juice)?". To measure life satisfaction, a subjective assessment scale was used in the form of a ladder, visually spanning from 0 to 10. On this scale, the topmost point (10) denotes the highest conceivable quality of life, whereas the bottom point (0) represents the worst imaginable quality of life. RESULTS: After adjusting for several covariates, the lowest estimated marginal mean of life satisfaction was identified in those participants who skipped breakfast (mean [M] = 5.6, 95% confidence interval [CI] 5.5 to 5.8). Conversely, the highest estimated marginal mean of life satisfaction was observed in those who had breakfast every day (M = 6.5, 95% CI 6.3 to 6.6). Overall, a nearly linear relationship between higher frequency of breakfast and greater life satisfaction in children and adolescents was identified (p-for-trend < 0.001). In addition, the highest estimated marginal mean of life satisfaction score was identified in those participants from Portugal who had breakfast every day (M = 7.7; 95% CI 6.9 to 8.5 points). Conversely, the lowest estimated marginal mean of life satisfaction was observed in those participants from Romania who no breakfast (M = 3.5; 95% CI 2.6 to 4.4 points). CONCLUSIONS: There is a nearly linear relationship between higher frequency of breakfast and greater life satisfaction in children and adolescents. Considering the potential health advantages associated with breakfast during this critical age phase, these findings imply the necessity for additional global efforts to promote increased breakfast consumption among children and adolescents.

Correlation of Self-Efficacy for Pelvic Floor Muscle Exercise with Symptoms of Stress Urinary Incontinence in Women.

INTRODUCTION AND HYPOTHESIS: Self-efficacy for pelvic floor exercises, i.e. confidence in achieving pelvic floor contractions, may predict adherence to treatment. However, there is a paucity of literature investigating the clinical relevance of this outcome. The aim was to determine the relationship between self-efficacy for pelvic floor exercise and symptom severity, pelvic floor distress and impact on quality of life, as well as sociodemographic characteristics and pelvic floor muscle strength in women with stress urinary incontinence (SUI). METHODS: A cross-sectional study was conducted in women with SUI. The Spanish version of the Broome Pelvic Muscle Self-Efficacy Scale was used to assess self-efficacy for pelvic floor exercise. The dependent variables were: urinary incontinence symptoms using the International Consultation on Incontinence Questionnaire, short form (ICIQ-SF), pelvic floor distress symptoms using the Urogenital Distress Inventory-6, impact on quality of life using the Incontinence Impact Questionnaire (IIQ-7), leakage using the 1-h pad test, number of SUI episodes per week and pelvic floor muscle strength. RESULTS: A total of 56 women with a median age of 44.5 years were included. Self-efficacy for pelvic floor exercise correlated negatively and moderately with the ICIQ-SF (r = -0.529; p < 0.001), IIQ-7 (r = -0.442; p = 0.001), 1-h pad test (rs = -0.467; p < 0.001); and number of SUI episodes/week (rs = -0.489; p < 0.001). Correlation with the other outcomes was weak or non-existent. Linear regression with forward selection showed that the ICIQ-SF was the variable most related to self-efficacy (ß: -3.01, 95% CI: -4.03 to -1.69). CONCLUSIONS: These findings highlight the importance of assessing self-efficacy for pelvic floor exercise in the treatment of women with SUI. Future prospective studies are needed to confirm these findings.

The Effectiveness of Adding Transvaginal Radiofrequency to Pelvic Floor Muscle Training for 6 Weeks in Women with Stress Urinary Incontinence. A Double-Blind Randomised Controlled Trial.

