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Small fiber neuropathy (SFN) is one of the main neurological manifestations in primary Sjögren's Syndrome (pSS). For the detection of SFN, cutaneous silent period (CSP) measurement is gaining popularity recently due to its non-invasiveness and practical application. Evaluating SFN involvement in patients with pSS using CSP and evaluating its relationship with clinical parameters. Patients with a diagnosis of pSS and healthy volunteers demographically homogeneous with the patient group were included in the study. The CSP responses were recorded over the abductor pollicis brevis muscle. The latency and duration values of the responses were obtained. In patient group, EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), Hospital Anxiety and Depression Scale (HADS), Short Form-36 (SF-36) questionnaire, Leeds Assessment of Neuropathic Symptoms and Signs (LANSS) and Central Sensitization Inventory (CSI) were applied for the evaluation of symptom severity, mood, quality of life, presence of neuropathic pain and central sensitization, respectively. The mean CSP latency was significantly longer in patient group compared to control group (p < 0.001). Mean CSP duration was also significantly shorter in patient group (p < 0.001). There were no significant differences in CSP parameters according to patients' neuropathic pain or central sensitization profile. There were significant correlations of CSP parameters (latency and duration, respectively) with ESSPRI dryness (ρ = 0.469, p = 0.004; ρ = -0.553, p < 0.001), fatigue (ρ = 0.42, p = 0.011; ρ = -0.505, p = 0.002), pain (ρ = 0.428, p = 0.009; ρ = -0.57, p < 0.001) subscores and mean ESSPRI score (ρ = 0.631, p < 0.001; ρ = -0.749, p < 0.001). When SF-36 subscores and CSP parameters were investigated, a significant correlation was found only between "bodily pain" subscore and CSP duration (ρ = -0.395, p = 0.017). In HADS, LANSS and CSI evaluations, a significant correlation was found only between HADS anxiety score and the CSP duration (ρ = 0.364, p = 0.02). As indicated by CSP measurement, SFN is more prominent in patients with pSS than in the healthy population. It is important to investigate the presence of SFN because of its correlation with the leading symptoms in the clinical spectrum of pSS.
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Neuralgia , Síndrome de Sjögren , Humanos , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Síndrome de Sjögren/epidemiología , Calidad de Vida , Estado de Salud , Neuralgia/complicaciones , Fatiga/epidemiologíaRESUMEN
BACKGROUND: Respiratory failure is the leading cause of mortality in spinal muscular atrophy type 1 (SMA1) children. The current study aims to evaluate the effect of nusinersen treatment on respiratory outcome of the patients with SMA1. METHODS: In this retrospective, single-center study, 52 SMA1 patients treated with nusinersen were included in the analysis. Patients were divided into two groups based on their age at the time of their first nusinersen treatment (Group 1: ≤6 months, Group 2: >6 months). Respiratory outcome on the 180th day of treatment is defined as the type of ventilation support (spontaneous breathing, noninvasive ventilation (NIV), and tracheostomized or intubated on invasive mechanical ventilation). Demographic data, respiratory outcome, and Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scores were obtained from medical records. RESULTS: On the 180th day of treatment, 46 of the 52 (88.4%) children were alive. Prevalence of the mortality was similar in both groups (P = 0.65). The comparison of respiratory outcome in patients between group 1 and group 2 was as follows: spontaneous breathing, 7 (43.7%) versus 4 (13.3%) (P = 0.03); NIV <16 h/day, 3 (18.7%) versus 4 (13.3%) (P = 0.68); invasive mechanical ventilation, 6 (37.5%) versus 22 (73.3%) (P = 0.01). There were no patients using NIV ≥16 h/day. There were significant improvements in Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scores of the patients at day 180 in comparison with the baseline (P < 0.001). CONCLUSIONS: Early initiation of nusinersen treatment in SMA1 patients may alter the disease's natural course.
