Enzyme Replacement Therapy Decreases Left Ventricular Mass Index in Patients with Hunter Syndrome?

Although enzyme replacement therapy (ERT) has shown benefit in improving cardiac systolic function in a murine model of cardiomyopathy associated with Hunter syndrome, few studies have analyzed its effect in humans. We evaluated the effect of ERT on patients with Hunter syndrome-related cardiomyopathy. We performed a retrospective analysis of serial transthoracic echocardiograms performed before and over the first 5 years after treatment initiation, in 14 patients with Hunter syndrome. An important cardiac remodeling occurred in all patients in this study. There was a significant reduction in left ventricular mass index from 70.88 to 26.75 g/m2.7 (p = 0.003), with a trend towards a decrease in relative wall thickness from 0.515 at baseline to 0.370 after 5 years of enzyme therapy (p = 0.140). No differences were observed in aortic root diameter, left atrial diameter, left ventricular diastolic and systolic diameters, left ventricular ejection fraction, or myocardial performance index. Our findings demonstrate that ERT contributes to reducing left ventricular mass index in patients with Hunter syndrome.

Predictors of early left ventricular systolic dysfunction in duchenne muscular dystrophy patients.

INTRODUCTION: Early detection of left ventricular systolic dysfunction (LVSD) is important for therapeutic strategies for Duchenne muscular dystrophy (DMD) patients. We analyzed myocardial strain using echocardiography for early detection of LVSD and determined the predictors of early LVSD. METHODS: This investigation was a cross-sectional study of 40 DMD patients with normal left ventricular ejection fraction. Global longitudinal strain (GLS) was used to analyze subtle disturbances in longitudinal contraction of the myocardium. Patients were determined to have early LVSD (GLS > -18) or normal left ventricular systolic function (GLS ≤ -18). RESULTS: Patients who had early LVSD were older and had a higher frequency of corticosteroid therapy and of mutations in exons 45, 46, 47, 48, 49, 50, and 52. DISCUSSION: Myocardial strain measurements are useful for the early diagnosis of LVSD in DMD patients. Older age, use of corticosteroids, and mutations within the "hot-spot" region of the DMD gene are associated with early LVSD. Muscle Nerve, 2018.

Stroke-related mortality analysis in Paraná, Brazil, over 10 years.

BACKGROUND: Stroke is the second leading cause of death and disability around the world. OBJECTIVE: The purpose of this study is to evaluate the age- and sex-specific mortality rates related to stroke in the state of Paraná, Brazil, between 2007 and 2016. METHODS: In this cross-sectional study, residents in the state of Paraná were selected by death certificates (from 2007-2016); the basic cause of death was stroke. A descriptive analysis was performed, and mortality rates were calculated with a 95% confidence interval (95% CI) for each year. RESULTS: From 2007 to 2016, there were 62,607 deaths in the state of Paraná due to stroke. Most individuals had medical assistance before death (85.7% in 2007 versus 83.9% in 2016), and most of these deaths occurred in hospitals (73.6% in 2007 versus 74.8% in 2016). Death rates due to stroke increased from 138 (95% CI 135-142) to 163 (95% CI 159-166) per 100,000 inhabitants. This raise occurred mainly in those over 79 years old. For the ages groups of 34 to 44 and 44 to 54 years, mortality rate decreased. CONCLUSIONS: In the past 15 years, despite the advances in the diagnosis and treatment of stroke, there has been an increase in mortality due to stroke in the state of Paraná. This fact is possibly associated with the aging of the population because there was a more pronounced increase in the group over 79 years old. Thus, new health strategies are necessary to improve the survival and quality of life of poststroke individuals.

ANTECEDENTES: O acidente vascular cerebral (AVC) é a segunda principal causa de morte e invalidez em todo o mundo. OBJETIVO: O objetivo desse estudo é avaliar as taxas de mortalidade específicas por idade e sexo relacionadas ao AVC no estado do Paraná, Brasil, entre 2007 e 2016. MéTODOS: Neste estudo transversal, foram selecionados residentes no estado do Paraná por certidões de óbito (de 2007­2016); a causa básica da morte foi AVC. Uma análise descritiva foi realizada e as taxas de mortalidade calculadas com intervalo de confiança de 95% (IC 95%) para cada ano. RESULTADOS: De 2007 a 2016, ocorreram 62.607 óbitos no estado do Paraná por AVC. A maioria dos indivíduos teve assistência médica antes do óbito (85,7% em 2007 contra 83,9% em 2016), e a maioria desses óbitos ocorreu em hospitais (73,6% em 2007 contra 74,8% em 2016). As taxas de mortalidade por AVC aumentaram de 138 (IC 95% 135­142) para 163 (IC 95% 159­166) por 100.000 habitantes. Esse aumento ocorreu principalmente em maiores de 79 anos. Para as faixas etárias de 34 a 44 e 44 a 54 anos, a taxa de mortalidade diminuiu. CONCLUSõES: Nos últimos 15 anos, apesar dos avanços no diagnóstico e tratamento do AVC, houve aumento da mortalidade no estado do Paraná. Este fato está possivelmente associado ao envelhecimento da população, pois houve um aumento mais acentuado no grupo acima de 79 anos. Assim, novas estratégias de saúde são necessárias para melhorar a sobrevida e a qualidade de vida dos indivíduos pós-AVC.

