979 resultados para Machado-Joseph disease


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Aims - To investigate the effect of a range of demographic and psychosocial variables on medication adherence in chronic obstructive pulmonary disease (COPD) patients managed in a secondary care setting. Methods - A total of 173 patients with a confirmed diagnosis of COPD, recruited from an outpatient clinic in Northern Ireland, participated in the study. Data collection was carried out via face-to-face interviews and through review of patients’ medical charts. Social and demographic variables, co-morbidity, self-reported drug adherence (Morisky scale), Hospital Anxiety and Depression (HAD) scale, COPD knowledge, Health Belief Model (HBM) and self-efficacy scales were determined for each patient. Results - Participants were aged 67 ± 9.7 (mean ± SD) years, 56 % female and took a mean (SD) of 8.2 ± 3.4 drugs. Low adherence with medications was present in 29.5 % of the patients. Demographic variables (gender, age, marital status, living arrangements and occupation) were not associated with adherence. A range of clinical and psychosocial variables, on the other hand, were found to be associated with medication adherence, i.e. beliefs regarding medication effectiveness, severity of COPD, smoking status, presence of co-morbid illness, depressed mood, self-efficacy, perceived susceptibility and perceived barriers within the HBM (p < 0.05). Logistic regression analysis showed that perceived ineffectiveness of medication, presence of co-morbid illness, depressed mood and perceived barriers were independently associated with medication non-adherence in the study (P < 0.05). Conclusions - Adherence in COPD patients is influenced more by patients’ perception of their health and medication effectiveness, the presence of depressed mood and co-morbid illness than by demographic factors or disease severity.

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Background: The Unified Huntington’s Disease Rating Scale (UHDRS) is the principal means of assessing motor impairment in Huntington disease but is subjective and generally limited to in-clinic assessments. Objective: To evaluate the feasibility and ability of wearable sensors to measure motor impairment in individuals with Huntington disease in the clinic and at home. Methods: Participants with Huntington disease and controls were asked to wear five accelerometer-based sensors attached to the chest and each limb for standardized, in-clinic assessments and for one day at home. A secondchest sensor was worn for six additional days at home. Gait measures were compared between controls, participants with Huntington disease, and participants with Huntington disease grouped by UHDRS total motor score using Cohen’s d values. Results: Fifteen individuals with Huntington disease and five controls completed the study. Sensor data were successfully captured from 18 of the 20 participants at home. In the clinic, the standard deviation of step time (timebetween consecutive steps) was increased in Huntington disease (p<0.0001; Cohen’s d=2.61) compared to controls. At home with additional observations, significant differences were observed in seven additional gait measures. The gait of individuals with higher total motor scores (50 or more) differed significantly from those with lower total motor scores (below 50) on multiple measures at home. Conclusions: In this pilot study, the use of wearable sensors in clinic and at home was feasible and demonstrated gait differences between controls, participants with Huntington disease, and participants with Huntington diseasegrouped by motor impairment.

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BACKGROUND: -There are few contemporary data on the mortality and morbidity associated with rheumatic heart disease (RHD) or information on their predictors. We report the two year follow-up of individuals with RHD from 14 low and middle income countries in Africa and Asia.

METHODS: -Between January 2010 and November 2012, we enrolled 3343 patients from 25 centers in 14 countries and followed them for two years to assess mortality, congestive heart failure (CHF), stroke or transient ischemic attack (TIA), recurrent acute rheumatic fever (ARF), and infective endocarditis (IE).

RESULTS: -Vital status at 24 months was known for 2960 (88.5%) patients. Two thirds were female. Although patients were young (median age 28 years, interquartile range 18 to 40), the two year case fatality rate was high (500 deaths, 16.9%). Mortality rate was 116.3/1000 patient-years in the first year and 65.4/1000 patient-years in the second year. Median age at death was 28.7 years. Independent predictors of death were severe valve disease (hazard ratio (HR) 2.36, 95% confidence interval (CI) 1.80-3.11), CHF (HR 2.16, 95% CI 1.70-2.72), New York Heart Association functional class III/IV (HR 1.67, 95% CI 1.32-2.10), atrial fibrillation (AF) (HR 1.40, 95% CI 1.10-1.78) and older age (HR 1.02, 95% CI 1.01-1.02 per year increase) at enrolment. Post-primary education (HR 0.67, 95% CI 0.54-0.85) and female sex (HR 0.65, 95%CI 0.52-0.80) were associated with lower risk of death. 204 (6.9%) had new CHF (incidence, 38.42/1000 patient-years), 46 (1.6%) had a stroke or TIA (8.45/1000 patient-years), 19 (0.6%) had ARF (3.49/1000 patient-years), and 20 (0.7%) had IE (3.65/1000 patient-years). Previous stroke and older age were independent predictors of stroke/TIA or systemic embolism. Patients from low and lower-middle income countries had significantly higher age- and sex-adjusted mortality compared to patients from upper-middle income countries. Valve surgery was significantly more common in upper-middle income than in lower-middle- or low-income countries.