INTRODUCTION AND HYPOTHESIS: The objective was to evaluate the clinically beneficial effect of adding transvaginal monopolar non-ablative radiofrequency (RF) to pelvic floor muscle training (PFMT) on leakage severity, quality of life and urinary incontinence-related symptoms in women with stress urinary incontinence (SUI). METHODS: A double-blind randomised controlled trial was conducted, with a 6-week intervention and a 6-month follow-up. Participants were randomly assigned to the experimental group (PFMT plus RF; n = 18) or the control group (PFMT plus placebo; n = 20). The primary outcome was the International Consultation on Incontinence Questionnaire Short Form (ICIQ-SF). Secondary outcomes included the Pelvic Floor Distress Inventory-20 (PFDI-20) and the Pelvic Floor Impact Questionnaire-7 (PFIQ-7), self-efficacy, female sexual function, pelvic floor muscle dynamometry, 1-h pad test and number of SUI episodes per week. Inferential analyses of the data were performed using a two-factor analysis of variance. RESULTS: Both groups achieved a statistically significant improvement in ICIQ-SF over time. However, the differences observed in the experimental group exceeded the minimal clinically important differences by 4 points (MD = -9.4, 95% CI = -12.6 to -6.3), which was not observed in the control group (MD = -3.9, 95% CI = -6.9 to -1.0). This was maintained at the 6-month follow-up with a significant time*group interaction (p < 0.001, ηp2 = 0.150). There was no time*group interaction in the other variables (p > 0.05). Additionally, a significant difference in favour of the experimental group was observed in the 1-h pad test and episodes of SUI per week (p < 0.05). CONCLUSIONS: This study highlights the beneficial effects of adding transvaginal RF to PFMT on the severity and amount of leakage, as well as on the quality of life and urinary incontinence-related symptoms in women with moderate SUI. Future trials are needed to assess the effects of this intervention in women with severe SUI.

Association Between Pain Coping and Symptoms of Anxiety and Depression, and Work Absenteeism in People With Upper Limb Musculoskeletal Disorders: A Systematic Review and Meta-analysis.

OBJECTIVE: To determine the prospective association of pain coping strategies and symptoms of anxiety and depression with work absenteeism in people with upper limb musculoskeletal disorders. DATA SOURCES: A systematic search of PubMed, Web of Science, Embase, Cochrane Library, and Scopus databases was conducted from inception to September 23, 2022. STUDY SELECTION: Prospective observational studies of adults with upper limb musculoskeletal disorders were included. Included studies had to provide data on the association of pain coping strategies (catastrophizing, kinesiophobia, self-efficacy or fear avoidance) or symptoms of anxiety and depression with work absenteeism. DATA EXTRACTION: Study selection, data extraction, and assessment of methodological quality (Newcastle Ottawa Scale) were performed by 2 independent authors. Random-effects models were used for quantitative synthesis. DATA SYNTHESIS: Eighteen studies (n=12,393 participants) were included. Most studies (77.8%) reported at least 1 significant association between 1 or more exposure factors (pain coping strategies or symptoms of anxiety and depression) and work absenteeism. Meta-analyses showed a statistically significant correlation between the exposure factors of catastrophizing (r=0.28, 95% confidence interval [CI]: 0.15 to 0.40; P<.0001) and symptoms of anxiety and depression (r=0.23, 95% CI: 0.10 to 0.34; P=.0003) with work absenteeism. The correlation between self-efficacy and work absenteeism was non-significant (r=0.24, 95% CI: -0.02 to 0.47; P=.0747). CONCLUSIONS: Rehabilitation teams should consider assessing catastrophizing and symptoms of anxiety and depression to identify patients at risk for work absenteeism. Addressing these variables may also be considered in return-to-work programs for individuals with upper limb disorders.

Individuals with chronic ankle instability show altered regional activation of the peroneus longus muscle during ankle eversion.

Individuals with chronic ankle instability (CAI) present muscular weakness and potential changes in the activation of the peroneus longus muscle, which likely explains the high recurrence of ankle sprains in this population. However, there is conflicting evidence regarding the role of the peroneus longus activity in CAI, possibly due to the limited spatial resolution of the surface electromyography (sEMG) methods (i.e., bipolar sEMG). Recent studies employing high-density sEMG (HD-sEMG) have shown that the peroneus longus presents differences in regional activation, however, it is unknown whether this regional activation is maintained under pathological conditions such as CAI. This study aimed to compare the myoelectric activity, using HD-sEMG, of each peroneus longus compartment (anterior and posterior) between individuals with and without CAI. Eighteen healthy individuals (No-CAI group) and 18 individuals with CAI were recruited. In both groups, the center of mass (COM) and the sEMG amplitude at each compartment were recorded during ankle eversion at different force levels. For the posterior compartment, the sEMG amplitude of CAI group was significantly lower than the No-CAI group (mean difference = 5.6% RMS; 95% CI = 3.4-7.6; p = 0.0001). In addition, it was observed a significant main effect for group (F1,32 = 9.608; p = 0.0040) with an anterior displacement of COM for the CAI group. These findings suggest that CAI alters the regional distribution of muscle activity of the peroneus longus during ankle eversion. In practice, altered regional activation may impact strengthening programs, prevention, and rehabilitation of CAI.

