X-linked variations in SHROOM4 are implicated in congenital anomalies of the urinary tract and the anorectal, cardiovascular and central nervous systems.

BACKGROUND: SHROOM4 is thought to play an important role in cytoskeletal modification and development of the early nervous system. Previously, single-nucleotide variants (SNVs) or copy number variations (CNVs) in SHROOM4 have been associated with the neurodevelopmental disorder Stocco dos Santos syndrome, but not with congenital anomalies of the urinary tract and the visceral or the cardiovascular system. METHODS: Here, exome sequencing and CNV analyses besides expression studies in zebrafish and mouse and knockdown (KD) experiments using a splice blocking morpholino in zebrafish were performed to study the role of SHROOM4 during embryonic development. RESULTS: In this study, we identified putative disease-causing SNVs and CNVs in SHROOM4 in six individuals from four families with congenital anomalies of the urinary tract and the anorectal, cardiovascular and central nervous systems (CNS). Embryonic mouse and zebrafish expression studies showed Shroom4 expression in the upper and lower urinary tract, the developing cloaca, the heart and the cerebral CNS. KD studies in zebrafish larvae revealed pronephric cysts, anomalies of the cloaca and the heart, decreased eye-to-head ratio and higher mortality compared with controls. These phenotypes could be rescued by co-injection of human wild-type SHROOM4 mRNA and morpholino. CONCLUSION: The identified SNVs and CNVs in affected individuals with congenital anomalies of the urinary tract, the anorectal, the cardiovascular and the central nervous systems, and subsequent embryonic mouse and zebrafish studies suggest SHROOM4 as a developmental gene for different organ systems.

Koumiss and immunity: A thorough investigation of fermentation parameters and their impact on health benefits.

The aim of this study has been to determine the components, cytokine level and immunoglobulin level of koumiss during different fermentation periods, and to reveal the interrelation between these parameters. For achieving this objective, 10 samples of koumiss were prepared and randomly divided into 2 groups: the first group was sampled at 0, 1, 5, 12, and 24 h of incubation at room temperature for analysis. The second group was stored at +4°C, and samples were taken on the 5th, 10th, 15th, and 20th days. The count of Enterobacteriaceae spp, Staphylococcus, and Micrococcus spp progressively decreased with the period of fermentation until in the final samples of both groups they became undetectable. There were positive or negative correlations between cytokine and immunoglobulin levels and the physicochemical and microbiological parameters in the koumiss samples in both groups. However, the levels of IFN-γ, IL-2, TNF-α, and IgG did not change significantly over time in both group. In conclusion, it is clear that traditionally prepared koumiss with different fermentation time and temperature does not show any discrepancy in the concentrations of cytokine and immunoglobulin.

The relationship between Th1/Th2 cytokine polarization and milk composition in the postnatal period in Kyrgyz mares and foals.

Immune compatibility between mare and foal is one of the important topics of reproductive immunology. At this point, although there are many studies on antibodies, there are not many publications on the relationship between the cytokine levels of mare, foal and milk and the effects of milk composition on this relationship. Here we investigate the relationship between Th1/Th2 cytokine balance and milk composition in Kyrgyz mares and foals. Samples were taken soon after the foal was born and on days 5, 10 and 20 after birth. Th1 (IFN-γ, IL-2, TNF-α) and Th2 (IL-4, IL-5, IL-10) cytokine levels in blood samples were measured using a commercial ELISA kit. Dry matter, fat, protein and non-fat dry matter ratios were determined in the mare's milk and colostrum. We detected no Th1/Th2 polarization in the mare's milk on the day of the foal's birth (day 0) and day 10, but there was Th1 polarization on day 5 and Th2 cytokine polarization on day 20. There was no polarization in the blood sera of the mares on days 0, 5, and 10, and Th1 cytokine polarization was also detected on day 20. We detected no Th1/Th2 cytokine polarization in the blood sera of the foals on any of the days. Dry matter (19.66 ± 0.39%), protein (16.56 ± 0.18%), fat (2.13 ± 0.17), and non-fat dry matter (17.59 ± 0.44%) were higher in colostrum than the other days. When the correlations between cytokine levels in milk samples and milk composition were examined, there was a positive correlation between IL-5 level and protein ratio on day 10. We detected a positive correlation between IL-2 level and the fat rate on day 20. There was a positive correlation between the IL-2 level and the non-fat dry matter ratio on day 20. Further studies are now needed to determine the relationship between the changes in the composition of mare's milk in the postnatal period and Th1/Th2 cytokine polarization in mares and foals at the time of birth and in the postnatal period. Our finding that protein and fat ratios in mare's milk in the postnatal period are positively correlated with IL-2 levels should be considered in terms of foal and human nutrition.

