Sleep quality and daytime sleepiness amongst family caregivers of children with Spinal Muscular Atrophy.

BACKGROUND: While there are limited studies focusing on sleep quality of family caregivers of children with Spinal Muscular Atrophy (SMA), there are no studies on daytime sleepiness in SMA. AIMS: This study aimed a) to compare the sleep quality and daytime sleepiness between caregivers of children with SMA and those of healthy peers and b) to investigate the sleep quality and daytime sleepiness of family caregivers of children with different types of SMA. METHODS AND PROCEDURES: This study included 30 family caregivers of children with SMA (SMA Type 1:12, SMA Type 2:10, and SMA Type 3:8) and 31 family caregivers of healthy peers. Sleep quality and daytime sleepiness of family caregivers were evaluated using the Pittsburg Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (EPS), consecutively. OUTCOMES AND RESULTS: Demographic characteristics of children with SMA and healthy peers were similar (p > 0.05). The mean ages of family caregivers of children with SMA and healthy peers were 36.07 ± 5.84 and 35.26 ± 5.02 years, respectively (p = 0.6). The PSQI scores of family caregivers of children with SMA (7.50 ± 3.90 points) were lower than those of healthy peers (4.09 ± 1.97 points) (p < 0.001). There was no difference in PSQI scores between SMA types (p = 0.8). Also, no difference was found between SMA types and between SMA and healthy peers in terms of EPS (p > 0.05). CONCLUSIONS AND IMPLICATIONS: Family caregivers of children with SMA had poor sleep quality but similar daytime sleepiness compared with those of healthy peers. Among SMA types, family caregivers had similar sleep quality and daytime sleepiness. It was demonstrated that the sleep quality of family caregivers should be taken into consideration in the disease management of SMA.

A New Instrument to Assess Dynamic Balance in Children with Duchenne Muscular Dystrophy: Four Square Step Test and Its Validity, Reliability and Feasibility.

AIM: The aim of this study was to investigate validity, reliability and feasibility of the Four Square Step Test (FSST) in children with Duchenne Muscular Dystrophy (DMD). METHODS: The study included 44 children with DMD aged 5-12 years. The functional level of the children was determined by using the Brooke Lower Extremity Functional Classification (BLEFC). The concurrent validity of the FSST, a dynamic balance assessment, was investigated by analyzing the association between FSST and Timed Up and Go Test (TUG), North Star Ambulatory Assessment (NSAA) and Six-Minute Walk Test (6MWT) distance. The intra- and inter-rater reliability of the FSST was determined by using the Intra Class Correlation Coefficient (ICC) while the feasibility of the test was determined by calculating the percentage of children who successfully completed the test in accordance with the test instructions. RESULTS: The FSST score of the children (mean age 9.25 ± 2.14 years) was 11.91 ± 3.43 and the BLEFC level ranged from Level 1 (65.9%) to Level 3 (9.1%). FSST had positive, moderate relation with BLEFC (r = 0.447; p = .002); positive, strong relation with TUG (rs = 0.623), and negative, moderate correlations with NSAA (rs = -0.529) and 6MWT distance (rs = -0.592) (p < .001). Intra-rater (ICC: 0.965) and inter-rater (ICC: 0.991) reliability was "excellent," and the test was feasible with successful performance of 88% children. DISCUSSION: The FSST is a valid, reliable and feasible instrument to assess dynamic balance as well as having the ability to reflect falling risk and different functional levels of children with DMD.

Examination of the relationship between foot-body posture and balance and gait in Duchenne muscular dystrophy.