Neuromuscular Responses and Perceptions of Health Status and Pain-Related Constructs in End-Stage Knee Osteoarthritis During Resistance Training With Blood Flow Restriction.

ABSTRACT: Ogrezeanu, DC, López-Bueno, L, Sanchís-Sánchez, E, Carrasco, JJ, Cuenca-Martínez, F, Suso-Martí, L, López-Bueno, R, Cruz-Montecinos, C, Martinez-Valdes, E, Casaña, J, and Calatayud, J. Neuromuscular responses and perceptions of health status and pain-related constructs in end-stage knee osteoarthritis during resistance training with blood flow restriction. J Strength Cond Res 38(4): 762-772, 2024-We aimed to evaluate the neuromuscular responses and their relationship with health status, kinesiophobia, pain catastrophizing, and chronic pain self-efficacy in patients with end-stage knee osteoarthritis during acute resistance training with different levels of blood flow restriction (BFR). Seventeen patients with end-stage knee osteoarthritis participated in 3 experimental sessions separated by 3 days, performing 4 sets of knee extensions with low load and 3 levels of concurrent BFR performed in a random order: control (no BFR), BFR at 40% arterial occlusion pressure (AOP), and BFR at 80% AOP. Normalized root-mean-square (nRMS), nRMS spatial distribution (centroid displacement, modified entropy, and coefficient of variation), and normalized median frequency (nFmed) were calculated from the vastus medialis (VM) and lateralis (VL) using high-density surface electromyography. Subjects were asked to report adverse effects after the sessions. In the VM, nRMS was higher with 80% AOP than with 40% AOP ( p = 0.008) and control ( p < 0.001), whereas there were no differences between conditions in the VL. Normalized root-mean-square also showed an association with pain catastrophizing, chronic pain self-efficacy, and health status (VM: -0.50, 0.49, -0.42; VL: -0.39, 0.27, -0.33). Spatial distribution varied between conditions but mostly in the VL. Overall, nFmed did not vary, with only a slight increase in the VL with 40% AOP, between set 3 and 4. BFR during knee extensions at 80% AOP increases VM activity and VL amplitude distribution more than 40% AOP and control. Importantly, muscle activity increases are modulated by pain catastrophizing, chronic pain self-efficacy, and health status in these patients, and kinesiophobia seems to especially modulate entropy.

Comorbidity burden and nutritional status are associated with short-term improvement in functional independence and pain intensity after hip fracture surgery in older adults with in-hospital rehabilitation.

PURPOSE: Hip fracture is a common condition among older adults. The aim of this study was to explore the influence of nutritional status and comorbidity burden on changes in functionality, fall risk, and pain intensity one month after hip surgery in older adults with in-hospital rehabilitation. METHODS: Thirty-six hip fracture patients (55.6% female) aged 65 years or older with indication for surgical resolution were recruited. The main outcomes were functional independence (Barthel Index), risk of falls (Downton Falls Risk Index) and pain intensity (Visual Analogue Scale), assessed preoperatively and one month after discharge. Covariates included age, sex, BMI, Charlson Comorbidity Index (CCI) and nutritional status (Mini Nutritional Assessment). For the inferential analysis, a one-way analysis of covariance (ANCOVA) was applied. RESULTS: Significant improvements were observed in functional independence (11.0 points, 95% CI: 1.7 to 20.3), risk of falls (-2.8 points, 95% CI: -4.0 to -1.7) and pain intensity (-2.6 points, 95% CI: -3.4 to -1.9). Among the covariates, a significant interaction was found between the CCI and improvements in functional independence (F=7.03, p=0.010, η2p=0.093), while nutritional status showed a significant interaction with pain reduction (F=5.65, p=0.020, η2p=0.075). CONCLUSION: A lower comorbidity burden was associated with greater postoperative functional independence, while better nutritional status was associated with a greater reduction in postoperative pain intensity.