Rare Variants in BNC2 Are Implicated in Autosomal-Dominant Congenital Lower Urinary-Tract Obstruction.

Congenital lower urinary-tract obstruction (LUTO) is caused by anatomical blockage of the bladder outflow tract or by functional impairment of urinary voiding. About three out of 10,000 pregnancies are affected. Although several monogenic causes of functional obstruction have been defined, it is unknown whether congenital LUTO caused by anatomical blockage has a monogenic cause. Exome sequencing in a family with four affected individuals with anatomical blockage of the urethra identified a rare nonsense variant (c.2557C>T [p.Arg853∗]) in BNC2, encoding basonuclin 2, tracking with LUTO over three generations. Re-sequencing BNC2 in 697 individuals with LUTO revealed three further independent missense variants in three unrelated families. In human and mouse embryogenesis, basonuclin 2 was detected in lower urinary-tract rudiments. In zebrafish embryos, bnc2 was expressed in the pronephric duct and cloaca, analogs of the mammalian lower urinary tract. Experimental knockdown of Bnc2 in zebrafish caused pronephric-outlet obstruction and cloacal dilatation, phenocopying human congenital LUTO. Collectively, these results support the conclusion that variants in BNC2 are strongly implicated in LUTO etiology as a result of anatomical blockage.

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.

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 ).

The effect of trunk training on trunk control, upper extremity, and pulmonary function in children with Duchenne muscular dystrophy: A randomized clinical trial.

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.

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.

A novel approach in the prevention of mastitis: electrical teat dipping.

Teat dipping is widely used in dairy cattle, especially to protect against contagious mastitis. Here we determine the effect of the device called 'Electrical Teat Dipping' (ETD), which was developed by combining teat dipping application and electrical field stimulation technique on teats. For this purpose, the front teats of 100 Holstein breed milking cows were evaluated in two groups, with ETD being applied once to the left front teat of these cows, and conventional teat dipping (CTD) being applied once to the right front teat, both after milking. Ultrasonographic measurements of the teats were made before milking and after teat dipping. We found that the width of the teat canal (1.88 ± 0.07 mm) in the teat using ETD was narrower after the application compared to those with CTD (2.28 ± 0.05 mm). Based on our findings, we conclude that the effects of ETD on the teat are very positive and can potentially be used as a new approach in the preventative control of mastitis in cows.

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.

Effect of modulating glutamate signaling on myelinating oligodendrocytes and their development-A study in the zebrafish model.

