Objectives:
To investigate the effect of trunk training on trunk control, arm, and pulmonary function in children with Duchenne muscular dystrophy.
Design:
A randomised controlled trial.
Settings:
...Neuromuscular diseases clinic of university hospital.
Subjects:
Twenty-six children with Duchenne muscular dystrophy aged 5–16 were included in the study.
Intervention:
Participants were randomly allocated into two groups. The study group (N = 13) exercised with the trunk-oriented exercise program and the conventional exercise program, whereas the control group (N = 13) underwent the conventional exercise program for eight weeks.
Main measures:
The primary outcomes were trunk control was assessed using the Trunk Control Measurement Scale, the arm function was assessed using Performance of Upper Limb, and respiratory function using the pulmonary function test. Data collection was conducted at baseline, and eighth week. The differences in trunk control scores, arm function scores, and respiratory function values before and after the training were calculated for the intergroup comparison.
Results:
The mean age of the participants was 11.6 (2.6) in the study group and 10.6 (3.4) in the control group. The changes between trunk control score, arm function score (total and distal level score), and respiratory function value (Forced Vital Capacity, Forced Expiratory Volume in one second, and Peak Expiratory Flow Volume percentage values) were compared and significant differences were found after eight week periods in the study and control groups.
Conclusions:
Trunk-oriented exercise program in Duchenne muscular dystrophy might be effective for trunk control, arm, and respiratory function.
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NUK, OILJ, SAZU, UKNU, UL, UM, UPUK
Previously genome-wide association methods in patients with classic bladder exstrophy (CBE) found association with ISL1, a master control gene expressed in pericloacal mesenchyme. This study sought ...to further explore the genetics in a larger set of patients following-up on the most promising genomic regions previously reported. Genotypes of 12 markers obtained from 268 CBE patients of Australian, British, German Italian, Spanish and Swedish origin and 1,354 ethnically matched controls and from 92 CBE case-parent trios from North America were analysed. Only marker rs6874700 at the ISL1 locus showed association (p = 2.22 × 10
). A meta-analysis of rs6874700 of our previous and present study showed a p value of 9.2 × 10
. Developmental biology models were used to clarify the location of ISL1 activity in the forming urinary tract. Genetic lineage analysis of Isl1-expressing cells by the lineage tracer mouse model showed Isl1-expressing cells in the urinary tract of mouse embryos at E10.5 and distributed in the bladder at E15.5. Expression of isl1 in zebrafish larvae staged 48 hpf was detected in a small region of the developing pronephros. Our study supports ISL1 as a major susceptibility gene for CBE and as a regulator of urinary tract development.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Background
Kinesiophobia can be a barrier for physical activity in patients with multiple sclerosis (PwMS) and it can develop as a result of fear and avoidance reactions due to fatigue. However, ...there is no valid and reliable scale available to assess kinesiophobia due to fatigue in PwMS.
Aims
To investigate the test–retest reliability and construct validity of the Tampa Scale of Kinesiophobia-Fatigue (TSK-F) in PwMS.
Methods
Eighty-seven PwMS were included in the study. In addition to TSK-F, the following measurements were used for construct validity: Expanded Disability Status Scale (EDSS), Fatigue Severity Scale (FSS), Fatigue Impact Scale (FIS), 6-Minute Walking Test (6MWT), International Physical Activity Questionnaire (IPAQ), Beck Depression Inventory (BDI), Multiple Sclerosis Quality of Life Scale-54 (MSQoL-54). TSK-F was administered twice (3–7 days apart) to measure test–retest reliability.
Results
The intraclass correlation coefficient of the TSK-F was 0.867. It had a weak correlation with the IPAQ and EDSS, moderate correlation with the MSQoL-54 and 6MWT, and strong correlation with the BDI, FSS, and FIS (respectively, rho − 0.345, rho 0.365, rho 0.544, rho − 0.449, rho 0.690, rho 0.602, rho 0.650). The scale had good performance to discriminate the disease severity with the area under the curve (AUC) value 0.730.
Conclusions
TSK-F has excellent reliability and moderate-to-good validity in evaluating kinesiophobia and the scale may be a useful outcome measurement for assessment of kinesiophobia due to fatigue in PwMS.
Objective
To explore the effects of aerobic training adding to home-based exercise program on motor function and muscle architectural properties in children with Duchenne muscular dystrophy.
Design
...This is a prospective randomized controlled study.
Setting
Pediatric neuromuscular clinic in a tertiary care center.
Subjects
Children with Duchenne muscular dystrophy.
Interventions
Children were randomly divided into two groups whereby 12-weeks aerobic training was additionally given in treatment group in contrast to the control group which received only home-based exercise program.
Main Measures
Motor Function Measure and Six Minute Walk Test were used for clinical evaluation, and muscle architectural properties (thickness, pennation angle and fascicle length) were measured by ultrasound imaging. Both groups were assessed at baseline and after 12-weeks of training.