BACKGROUND: Although the gait and balance disturbances of Duchenne muscular dystrophy (DMD) patients were evaluated by using different methods in literature, the impact of the foot and body posture on gait and balance has not been clearly described yet in DMD. AIM: The aim of this study was to examine the relationship between foot-body posture and gait and balance in patients with DMD. METHODS: Ambulatory patients with DMD who had > 90° range of motion at ankle joint were included in the study. Foot and body posture were evaluated with the Foot Posture Index (FPI-6) and the New York Posture Rating (NYPR). The limitation degree at the ankle joint (ALD) was recorded. Gait characteristics and balance were evaluated objectively by using the GAITRite system and the Bertec Balance Check Screener™ force platform system, respectively. RESULTS: A total of 38 ambulatory patients with DMD (age: 92.44 ± 17.91 months) were recruited. Both the right and left foot FPI-6 scores were correlated with GAITRite parameters such as ambulation time, gait speed, number of steps, and left and right stride lengths (p < 0.05). A relationship was determined between right foot FPI-6 score and anterior-posterior limits of stability (LoS) obtained by force platform (p < 0.05). NYPR score was also correlated with the postural control parameters such as left and right base of support and anterior posterior LoS (p < 0.05). CONCLUSION: The current findings indicate a contribution of foot and body posture to gait and balance disorders of children with DMD. CLINICAL TRIAL NUMBER: NCT04353167, Date of registration: April 16, 2020.

The Impact of "Fear of Falling" on Physical Performance, Balance, and Ambulation in Duchenne Muscular Dystrophy.

OBJECTIVE: The aim of this study was to investigate falls and the fear of falling (FOF) in children with Duchenne muscular dystrophy (DMD) and to determine the relationships between the FOF and physical performance, balance, and ambulation. METHODS: Thirty-eight ambulatory children with DMD were included in the study. The functional level, falling history, FOF, physical performance, balance, and ambulation were assessed by using Brooke Lower Extremity Functional Classification, History of Falls Questionnaire, Pediatric Fear of Falling Questionnaire (Ped-FOF), timed performance tests, Timed "Up and Go" (TUG) test, and North Star Ambulatory Assessment (NSAA), respectively. RESULTS: Of the 38 children (mean age: 9.00 ± 2.03 years) 97.4% had a history of serious fall last year and 62.2% were injured due to this fall. The Ped-FOF score was 13.79 ± 7.20. Weak to moderate relations were determined between Ped-FOF and functional level (r = 0.33), frequency of falls (r = 0.41), duration of climbing 4-steps (r = 0.38), TUG (r = 0.36), and NSAA (r = -0.32) (p < 0.05). CONCLUSION: Ambulatory children with better performance scores had lower levels of FOF despite their history of serious falls and injuries. FOF tends to increase as the symptoms of the disease progresses. Investigating the history of falls and FOF from the earliest period will guide to take precautions and make necessary interventions on time in treatment programs.

Synthesis, antimicrobial activity and modeling studies of thiazoles bearing pyridyl and triazolyl scaffolds.

In this study, novel 4-(5-((2/3/4-substituted benzyl)thio)-4-(4-substituted phenyl)-4H-1,2,4-triazol-3-yl)-2-(pyridin-3/4-yl)thiazoles were synthesized following a multi-step synthetic procedure. All the compounds were screened with a panel of gram positive/negative bacteria, yeasts, and molds for antimicrobial activity using the disc diffusion method. Then, the minimum inhibitor concentration (MIC) and the minimum bactericidal concentration (MBC) values of active compounds were determined against Micrococcus luteus, Bacillus cereus, Listeria monocytogenes, and Staphylococcus aureus using the broth microdilution technique. These compounds were also screened for their inhibitory activities against S. aureus DNA gyrase by supercoiling assay. Furthermore, the crystal structure of S. aureus DNA gyrase B ATPase was subjected to a docking experiment to identify the possible interactions between the most active ligand and the active site. Lastly, the in silico technique was performed to analyze and predict the drug-likeness, molecular and ADME properties of the synthesized molecules.

Feasibility and Safety of Applying the Functional Electrical Stimulation to Child with Facioscapulohumeral Dystrophy: A Case Report.