Myelination is crucial for the development and maintenance of axonal integrity, especially fast axonal action potential conduction. There is increasing evidence that glutamate signaling and release through neuronal activity modulates the myelination process. In this study, we examine the effect of manipulating glutamate signaling on myelination of oligodendrocyte (OL) lineage cells and their development in zebrafish (zf). We use the "intensity-based glutamate-sensing fluorescent reporter" (iGluSnFR) in the zf model (both sexes) to address the hypothesis that glutamate is implicated in regulation of myelinating OLs. Our results show that glial iGluSnFR expression significantly reduces OL lineage cell number and the expression of myelin markers in larvae (zfl) and adult brains. The specific glutamate receptor agonist, L-AP4, rescues this iGluSnFR effect by significantly increasing the expression of the myelin-related genes, plp1b and mbpa, and enhances myelination in L-AP4-injected zfl compared to controls. Furthermore, we demonstrate that degrading glutamate using Glutamat-Pyruvate Transaminase (GPT) or the blockade of glutamate reuptake by L-trans-pyrrolidine-2,4-dicarboxylate (PDC) significantly decreases myelin-related genes and drastically declines myelination in brain ventricle-injected zfl. Moreover, we found that myelin-specific ClaudinK (CldnK) and 36K protein expression is significantly decreased in iGluSnFR-expressing zfl and adult brains compared to controls. Taken together, this study confirms that glutamate signaling is directly required for the preservation of myelinating OLs and for the myelination process itself. These findings further suggest that glutamate signaling may provide novel targets to therapeutically boost remyelination in several demyelinating diseases of the CNS.

CNS myelin protein 36K regulates oligodendrocyte differentiation through Notch.

In contrast to humans and other mammals, zebrafish can successfully regenerate and remyelinate central nervous system (CNS) axons following injury. In addition to common myelin proteins found in mammalian myelin, 36K protein is a major component of teleost fish CNS myelin. Although 36K is one of the most abundant proteins in zebrafish brain, its function remains unknown. Here we investigate the function of 36K using translation-blocking Morpholinos. Morphant larvae showed fewer dorsally migrated oligodendrocyte precursor cells as well as upregulation of Notch ligand. A gamma secretase inhibitor, which prevents activation of Notch, could rescue oligodendrocyte precursor cell numbers in 36K morphants, suggesting that 36K regulates initial myelination through inhibition of Notch signaling. Since 36K like other short chain dehydrogenases might act on lipids, we performed thin layer chromatography and mass spectrometry of lipids and found changes in lipid composition in 36K morphant larvae. Altogether, we suggest that during early development 36K regulates membrane lipid composition, thereby altering the amount of transmembrane Notch ligands and the efficiency of intramembrane gamma secretase processing of Notch and thereby influencing oligodendrocyte precursor cell differentiation and further myelination. Further studies on the role of 36K short chain dehydrogenase in oligodendrocyte precursor cell differentiation during remyelination might open up new strategies for remyelination therapies in human patients.

Comparison of motor proficiency and functional ambulation in Duchenne muscular dystrophy with healthy peers.

AIMS: The study was aimed to investigate the difference between boys with Duchenne muscular dystrophy (DMD) and healthy peers in terms of gross and fine motor proficiency, and determine the relationship between motor proficiency and ambulation. METHODS: A total of 24 boys with DMD and 22 healthy peers were evaluated. Demographics and physical characteristics were recorded. The Bruininks Oseretsky Test of Motor Proficiency-Short Form (BOTMP-SF) was administered to evaluate gross and fine motor proficiency in both groups. The 6-minute walk test (6MWT) was performed to evaluate functional walking capacity and North Star Ambulatory Assessment (NSAA) was used to determine ambulatory status of boys with DMD. RESULTS: The gross motor proficiency score was significantly lower in boys with DMD (p = 0.001). No difference was found between the groups in terms of fine motor proficiency (p = 0.962). The gross motor function of BOTMP-SF was significantly related to 6MWT distance (r = 0.696, p = 0.001) and NSAA (r = 0.738, p = 0.001). No relationship was found between BOTMP-SF fine motor proficiency and 6MWT distance (r = 0.210, p = 0.361), and NSAA (r = -0.020, p = 0.928). There were significant correlations between running speed and agility with 6MWT distance (r = 0.585, p = 0.005) and NSAA (r = 0.650, p = 0.056). CONCLUSIONS: Boys with DMD were more affected in aspects of gross motor proficiency than healthy peers, while fine motor proficiency were found same. The gross motor proficiency was found more related to the level of ambulation.