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Atrofia Muscular Espinal , Atrofias Musculares Espinales de la Infancia , Niño , Humanos , Lactante , Oligonucleótidos/uso terapéutico , Respiración Artificial , Estudios Retrospectivos , Atrofias Musculares Espinales de la Infancia/tratamiento farmacológicoRESUMEN
BACKGROUND: To describe and understand the experiences and beliefs of caregivers of children with cerebral palsy following botulinum toxin injection. METHODS: A descriptive case study approach with focus group interviews was employed. A semi-structured questionnaire was conducted to collect data. Twenty-one caregivers of children (3-13 years old) with cerebral palsy were recruited with a maximum variation sampling strategy to gain insight through different perspectives. Qualitative analysis with verbatim transcripts was analysed using a thematic approach. FINDINGS: Four themes emerged from qualitative analyses: acceptance of diagnosis, perceptions about treatment, caregivers' experiences with the health environment, and feelings and thoughts after the treatment. CONCLUSIONS: This study highlights caregivers' requests for information about the possible long-term effect of botulinum toxin, as well as information and support to provide the best rehabilitation programme immediately after injection.
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Toxinas Botulínicas , Parálisis Cerebral , Adolescente , Cuidadores , Parálisis Cerebral/tratamiento farmacológico , Niño , Preescolar , Humanos , Investigación Cualitativa , Encuestas y CuestionariosRESUMEN
BACKGROUND/OBJECTIVE: With the COVID-19 (coronavirus disease 2019) pandemic, telemedicine applications gained momentum, and clinicians tried to develop various musculoskeletal examination methods to be used in telemedicine visits. The aim of this study is to investigate the interrater reliability, acceptability, and practicality of the real-time video Pediatric Gait, Arms, Legs, and Spine (v-pGALS) assessment used in the evaluation during the telemedicine visit. METHODS: The study was designed as cross-sectional. Twenty school-aged children who presented to outpatient clinics with musculoskeletal complaints were included. For interrater reliability, the children were evaluated by face-to-face examination with v-pGALS, and then the child was reevaluated by another physiatrist with real-time evaluation (online video call) with the help of a parent. For acceptability, the time taken and the discomfort caused were evaluated by patients/parents with the smiley face visual analog scale, whereas to assess practicality, the ratio of completeness to duration of examination completion was recorded. RESULTS: κ coefficient of the agreement was found to be 0.88 between the results of the face-to-face examination and online video examination, suggesting very good agreement between the 2 raters. Acceptability of v-pGALS by parents and patients was high; 60% of children and 80% of parents found the duration of examination acceptable, and 70% of the patients and 95% of parents reported no discomfort caused by examination. The duration of face-to-face examination was 5.75 ± 1.29 minutes, whereas the duration of online examination was 15.81 ± 4.9 minutes. CONCLUSIONS: Video pGALS is a reliable, acceptable, and practical examination system that can be used for musculoskeletal assessment of children in telemedicine visits.
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COVID-19 , Telemedicina , COVID-19/diagnóstico , Niño , Estudios Transversales , Marcha , Humanos , Pierna , Reproducibilidad de los ResultadosRESUMEN
Spasticity is the most common motor disturbance in cerebral palsy (CP). Lockdown in the COVID-19 outbreak has profoundly changed daily routines, and similarly caused the suspension of spasticity treatment plans. Besides, the delay in botulinum toxin (BoNT) injection, which is important in the management of focal spasticity, led to some problems in children. This consensus report includes BoNT injection recommendations in the management of spasticity during the COVID-19 pandemic in children with CP. In order to develop the consensus report, physical medicine and rehabilitation (PMR) specialists experienced in the field of pediatric rehabilitation and BoNT injections were invited by Pediatric Rehabilitation Association. Items were prepared and adapted to the Delphi technique by PMR specialists. Then they were asked to the physicians experienced in BoNT injections (PMR specialist, pediatric orthopedists, and pediatric neurologists) or COVID-19 (pediatric infectious disease, adult infectious disease). In conclusion, the experts agree that conservative management approaches for spasticity may be the initial steps before BoNT injections. BoNT injections can be administered to children with CP with appropriate indications and with necessary precautions during the pandemic.