AIMS: This study aims to investigate the feasibility and safety of short-term functional electrical stimulation (FES) training of the quadriceps femoris muscles in a child with facioscapulohumeral muscular dystrophy (FSHD). METHODS: A 7-year-old child with FSHD received treatment due to a decrease in functional performance and difficulty climbing stairs. The child was followed up with a home-based exercise program. FES was applied twice a week during stair climbing for six weeks. Muscle activation of the quadriceps femoris was measured using superficial electromyography, muscle strength was measured with a hand-held dynamometer, and functional performance was assessed with the 6-Minute Walk and the Stair Climb Tests before and after the treatment period. RESULTS: At the end of the treatment, there was an improvement in muscle activation. While muscle strength increased in the quadriceps femoris muscle of the non-dominant side, it remained constant on the dominant side. Functional performance test results also improved. CONCLUSIONS: FES was a feasible and safe tool to use in our case, a child with FSHD.

The effect of aerobic training on motor function and muscle architecture in children with Duchenne muscular dystrophy: A randomized controlled study.

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.

Ultrasonographic assessment of lower limb muscle architecture in children with early-stage Duchenne muscular dystrophy.

BACKGROUND: Muscle imaging methods such as ultrasound and magnetic resonance imaging have been used for many years to determine the dystrophic process in muscular dystrophies. However, the knowledge regarding muscle architecture in children at early-stage Duchenne muscular dystrophy (DMD) with different functional levels is limited. OBJECTIVE: To explore the effect of functional level on muscle architectural properties in children with early stage DMD and the difference between DMD and typically developing (TD) peers. METHODS: Thirty children with DMD (15 Grade 1 and 15 Grade 2 according to the Vignos Scale) and 5 TD peers were included. Ultrasound imaging was used to measure muscle thickness (MT), fascicle length (FL), and pennation angle (PA) of vastus lateralis (VL) and medial gastrocnemius (MG) muscles bilaterally. RESULTS: The MT and FL values for VL, and MT, FL and PA values for MG muscles were higher in children with DMD compared with those of TD peers (p<0.05). The FL of VL, and MT and FL of GM muscles of children with DMD Grade 2 were higher than those of children with DMD Grade 1 (p<0.05). CONCLUSIONS: MT and FL are increased in children with DMD compared with TD peers. Additionally, muscle architecture seems to be affected even at the early stages of the disease.

The association between trunk control and upper limb functions of children with Duchenne muscular dystrophy.

Introduction: The aim of this study was to investigate whether trunk control is associated with the upper limb function of children with Duchenne Muscular Dystrophy (DMD).Methods: The children included in the study were divided into two groups according to the Trunk Control Measurement Scale total scores. Twenty-five children whose trunk control levels were lower than 30.5 points were included in Group 1 while 31 children who had good levels of trunk control between 30.5 and 58 points were included in Group 2. General functional levels, muscular strength, and function of the upper limb were assessed and compared between the groups.Results: According to the results of the assessments, children in Group 2 were found to have better upper limb function and muscular strength than Group 1 (p < .001).Conclusions: The current study demonstrated better upper limb functional performance of children with DMD that had good-level trunk control thus suggesting the possible association between trunk stability and upper limb function. The results reveal the need to improve and protect trunk muscle strength and stability as well as upper limb strength, and to optimize trunk stability during upper limb movement in rehabilitation programs.

Investigation of surface electromyography amplitude values during stair climbing task in children with Duchenne muscular dystrophy.

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 ).

Pulmonary and upper limbs function in children with early stage Duchenne muscular dystrophy compared to their healthy peers.

BACKGROUND: Pulmonary and upper limbs function of children with Duchenne muscular dystrophy (DMD) are known to deteriorate throughout the disease process. However, there is a lack of information on the extent of impairments in the early stages of DMD when compared to healthy peers. OBJECTIVE: To investigate to what extent pulmonary and upper limbs function of children with early stage DMD are impaired. METHODS: Sixty-one children participated in the study: 31 with Grade 1 DMD (study group) according to the Brooke Upper and Lower Extremity Functional Classification Systems, and 30 age matched healthy peers (control group). Pulmonary function was determined with pulmonary function tests. The Performance of Upper Limb test was used to evaluate the upper limbs function. RESULTS: Study and control groups were homogenous in terms of physical characteristics (p>0.05). Pulmonary and upper limbs function of children with DMD were about 85% and 93% of healthy peers, respectively. CONCLUSION: This study provides evidence for deterioration of pulmonary and upper limbs function in children with early stage DMD. Better knowledge of deterioration rate over time may help therapists to better plan and update their plan of care.

