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[Purpose] The incidence of chronic obstructive pulmonary disease is rapidly increasing worldwide. In Nepal, it has the highest mortality rate among all noninfectious diseases. Since 2015, we have been involved in a project that aims to facilitate chest rehabilitation for patients with chronic obstructive pulmonary disease in Nepal. We compared the Nepali version of the St. George's Respiratory Questionnaire with the Chronic Obstructive Pulmonary Disease Assessment Test, the latter of which was translated into Nepali for this project. We also evaluated the extent to which patient quality of life improved after the rehabilitation program. [Participants and Methods] The Nepali St. George's Respiratory Questionnaire and Chronic Obstructive Pulmonary Disease Assessment Test were used to assess the health status of patients both before the intervention's initiation and one year after it. Between May and September of 2016, 122 patients with chronic obstructive pulmonary disease participated in this program. [Results] We collected valid responses from 57 patients both before and after the intervention. The scores of both screening tools were significantly lower after the intervention than before and showed a significant correlation with one another. [Conclusion] These results suggest that the Nepali version of the Chronic Obstructive Pulmonary Disease Assessment Test is a reliable tool for the evaluation of chronic obstructive pulmonary disease and that the intervention used in the project might be effective for patients afflicted with the disease. However, there are limitations to the research design, such as the limited number of participants used in the study.
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OBJECTIVE: To verify the effects of structured home-based exercises without supervision by a physical therapist in patients with early-stage amyotrophic lateral sclerosis (ALS). DESIGN: A historical controlled study that is part of a multicenter collaborative study. SETTING: Rehabilitation departments at general hospitals and outpatient clinics with a neurology department. PARTICIPANTS: Patients (N=21) with ALS were enrolled and designated as the home-based exercise (Home-EX) group, and they performed unsupervised home-based exercises. As a control group, 84 patients with ALS who underwent supervised exercise with a physical therapist for 6 months were extracted from a database of patients with ALS and matched with the Home-EX group in terms of their basic attributes and clinical features. INTERVENTION: The Home-EX group was instructed to perform structured home-based exercises without supervision by a physical therapist that consisted of muscle stretching, muscle training, and functional training for 6 months. MAIN OUTCOME MEASURES: The primary outcome was the score on the ALS Functional Rating Scale-Revised (ALSFRS-R), which is composed of 3 domains: bulbar function, limb function, and respiratory function. The score ranges from 0 to 48 points, with a higher score indicating better function. RESULTS: In the Home-EX group, 15 patients completed the home-based exercises for 6 months, and 6 patients dropped out because of medical reasons or disease progression. No adverse events were reported. The Home-EX group was found to have a significantly higher respiratory function subscore and total score on the ALSFRS-R than the control group at follow-up (P<.001 and P<.05, respectively). CONCLUSIONS: Structured home-based exercises without supervision by a physical therapist could be used to alleviate functional deterioration in patients with early-stage ALS.
Assuntos
Esclerose Lateral Amiotrófica/reabilitação , Terapia por Exercício/métodos , Autocuidado/métodos , Idoso , Esclerose Lateral Amiotrófica/psicologia , Progressão da Doença , Exercício Físico/psicologia , Terapia por Exercício/psicologia , Feminino , Serviços de Assistência Domiciliar , Humanos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Qualidade de Vida , Autocuidado/psicologia , Resultado do TratamentoRESUMO
BACKGROUND: There have been few descriptions in the literature on long-term enzyme replacement therapy (ERT) in patients with advanced late-onset Pompe disease (LOPD). OBJECTIVES: This study aimed to assess the efficacy and limitations of ERT in advanced LOPD patients. METHODS: We retrospectively reviewed the clinical courses of patients with advanced LOPD (two juvenile-onset and five adult-onset patients) who were treated with recombinant human alglucosidase alfa to examine improvements achieved with and limitations of ERT until their death or when switching to avalglucosidase alfa occurred. RESULTS: All patients were non-ambulant and ventilator dependent. The duration of follow-up ranged from 3.7 to 15.0 years (median 9.0 years). All patients reported improvements in their lives during the first two or three years of ERT. Vital capacity was clearly improved in patients with relatively spared respiratory function, although it deteriorated after respiratory complications such as pneumothorax. Pinch and grip power tended to be preserved during the treatment period. Muscle CT revealed progression of atrophy and fatty replacement predominantly in the proximal limb muscles without improvement after ERT. Four patients died due to aspergillosis, respiratory failure, ileus, and sudden death of unknown cause. CONCLUSIONS: Our findings demonstrate that patients undergoing ERT show certain improvements, even in the advanced stage of Pompe disease. Respiratory complications are lethal even during ERT, and early diagnosis and induction of therapy are critical. Muscle wasting progressed more severely in the proximal limbs, even after ERT.
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OBJECTIVE: The aim of this study was to validate the usefulness of the measurement of lung insufflation capacity (LIC) using the LIC TRAINER (LT) in patients with amyotrophic lateral sclerosis (ALS). METHODS: This retrospective study was conducted in the rehabilitation departments of the Japanese National Center of Neurology and Psychiatry and involved 20 ALS patients who underwent respiratory therapy between April 1, 2014, and December 2017. The vital capacity (VC), maximum insufflation capacity (MIC), and LIC measurements at the start of respiratory therapy were extracted from the medical records, and patients were divided into three groups: group A, VC could not be measured; group B, VC could be measured, but MIC was less than VC; and group C, MIC was larger than VC. LIC could be measured in all groups. In group C, paired t-tests were used to analyze whether there was a significant difference in the volumes measured using different methods. RESULTS: LIC was 950, 1863±595, and 2980±1176 ml in groups A (n=1), B (n=10), and C (n=9), respectively. In groups A and B, LIC could be measured in all patients, even when VC or MIC could not be measured. In group C, the measured LIC value was significantly greater than MIC (p=0.003). CONCLUSION: LIC could be successfully measured using the LT. By using the LT, it was feasible to conveniently perform LIC measurements, suggesting that it could be a useful device for performing respiratory therapy in ALS patients.