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Inhibidores de la Liberación de Acetilcolina/uso terapéutico , Toxinas Botulínicas/uso terapéutico , COVID-19/prevención & control , Parálisis Cerebral/rehabilitación , Espasticidad Muscular/tratamiento farmacológico , Parálisis Cerebral/fisiopatología , Niño , Control de Enfermedades Transmisibles , Técnica Delphi , Humanos , Control de Infecciones , Inyecciones Intramusculares/métodos , Espasticidad Muscular/etiología , Espasticidad Muscular/fisiopatología , Guías de Práctica Clínica como Asunto , SARS-CoV-2RESUMEN
PURPOSE: To investigate the effect of caregiver burden, resilience, and quality of life of the parents of patients who are followed-up in a tertiary pediatric rehabilitation clinic. DESIGN AND METHODS: This is a correlational cross-sectional study. Parents of patients who attend to utilize pediatric rehabilitation outpatient clinics of a tertiary center on a regular basis were invited to participate. All participants filled in the Zarit Caregiver Burden (ZCB) scale, Family Resilience scale (FRS) and Nottingham Health Profile (NHP). RESULTS: A total of 107 patient and caregiver dyads were included. Fifty-five (51%) patients had cerebral palsy, 10 (9%) had spina bifida and 42 (39%) had other neurodevelopmental disorders. Eighty-one (75%) of the caregivers were mothers. There was a significant difference in the caregiver burden between ambulatory children (Mean ZCB score 30.11⯱â¯13.56) and non-ambulatory patients (Mean ZCB score 37.22⯱â¯13.91) (pâ¯=â¯.01). There were moderate negative correlations between caregiver burden and FRS commitment to life and FRS self-sufficiency subscales. Caregiver burden significantly positively correlated with all NHP domains. CONCLUSIONS: Caregiver burden is significantly higher in parents of non-ambulatory children when compared to ambulatory children in the pediatric rehabilitation setting. Caregiver burden and resilience correlated on moderate levels, but the factors influencing them are still unclear. PRACTICE IMPLICATIONS: Healthcare and social support should be given to all caregivers in pediatric rehabilitation setting, but more so to the caregivers of non-ambulatory patients.
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Carga del Cuidador , Calidad de Vida , Cuidadores , Niño , Estudios Transversales , Femenino , Humanos , Apoyo SocialRESUMEN
STUDY DESIGN: Prospective single-blind, randomized controlled study. INTRODUCTION: Children with perinatal brachial plexus palsy (PBPP) have motion limitations in the affected upper extremity. Modified constraint-induced movement therapy (mCIMT) is one of the treatment options used for the improvement of the function of the affected limb. PURPOSE OF THE STUDY: The purpose of this study was to compare the effect of mCIMT and conventional therapy in improving active range of motion (ROM) and functional use of the affected upper extremity in children with PBPP with injuries to upper and middle trunks in the hospital environment. MATERIALS: 26 patients received conventional rehabilitation program (control group) and 13 patients participated in a mCIMT program (study group). Children had a mean age 56.3 months (range 4-10 years). The mCIMT included 1 hour therapy sessions emphasizing the affected arm use for 14 consecutive days during hospitalization. Their normal arm was also constrained for 6 hour per day. All the patients were assessed at the baseline, one day, one month, and three months after completion of therapy using active ROM, active movement scale, hand dynamometer, box and blocks test. RESULTS: The mCIMT group improved more than the control group in shoulder internal rotation, forearm supination, elbow flexion active ROMs, hand grip strength, and in upper extremity function. CONCLUSION: mCIMT has a potential to promote functional gains for children with PBPP; this approach should be widely applied within routine clinical practice.