Development of a questionnaire to assess fear of falling in children with neuromuscular diseases.

Fear of falling (FOF) indicates loss of confidence in the ability to perform daily living activities without falling. Although specific questionnaires do exist to assess FOF in different patient populations, any of them targets the pediatric patients with neuromuscular diseases which falling is a frequent symptom. This study aims to present the development of a self-report FOF questionnaire for children with neuromuscular diseases, pilot application of the questionnaire, and its preliminary psychometric properties. An International Classification of Functioning, Disability and Health (ICF)-based 'Pediatric Fear of Falling Questionnaire' for neuromuscular diseases (Ped-FOF) was created by the study team following the steps of developing an instrument. The questionnaire included 34 items which were organized based on the 'Activities and Participation' component of ICF-children and youth. Thirty children with Duchenne muscular dystrophy (DMD) were recruited to obtain preliminary reliability and validity results of the questionnaire. The mean Ped-FOF score of study population was 15.30 ± 7.03. According to the preliminary results, intraclass correlation coefficient was 0.715 [confidence interval (CI) 95%], and moderate correlations between Ped-FOF and functional performance and quality of life were determined (P < 0.05). Ped-FOF promises a practical assessment of FOF in pediatrics with neuromuscular diseases with understandable items that allow self-report of children. Ped-FOF also allows clinicians and therapists to assess FOF efficiently in limited clinical time. Its preliminary reliability and validity results are also sufficient to be used in DMD that falling is a frequent condition, which indicates that the use of questionnaire is promising in many other pediatric neuromuscular disorders.

Kinesiology Taping in Duchenne Muscular Dystrophy: Acute Effects on Performance, Gait Characteristics, and Balance.

AIM: This study was aimed to investigate the acute effects of kinesiology taping (KT) on physical performance, gait characteristics, and balance in early-stage Duchenne Muscular Dystrophy (DMD). METHOD: Forty-five children at early functional level of DMD were included. 6-minute walk test (6MWT), and timed performance tests were performed; gait characteristics, and balance were assessed before and one hour after taping. KT was applied to bilateral quadriceps and tibialis anterior muscles. The comparison of assessments was performed by using Wilcoxon Signed Ranks test. RESULTS: Significant increase in the distance of 6MWT, decrease in the duration of descending 4 steps, and 10 m walk timed performance tests, improvements in all of the gait characteristics, and balance were determined after taping (p < .05). CONCLUSIONS: KT has positive acute effects on performance and gait of children with DMD at early functional level which encourages therapists to use KT as a complementary approach in rehabilitation programs.

Anticancer and antimetastatic activity of Hypomyces chrysospermus, a cosmopolitan parasite in different human cancer cells.

The importance of microbial natural compounds in drug research is increasing every year and they are used to prevent or treat a variety of diseases. Hypomyces chrysospermus is a cosmopolitan parasite of many Boletaceae members. Since not much work has been conducted to date, this study is undertaken to explore the anticancer effect, including the antiproliferative and antimetastatic activity of Hypomyces chrysospermus. The aim of this study is to determine the antiproliferative and antimetastatic activity of Hypomyces chrysospermus ethyl acetate extract, having antioxidant activity, against A549, Caco2, MCF-7 human cancer and CCD-19 Lu and CCD 841 CoN healthy human cell lines. Firstly, cytotoxic activity was determined by the WST-1 assay. After cell proliferations and anti-metastatic effects were investigated by a real-time cell analysis system (RTCA-DP) and IC50 concentrations were calculated for each cell line. In addition, the expression levels of Apaf-1, TNF and NF-kB mRNA in cancer cells were investigated with RealTime-PCR. The ethyl acetate extract of Hypomyces chrysospermus presented anticancer activities including antiproliferative and antimetastatic effects. Hypomyces chrysospermus as a source of biologically active metabolites can be used as an important resource in the development of new anticancer effective agents.