Lower Limb Flexibility in Children With Duchenne Muscular Dystrophy: Effects on Functional Performance.

OBJECTIVE: To investigate the effects of lower limb flexibility on the functional performance of children with Duchenne muscular dystrophy. METHODS: Thirty children, whose functional levels were at 1 or 2 according to the Brooke Lower Extremity Functional Classification Scale, were included in this study. The flexibilities of the hamstrings, hip flexors, tensor fascia latae, and gastrocnemius muscles were evaluated in the children's dominant lower limbs. The children's functional performance was assessed using 6-minute walk tests and timed performance tests. The correlations between the flexibilities of the lower limb muscles and the performance tests were examined. RESULTS: The flexibilities of the lower extremity muscles were found to be correlated to the 6-minute walk tests and the timed performance tests. The flexibility of the hamstrings (r = -.825), the gastrocnemius muscles (r = .545), the hip flexors (r = .481), and the tensor fascia latae (r = .445) were found to be correlated with functional performance as measured by the 6-minute walk tests (P < .05). DISCUSSION: The results of the current study indicate that the flexibility of the lower limbs has an effect on functional performance in the early stages of Duchenne muscular dystrophy. More research is needed to determine the functional effects of flexibility on performance by adding long-term flexibility exercises to the physiotherapy programs of children with Duchenne muscular dystrophy.

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.

Comparison of Median Nerve Mechanosensitivity and Pressure Pain Threshold in Patients With Nonspecific Neck Pain and Asymptomatic Individuals.

OBJECTIVE: The purpose of this study was to investigate the presence of median nerve mechanosensitivity by comparing median nerve neurodynamic test results of patients with nonspecific neck pain (NNP) and asymptomatic individuals. METHODS: A total of 40 patients (30 women, 10 men) with NNP between the ages of 21 and 62 years (39.53 ± 10.18 years) and 38 asymptomatic individuals (23 women, 15 men) between the ages of 18 and 60 years (37.13 ± 9.64 years) participated in the study. Pressure pain threshold was assessed with digital pressure algometer, cervical joint range of motion was assessed with a universal goniometer, and median nerve mechanosensitivity was assessed with Upper Limb Neurodynamic Test 1 (ULNT1). The test step where the first sensory response was given, the location and character of the sensory response, and the final elbow extension angle were recorded during ULNT1. RESULTS: Patients with NNP had significantly decreased pressure pain threshold (P < .001), decreased range of motion of cervical flexion (P < .001), and decreased cervical lateral flexion (P = .001) compared with asymptomatic individuals, whereas no change was identified in range of motion of rotation (P = .100). In ULNT1, 45% of patients with NNP reported pain and 40% of them reported stretch. A total of 65% of asymptomatic individuals reported stretch, and 13% of them reported pain. It was identified in ULNT1 that final elbow extension angle was lower in the NNP group compared with asymptomatic individuals (P = .008). CONCLUSION: Median nerve mechanosensitivity increased, pressure pain threshold decreased, and active neck motion was limited in individuals with NNP compared with asymptomatic individuals.

How do physical capacity, fatigue and performance differ in children with duchenne muscular dystrophy compared with their healthy peers?

OBJECTIVE: To compare the fatigue levels and energy expenditure of children with Duchenne Muscular Dystrophy (DMD) at different functional levels with healthy children. METHODS: The cross-sectional study was carried out in the Unit of Pediatric Neuromuscular Diseases in the Department of Physiotherapy and Rehabilitation, Faculty of Health Science, Hacettepe University between March 2015 and January 2016. Fifty two children diagnosed with DMD in Level I-III according to the Brooke Functional Classification Scale and 17 healthy children were included in the study. The Six Minute Walk Test (6MWT), Northstar Ambulatory Assessment Scale (NSAA), Physiological Cost Index (PCI), and Timed performance tests were used to assess the children. RESULTS: Comparison in terms of PCI indicated a difference between Levels 2 and 3, and Levels 1 and 3 (p<0.0083). A difference was found in ascending and descending 4 stairs after 6MWT when fatigue after activity was evaluated. CONCLUSION: The walking distances, fatigue levels and energy expenditure of DMD patients were higher than the healthy peers. This difference was more prominent with decreasing functional level.

