Quality of life in patients with Charcot-Marie-Tooth disease type 1A

Avaliou-se o comprometimento funcional de pacientes com Charcot-Marie-Tooth provenientes da duplicação 17p11.2-p12 (CMT1A), utilizando o SF-36, que é um questionário para medir a qualidade de vida. Vinte e cinco pacientes de ambos os sexos com idades ≥10 anos e diagnóstico molecular de CMT1A foram selecionados. Idade, sexo, condições sociodemográficas e profissionais foram pareados com o Grupo Controle (sem histórico familiar de neuropatia). Os resultados mostraram que o maior impacto da CMT1A na qualidade de vida ocorreu nos domínios social e emocional dos pacientes avaliados. A capacidade funcional também tende a ser significativamente afetada, enquanto outros indicadores de deficiência física foram preservados. Por fim, os aspectos sociais e emocionais dos pacientes acometidos por CMT1A costumam ser negligenciados na assistência médica prestada aos pacientes brasileiros, e devem ser melhor compreendidos a fim de oferecer uma assistência global à saúde, resultando em adequada qualidade de vida.

Cardiovascular risk and mortality in end-stage renal disease patients undergoing dialysis: sleep study, pulmonary function, respiratory mechanics, upper airway collapsibility, autonomic nervous activity, depression, anxiety, stress and quality of life: a prospective, double blind, randomized controlled clinical trial

Background: Chronic kidney disease (CKD) is one of the most serious public health problems. The increasing prevalence of CKD in developed and developing countries has led to a global epidemic. The hypothesis proposed is that patients undergoing dialysis would experience a marked negative influence on physiological variables of sleep and autonomic nervous system activity, compromising quality of life.Methods/Design: A prospective, consecutive, double blind, randomized controlled clinical trial is proposed to address the effect of dialysis on sleep, pulmonary function, respiratory mechanics, upper airway collapsibility, autonomic nervous activity, depression, anxiety, stress and quality of life in patients with CKD. The measurement protocol will include body weight (kg); height (cm); body mass index calculated as weight/height(2); circumferences (cm) of the neck, waist, and hip; heart and respiratory rates; blood pressures; Mallampati index; tonsil index; heart rate variability; maximum ventilatory pressures; negative expiratory pressure test, and polysomnography (sleep study), as well as the administration of specific questionnaires addressing sleep apnea, excessive daytime sleepiness, depression, anxiety, stress, and quality of life.Discussion: CKD is a major public health problem worldwide, and its incidence has increased in part by the increased life expectancy and increasing number of cases of diabetes mellitus and hypertension. Sleep disorders are common in patients with renal insufficiency. Our hypothesis is that the weather weight gain due to volume overload observed during interdialytic period will influence the degree of collapsibility of the upper airway due to narrowing and predispose to upper airway occlusion during sleep, and to investigate the negative influences of haemodialysis in the physiological variables of sleep, and autonomic nervous system, and respiratory mechanics and thereby compromise the quality of life of patients.

Psoriasis: correlation between severity index (PASI) and quality of life index (DLQI) in patients assessed before and after systemic treatment

BACKGROUND: Psoriasis is a chronic inflammatory disease of the skin that affects patients of all ages and both genders. The impact of the disease on quality of life is greater among patients with moderate to severe psoriasis. OBJECTIVE: to establish a correlation between the psoriasis area and severity index (PASI) and the Dermatology Life Quality Index (DLQI) based on a quality of life questionnaire adapted to the Brazilian context for patients with plaque psoriasis before and after systemic treatment. METHODS: This was a cross-sectional, descriptive study of psoriasis patients who did not undergo treatment or who manifested clinical activity of the disease. Patients were evaluated according to the PASI and the quality of life questionnaire adapted to the Brazilian context before and 60 days after systemic treatment. RESULTS: Thirty-five patients participated in the study. Twenty-six were men, with a mean age of 46 years. There was no correlation between the PASI and the quality of life questionnaire adapted to the Brazilian context, but there was a correlation between the PASI and some items of the quality of life questionnaire adapted to the Brazilian context, such as jobs involving public contact. CONCLUSION: The non-correlation between the PASI and the quality of life questionnaire adapted to the Brazilian context in this work may be associated with a history of chronic disease, which implies greater acceptance of the illness, or may be related to the low income and social status of the patients studied. The correlation observed among patients with careers involving public contact suggests that some professions are more impacted by the disease. It may be necessary to adapt the quality of life questionnaire to patients with a low income and cultural and social limitations. The small sample size (n=35 patients) and the short follow-up period of 60 days were some of the limitations of this work.