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Terapia por Ejercicio/métodos , Parálisis Neonatal del Plexo Braquial/rehabilitación , Restricción Física , Niño , Preescolar , Femenino , Fuerza de la Mano , Hospitalización , Humanos , Masculino , Parálisis Neonatal del Plexo Braquial/fisiopatología , Rango del Movimiento Articular , Recuperación de la Función , Método Simple Ciego , Resultado del Tratamiento , Extremidad SuperiorRESUMEN
OBJECTIVE: To investigate the effect and superiority of fluoroscopy-guided intraarticular shoulder injection (IAI), suprascapular nerve block (SSNB), and combination treatment in hemiplegic shoulder pain (HSP). DESIGN: We included 30 patients diagnosed with HSP. Patients were divided into three groups: IAI, SSNB, and combination treatment. Patients were assessed using a visual analogue scale (VAS) prior to the injection and at hour 1, week 2, and month 2 after the injection, with goniometry at two angles at the moment that pain started and maximum passive range of motion (ROM) of the shoulder and Modified Barthel Index prior to the injection, at week 2 and month 2 after the injection. RESULTS: Significant decrease in the VAS and increase in shoulder passive ROMs were detected at all follow-ups in groups. In comparison, there was no significant difference in VAS scores. Change in the internal rotation at the moment that pain started was found to be higher in the patients treated with the combined method than the other methods. Change in maximum passive ROMs was similar between treatment groups. CONCLUSION: IAI, SSNB, and the combination treatments are reliable and effective treatment modalities that provide pain relief and an increase in shoulder passive ROMs in HSP.
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Fluoroscopía , Hemiplejía/tratamiento farmacológico , Bloqueo Nervioso , Dolor de Hombro/tratamiento farmacológico , Anciano , Método Doble Ciego , Femenino , Estudios de Seguimiento , Hemiplejía/diagnóstico por imagen , Humanos , Masculino , Persona de Mediana Edad , Bloqueo Nervioso/métodos , Dimensión del Dolor , Nervios Periféricos , Rango del Movimiento Articular , Escápula , Articulación del Hombro , Dolor de Hombro/diagnóstico por imagen , Resultado del TratamientoRESUMEN
OBJECTIVE: To investigate the effects of kinesiology taping and different types of application techniques of kinesiology taping in addition to therapeutic exercises in the treatment of congenital muscular torticollis. DESIGN: Prospective, single blind, randomized controlled trial. SETTING: An outpatient rehabilitation clinic in a tertiary university hospital. SUBJECTS: Infants with congenital muscular torticollis aged 3-12 months. INTERVENTIONS: Group 1 included 11 infants who only received exercises, Group 2 included 12 infants who received kinesiology taping applied on the affected side by using inhibition technique in addition to exercises. Group 3 included 10 infants who additionally received kinesiology taping applied on the unaffected side by using facilitation technique and on the affected side by using inhibition technique. MAIN MEASURES: Range of motion in lateral flexion and rotation of the neck, muscle function and degree of craniofacial changes were assessed at pretreatment, post treatment and, 1 month and 3 months' post treatment. RESULTS: Friedman analysis of within-group changes over time revealed significant differences for all of the outcome variables in all groups except cervical rotation in Group 3 ( P<0.05). No significant differences were found between groups at any of the follow-up time points for any of the outcome variables ( P>0.05). CONCLUSIONS: There is no any additive effect of kinesiology taping to exercises for the treatment of congenital muscular torticollis. Also different techniques of applying kinesiology taping resulted in similar clinical outcomes.