Evaluation of Left-Sided Heart Chambers With Novel Echocardiographic Techniques in Men With Duchenne or Becker Muscular Dystrophy.

Left ventricular systolic dysfunction (LVSD) is a common finding in patients with Duchenne (DMD) and Becker (BMD) muscular dystrophies. Novel echocardiographic techniques have been used for the detection of LVSD in several heart diseases. We aim to compare cardiac anatomic and functional data studied by three-dimensional (3DE) and two-dimensional (2DE) echocardiography and to analyze the myocardial strain for the detection of early LVSD in DMD and BMD patients. We performed a cross-sectional study of 46 DMD and 14 BMD patients. We measured left atrium volume and left ventricle volumes and ejection fraction using 3DE and 2DE techniques. Myocardial strain analysis was derived from global longitudinal strain (GLS) measurements. GLS was measured by 2DE with the speckle tracking technique. The correlation between 3DE and 2DE for the measurement of left atrium volume as well as left ventricle diastolic and systolic volumes was strong. 2DE presented larger left atrium and left ventricle volumes. Left ventricle ejection fraction was similar between the two techniques. Myocardial strain analysis was able to detect early LVSD in 50.0% of DMD patients and in 9.1% of BMD patients. In conclusion, two-dimensional echocardiography appears to be a good alternative for the anatomical and functional evaluation of the left heart chambers in DMD and BMD patients. Myocardial strain analysis detects early LVSD in a sizable portion of patients with dystrophinopathies.

Stroke-related mortality analysis in Paraná, Brazil, over 10 years / Análise da mortalidade relacionada ao acidente vascular cerebral no estado do Paraná, Brasil, ao longo de 10 anos

Abstract Background Stroke is the second leading cause of death and disability around the world. Objective The purpose of this study is to evaluate the age- and sex-specific mortality rates related to stroke in the state of Paraná, Brazil, between 2007 and 2016. Methods In this cross-sectional study, residents in the state of Paraná were selected by death certificates (from 2007-2016); the basic cause of death was stroke. A descriptive analysis was performed, and mortality rates were calculated with a 95% confidence interval (95% CI) for each year. Results From 2007 to 2016, there were 62,607 deaths in the state of Paraná due to stroke. Most individuals had medical assistance before death (85.7% in 2007 versus 83.9% in 2016), and most of these deaths occurred in hospitals (73.6% in 2007 versus 74.8% in 2016). Death rates due to stroke increased from 138 (95% CI 135-142) to 163 (95% CI 159-166) per 100,000 inhabitants. This raise occurred mainly in those over 79 years old. For the ages groups of 34 to 44 and 44 to 54 years, mortality rate decreased. Conclusions In the past 15 years, despite the advances in the diagnosis and treatment of stroke, there has been an increase in mortality due to stroke in the state of Paraná. This fact is possibly associated with the aging of the population because there was a more pronounced increase in the group over 79 years old. Thus, new health strategies are necessary to improve the survival and quality of life of poststroke individuals.