Timing of Dysphagia Screening in Alzheimer's Dementia.

BACKGROUND: Dysphagia is an important and frequent symptom in Alzheimer's dementia (AD). We hypothesized that dysphagia could be seen in the early stages of AD and sarcopenia presence rather than the severity of the AD affecting dysphagia. The main aim of this study was to investigate swallowing functions in AD patients according to stages. The second aim was to investigate the correlation between sarcopenia and dysphagia in AD. METHODS: This study involved 76 probable AD patients. For all participants, diagnosis of sarcopenia was based on definitions from the revised version of European Working Group on Sarcopenia in Older People at 2018. Dysphagia symptom severity was evaluated by the Turkish version of the Eating Assessment Tool, a videofluoroscopic swallowing study (VFSS) was performed for instrumental evaluation of swallowing. The patients were divided into 3 groups according to the clinical dementia rating (CDR) scale as CDR 1 (mild dementia), CDR 2 (moderate dementia), and CDR 3 (severe dementia). Swallowing evaluation parameters were analyzed between these groups. RESULTS: Mean age was 78.9 ± 6.4 years, and 56.4% were female. Twenty-six patients had mild dementia, 31 patients had moderate dementia, 19 patients had severe dementia (CDR 3). We found that sarcopenia rates were similar between AD stages according to CDR in our study population and dysphagia could be seen in every stage of AD. In a multivariate analysis, polypharmacy and sarcopenia were found to be independently associated factors for dysphagia, irrespective of stage of AD (OR: 6.1, CI: 1.57-23.9, P = 0.009; OR: 4.9, CI: 1.24-19.6, P = 0.023, respectively). CONCLUSION: Aspirations may be subtle so that AD patients and caregivers may not be aware of swallowing difficulties. Therefore, all AD patients, especially those who have polypharmacy and/or sarcopenia (probable-sarcopenia-severe sarcopenia), should be screened for dysphagia in every stage.

The Effects of Different Exercise Trainings on Suprahyoid Muscle Activation, Tongue Pressure Force and Dysphagia Limit in Healthy Subjects.

Suprahyoid muscle activation and tongue pressure force play a critical role for swallowing function. In addition, dysphagia limit is one of most important factors indicating swallowing efficiency. The purpose of this study was to compare the effects of 8-week training sessions of three different exercises including chin tuck against resistance (CTAR), Shaker exercises and chin tuck exercise with theraband on suprahyoid muscle activity, anterior tongue pressure and dysphagia limit in healthy subjects. Thirty-six healthy volunteers aged between 18 and 40 years who scored below 3 points from Turkish version of Eating Assessment Tool (T-EAT-10) were included in the study, and all participants were divided into three groups randomly. Maximal suprahyoid muscle activations and dysphagia limit of participants were assessed by superficial electromyography. CTAR and chin tuck exercise with theraband increased the maximum suprahyoid muscle activation (p1 = 0.004, p2 = 0.018), whereas Shaker exercise did not increase maximal suprahyoid muscle activation (p = 0.507) after exercise training. CTAR and chin tuck exercise with theraband increased tongue pressure (p1 = 0.045, p2 = 0.041), while Shaker exercise did not increase anterior tongue pressure (p = 0.248). There was no statistically significant difference in dysphagia limits in three groups between before and after exercise training (p > 0.05). As a result, although CTAR seems to be the most effective exercise in most parameters, chin tuck exercise with theraband can also be used as an alternative to CTAR to improve suprahyoid muscle activity and tongue pressure.

Is the proprioceptive neuromuscular facilitation technique superior to Shaker exercises in swallowing rehabilitation?