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Cinta Atlética/estadística & datos numéricos , Terapia por Ejercicio/métodos , Músculos del Cuello/fisiopatología , Tortícolis/congénito , Terapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Lactante , Quinesiología Aplicada/métodos , Masculino , Proyectos Piloto , Recuperación de la Función , Medición de Riesgo , Índice de Severidad de la Enfermedad , Método Simple Ciego , Factores de Tiempo , Tortícolis/diagnóstico , Tortícolis/rehabilitación , Resultado del TratamientoRESUMEN
BACKGROUND: The pediatric Gait, Arms, Leg, and Spine (pGALS) is a practical questionnaire for musculoskeletal (MSK) system evaluation in school-age children. OBJECTIVE: The aim of this study was to evaluate the acceptability/practicality of pGALS Turkish translation in Turkey (cross-sectional study). METHODS: The Turkish translation of pGALS was administered to children (4-18 years) who attended to the Pediatric Emergency Department of Hacettepe University, Ankara, Turkey, and the outpatient clinic of the Physical Therapy and Rehabilitation Department of Marmara University, Istanbul, Turkey, during 1 month in 2016. The demographics, complaints, final diagnoses, and pGALS parameters were noted. The acceptability of pGALS was evaluated using visual analog scale. RESULTS: Ninety-five patients (median age, 108 months; male/female, 1.1) were enrolled. Sixteen patients (16.8%) had MSK diagnosis, whereas 79 (83.2%) had non-MSK diagnoses. Musculoskeletal diagnoses were as follows: scoliosis (n = 4), metatarsus adductus (n = 4), soft tissue injury (n = 3), lumber disk herniation (n = 2), muscle spasm (n = 1), Achilles tendinitis (n = 1), and tibia torsion (n = 1). The sensitivity was 64.7%, and specificity was 89.7% for positive response to 1 or more pGALS screening questions to detect abnormal pGALS. The most sensitive question was pain question. The most common abnormal pGALS components were spine and posture. The sensitivity and specificity of pGALS for detecting MSK diagnosis were 93.7% and 97.4%, respectively. The median duration of pGALS examination was 4 minutes. Most patients/parents found the duration acceptable (94.7%/97.9%, respectively) and reported that pGALS caused little/no discomfort (97.9%/96.8%, respectively). CONCLUSION: This is the first study showing the Turkish version of pGALS as a valid, acceptable, and practical screening test in Turkey.
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Extremidades , Enfermedades Musculoesqueléticas/diagnóstico , Columna Vertebral , Niño , Estudios Transversales , Extremidades/fisiopatología , Femenino , Marcha , Humanos , Masculino , Tamizaje Masivo/métodos , Enfermedades Musculoesqueléticas/fisiopatología , Pediatría/métodos , Reproducibilidad de los Resultados , Sensibilidad y Especificidad , Columna Vertebral/fisiopatología , Encuestas y Cuestionarios , Traducción , Turquía , Escala Visual AnalógicaRESUMEN
[Purpose] Postural stability is the ability of to maintain the position of the body within the support area. This function is affected in cerebral palsy. The aim of the present study was to compare static and dynamic postural stability between children with hemiplegic cerebral palsy and healthy controls. [Subjects and Methods] Thirty-seven children between the ages of 5 and 14 diagnosed with hemiplegic cerebral palsy (19 right, 18 left) and 23 healthy gender- and age-matched controls were included in the study. Postural stability was evaluated in both of the groups using a Neurocom Balance. Sway velocity was measured both with the eyes open and closed. Sit to stand and turning abilities were also assessed. [Results] The sway velocities with the eyes open and closed were significantly different between the groups. The weight transfer time in the Sit to Stand test was also significantly slower in children with cerebral palsy. Children with cerebral palsy also showed slower turning times and greater sway velocities during the Step and Quick Turn test on a force plate compared with their healthy counterparts. [Conclusion] Both static and dynamic postural stability parameters are affected in hemiplegic cerebral palsy. Further research is needed to define rehabilitation interventions to improve these parameters in patients.
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Physical activity has been shown to reduce the risk for first-ever stroke as well as recurrent stroke with positive effects on almost all known modifiable risk factors. However, the perceived barriers and facilitators for engaging in physical activity have been insufficiently studied and may differ between cultures. Therefore, the aim of this study was to explore the perceptions of community-dwelling physically inactive individuals with stroke about barriers and facilitators to their participation in physical activity. This qualitative study included two focus groups of ten individuals with stroke classified as physically inactive based on accelerometer recordings. A semi-structured set of questions was posed in each focus group session and the transcripts were analysed using thematic analysis. The results revealed three main themes and subthemes: individual factors (physical impairments, psychological factors, spirituality), interaction with the family (attitudes of family members/close community) and social and environmental factors (hobbies, lack of or presence of facilities, the use of orthosis). We conclude that physical activity participation is affected by a multitude of factors as well as cultural differences. Thus, interventions aimed at increasing participation in physical activity after stroke should not only be planned according to individual clinical characteristics but also take into account a range of personal-to-social factors, including cultural differences.
