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.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Purpose:
The aim of this study was to investigate the effects of upper extremity dynamic exercise on respiratory function and quality of life in children with Duchenne Muscular Dystrophy (DMD).
...Methods:
In this study, children who diagnosed DMD scored as Level 1 or 2 according to the Brooke Upper Extremity Functional Classification (BUEFC) were divided into study and control groups with coin toss method randomly. Demographic datas were recorded. Timed performance tests (standing from supine, T-Shirt wearing/removing), electronic respiratory muscle strength device and Pediatric Quality of Life-3.0 (PedsQL-3.0) Neuromuscular Module child/parent questionnaire were used for evaluation of upper extremity performances, peak expiratory flow (PEF) and health-related quality of life, respectively. Study group had trained in arm ergometer, 8 weeks, 3 days per week, mean 40 minutes. Control group was applied normal range of motion upper extremity exercises as home programme. Assessments were repeated after 8 weeks.
Results:
24 DMD children, 12 control and 12 study subjects, respectively with mean age 9.33±1.37 and 9.50±1.38 were evaluated. Statistically significant differences were found in favor of study group in standing from supine position and T-shirt wearing time after exercise program (p<0.05). Significant increase was found in PEF liter value in study group (p<0.01). There were positive, moderate correlation between PedsQL-child scores and PEF (r=0.590; p<0.05); negative, strong correlation between PEF and T-Shirt wearing time (r=-0.629; p<0.05).
Discussion:
It was found that upper extremity dynamic exercise with arm ergometer increased respiratory function and showed positive effect on quality of life of children with DMD.
► A new method was developed to determine inorganic Se species by CPE–FAAS. ► A method for determination of selenium using CPE/FAAS system has not been published previously. ► The proposed method is ...fairly simple, cheap, environmental friendly and useful. ► The linear range of the method is highly wide and suitable for its application to real food samples.
A useful preconcentration and determination method was proposed for trace selenium in food samples. The procedure is based on complex formation of Pyronine B with Se(IV) ions in the presence of sodium dodecyl sulphate (SDS) and Ponpe 7.5. The variables affecting complex formation, extraction and phase separation were studied and optimised. Under the experimental conditions used, the calibration graph was linear in the range of 20–1700μgL−1 for Se(IV) ions. The limit of detection was 3.81μgL−1 of Se(IV) and the relative standard deviation for 5 replicate determinations at 250μgL−1concentration level was 2.45%. Recovery values were obtained between 97.8% and 102.8% for spiked samples. The method was successfully applied to the determination of total selenium in some food samples and alcoholic and nonalcoholic beverages. Its validity was checked by the analysis of four certified reference materials. The results obtained by the proposed method were quantitatively in good agreement with the certified values.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
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.
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.
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).
Median nerve mechanosensitivity increased, pressure pain threshold decreased, and active neck motion was limited in individuals with NNP compared with asymptomatic individuals.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NLZOH, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
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).
...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.
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).
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.
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DOBA, IJS, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK, VSZLJ
Background. Tricho-rhino-phalangeal syndrome (TRPS) is a rare, autosomal dominant disorder characterized by typical craniofacial features, ectodermal and skeletal findings. TRPS type 1 (TRPS1) is ...caused by pathogenic variations in the TRPS1 gene, which relates to the vast majority of cases. TRPS type 2 (TRPS2) is a contiguous gene deletion syndrome involving loss of functional copies of the TRPS1, RAD21, and EXT1. Herein, we reported the clinical and genetic spectrum of seven TRPS patients with a novel variant. We also reviewed the musculoskeletal and radiological findings in the literature. Methods. Seven Turkish patients (three female, four male) from five unrelated families aged between 7 to 48 years were evaluated. The clinical diagnosis was confirmed by either molecular karyotyping or TRPS1 sequencing analysis via next-generation sequencing. Results. Both TRPS1 and TRPS2 patients had some common distinctive facial features and skeletal findings. All patients had a bulbous nose with hypoplastic alae nasi, brachydactyly, short metacarpals and phalanges in variable stages. Low bone mineral density (BMD) was identified in two TRPS2 family members presenting with bone fracture, and growth hormone deficiency was detected in two patients. Skeletal X-ray imaging revealed cone-shaped epiphysis of the phalanges in all, and multiple exostoses were present in three patients. Cerebral hamartoma, menometrorrhagia and long bone cysts were among the new/rare conditions. Three pathogenic variants in TRPS1 were identified in four patients from three families, including a frameshift (c.2445dup, p.Ser816GlufsTer28), one missense (c.2762G > A), and a novel splice site variant (c.2700+3A > G). We also reported a familial inheritance in TRPS2 which is known to be very rare. Conclusions. Our study contributes to the clinical and genetic spectrum of patients with TRPS while also providing a review by comparing with previous cohort studies.