PURPOSE: This prospective study was planned to investigate whether the combined isotonic technique of proprioceptive neuromuscular facilitation (PNF) is superior to Shaker exercises in improving the function of swallowing muscles. METHODS: Fifty individuals (30 females and 20 males; mean age 68 ± 3.89 years) with swallowing difficulties were separated into two groups randomly. The treatment groups were Shaker and PNF groups, which performed these exercises three times in a week for6 weeks. Swallowing difficulties were determined with the Turkish version of the eating assessment tool (T-EAT-10). The 100 ml-water swallow test was used to measure capacity, volume, and speed of swallowing. Contraction amplitude changes used as a universal measurement of motor unit activity during the muscle action were measured with superficial electromyography. RESULT: After 6 weeks of exercise training, T-EAT-10 scores decreased in both groups (p < 0.001). Water swallowing capacity and volume improved in both groups (p < 0.001). There was no change in swallowing speed in both groups (p > 0.05). Maximal voluntary contraction values of suprahyoid muscles were higher in PNF than the Shaker group (p < 0.05). CONCLUSION: Both the types of exercise can be used in the rehabilitation of swallowing difficulties. However, the PNF technique increased the contraction amplitude values that occur during maximum contraction more than the Shaker exercises. Different functional evaluations are needed to determine the effectiveness of PNF on swallowing difficulty.

Reliability and validity of the turkish translation of pedsqlTM multidimensional Fatigue scale in Duchenne Muscular Dystrophy.

OBJECTIVE: To perform the Turkish translation, reliability, and validity study of the PedsQLTM-3.0 Multidimensional Fatigue Scale (PedsQL-MFS) in patients with Duchenne Muscular Dystrophy (DMD). METHODS: This prospective, cross-sectional, observational study was held in Hacettepe University, Faculty of Physical Therapy and Rehabilitation between January 2016-August 2018. Turkish translation of the PedsQL-MFS was conducted based on the steps addressed in the translation manual of the original research. The psychometric features of the Turkish version of PedsQL-MFS including feasibility, internal consistency, and test-retest reliability, construct, and criterion-related validity as well as parent/child agreement were investigated on a total of 71 children and their parents. RESULTS: The mean age of boys with DMD included in the study was 102.94+/-23.23 months with a mean 17.15+/-2.98 BMI. Internal consistencies of Child Self Report General Fatigue, Sleep/rest Fatigue, and Cognitive Fatigue items were 0.74, 0.65, and 0.83 while, 0.89, 0.84, and 0.91 in Parent Proxy Report. The ICC values of Child Self Report and Parent Proxy Report were 0.87 and 0.91, respectively. Parent Proxy Report succeded more acceptable fit indices than Child Self Report. A statistically significant correlation was found between PedsQL-MFS and PedsQL-Neuromuscular Module (p<0.05). Moderate agreement was detected between parent and child. CONCLUSION: The Turkish version of PedsQL-MFS was determined to be a reliable and valid tool to evaluate fatigue in 5-12 years old, ambulant children with DMD.

The association of hand grip strength with functional measures in non-ambulatory children with Duchenne muscular dystrophy.

INTRODUCTION: 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. OBJECTIVE: The aim of this study was to determine the associations between hand grip strength and functional measures in non-ambulatory children with DMD. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

The effect of two different aerobic training modalities in a child with spinal muscular atrophy type II: a case report.

This study examined the effect of two different aerobic training modalities in a boy with spinal muscular atrophy type II. Motor functions were measured with Hammersmith Functional Motor Scale (HFMS) and Gross Motor Function Measure (GMFM). Spirometry was utilized for assessing pulmonary functions and PedsQL 3.0 Neuromuscular module was utilized for quality of life of child. Ergometer training was applied 3 times per week for the duration of 12 weeks. After 6 weeks of wash-out period, aquatherapy was applied for twice a week for the duration of 12 weeks. HFMS and GMFM scores, and pulmonary functions of the child and quality of life scores of his parents were improved during both modalities. These improvements were largely maintained during 1-year follow-up. This study showed that both modalities had similar effects on our case's pulmonary, motor functions and quality life of himself and parents. It will be a guide for researchers working in the area of children with neuromuscular disorders regarding plan and diversification of therapy program.