Oral health associated with quality of life of people living with HIV/AIDS in Brazil

Background: The epidemic of HIV/AIDS enters into its fourth decade and is still considered an important public health problem in developed and developing countries. The purpose is verify the oral health and other factors that influence the quality of life of people living with HIV/AIDS attending a public service reference in Brazil.Methods: The participants answered the questionnaire on socio-demographic conditions, issues related to HIV and daily habits. The quality of life was analyzed by the HIV/AIDS Targeted Quality of Life (HAT-QoL) instrument with 42 items divided into nine domains: General Activity, Sexual Activity, Confidentiality concerns, Health Concerns, Financial Concern, HIV Awareness, Satisfaction with Life Issues related to medication and Trust in the physician. The oral health data were collected by means of the DMFT index, use and need of dentures and the Community Periodontal Index, according to the criteria proposed by the World Health Organization, by a calibrated researcher. Bivariate and multiple linear regressions were performed.Results: Of the participants, 53.1% were women and had a mean age of 42 years, 53.1% had eight years or less of schooling and 20.3% were not employed. In analyzing the quality of life domain of the HAT-QoL, with a lower average there was: Financial concern (39.4), followed by Confidentiality concern (43.2), Sexual activities (55.2) and Health concerns (62. 88). There was an association between the variables: do not have link to employment (p < 0.001), is brown or black (p = 0.045), alcohol consumption (p = 0.041), did not make use of antiretroviral therapy (p = 0.006), high levels of viral load (p = 0.035) and need for dentures (p = 0.025), with the worse quality of life scores.Conclusion: Socioeconomic and inadequate health conditions had a negative impact on the quality of life of people with HIV/AIDS.

Transformation of wealth produced into quality of life: analysis of the social efficiency of nation-states with the DEA's triple index approach

Although an essential condition for the occurrence of human development, economic growth is not always efficiently converted into quality of life by nation-states. Accordingly, the objective of this study is to measure the social efficiency-the ability of a nation-state to convert its produced wealth into quality of life-of a set of 101 countries. To achieve this goal, the Data Envelopment Analysis method was used in its standard, cross-multiplicative and inverted form, by means of a new approach called 'triple index'. The main results indicated that the former Soviet republics and Eastern European countries stood out in terms of social efficiency. The developed countries, notwithstanding their high social indicators, did not excel in efficiency; however, the countries of south of Africa, despite having the worst social conditions, were also the most inefficient.

Health related quality of life is differently associated with leisure-time physical activity intensities according to gender: a cross-sectional approach

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Health related quality of life measure in systemic pediatric rheumatic diseases and its translation to different languages: an international collaboration

Background: Rheumatic diseases in children are associated with significant morbidity and poor health-related quality of life (HRQOL). There is no health-related quality of life (HRQOL) scale available specifically for children with less common rheumatic diseases. These diseases share several features with systemic lupus erythematosus (SLE) such as their chronic episodic nature, multi-systemic involvement, and the need for immunosuppressive medications. HRQOL scale developed for pediatric SLE will likely be applicable to children with systemic inflammatory diseases.Findings: We adapted Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY (c)) to Simple Measure of Impact of Illness in Youngsters (SMILY (c)-Illness) and had it reviewed by pediatric rheumatologists for its appropriateness and cultural suitability. We tested SMILY (c)-Illness in patients with inflammatory rheumatic diseases and then translated it into 28 languages. Nineteen children (79% female, n= 15) and 17 parents participated. The mean age was 12 +/- 4 years, with median disease duration of 21 months (1-172 months). We translated SMILY (c)-Illness into the following 28 languages: Danish, Dutch, French (France), English (UK), German (Germany), German (Austria), German (Switzerland), Hebrew, Italian, Portuguese (Brazil), Slovene, Spanish (USA and Puerto Rico), Spanish (Spain), Spanish (Argentina), Spanish (Mexico), Spanish (Venezuela), Turkish, Afrikaans, Arabic (Saudi Arabia), Arabic (Egypt), Czech, Greek, Hindi, Hungarian, Japanese, Romanian, Serbian and Xhosa.Conclusion: SMILY (c)-Illness is a brief, easy to administer and score HRQOL scale for children with systemic rheumatic diseases. It is suitable for use across different age groups and literacy levels. SMILY (c)-Illness with its available translations may be used as useful adjuncts to clinical practice and research.

Correlation between sleep and quality of life in cerebral palsy

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)