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Vida Independiente , Accidente Cerebrovascular , Humanos , Infarto Cerebral , Ejercicio Físico , Factores de RiesgoRESUMEN
Previous literature has shown that basic body awareness therapy (BBAT) may be an effective treatment for fibromyalgia syndrome (FMS). This study constitutes the first case study evaluating internet-based BBAT for FMS. The purpose of this case study was to describe the feasibility and preliminary outcomes of an internet-based BBAT training for a total of 8 weeks in three patients with FMS. The patients underwent a synchronous individual internet-based BBAT training. Outcomes were assessed using Fibromyalgia Impact Questionnaire Revised, Awareness-Body-Chart, Short-Form McGill Pain Questionnaire (SF-MPQ), and plasma fibrinogen level. These measures were administered at baseline and after the treatment. Patient satisfaction with the treatment was evaluated using a structured questionnaire. At the post-treatment evaluation, each patient showed improvements in all outcome measures. All patients had clinically significant changes in Fibromyalgia Impact Questionnaire Revised. The SF-MPQ total score in Patients 1 and 3 exceeded the minimal clinically important difference. All patients' pain severity exceeded the minimal clinically important difference for Visual Analogue Scale (SF-MPQ). Besides, we found some benefits in body awareness and the level of dysautonomia. At the end of the treatment, patient satisfaction with the programme was very high. Based on this case study, the application of internet-based BBAT seems feasible and promising for clinical benefits.
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Fibromialgia , Humanos , Fibromialgia/diagnóstico , Fibromialgia/terapia , Concienciación , Resultado del TratamientoRESUMEN
PURPOSE: To translate the Hand-Use-at-Home questionnaire (HUH), assesses the amount of spontaneous use of the affected hand in children with 18 bimanual activities, into Turkish and examine its validity and reliability on children with neonatal brachial plexus palsy (NBPP) or unilateral cerebral palsy (UCP). MATERIALS AND METHODS: The HUH was translated and cross-culturally adapted to Turkish and administered to children with NBPP (n = 25) and UCP (n = 42) between 3 and 10 years. The psychometric analyses included reliability by internal consistency (Cronbach's alpha) and test/retest reliability (intraclass correlation coefficient, ICC) structural validity was evaluated with exploratory factor analysis, and construct validity was investigated by matching the HUH with the Pediatric Outcome Data Collection Instrument Upper Extremity Scale (PODCI) (NBPP only), and Children's Hand-Use Experience Questionnaire (CHEQ) (UCP only). RESULTS: HUH showed excellent test-retest reliability (ICC2,1 = 0.988 Cl (0.977-0.992)), excellent internal consistency (Cronbach's-α = 0.989), and moderate correlation with CHEQ (rs = 0.558) in UCP and high correlation with PODCI Scale (rs = 0.789) in NBPP group. The HUH had low and moderate correlation respectively lesion-extent levels (r=-0.457) in NBPP and 5 Manual Ability Classification System levels (r=-0.688) in the UCP group. CONCLUSION: The HUH is a valid and reliable tool to assess the amount of spontaneous use of the affected hand in Turkish children with NBPP and UCP.
The Hand-Use-at-Home (HUH) questionnaire is a reliable and good valid outcome measure to evaluate the amount of spontaneous use of the affected hand.We suggest the Turkish version of the HUH be used in the Turkish children to indicate small changes in the severity of disorder of children until a normal quality of life is achieved.The HUH can be used with high reliability and validity by experienced and inexperienced doctors and physiotherapists.
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Objectives: The study aimed to determine the impact of the pandemic on parents/caregivers and children with neurologic disabilities. Patients and methods: This multi-center cross-sectional study was conducted with 309 parents/caregivers (57 males, 252 females) and their 309 children (198 males, 111 females) with disabilities between July 5, 2020, and August 30, 2020. The parents/caregivers were able to answer the questions and had internet access. The survey included questions on the utilization of educational and health care services (whether they could obtain medicine, orthosis, botulinum toxin injection, or rehabilitation) during the pandemic. A Likert scale was used to evaluate the effect of the specific health domains, including mobility, spasticity, contractures, speech, communication, eating, academic, and emotional status. The Fear of COVID-19 Scale was used to assess fear of COVID-19. Results: Among the children, 247 needed to visit their physician during the pandemic; however, 94% (n=233) of them could not attend their physician appointment or therapy sessions. The restricted life during the first wave of the pandemic in Türkiye had negatively affected 75% of the children with disabilities and 62% of their parents. From the perspective of the parents/caregivers, mobility, spasticity, and joint range of motion of the children were affected. Forty-four children required repeated injections of botulinum toxin; however, 91% could not be administered. The Fear of COVID-19 Scale scores were significantly higher in the parents who could not bring their children to the routine physician visit (p=0.041). Conclusion: During the pandemic, access to physical therapy sessions was disrupted in children with neurological disabilities, and this may have harmful consequences on the functional status of children.