Comparison of pain, kinesiophobia and quality of life in patients with low back and neck pain.

[Purpose] The purpose of this study was to compare patients with low back and neck pain with respect to kinesiophobia, pain, and quality of life. [Subjects and Methods] Three-hundred patients with low back (mean age 43.2±11 years) and 300 with neck pain (mean age 42.8±10.2 years) were included in this study. Pain severity was evaluated by using the Short-Form McGill Pain Questionnaire, which includes a Visual Analogue Scale, quality of life by the Nottingham Health Profile, and kinesiophobia by the Tampa Scale for Kinesiophobia. [Results] Pain severity was similar in both groups, with a Visual Analogue Scale score of 6.7±2 in the low back pain and 6.8±2 in the neck pain group. Nottingham Health Profile pain [z=-4.132] and physical activity scores [z=-5.640] in the low back pain group were significantly higher. Kinesiophobia was also more severe in the low back pain group, with a mean 42.05±5.91 versus 39.7±6.0 Tampa Scale for Kinesiophobia score [z=-4.732]. [Conclusion] Patients with low back pain developed more severe kinesiophobia, regardless of the pain severity, and had greater pain perception and lower physical activity levels. Kinesiophobia adversely affects the quality of life and requires effective management of low back pain.

Dasatinib attenuated bleomycin-induced pulmonary fibrosis in mice.

Anti-fibrotic effect of dasatinib, a platelet-derived growth factor receptor (PDGFR) and Src-kinase inhibitor, was tested on pulmonary fibrosis (PF). Adult mice were divided into four groups: mice dissected 21 d after the bleomycin (BLM) instillation (0.08 mg/kg in 200 µl) (I) and their controls (II), and mice treated with dasatinib (8 mg/kg in 100 µl, gavage) for one week 14 d after BLM instillation and dissected 21 d after instillation (III) and their controls (IV). The fibrosis score and the levels of fibrotic markers were analyzed in lungs. BLM treatment-induced cell proliferation and increased the levels of collagen-1, alpha smooth muscle actin, phospho (p)-PDGFR-alpha, p-Src, p-extracellular signal-regulated kinases1/2 and p-cytoplasmic-Abelson-kinase (c-Abl) in lungs, and down-regulated PTEN expression. Dasatinib reversed these alterations in the fibrotic lung. Dasatinib limited myofibroblast activation and collagen-1 accumulation by the inhibition of PDGFR-alpha, and Src and c-Abl activations. In conclusion, dasatinib may be a novel tyrosine and Src-kinase inhibitor for PF regression in mice.

Different types of upper extremity exercise training in Duchenne muscular dystrophy: effects on functional performance, strength, endurance, and ambulation.

INTRODUCTION: We investigated and compared the effects of 2 different types of upper extremity exercise training on upper extremity function, strength, endurance, and ambulation in patients with early-stage Duchenne muscular dystrophy (DMD). METHODS: The study group (n = 12) exercised with an arm ergometer under the supervision of a physiotherapist, whereas the control group (n = 12) underwent a strengthening range-of-motion (ROM) exercise program under the supervision of their families at home for 8 weeks. Upper extremity functional performance, strength, endurance, and ambulatory status were assessed before and after the training. RESULTS: Ambulation scores, endurance, and arm functions, as well as proximal muscle strength, were improved after the training in the study group (P < 0.05). CONCLUSIONS: These results demonstrate that upper extremity training with an arm ergometer is more effective in preserving and improving the functional level of early-stage DMD patients compared to ROM exercises alone.