Resumo Antecedentes O acidente vascular cerebral (AVC) é a segunda principal causa de morte e invalidez em todo o mundo. Objetivo O objetivo desse estudo é avaliar as taxas de mortalidade específicas por idade e sexo relacionadas ao AVC no estado do Paraná, Brasil, entre 2007 e 2016. Métodos Neste estudo transversal, foram selecionados residentes no estado do Paraná por certidões de óbito (de 2007-2016); a causa básica da morte foi AVC. Uma análise descritiva foi realizada e as taxas de mortalidade calculadas com intervalo de confiança de 95% (IC 95%) para cada ano. Resultados De 2007 a 2016, ocorreram 62.607 óbitos no estado do Paraná por AVC. A maioria dos indivíduos teve assistência médica antes do óbito (85,7% em 2007 contra 83,9% em 2016), e a maioria desses óbitos ocorreu em hospitais (73,6% em 2007 contra 74,8% em 2016). As taxas de mortalidade por AVC aumentaram de 138 (IC 95% 135-142) para 163 (IC 95% 159-166) por 100.000 habitantes. Esse aumento ocorreu principalmente em maiores de 79 anos. Para as faixas etárias de 34 a 44 e 44 a 54 anos, a taxa de mortalidade diminuiu. Conclusões Nos últimos 15 anos, apesar dos avanços no diagnóstico e tratamento do AVC, houve aumento da mortalidade no estado do Paraná. Este fato está possivelmente associado ao envelhecimento da população, pois houve um aumento mais acentuado no grupo acima de 79 anos. Assim, novas estratégias de saúde são necessárias para melhorar a sobrevida e a qualidade de vida dos indivíduos pós-AVC.

Smoking cessation among patients at a university hospital in Curitiba, Brazil.

OBJECTIVE: To determine the prevalence of smoking and the frequency of smoking cessation counseling among patients at a university hospital, as well as to compare smokers and former smokers in terms of smoking history. METHODS: A cross-sectional study involving 629 patients at the Federal University of Paraná Hospital de Clínicas, located in the city of Curitiba, Brazil. RESULTS: Of the 629 patients, 206 (32.7%) were male, 76 (12.1%) were smokers, 179 (28.5%) were former smokers, and 374 (59.5%) were nonsmokers. The mean age of the patients was 49.9 ± 15.0 years (range, 18-84 years). Of the 76 smokers and 179 former smokers, 72 (94.7%) and 166 (92.7%), respectively, were questioned about tobacco use. Smoking history and degree of nicotine dependence were higher among the former smokers (p = 0.0292 and p = 0.0125, respectively). Gender, age at smoking initiation, physician inquiry about tobacco use, and smoking cessation counseling were comparable between the two groups. The smoking cessation rate was 0.70. The prevalence of heavy smoking varied by gender and by age bracket, being higher in males and in the 41-70 year age bracket. CONCLUSIONS: The smoking prevalence in this group of patients was lower than that reported for patients at another university hospital, for adults in Curitiba, and for adults in Brazil. The smoking cessation rate was higher in these patients than in the general population of Curitiba. Smokers and former smokers differed regarding age, smoking history, and degree of nicotine dependence. Heavy smoking and a moderate or high degree of nicotine dependence were not obstacles to smoking cessation.

General practice physician knowledge about headache: evaluation of the municipal continual medical education program.

OBJECTIVE: Headache is a common condition not always managed satisfactorily by primary care providers (PCPs). In an effort to improve headache care, the Curitiba City Hall in consortia with Hospital de Clínicas da Universidade Federal do Paraná - Brazil developed an educational program directed to the PCPs. The goal of the project was to evaluate, to update and to train the PCP on headache knowledge and care. METHOD: The program was designed to have a theoretical phase and a practical phase. Knowledge on headache and medical care of headache were surveyed before and after the theoretical phase thorough a specific questionnaire. RESULTS: Significant improvement in post-CME scores on headache prevalence (p<0.001), migraine diagnosis (p<0.001) and management (p=0.01), secondary headache diagnosis (p=0.005) and management (p=0.005) was reached by the respondents. CONCLUSION: Improvement in post-CME scores confirms that the program had a significant immediate impact on the PCPs knowledge directly affecting the patient's health.

Cessação de tabagismo em pacientes de um hospital universitário em Curitiba / Smoking cessation among patients at a university hospital in Curitiba, Brazil

OBJETIVO: Determinar a prevalência de tabagismo e do aconselhamento para a cessação do tabagismo em um grupo de pacientes de um hospital universitário, assim como comparar a carga tabágica entre fumantes e ex-fumantes. MÉTODOS: Estudo transversal com 629 pacientes do Hospital de Clínicas da Universidade Federal do Paraná, localizado em Curitiba. RESULTADOS: Dos 629 pacientes, 206 (32,7 por cento) eram do sexo masculino, 76 (12,1 por cento) eram fumantes, 179 (28,5 por cento) eram ex-fumantes, e 374 (59,5 por cento) eram não fumantes. A média de idade dos pacientes foi de 49,9 ± 15,0 anos (variação: 18-84 anos). Dos 76 fumantes e 179 ex-fumantes, 72 (94,7 por cento) e 166 (92,7 por cento), respectivamente, foram indagados sobre o hábito de fumar. A carga tabágica e o grau de dependência de nicotina foram maiores entre os ex-fumantes (p = 0,0292 e p = 0,0125, respectivamente). Gênero, idade ao início do tabagismo, questionamento médico sobre hábito de fumar e orientação para cessação do fumo foram semelhantes entre os dois grupos. O índice de cessação de tabagismo foi de 0,70. A prevalência de fumo pesado variou entre os gêneros e as faixas etárias - maior entre os homens e na faixa etária de 41-70 anos. CONCLUSÕES: A prevalência de tabagismo neste grupo de pacientes foi menor do que a observada em pacientes de outro hospital universitário e na população adulta de Curitiba e do Brasil. O índice de cessação do tabagismo foi maior do que o da população de Curitiba. Fumantes e ex-fumantes diferiram em relação à idade, à carga tabágica e ao grau de dependência. Fumo pesado e dependência média ou elevada não impediram cessação do tabagismo.