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INTRODUCTION: Airway clearance techniques, which include positive expiratory pressure (PEP) devices, are essential in the pulmonary rehabilitation of cystic fibrosis (CF). Bottle-PEP is a low-cost but an effective alternative. OBJECTIVE: The aim of this study is to document the sustainability and safety of Bottle-PEP therapy as a home rehabilitation aid. METHODS: The study has been designed as a prospective case series. Patients with CF at the age of 6-18 years in acute exacerbation period were included in the study. Bottle-PEP training was given by a competent physiotherapist to those patients who did not use any method, and those who currently use another device were followed up with their existing devices. Thus, patients divided into two groups were followed up for 1 year. The patients were evaluated by phone every 2 weeks for exacerbation, regular and proper use of the device, and satisfaction during their follow-up. The patients were evaluated every 3 months with pulmonary function tests, 6-minute walking test (6MWT) and quality of life. RESULTS: Thirty-four patients were included in the study. The acute exacerbation score of the patients was 4.5 in the Bottle-PEP group and 6 in the other group, showing no significant difference (p = .1). Treatment compliance scores were compared, the median value of the Bottle-PEP group was 24 the other group was 27 and there was no significant difference (p = .6). During follow-up of, there were no significant differences in FEV1, 6MWT and quality of life data (p > .05). CONCLUSION: Bottle-PEP treatment is not different from other devices in terms of long-term usability and safety in patients diagnosed with cystic fibrosis.
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Fibrosis Quística , Humanos , Niño , Adolescente , Fibrosis Quística/terapia , Respiración con Presión Positiva/métodos , Calidad de Vida , Respiración Artificial , Terapia Respiratoria/métodosRESUMEN
Even though the effect of several factors on sit-to-stand (STS) performance of children with CP has been previously explored, the potential role of lower extremity selective control, trunk control and sitting function on the performance of STS has not been examined. This study aimed to investigate the association of trunk control and lower extremity selective motor control with STS performance in children with CP. We recruited 28 children with CP aged between 4 and 10 years whose Gross Motor Function Classification System levels were I and II and 32 age-matched typically developing (TP) children. Trunk control, sitting function, selective control of the lower extremities and STS were evaluated with Trunk Control Measurement Scale (TCMS), sitting section of Gross Motor Function Measure-88 (GMFM-88), Selective Control Assessment of the Lower Extremity (SCALE) and the STS outcomes of a force platform [weight transfer time, rising index, and center of gravity (COG) sway velocity], respectively. In all evaluations, children with CP demonstrated lower scores than TD children. A moderate correlation was found between total scores of TCMS, GMFM-88 sitting section scores and COG sway velocity during STS and a fair correlation between SCALE total scores and COG sway velocity in the CP group ( r = -0.51, r = -0.52, r = -0.39, respectively). A fair correlation was found between SCALE total scores and the weight transfer time during STS in children with CP ( r = -0.39). Based on these results, improving trunk and lower extremity selective control may enhance STS performance in children with CP.
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Parálisis Cerebral , Niño , Preescolar , Estudios Transversales , Humanos , Extremidad Inferior , Movimiento , Equilibrio PosturalRESUMEN
Sjögren-Larsson syndrome (SLS) is a rare neurocutaneous disorder characterized by the presence of congenital ichthyosis, spasticity, and mental retardation. As with other rare genetic diseases, treatment is mainly symptomatic. Due to the absence of definitive treatment, lifelong follow-up and support of patients are important to improve the quality of life. A 7-year-old female child who was diagnosed as having SLS was referred to the rehabilitation clinic. After 20 sessions of a rehabilitation program, she started walking independently with the additional contribution of ankle-foot orthoses (AFOs). The contribution of the short-term rehabilitation approach and especially the administration of AFOs to the independence level of the patient is emphasized herein.
