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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
Skeletal and respiratory muscle dysfunction has been previously described in patients with other etiologic subgroups of pulmonary arterial hypertension (PAH) but has never been investigated in patients with PAH due to congenital heart diseases (CHD). This study aims to show the involvement of skeletal and respiratory muscles in these patients. This cross-sectional study included patients with PAH due to CHD and healthy controls. Patients' demographic properties, six-minute walk tests; shoulder abduction, handgrip, knee extension, and ankle dorsiflexion muscle strength, maximum inspiratory (MIP) and expiratory pressures (MEP) were measured. Deltoid, flexor digitorum superficialis, and profundus, tibialis anterior and rectus femoris muscles were visualized with ultrasonography and their cross-sectional areas (CSA) were also measured in both groups. 12 patients and 12 controls were included. Mean MIP was 104.22±32.57 cm H2O for healthy participants while 61.33±29.74 cm H2O for patients (p<0.001). For mean MEP, it was 100.08±26.05 cm H2O in healthy participants and 69.75±39.79 cmH2O in controls (p=0.004). When the strength of skeletal muscles was compared, there were significant differences between the groups in all measurements except for bilateral grip strength. In the correlation analysis, MIP and MEP values showed no significant correlations with clinical parameters. They showed significant moderate correlations with skeletal muscle strength. When CSAs of the muscles were compared, there were significant differences in all measurements except for left FDS and FDP and bilateral rectus femoris. This study showed that in patients with pulmonary arterial hypertension due to CHD, respiratory muscle strength is significantly worse than healthy participants. Patients had also significantly worse skeletal muscle strength except for grip strength.
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Cardiopatías Congénitas , Hipertensión Arterial Pulmonar , Estudios Transversales , Fuerza de la Mano , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico por imagen , Humanos , Fuerza Muscular , Músculos RespiratoriosRESUMEN
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
Rheumatoid arthritis (RA) and spondyloarthropathies (SpA) are among the most common inflammatory rheumatic diseases, which might induce chronic pain for their sufferers. Mind-body interventions like Tai Chi and yoga are among the many alternative therapies for combatting chronic pain. This review aims to overview the articles about their effectiveness in RA and SpA. We searched PubMed/MEDLINE, Scopus, and Web of Science for English-language sources from their inception through September 2017. Case-control studies, interventional studies, and case series that included more than three cases and randomized crossover studies were included. The literature search retrieved 133 non-duplicate records, and 15 of them were eligible and were included in this review. The influence of Tai Chi remains debatable in RA, while there is only one study that investigated its efficacy in SpA. Yoga seems effective in decreasing pain and inflammation while increasing quality of life. There are no data available about its effect on SpA. Even after a thorough research, the number of articles is quite limited on the effectiveness of Tai Chi and yoga in RA and SpA. While these complementary approaches still show some promise as alternative therapies in RA and SpA, the literature lacks long-term studies with larger patient groups.
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Artritis Reumatoide/terapia , Espondiloartropatías/terapia , Taichi Chuan , Yoga , Adulto , Anciano , Anciano de 80 o más Años , Artritis Reumatoide/diagnóstico , Artritis Reumatoide/fisiopatología , Artritis Reumatoide/psicología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Calidad de Vida , Espondiloartropatías/diagnóstico , Espondiloartropatías/fisiopatología , Espondiloartropatías/psicología , Resultado del TratamientoRESUMEN
AIM OF THE STUDY: To define the effectiveness of ganglion Impar block in improving neuropathic pain. MATERIALS AND METHODS: Patients who had pain around the coccyx for more than three months and did not respond to conservative treatment were included in this study. All the patients underwent fluoroscopy guided transsacrococcygeal ganglion Impar block with injecting 3 mL of 0.5% bupivacaine, 2 mL saline, and 1 mL (40 mg) of methylprednisolone. Patients were evaluated with visual analog scale (VAS) for pain, Leeds assessment of neuropathic symptoms and signs scale (LANSS) for neuropathic pain, Beck depression Inventory (BDI) for mood and Short-form 12 (SF-12) for quality of life before, 1 month 3 months and 6 months after the injection. Patients' painless sitting duration was also recorded. RESULTS: A total of 28 patients were included in the final analyses. VAS and LANSS scores improved significantly throughout the follow-up periods. BDI scores also improved while SF-12 scores did not show significant changes. Painless sitting period of the patients' improved significantly. CONCLUSIONS: Ganglion Impar block is effective in decreasing the neuropathic component of chronic coccygodynia. This improves painless sitting in patients but its reflections on quality of life is not clear.