Risk and Preventive Factors for Depressive Symptoms Among Older Chilean Adults During the SARS-CoV-2 Outbreak: A Longitudinal Study.

OBJECTIVE: To specifically examine the multiple factors related to the increase in depressive symptoms during the COVID-19 outbreak in older adults in Chile. METHOD: A longitudinal study was conducted using a dataset from a nationally representative survey cohort of Chilean older adults followed at three time points during the COVID-19 outbreak. The main outcome was depressive symptoms (Patient Health Questionnaire scale). The independent variables included: age, sex, educational level, geographic area, living alone, self-perceived health, self-reported resilience, loneliness, and social isolation. RESULTS: A total of 424 older adults were included. Female sex (ß = 0.95, 95% CI: 0.22 to 1.68) and loneliness (ß = 1.21, 95% CI: 1.05 to 1.37) were the main risk factors for an increase in depressive symptoms in older adults. In contrast, living outside the metropolitan region (ß=-0.70, 95% CI: -1.39 to -0.02), living in company (ß=-0.34, 95% CI:-1.24 to 0.56), having better self-perceived health (ß=-5.04, 95% CI:-6.33 to -3.75) and greater resilience (ß=-0.30, 95% CI: -0.38 to -0.23) were preventive factors. CONCLUSION: These results provide useful evidence to develop mental health prevention or control strategies for older adults. CLINICAL IMPLICATIONS: The findings highlight the importance of a holistic approach to health care for older adults that integrates strategies to address loneliness, foster resilience, and promote an active social life.

Gender differences on effects of forearm rotation on compressive stiffness of flexor carpi ulnaris during submaximal handgrip contractions.

Little is known about gender differences in stiffness of forearm muscles during voluntary actions. This study aimed to investigate the effect of forearm rotation on flexor carpi ulnaris (FCU) stiffness in men and women during submaximal handgrip contractions. During a single session, measurements were made on 20 young participants (9 females). Two positions of the forearm were compared in random order with the elbow flexed 90 degrees: (i) neutral position and (ii) maximal supination. In each position, participants performed two submaximal handgrip contractions at 25% and 50% of maximal voluntary contraction, while compressive stiffness was collected using a hand myometer (MyotonPRO). A mixed repeated measurement ANOVA was applied to assess the interaction between gender, forearm position, and contraction intensity. The FCU stiffness is affected by handgrip contraction intensity (p < 0.001), gender (p < 0.001), BMI (p = 0.009), and forearm rotation (p = 0.007). Only the gender factor was found to have significant interaction with forearm rotation (p = 0.037). Men's FCU was stiffer than women's in both positions and contraction intensities (p < 0.05). Only in men a significant increase in FCU stiffness was observed when comparing contraction intensities at both forearm positions (p < 0.05), as well as when the forearm was rotated from neutral to supine at both intensities (p < 0.05). In conclusion, FCU stiffness during handgrip contraction differed significantly between men and women. Women have fewer stiffness changes in FCU when performing different levels of handgrip contraction. We also observed that only men increased FCU stiffness by changing the forearm position from neutral to supine position for both handgrip intensities.

Dose-response association of handgrip strength and risk of depression: a longitudinal study of 115 601 older adults from 24 countries.

BACKGROUND: Prior research has solely focused on the association between handgrip strength and risk of depression in single countries or general populations, but more knowledge is required from wider-spread cohorts and target populations. AIMS: This study aimed to investigate the association between handgrip strength and risk of depression using repeated measures in adults aged 50 years and over. METHOD: Data on handgrip strength and risk of depression were retrieved from the Survey of Health, Ageing and Retirement in Europe (SHARE) waves 1, 2, 4, 5, 6 and 7, using a hand dynamometer (Smedley, S Dynamometer, TTM) and the EURO-D 12-item scale, respectively. Time-varying exposure and covariates were modelled using both Cox regression and restricted cubic splines. RESULTS: A total of 115 601 participants (mean age 64.3 years (s.d. = 9.9), 54.3% women) were followed-up for a median of 7.3 years (interquartile range: 3.9-11.8) and 792 459 person-years. During this period, 30 208 (26.1%) participants experienced a risk of depression. When modelled as a continuous variable, we observed an inverse significant association for each kg increase of handgrip strength and depression up to 40 kg in men and up to 27 kg in women. CONCLUSIONS: Being physically strong may serve as a preventive factor for depression in older adults, but this is limited up to a maximum specific threshold for men and women.