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Síndrome de Sjögren-Larsson , Niño , Femenino , Humanos , Espasticidad Muscular , Calidad de Vida , Síndrome de Sjögren-Larsson/diagnóstico , Síndrome de Sjögren-Larsson/genéticaRESUMEN
Objectives: This study aims to assess psychosocial functioning in relation to lesion level and ambulatory status in children with spina bifida (SB) and compare them to their peers. Patients and methods: Between March 2013 and May 2013, a total of 31 patients with SB (11 males, 20 females; mean age: 9.4 years; range, 6 to 14.7 years) and 36 typically developing peers (16 males, 20 females; mean age: 9.8 years; range, 6.5 to 14.8 years) were included in the study. All participants were assessed using a semi-structured psychiatric diagnostic interview via the Kiddie Schedule for Affective Disorders and Schizophrenia Present and Lifetime Version (K-SADS-PL), Wechsler Intelligence Scale for Children-Revised (WISC-R), Behavioral Rating Inventory of Executive Functions (BRIEF) parent form, Social Responsiveness Scale (SRS), and Aberrant Behavior Checklist (ABC). Results: In the SB group, the rate of psychiatric disorders was significantly higher (p=0.001) and the SRS scores and the planning and organizational components of the executive function were higher than their peers (p=0.02 and p=0.007, respectively). The psychiatric diagnosis rate, BRIEF, and SRS total scores did not significantly differ according to lesion level and ambulatory status. The BRIEF initiate and organization of materials subtest scores and ABC scores were significantly lower at high lesion levels (p=0.02, p=0.02, and p=0.02, respectively) and non-community walkers (p=0.002, p=0.03, and p=0.003, respectively). Conclusion: Psychiatric disorders, impairment in social responsiveness, and planning and organization components of the executive function are prevalent in children with SB with no intellectual disabilities, compared to their peers. Therefore, psychosocial counseling and multidisciplinary follow-up for SB patients seem to be beneficial.
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INTRODUCTION: Patients with Duchenne muscular dystrophy (DMD) have lost their access to on-site rehabilitation due to the COVID-19 pandemic. Telerehabilitation can be a viable approach for these patients to protect their muscle strength and functional status. The aim of this study is to compare telerehabilitation with home-based video exercises. PATIENTS AND METHODS: Male, ambulatory DMD patients were randomized into telerehabilitation and video-exercise groups. Nineteen patients were included in the final analyses. Telerehabilitation consisted of live online exercises, while video exercise implemented a pre-recorded video as a home-based program. Both programs spanned 8 weeks, three times a week. Patients' muscle strength with a hand-held dynamometer, Quick Motor Function Test, North-Star Ambulatory Assessment (NSAA), 6-Minute Walk Test (6MWT) and Caregiver Burden were recorded before and after treatment. RESULTS: The 6MWT of the telerehabilitation group was391.26 ± 95.08 m before and387.75 ± 210.93 after treatment (p = 0.94) and 327.46 ± 103.88 m before treatment and313.77 ± 114.55 after treatment in video group (p = 0.63). The mean NSAA score of the telerehabilitation group were26.70 ± 8.04 before treatment and 25.20 ± 11.33 after treatment (p = 0.24). In the video group scores were 21.66 ± 6.65 before to 22.00 ± 8.61 after treatment (p = 0.87). There were no significant changes between groups at the end of the treatments. The telerehabilitation group's neck extension, bilateral shoulder abduction, and left shoulder flexion, bilateral knee flexion and extension, bilateral ankle dorsiflexion, and left ankle plantar flexion strength improved significantly and were better than the video group (p < 0.05 for all measurements). CONCLUSION: A telerehabilitation approach is superior in improving muscle strength than a video-based home exercise, but none of the programs improved functional outcomes in ambulatory patients with DMD.