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Neuralgia , Calidad de Vida , Ganglios Simpáticos , Humanos , Dimensión del Dolor , Región SacrococcígeaRESUMEN
BACKGROUND: Systemic sclerosis can affect peripheral nerves, but the extent and the nature of this involvement are not well defined. The aim of this study is to compare the sonoelastrographic measurements of median nerves in systemic sclerosis (SSC), idiopathic carpal tunnel syndrome (CTS) and healthy individuals. METHODS: The clinical, electrophysiological and ultrasonographic assessments were done. Patients with SSC and CTS were assessed with nerve conduction studies. The measurements of cross sectional areas (CSA) were performed at psiform and forearm level from axial US images. The elastic ratio is the ratio of strain distribution in two selected region of interests (ROI) done via comparing the median nerve to flexor digitorum superfcialis tendon. The ROIs were fixed to 2 mm. RESULTS: The study was completed with 47 hands of 24 patients with SSC, 53 hands of 27 patients with CTS and 38 hands of health controls. The CSA of CTS group was significantly higher than systemic sclerosis and control groups. The elastic ratio at psiform level and forearm levels of systemic sclerosis group were significantly higher than the CTS and control groups. CONCLUSION: Median nerves lose the elasticity while the CSA's are in the normal range in patients with SSC. These results suggested that the increased peripheral nerve involvement in SSC is about the increased stiffness of the nerves.
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Síndrome del Túnel Carpiano/etiología , Nervio Mediano/fisiopatología , Esclerodermia Sistémica/complicaciones , Síndrome del Túnel Carpiano/diagnóstico por imagen , Síndrome del Túnel Carpiano/fisiopatología , Estudios de Casos y Controles , Elasticidad , Diagnóstico por Imagen de Elasticidad , Femenino , Humanos , Masculino , Nervio Mediano/diagnóstico por imagen , Esclerodermia Sistémica/diagnóstico por imagen , Esclerodermia Sistémica/fisiopatologíaRESUMEN
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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OBJECTIVE: Coccygodynia is a distressing condition that presents with pain around the coccyx. Impar (Walther) ganglion is a sympathetic ganglion located at the end of lumbosacral sympathetic chain. The objective of this study is to share our results and follow up of 34 ganglion impar blocks in 22 patients. DESIGN: Retrospective pilot study. SETTING: Interventional Pain Clinic in the Department of Physical Medicine and Rehabilitation in a university hospital. SUBJECTS: Twenty-two patients with coccygodynia who did not respond to conservative treatment and then presented to interventional pain clinic of a PM&R department in a university hospital METHODS: Pain was evaluated via 10-cm visual analog scale (VAS). VAS values were obtained before, 1 hour and 3 weeks after injection and during this study was conducted. RESULTS: For achieving at least 50% relief of pain, the success rate of a first injection was 82%, but accounted for three technical failures. In patients with a successful outcome, relief lasted for a median duration of 6 months. Relief was reinstated for a median period of 17 months by a second injection in nine patients who presented for repeat treatment. No relief was achieved in two of these patients when they presented for a third treatment. CONCLUSIONS: Ganglion impar block appears to be effective in patients who have coccygodynia resistant to conservative therapy, with high success rates and prolonged duration of effect. Controlled studies are required to reveal the mechanism of this effect.