Results
Median age of children was 7.9 years in the treatment group and 8.6 years in the control group (p > 0.05). Significant improvements were obtained for Motor Function Measure and Six Minute Walk Test from baseline to 12-weeks in the treatment group; Motor Function Measure total score changed from 83.2 (6.1) to 86.9 (4.0) vs. 82.3 (10.2) to 80.4 (9.4) points in the control group (p = 0.006); 6 Minute Walk Test distance changed from 395.3 (46.6) to 413.0 (52.3) vs. 421.7 (64.4) to 393.8 (68.2) meters in the control group (p < 0.001). However, muscle architectural parameters did not change during study period (p > 0.05).
Conclusion
Aerobic training may be of additional value in improving motor function and performance with no remarkable effect on muscle architectural properties.
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NUK, OILJ, SAZU, UKNU, UL, UM, UPUK
Objective
The aims of this study were (a) to examine the surface electromyography (sEMG) amplitude values of the lower limb muscles during stair climbing both between different functional levels of ...Duchenne muscular dystrophy (DMD), in comparison with healthy children, and (b) to investigate the relationships between sEMG amplitudes and physical performance.
Methods
sEMG amplitudes of the lower limbs of twenty-one children with DMD between levels I and III according to the Brooke Lower Extremity Functional Classification Scale and eleven healthy peers were evaluated by using sEMG during stair climbing task. Physical performance was evaluated by 6-min walk test and ascending 4-step timed performance test.
Results
The lower limb sEMG amplitude values of children with DMD were statistically higher than healthy children (
p
< 0.001). sEMG amplitudes of the right (
p
= 0.01) and left (
p
= 0.003) biceps femoris, the right (
p
< 0.001) and left (
p
= 0.001) gastrocnemius medialis, and the right vastus lateralis (
p
= 0.02) muscles were higher in children with levels 2–3 than those in level 1. Moderate-to-strong relations were found between the gastrocnemius medialis and biceps femoris sEMG amplitudes and physical performance assessments (
p
< 0.05).
Conclusion
Increased sEMG amplitude values in the lower limbs during stair climbing task are thought to be caused by the effort to compensate for progressive muscle weakness and are associated with lower physical performance in children with DMD. Further, sEMG amplitude values are determined to increase as the functional level deteriorates.
Clinical trial registration number and URL
NCT04287582 (
https://clinicaltrials.gov/ct2/show/NCT04287582?term=merve+bora&draw=2&rank=1
).
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DOBA, EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, IZUM, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, SIK, UILJ, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Patients with Duchenne Muscular Dystrophy (DMD) have gait disorders. Therefore, specific gait assessment tools are needed.
The aim of this study was to develop a gait assessment instrument for DMD ...patients (DMD-GAS), and investigate its validity and reliability.
The scale was developed considering the expert opinions which included 10 physiotherapists who had experience in the management of patients with DMD, and the Content Validity Index (CVI) was calculated. The final version of the DMD-GAS that was agreed upon the experts consisted of 10 items, and each item scored between 0 and 2. The intra-rater reliability was established by the video analysis of children with a 1-month interval and inter-rater reliability was determined by the scores of 3 physiotherapists.
The study included 56 patients with DMD.
The criterion validity was determined by investigating the relationship between the total score of the DMD-GAS and Motor Function Measure (MFM), 6 Minute Walk Test (6MWT), and the data obtained from GAITRite.
The CVI of the DMD-GAS was 0.90 (p < 0.05). The construct validity and internal consistency of the DMD-GAS were excellent as well as the intra- and inter-rater reliability (>0.90). Moderate-to-very strong correlations were found between the total score of the DMD-GAS and the MFM-total score (r = 0.78), 6MWT (r = 0.71), gait speed (r = 0.50), stride length (r = 0.56), and base of support (r = −0.70) (p < 0.01).
The results indicated that DMD-GAS was a reliable and valid instrument to determine gait characteristics of the patients with DMD in clinical settings.
NCT05244395.
•This study presents a new scale that can assess the kinetic properties of each part of the body during walking and the spatio-temporal characteristics of gait in a disease-specific manner.•DMD-GAS was a reliable, valid and practical assessment instrument for gait deviations.•DMD-GAS promises a practical assessment of gait deviations of children with DMD.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UILJ, UL, UM, UPCLJ, UPUK, ZAGLJ, ZRSKP
The purpose of this study was to investigate the effects of electrotherapy and exercise on pain intensity and mobility of connective tissue in patients with myofascial pain syndrome (MPS) in their ...cervical region.
60 patients were divided into 3 groups using a random allocation programme method. A hotpack was applied, and ultrasound was carried out on the patients in the treatment group who were also given exercise training. Exercise training was only given to the exercise group while the control group was given two weeks rest. The demographic characteristics, autonomic symptoms, and other MPS-associated symptoms of patients were recorded. The Short-Form McGill Pain Questionnaire was used to evaluate the intensity and quality of pain, and a skin-roll test was used to evaluate connective tissue mobility.