Exercise variables and pain threshold reporting for strength training protocols in people with haemophilia: A systematic review of clinical trials.

INTRODUCTION: Although strength exercise is often prescribed for people with haemophilia (PWH), it remains unknown how exercise variables and pain thresholds are used to prescribe strength training in PWH. AIM: To analyse how strength exercise variables and pain thresholds have been used to prescribe strength training in PWH. METHODS: A systematic search was conducted in PubMed, Embase, Web of Science, CENTRAL and CINAHL databases from inception to 7 September 2022. Studies whose intervention included strengthening training in adults with haemophilia were included. Two independent reviewers were involved in study selection, data extraction and risk of bias assessment. RESULTS: Eighteen studies were included. The least reported variables among the studies were: prophylactic factor coverage (11.1%), pain threshold/tolerability (5.6%), intensity (50%), total or partial range of motion (27.8%), time under tension (27.8%), attentional focus modality (0%), therapist experience in haemophilia (33.3%) and adherence assessment (50%). In contrast, weekly frequency (94.4%), duration (weeks) (100%), number of sets/repetitions (88.9%), repetitions to failure/not to failure (77.8%), types of contraction (77.8%), rest duration (55.6%), progression (55.6%), supervision (77.8%), exercise equipment (72.2%) and adverse event record (77.8%) had a higher percentage of reported (>50% of studies). CONCLUSION: Future research on strength training for PWH should improve information on pain threshold and other important variables such as prophylactic factor coverage, intensity, range of motion, time under tension, attentional focus modality, therapist experience in haemophilia and adherence assessment. This could improve clinical practice and comparison of different protocols.

Barriers and facilitators of physical activity in adults with severe haemophilia: A qualitative study.

BACKGROUND: People with haemophilia (PWH) tend to be less physically active than the general population, but there is a lack of research on the specific barriers and facilitators affecting their participation in physical activities. OBJECTIVES: This study aims to explore perceived barriers and facilitators to physical activity in severe PWH. DESIGN: An explorative qualitative study based on focus groups. METHODS: Four focus groups including 16 participants (severe haemophilia A patients) were conducted to examine the factors perceived as facilitators or barriers to haemophiliacs engaging in physical activity. One researcher conducted a thematic analysis of all data. RESULTS: Three themes were identified: body function, personal factors, and environmental factors. Key facilitators identified were access to prophylaxis treatment to reduce the risk of bleeding(s), the enjoyability of physical activity, fitness and health motives, social interaction, support, and low cost. PWH faced additional barriers to being physically active including hurtful joints, mobility issues, haemophilic arthropathy, dislike or disinterest, lack of motivation, fear of injury, tiredness, lack of time, lack of guidance, negative social influence, restriction, and lack of coordination of prophylaxis treatment. CONCLUSION: This exploratory study demonstrated that participation in physical activity in PWH is influenced not only by their own abilities and attitudes, but also by external variables, including family, friends, healthcare professionals, structures, and communities. The results of this study may be used to assist caregivers and health professionals, inform programs, interventions, and policies to promote physical activity and health in severe PWH.

Effectiveness of progressive moderate-vigorous intensity elastic resistance training on quality of life and perceived functional abilities in people with hemophilia: Secondary analysis of a randomized controlled trial.