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Bloqueo Nervioso Autónomo/métodos , Ganglios Simpáticos/fisiopatología , Dolor de la Región Lumbar/terapia , Manejo del Dolor/métodos , Región Sacrococcígea/inervación , Adulto , Anciano , Enfermedad Crónica , Femenino , Estudios de Seguimiento , Humanos , Dolor de la Región Lumbar/fisiopatología , Masculino , Persona de Mediana Edad , Clínicas de Dolor , Dimensión del Dolor/métodos , Proyectos Piloto , Estudios Retrospectivos , Factores de Tiempo , Resultado del TratamientoRESUMEN
BACKGROUND: When a patient with a prior history of malignancy and radiotherapy develops progressive weakness as a presentation of plexus involvement, the differential diagnosis usually rests between radiation-induced plexopathy and invasion from recurrent tumor. The presence of myokymic discharges is helpful in differentiating radiation-induced from neoplastic plexopathy. OBJECTIVE: To present a case report of a patient with chordoma, a locally aggressive tumor, who was diagnosed with recurrent tumor accompanied by the occurrence of myokymia in needle electromyographic examination. METHOD: A 55-year-old male patient with a history of chordoma and radiotherapy presented to our outpatient clinic with complaints of foot drop, and impaired walking for two months. His latest magnetic resonance imaging (MRI) which was performed three months earlier did not show recurrence. Upon electromyographic evaluation, myokymia, the pathognomic electromyography abnormal wave for radiation plexopathy was detected supporting a diagnosis of radiation plexitis rather than recurrent neoplastic invasion. One month later he presented with more severe pain and was re-evaluated by an MRI, on which a mass was detected indicating relapse. CONCLUSION: With this case report, we would like to emphasize that the behaviour of the tumor should be considered and imaging should be repeated when tumors display aggressive or recurrent behaviour.
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Cordoma , Miocimia , Neoplasias de la Columna Vertebral , Masculino , Humanos , Persona de Mediana Edad , Recurrencia Local de Neoplasia , DolorRESUMEN
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
Objectives: This study aimed to evaluate the muscle thickness, strength, and diaphragmatic function and relate them with clinical findings. Patients and methods: The cross-sectional study was conducted with 54 participants (27 males, 27 females; mean age; 24.5±5.3 years; range, 18 to 45 years) between January 2017 and October 2017. Of the participants, 31 were adult CF patients, and 23 were age-and sex-matched controls. Patient demographics, 6-min walk distance, body mass index (BMI), and fat-free mass index (FFMI) were evaluated. Each patient underwent pulmonary function tests. Quadriceps femoris thickness and diaphragm thickening fraction were assessed by ultrasonography. Upper extremity strength was measured with a handheld dynamometer. Results: There was no difference between the patients' and controls' BMI (p=0.052). However, patients' FFMI was lower than the controls' (p=0.010). The FFMI correlated with pulmonary function tests. Patients' both right and left quadriceps femoris muscles were thinner than the controls (p=0.001 and p=0.001, respectively). Patients with pancreatic insufficiency had thinner muscles than patients without pancreatic insufficiency. The control group had a stronger handgrip than CF patients (33.5±10.1 vs. 24.5±9.2 kg, p=0.003). Conclusion: Peripheral muscle wasting and weakness and lower functional capacity are highly prevalent in CF patients.
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BACKGROUND: A collaboration between the University of Michigan (U of M) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated to improve the health status of people with cystic fibrosis (pwCF) at MU through implementing Quality Improvement (QI) initiatives. The main aim was to improve lung function in children with FEV1pp <80. The secondary aim was to assess the changes in health related quality of life. METHODS: Included in the project were pwCF who received cystic fibrosis (CF) care at the MU CFC and were 6-18 years of age with an FEV1pp <80. Flow charts were created and a standardized CF care algorithm was implemented. Weekly case review were done to develop individualized treatment plans. Appropriate intervention was applied and patient data were assessed at baseline, 3, 6, 9 and 12 months. The Cystic Fibrosis Revised Questionnaire (CFQ-R) was completed. RESULTS: 55 pwCF were included (mean age:11.8 ± 3.3 years). Mean FEV1pp (SD) at baseline, 6 and 12 month was 63.7 (14.6), 66.9 (16.6), 70.4 (19.2), respectively, with a relative increase of 5.0% in 6 months (p:0.002) and 10.5% in 12 months compared to baseline (p<0.001). Physical functioning, eating problems and respiratory symptoms domains of the CFQ-R questionnaire were improved at the end of the one year for 6-13 (p = 0.024, p = 0.009, p = 0.002) and 13-18 year olds (p = 0.013, p = 0.002, p = 0.038). CONCLUSION: There was significant improvement in pwCF with FEV1<80%pp after implementing this QI project. The processes and assessments used can be adopted by other low-middle income countries to improve similar measures.