Tow bone mineral density (BMD) was identified in two TRPS2 family members presenting with bone fracture, and growth hormone deficiency was detected in two patients. The exact prevalence of TRPS is ...unknown; the estimated prevalence is Q.2-1/100,000.1'2 It was first described in 1966 by Giedion, and less than 250 TRPS patients have been reported.34 There are two clinical subtypes of TRPS; one of them is TRPS type 1 (TRPS1) (OMIM #190350) caused by pathogenic variants in the TRPS1 gene located on chromosome 8q23.3, and the other one is TRPS type 2 (Langer-Giedion syndrome, LGS), (OMIM #150230), a contiguous gene deletion syndrome is caused by submicroscopic deletion of the chromosomal segment 8q23.3-8q24.11, containing TRPS1, RAD21, and EXT1. A different subtype of TRPS has also been described; however, TRPS type 3 is now accepted within the spectrum of TRPS1 with more severe brachydactyly and short stature.25 TRPS1 gene is a zinc finger transcription factor expressed in cartilage, kidneys, and hair follicles that represses GATA-regulated genes and regulates bone perichondrium mineralization, chondrocyte proliferation, differentiation, and apoptosis.6/7TRPSl has high penetrance and wide phenotypic variability. Molecular Analysis Molecular karyotyping was performed using the DNA samples of patients obtained from peripheral whole blood.
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.
Abstract Introduction This study was planned to compare the static and dynamic balance in children with DMD at different functional levels with each other and with healthy peers. Material and Methods ...Sixty nine children between the ages of 6 and 11 were included in this study where 52 of them were diagnosed with Duchenne Musclar Dysthropy (DMD) in Level I (18 patients), Level II (17 patients), and Level III (17 patients) according to Brooke Functional Classification Scale and 17 of them healthy peers were included. In order to assess static and dynamic balance pediatric functional reach test (PFRT) and timed up and go test (TUGT) were used. Results When compared in terms of the TUGT, differences were found between all groups, i.e. Level 1 and 2, Level 2 and 3, Level 1 and 3, Healthy peers and Level 1, Healthy peers and Level 2, and Healthy peers and Level 3 (p<0.0083). When compared in terms of the PFRT in the standing positions, there was difference between level 3 and healthy peers(p<0.0083), but not between the other groups. Conclusions We found poor functional level in DMD to affect the dynamic and static balance parameters in this study. The dynamic balance of a child with DMD at Level 3 is decreased to a third of a healthy peer.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK, ZRSKP
Vaginal hyperplasia is characterized clinically as the protrusion of edematous hyperplastic mucosa tissue through the vulvar lips. Generally, it is formed during the proestrus and estrus periods in ...dogs. In this case report, 12 months old, pregnant Kangal breed dog with a mass in the vagina was presented with pre and postoperative findings. The bitch without any genital problems at pro/estrus stages was mated about two months ago. During the clinical examination, it was detected that bitch was in the second stage of labor and had type III vaginal hyperplasia. After the delivery of nine puppies without complication, the bitch was placed under general anesthesia and the hyperplasic tissue was removed surgically. Although no hyperplasia history at pro/estrus stages of the bitch, the fact that vaginal hyperplasia was observed in the last trimester of pregnancy is remarkable. Interestingly, this hyperplastic mass did not cause dystocia during all the puppies’ delivery in this case. In conclusion, in pregnant dogs, routine genital exams should be performed once at least until parturition to control vaginal structural integrity.
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