There was a statistically significant difference between treatment and control group on the sensory pain, total pain, and Visual Analog Scale measurements (p< 0.05). There was a decrease in connective tissue sensitivity measurements in the treatment and control groups. The connective tissue tension measurements were also decreased after treatment in the treatment and exercise groups.
We concluded that combined treatment was more effective to decrease pain intensity, and increase connective tissue mobility.
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DOBA, FSPLJ, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Periparturient dönemde, süt sentezinin başlaması ve yem tüketiminin azalması sonucu enerji ihtiyacının artması, ineklerde negatif enerji dengesinin (NED) ortaya çıkmasına neden olur. İneklerin NED’e ...uyum sağlayamaması sonucu, yağlı karaciğer ve ketozis gibi metabolik hastalıklar ortaya çıkmaktadır. Karaciğer yağlanması sonucu ketozis, aboma-sum deplasmanı, metritis gibi hastalıklar, immün sistemin baskılanması ve reprodüktif performansta azalma görülmek-tedir. Süt ineklerinin bu dönemde maruz kaldıkları metabolik stres hem doğal hem de kazanılmış bağışıklık üzerinde birçok olumsuzluklara sebep olmaktadır. Hem doğal hem de kazanılmış bağışıklık sisteminin düzenli çalışması için gerekli bir molekül olan sitokinlerin organizmadaki birçok önemli fizyolojik ve patolojik olayda görevleri vardır. Sitokinler fonksiyonlarına göre proinflamatuvar ve antiinflamatuvar olarak sınıflandırılabilir. Proinflamatuvar sitokinler (İnterlökin-1 Beta (IL-1β), Tümör Nekrozis Faktör-Alfa (TNF-α), IL-6, IL-15, IL-8) özellikle lökositleri aktive ederek organizmanın patojen mikroorganizmalara ve tümörlere karşı savunmasında rol alırlar. Antiinflamatuvar sitokinler ise (IL-4, IL-10, IL-13) inflamatuvar cevabı sınırlandırırlar. Bu derlemede, süt inekçiliğinde periparturient dönemdeki immün sistem fonksi-yonlarının önemli göstergelerinden biri olan sitokin konsantrasyonlarının, metabolik değişiklikler ve bu dönemde gözle-nen hastalıklarla arasındaki ilişkisi hakkında bilgi verilmesi amaçlandı.
The increase in energy requirement as a result of the start of milk synthesis and decrease in feed con-sumption causes negative energy balance (NEB) in cows in periparturient period. Inability to adapt to the NEB results in metabolic diseases such as fatty liver and ketosis. Ketosis, abomasum displacement, metritis, immune system sup-pression and decrease in reproductive performance are seen as a result of fatty liver. The metabolic stress experi-enced by dairy cows during this period causes dysfunction in many ways on both natural and acquired immunity. The metabolic stress of dairy cows during this period cause many negative effects on both natural and acquired immunity. Cytokines, a molecule necessary for the functioning of both the innate and acquired immune system, have roles in many important physiological and pathological events in the organism. Cytokines can be classified as pro-inflammatory and anti-inflammatory according to their functions. Pro-inflammatory cytokines (Interleukine-1 Beta (IL-1β), Tumor Ne-crosis Factor Alpha (TNF-α), IL-6, IL-15, IL-8) play a role in the defence of the organism against pathogenic microor-ganisms and tumours, in particular by activating leukocytes. Anti-inflammatory cytokines (IL-4, IL-10, IL-13) limit the inflammatory responses. In this review, it was aimed to give information about cytokine concentrations, which is one of the important indicators of immune system functions in dairy cattle periparturient period, its relationship with metabolic changes and diseases observed during this period.
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IZUM, KILJ, NUK, PILJ, PNG, SAZU, UL, UM, UPUK
Duchenne muscular dystrophy (DMD) is a disease characterized by progressive loss of muscle fiber, gradually from proximal to distal. Although a few studies have investigated hand grip strength in ...non-ambulatory DMD patients, a lack of literature was found determining its relationship with functional capacity.
The aim of this study was to determine the associations between hand grip strength and functional measures in non-ambulatory children with DMD.
Hand grip strength was evaluated using a dynamometer in children with DMD. The children with DMD were evaluated with the Turkish version of the Egen Klassifikation Scale Version 2 (EK2) for global functional capacity, the Performance of Upper Limb (PUL) for upper limb functional performance and the ABILHAND-Kids for hand ability.
The mean age of 38 DMD children was 12.02 ± 1.99 years. Dominant hand grip strength of the children with DMD was higher than the non-dominant hand (p < 0.05). The EK2 was 13.02 ± 5.50, PUL was 49.86 ± 14.34 and ABILHAND-Kids was 26.81 ± 7.59. Hand grip strength was found to be correlated with the EK2 (p < 0.05).
It is known that measuring functional ability and strength in very weak children with DMD has been difficult and complex for therapists/clinicians in the clinical environment. Although there is a moderate correlation, hand grip strength may be used in clinical practice as a practical assessment tool to have an immediate insight into the global functional capacity in non-ambulatory DMD children.
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