CONCLUSIONS: -Patients with clinical RHD have high mortality and morbidity despite being young; those from low and lower-middle income countries had a poorer prognosis associated with advanced disease and low education. Programs focused on early detection and treatment of clinical RHD are required to improve outcomes.

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Background: Adolescents with chronic disease (CD) can be more vulnerable to adverse psychosocial outcomes. This study aims: 1) to identify differences in psychosocial variables (health-related quality of life, psychosomatic complaints, resilience, self-regulation and social support) among adolescents who feel that CD affects or does not affect school/peers connectedness (measured by self-reported participation in school and social activities); and 2) to assess the extent to which psychosocial variables are associated with connectedness in school and peer domains. Methods: A cross-sectional study was conducted in 135 adolescents with CD (51.9% boys), average age of 14 ± 1. 5 years old (SD = 1.5). Socio-demographic, clinical, and psychosocial variables were assessed, using a self-reported questionnaire, which included the Chronic Conditions Short Questionnaire, KIDSCREEN-10 Index, Symptoms Check-List, Healthy Kids Resilience Assessment Module Scale, Adolescent Self-Regulatory Inventory, and Satisfaction with Social Support Scale. Descriptive statistics, GLM-Univariate ANCOVA and Logistic Regression were performed using the IBM Statistical Package for Social Sciences (SPSS), version 22.0. The significance level was set at p < 0.05. Results: Thirteen to eighteen percent of the adolescents felt that CD affected participation at school (PSCH) and participation in leisure time with friends (PLTF). These adolescents presented lower results for all psychosocial study variables, when compared with adolescents who did not feel affected in both areas of participation. From the studied psychosocial variables, the most important ones associated with PSCH (after controlling for age, gender, diagnosis, and education level of father/mother) were self-regulation and psychosomatic health. Concerning the PLTF, social support was the sole variable explaining such association. Conclusions: The present study pointed out the association between psychosocial variables; and living with a CD and school/peers connectedness. The need to focus on the assessment of the effects of a CD on adolescents’ lives and contexts is suggested, as well as on the identification of vulnerable adolescents. Such identification could help to facilitate the maximization of social participation of adolescents with CD, and to plan interventions centered on providing support and opportunities for a healthy youth development. For that purpose, a complex and multifactorial approach that includes clinicians, schools, family, and peers may be proposed.

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Stroke stands for one of the most frequent causes of death, without distinguishing age or genders. Despite representing an expressive mortality fig-ure, the disease also causes long-term disabilities with a huge recovery time, which goes in parallel with costs. However, stroke and health diseases may also be prevented considering illness evidence. Therefore, the present work will start with the development of a decision support system to assess stroke risk, centered on a formal framework based on Logic Programming for knowledge rep-resentation and reasoning, complemented with a Case Based Reasoning (CBR) approach to computing. Indeed, and in order to target practically the CBR cycle, a normalization and an optimization phases were introduced, and clustering methods were used, then reducing the search space and enhancing the cases re-trieval one. On the other hand, and aiming at an improvement of the CBR theo-retical basis, the predicates` attributes were normalized to the interval 0…1, and the extensions of the predicates that match the universe of discourse were re-written, and set not only in terms of an evaluation of its Quality-of-Information (QoI), but also in terms of an assessment of a Degree-of-Confidence (DoC), a measure of one`s confidence that they fit into a given interval, taking into account their domains, i.e., each predicate attribute will be given in terms of a pair (QoI, DoC), a simple and elegant way to represent data or knowledge of the type incomplete, self-contradictory, or even unknown.

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In this study we examined the impact of weather variability and tides on the transmission of Barmah Forest virus (BFV) disease and developed a weather-based forecasting model for BFV disease in the Gladstone region, Australia. We used seasonal autoregressive integrated moving-average (SARIMA) models to determine the contribution of weather variables to BFV transmission after the time-series data of response and explanatory variables were made stationary through seasonal differencing. We obtained data on the monthly counts of BFV cases, weather variables (e.g., mean minimum and maximum temperature, total rainfall, and mean relative humidity), high and low tides, and the population size in the Gladstone region between January 1992 and December 2001 from the Queensland Department of Health, Australian Bureau of Meteorology, Queensland Department of Transport, and Australian Bureau of Statistics, respectively. The SARIMA model shows that the 5-month moving average of minimum temperature (β = 0.15, p-value < 0.001) was statistically significantly and positively associated with BFV disease, whereas high tide in the current month (β = −1.03, p-value = 0.04) was statistically significantly and inversely associated with it. However, no significant association was found for other variables. These results may be applied to forecast the occurrence of BFV disease and to use public health resources in BFV control and prevention.