INTRODUCTION AND AIM: Strength exercise training is advised for people with hemophilia (PWH); however, few studies have been published and have methodological limitations. The purpose of this study was to evaluate the effectiveness of progressive elastic resistance training on quality of life and perceived functional abilities in PWH. METHODS: Participants were randomly allocated to the intervention (n = 10) or control (n = 10) group. The intervention group performed progressive moderate-vigorous elastic resistance training (2 days/week, a total of 8 weeks), focusing on the muscles of the knee, elbow, and ankle joints. The control group continued its usual daily activities for 8 weeks. Quality of life (A36 Hemofilia-QoL®) and perceived functional abilities (Haemophilia Activities List) were assessed at baseline and an 8-week follow-up. RESULTS: The intervention group improved the quality-of-life dimension of joint damage perception in comparison to the control group (p = .015, large effect size). Regarding perceived functional abilities, the intervention group improved lying, sitting, kneeling, and standing (p = .006, small effect size), and complex lower extremities activities (i.e., walking short and long-distance, and up-down stairs) (p = .006, small effect size) compared to the control group. No other significant differences were observed. CONCLUSIONS: Eight weeks of progressive moderate-vigorous elastic resistance training in PWH improve the quality-of-life dimension of joint damage perception and perceived functional abilities (lying/sitting/kneeling/standing, and complex lower extremities activities). Our results suggest a limited effect of the program on the other items of quality of life measured by the questionnaire as emotional functioning, mental health, and social relationships. Importantly, no serious adverse effects occurred.

Safety, feasibility, and neuromuscular activity of acute low-load resistance exercise with or without blood flow restriction in patients with severe hemophilia.

OBJECTIVE: To compare the safety, feasibility, and neuromuscular activity of acute low-load resistance exercise with/without blood flow restriction (BFR) in people with severe hemophilia (PwH). METHODS: Eight PwH under prophylaxis (5 with resistance training experience) performed 6 randomly ordered conditions of 3 intensity-matched knee extensions: no external load and no BFR, no external load and light BFR (20% of arterial occlusion pressure [AOP]), no external load and moderate BFR (40% AOP), external low load and no BFR, external low load with light BFR, and external low load with moderate BFR. Rated perceived exertion, pain, exercise tolerability, and adverse effects were assessed. Normalized root-mean-square (nRMS), nRMS spatial distribution, and muscle fiber-conduction velocity (MFCV) were determined using high-density surface electromyography for the vastus medialis and lateralis. RESULTS: Exercises were tolerated, without pain increases or adverse events. Externally resisted conditions with/without BFR provided greater nRMS than nonexternally resisted conditions (p < 0.05). Spatial distribution and MFCV did not vary between conditions. CONCLUSIONS: In these patients, knee extensions with low external resistance and BFR at 20% or 40% AOP appear safe, feasible and do not cause acute/delayed pain. However, BFR during three consecutive repetitions does not increase nRMS nor changes nRMS spatial distribution or MFCV.

Musculoskeletal pain intensity and risk of long-term sickness absence in the general working population: A prospective cohort study with register follow-up.

Determining predictors of sickness absence could allow for better screening, guidance, and development of preventive efforts aimed at those in increased risk. This study aimed to determine the prospective association between musculoskeletal pain intensity and risk of incident register-based long-term sickness absence in the general working population, as well as to determine the population attributable fraction. Drawing on data from a nation-wide questionnaire survey, this prospective cohort study followed a representative sample of the Danish general working population without recent long-term sickness absence (≥6 consecutive weeks) (n = 69,273) for long-term sickness absence up to two years (mean follow-up: 93 weeks) in a national register. The predictor was musculoskeletal pain intensity in the neck/shoulder and low-back during the preceding three months rated on an 11-point numerical rating scale from 0 to 10. The weighted incidence of long-term sickness-absence was 8.9% during two-year follow-up (n = 6165). We observed a clear dose-response association between musculoskeletal pain intensity of the neck/shoulder or low-back and the risk of incident long-term sickness absence, with a lower threshold of increased risk of 4 and 3 (scale 0-10) for neck/shoulder (HR (95% CI): 1.25 (1.09-1.42)) and low-back pain (HR (95% CI): 1.13 (1.00-1.29)), respectively. Prevention of pain intensities at or above 4 out of 10 could potentially prevent 17% (population attributable fraction, PAF (95% CI): 16.8 (13.6-20.1)) of the total long-term sickness absence in the general working population. Large-scale interventions to prevent and manage musculoskeletal pain need to be documented and implemented.