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Fibrosis Quística , Niño , Humanos , Adolescente , Fibrosis Quística/diagnóstico , Fibrosis Quística/terapia , Fibrosis Quística/complicaciones , Calidad de Vida , Mejoramiento de la Calidad , Estado de Salud , Encuestas y CuestionariosRESUMEN
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
BACKGROUND: Coccygodynia is one of the chronic, refractory painful musculoskeletal disorders. Interventional procedures are applied to patients unresponsive to initial treatment in coccygodynia. This study aims to compare the treatment outcomes of ganglion impar block (GIB) and caudal epidural steroid injection (CESI) in patients with chronic coccygodynia. METHODS: This study was a prospective randomized comparison study conducted between June 2019 and January 2021. Patients diagnosed with chronic coccygodynia were randomly divided into two groups: the GIB group and the CESI group. The severity of pain, presence of neuropathic pain, and quality of life were evaluated using the Numeric Rating Scale, Leeds Assessment of the Neuropathic Symptoms and Signs Scale, and Short Form-12 Health Survey (SF-12), respectively. RESULTS: A total of 34 patients in each group were included in the final analyses. While there was a significant decrease in pain intensity in both groups in the 3-month follow-up, this decrease was more significant in the GIB group at the 3rd week. There was a significant improvement in the SF-12 physical score and the number of patients with neuropathic pain in both groups in the 3rd week, but this improvement was not observed in the 3rd month. CONCLUSIONS: Although GIB may provide more pain relief in short term, both GIB and CESI are useful treatment methods in coccygodynia unresponsive to more conservative treatments.
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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.
Asunto(s)
COVID-19 , Distrofia Muscular de Duchenne , Telerrehabilitación , Terapia por Ejercicio , Humanos , Masculino , Pandemias , Método Simple CiegoRESUMEN
Objectives: This study aimed to investigate the long-term use of bottle-positive expiratory pressure (PEP) in addition to breathing exercises as a home-based rehabilitation aid on exercise capacity, spirometric parameters, and quality of life in chronic obstructive pulmonary disease (COPD) patients. Patients and methods: From a total of 30 patients with stable moderate-to-severe COPD, 24 (22 males, 2 females; mean age: 62.4+7.2 years; range, 40 to 75 years) were included in the final study and randomized into two groups: the group that performed breath retaining techniques and the group that was instructed to use the bottle-PEP in addition to these techniques. Patients were evaluated with modified Medical Research Council scale, COPD assessment test (CAT), spirometry, St. George`s Respiratory Questionnaire (SGRQ), and 6-min walk distance (6MWD) before, three months and six months after the initiation of the program. Results: In the bottle-PEP group, patients` mean 6MWD increased from 380.6±67.6 to 444.1±22.0 m (p=0.002), the mean CAT score decreased from 17.8±36.8 to 12.9±6.2 (p=0.03), and the mean SGRQ total score significantly decreased from 57.1±23.1 to 47.6±21.9 (p<0.05) after three months. The improvement in 6MWD continued in six months but disappeared in SGRQ and CAT scores. In the exercise group, only the 6MWD improved, and there were no significant improvements in other parameters regardless of time. There were no significant differences between the groups in any of the parameters at any follow-up session. Conclusion: While bottle-PEP does not significantly contribute when added to breathing exercises in patients with moderate-to-severe COPD in improving function and quality of life, it can be used as a safe choice in patients` home rehabilitation programs.