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Background: Balance alterations are one of the main problems in people with intellectual disabilities (ID), increasing their risk of falls and impacting their life. Aims: To describe a vestibular rehabilitation programme (VRP) and evaluate its effects on the ability to maintain balance and risk of suffering a fall. Methods: Forty-seven adults with mild to moderate ID were randomly assigned to two groups--a control group (CG, N = 24), which performed a general physical exercise only, and an experimental group (EG, N = 23) which also completed a VRP. The variables, used pre- and post-training and 1 month after the intervention, were as follows: Center of Pressure Displacement, Berg Scale, Timed Up and Go Test, and the Modified Clinical Test of Sensory Interaction and Balance. Results: The EG improved significantly in each variable. The CG did not show changes for any of the parameters. Conclusions: A programme based on VRP may improve balance and reduce the risk of falling.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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

Introduction: Eccentric training has been associated with several specific physiological adaptations. The flywheel machine is one of the easiest ways of performing eccentric overload training. However, no studies evaluated its feasibility, safety and muscle activity in patients with haemophilia (PWH). Aim: To evaluate feasibility and safety and compare muscle activity during flywheel vs weight machine knee extension exercise in severe PWH. Methods: Eleven severe PWH [mean age of 33.5 (8.1) years] participated in this cross‐sectional study after receiving prophylactic treatment. Surface electromyography (EMG) signals were recorded for the rectus femoris during the knee extension exercise performed with 2 different conditions (flywheel and weight machine) with matched intensity (6 on the Borg CR10 scale). Kinesiophobia was assessed before and after the experimental session. Participants were asked to rate tolerability of each condition. Adverse effects were evaluated 24 and 48 hours after the session. Results: Kinesophobia did not increase after the experimental session, and no adverse effects were reported. At 60%‐70% of the contraction cycle, the flywheel exercise showed higher (P =.024) eccentric rectus femoris muscle activity than the weight machine. In contrast, during the last 90%‐100% of the contraction cycle, the traditional weight machine showed higher (P =.004) rectus femoris activity than the flywheel. Conclusion: The knee extension exercise performed with the flywheel at moderate intensity is safe and well tolerated among severe PWH under adequate factor coverage. Importantly, the flywheel variation provides higher eccentric rectus femoris activity at the breaking force moment, while it provides lower eccentric muscle activity at the end of the cycle. [ABSTRACT FROM AUTHOR]

We report the case of a 39-year-old male patient affected by type B Niemann-Pick disease, in whom pulmonary involvement became evident 15 years after the initial diagnosis. Pulmonary involvement was discovered incidentally during the evaluation of a dry cough and exertional dyspnoea which occurred in the context of an acute febrile, self-limiting illness. In this case, the pulmonary involvement is clinically mild, with minimal alteration of the diffusing capacity for carbon monoxide (DL(CO)), despite moderate fibrosis and widespread infiltration of both alveoli and interstitium by sea blue histiocytes. [ABSTRACT FROM AUTHOR]

Patients with hemophilia need to strictly follow exercise routines to minimize their risk of suffering bleeding in joints, known as hemarthrosis. This paper introduces and validates a new exergaming software tool called HemoKinect that intends to keep track of exercises using Microsoft Kinect V2’s body tracking capabilities. The software has been developed in C++ and MATLAB. The Kinect SDK V2.0 libraries have been used to obtain 3D joint positions from the Kinect color and depth sensors. Performing angle calculations and center-of-mass (COM) estimations using these joint positions, HemoKinect can evaluate the following exercises: elbow flexion/extension, knee flexion/extension (squat), step climb (ankle exercise) and multi-directional balance based on COM. The software generates reports and progress graphs and is able to directly send the results to the physician via email. Exercises have been validated with 10 controls and eight patients. HemoKinect successfully registered elbow and knee exercises, while displaying real-time joint angle measurements. Additionally, steps were successfully counted in up to 78% of the cases. Regarding balance, differences were found in the scores according to the difficulty level and direction. HemoKinect supposes a significant leap forward in terms of exergaming applicability to rehabilitation of patients with hemophilia, allowing remote supervision. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study was to evaluate the differences between primiparous and multiparous women at 6 months postpartum and nulliparous women regarding the thickness (including thickness pattern) and contractile function of the abdominal and lumbar muscles., Methods: A cross-sectional observational study of 80 women, both primiparous (n = 29) and multiparous (n = 25), as well as including nulliparous as a comparison group (n = 26), was conducted. B-mode ultrasound imaging was used for the measurement of abdominal and lumbar muscle thickness in 2 conditions (resting and activation). Moreover, the contractile function of these muscles was determined by the thickness ratio., Results: Significant differences between groups were observed in abdominal muscle thickness. During activation, the obliques showed a lower thickness in both groups of postpartum women compared to nulliparous women, while lower values for the thickness for the transverse abdominis (TrA) were only observed in primiparous women. The thickness pattern observed in nulliparous women both in resting and in activation (internal oblique [IO] > external oblique [EO] > TrA) was also achieved among postpartum women, despite no statistical differences observed between IO and EO in these groups. No significant differences between groups were observed for contractile function, showing the TrA greater values, followed by IO and EO in both nulliparous and postpartum women., Conclusions: This study suggests that the thickness (and the thickness pattern) of the abdominal muscles in women at 6 months postpartum differs from nulliparous, while for the lumbar muscles, it is comparable to nulliparous. However, despite the obliques being thinner in postpartum women, the abdominal muscles are functionally competent., Impact: This is believed to be the first study to compare abdominal and lumbar muscles thickness and contractile function specifically between primiparous and multiparous women. Although at 6 months' postpartum abdominal thickness differs from nulliparous women, these muscles are functionally competent. These findings provide valuable insights for use in the development of postpartum interventions., (© 2024 American Physical Therapy Association. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Testicular Leydig cells (LC) regulate the proper development of male individuals, both during fetal life (fetal LC) and puberty (adult LC). In the ovaries of adult women, there are cells that are very similar to Leydig cells, the ovarian hilus cells (OHC), which also produce testosterone. The origin of these cells, in both sexes, remains unknown and is still a matter of debate. We have studied the location, characteristics and relationships of the OHC in 90 patients. The indications for oophorectomy were: metrorrhagia (n=9), prolapse (n=8), endometrial hyperplasia (n=14), cancer (endometrial, myometrial, or cervical) (n=35), uterine leiomyomata (n=14), and various ovarian tumors (cysts and benign tumors, borderline and malignant) (n=10). In addition to the hilus, occasionally the nodules, nests and clusters of OHC were located in the mesovarium, the mesosalpinx, and in the medullar and cortical regions of the ovaries. The morphological (including crystalloids of Reinke) and immunohistochemical (positivity for calretinin and alpha-inhibin) findings were similar to those described for testicular LC. Therefore, OHC can be considered ovarian Leydig cells (OLC). LC are usually found in small numbers in the ovaries, but if one looks for them intentionally, one always finds them. Close relationships were observed between the OLC with nerves and vessels. Moreover, an intraneural location of the OLC was demonstrated in all cases, and these intraneural cells showed similar characteristics to extraneural OLC, suggesting that they derive from endoneural cells which are present in the vegetative nerves of the ovaries.