OBJECTIVE: To determine the prevalence of smoking and the frequency of smoking cessation counseling among patients at a university hospital, as well as to compare smokers and former smokers in terms of smoking history. METHODS: A cross-sectional study involving 629 patients at the Federal University of Paraná Hospital de Clínicas, located in the city of Curitiba, Brazil. RESULTS: Of the 629 patients, 206 (32.7 percent) were male, 76 (12.1 percent) were smokers, 179 (28.5 percent) were former smokers, and 374 (59.5 percent) were nonsmokers. The mean age of the patients was 49.9 ± 15.0 years (range, 18-84 years). Of the 76 smokers and 179 former smokers, 72 (94.7 percent) and 166 (92.7 percent), respectively, were questioned about tobacco use. Smoking history and degree of nicotine dependence were higher among the former smokers (p = 0.0292 and p = 0.0125, respectively). Gender, age at smoking initiation, physician inquiry about tobacco use, and smoking cessation counseling were comparable between the two groups. The smoking cessation rate was 0.70. The prevalence of heavy smoking varied by gender and by age bracket, being higher in males and in the 41-70 year age bracket. CONCLUSIONS: The smoking prevalence in this group of patients was lower than that reported for patients at another university hospital, for adults in Curitiba, and for adults in Brazil. The smoking cessation rate was higher in these patients than in the general population of Curitiba. Smokers and former smokers differed regarding age, smoking history, and degree of nicotine dependence. Heavy smoking and a moderate or high degree of nicotine dependence were not obstacles to smoking cessation.

General practice physician knowledge about headache: evaluation of the municipal continual medical education program / Conhecimento dos médicos generalistas sobre cefaléias: avaliação de um programa municipal de educação médica continuada

OBJECTIVE: Headache is a common condition not always managed satisfactorily by primary care providers (PCPs). In an effort to improve headache care, the Curitiba City Hall in consortia with Hospital de Clínicas da Universidade Federal do Paraná - Brazil developed an educational program directed to the PCPs. The goal of the project was to evaluate, to update and to train the PCP on headache knowledge and care. METHOD: The program was designed to have a theoretical phase and a practical phase. Knowledge on headache and medical care of headache were surveyed before and after the theoretical phase thorough a specific questionnaire. RESULTS: Significant improvement in post-CME scores on headache prevalence (p<0.001), migraine diagnosis (p<0.001) and management (p=0.01), secondary headache diagnosis (p=0.005) and management (p=0.005) was reached by the respondents. CONCLUSION: Improvement in post-CME scores confirms that the program had a significant immediate impact on the PCPs knowledge directly affecting the patient's health.

OBJETIVO: Cefaléia é uma condição comum nem sempre tratada de forma adequada pelos médicos generalistas (MG). Com o objetivo de melhorar essa situação, a Prefeitura de Curitiba em parceria com o Hospital de Clínicas da Universidade Federal do Paraná - Brasil desenvolveu um programa para os MG. O objetivo principal desse projeto foi avaliar, atualizar e capacitar médicos a respeito do conhecimento e manejo das cefaléias. MÉTODO: O programa consistiu de uma fase teórica e uma fase prática. O conhecimento sobre cefaléia e seu manejo foi avaliado através de um questionário específico antes e depois da fase teórica. RESULTADOS: Significativa melhora no pós-teste nos itens de prevalência de cefaléias (p<0,001), diagnóstico de migrânea (p<0,001) e seu manejo (p=0,01), diagnóstico de cefaléia secundária (p=0,005) e seu manejo (p=0,005) foram alcançados pelos participantes. CONCLUSÃO: Melhorias na pontuação dos pós-testes confirmam que o programa teve um impacto imediato e significante no conhecimento dos MG afetando diretamente a saúde dos pacientes.