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.

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.

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.

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.

Mediastinal lymph node amyloidosis in a patient with sarcoidosis.

A 27-year-old male patient presented with respiratory symptoms, bilateral enlargement of the cervical lymph nodes and enlarged liver. In the imaging studies, bilateral enlargement of the hilar nodes was observed, together with pulmonary infiltrate. The patient was submitted to lung and liver biopsies, which revealed noncaseating granulomas. The clinical, radiological and histopathological findings were consistent with sarcoidosis and lymph node amyloidosis. The combination of sarcoidosis and amyloidosis has rarely been reported.

Risk factors for nosocomial infection in trauma patients.

Several factors are implicated in the increased vulnerability of multiple trauma victims to infection, especially in intensive care-units (ICU). This cohort study was designed to report the incidence, the topography, the etiology and to identify the risk factors for infection in trauma patients admitted in an ICU. From January 2000 to December 2001, 416 trauma patients were admitted to the ICU for more than 24 hours, the mean length of stay was 9.3 days (range 2-65) and 188 (45%) patients developed a total of 290 NI. The most prevailing infections were pneumonia (49%), bloodstream (19%) and urinary tract infections (12%). The variables studied were: the demographic data, diagnosis on admission, site and mechanism of injury, type and number of surgeries, use of invasive devices, days under mechanical ventilation (MV) and site and number of NI. These variables were analyzed with a univariable and multivariable regression analysis. The NI was associated with injury in more than 1 anatomic segment (OR=1.6; CI95%1.06-2.40); mechanical ventilation for more than 3 days (OR=12; CI95% 6.87-24.02); more than 1 surgery (OR=3.13;CI95%1.75-5.65) and more than 2 invasive devices (OR=4.7; CI95%2.99-7.37). Deaths over the first 5 days had high association (RR=3.18) with NI. Three significant variables were identified in the logistic regression, which are: more than 3 days under MV, number of invasive devices and number of surgeries.

Doença intersticial pulmonar associada à doença inflamatória intestinal: relato de dois casos / Interstitial pulmonary disease and inflammatory bowel disease association: 2 cases report

O acometimento pulmonar na doença inflamatória intestinal é raro e não está bem caracterizado. Relatamos os casos de duas pacientes com sintomatologia pulmonar inespecífica, em tratamento prévio de doença inflamatória intestinal – doença de Crohn (caso 1) e retocolite ulcerativa (caso 2). Os achados tomográficos foram compatíveis com pneumonia intersticialusual (PIU). Foi realizada revisão da literatura sobre o assunto.

The lung involvement in the inflammatory bowel disease is rare and it is not well characterized. We describe two cases of female patients with un specific pulmonary symptomatology, and previous treatment for inflammatory bowel disease - Crohn‘s disease (case1) and ulcerative rectocolitis. A chest computerized tomography was compatible with usual interstitial pneumonia (UIP). Related literature was reviewed.

Risk factors for nosocomial infection in trauma patients

Several factors are implicated in the increased vulnerability of multiple trauma victims to infection, especially in intensive care-units (ICU). This cohort study was designed to report the incidence, the topography, the etiology and to identify the risk factors for infection in trauma patients admitted in an ICU. From January 2000 to December 2001, 416 trauma patients were admitted to the ICU for more than 24 hours, the mean length of stay was 9.3 days (range 2-65) and 188 (45 percent) patients developed a total of 290 NI. The most prevailing infections were pneumonia (49 percent), bloodstream (19 percent) and urinary tract infections (12 percent). The variables studied were: the demographic data, diagnosis on admission, site and mechanism of injury, type and number of surgeries, use of invasive devices, days under mechanical ventilation (MV) and site and number of NI. These variables were analyzed with a univariable and multivariable regression analysis. The NI was associated with injury in more than 1 anatomic segment (OR=1.6; CI95 percent1.06-2.40); mechanical ventilation for more than 3 days (OR=12; CI95 percent 6.87-24.02); more than 1 surgery (OR=3.13;CI95 percent1.75-5.65) and more than 2 invasive devices (OR=4.7; CI95 percent2.99-7.37). Deaths over the first 5 days had high association (RR=3.18) with NI. Three significant variables were identified in the logistic regression, which are: more than 3 days under MV, number of invasive devices and number of surgeries.

Amiloidose ganglionar mediastinal em paciente com sarcoidose / Mediastinal lymph node amyloidosis in a patient with sarcoidosis

Paciente masculino, 27 anos, com sintomas respiratórios, linfonodomegalia cervical anterior bilateral e hepatomegalia. Os estudos de imagem evidenciaram linfonodomegalia hilar bilateral e infiltrado pulmonar. O paciente foi submetido a biópsias pulmonar e hepática, que evidenciaram presença de granulomas não caseosos. Também foi submetido à biópsia de linfonodo hilar, que revelou a presença de material amilóide. Os achados clínicos, radiológicos e histopatológicos foram compatíveis com sarcoidose e amiloidose ganglionar. A associação entre sarcoidose e amiloidose é raramente descrita.

A 27-year-old male patient presented with respiratory symptoms, bilateral enlargement of the cervical lymph nodes and enlarged liver. In the imaging studies, bilateral enlargement of the hilar nodes was observed, together with pulmonary infiltrate. The patient was submitted to lung and liver biopsies, which revealed noncaseating granulomas. The clinical, radiological and histopathological findings were consistent with sarcoidosis and lymph node amyloidosis. The combination of sarcoidosis and amyloidosis has rarely been reported.

Celiac disease prevalence in Brazilian dilated cardiomyopathy patients.

Celiac disease (CD) is a permanent condition of gluten intolerance and a number of autoimmune diseases have been associated with it. In the past few years, a relation between CD and dilated cardiomyopathy (CM) was described in Europe and United States. The aim of this study was to evaluate the prevalence of CD among south Brazilian precardiac transplant patients with advanced CM. A total of 74 patients on a list for heart transplantation were evaluated for the presence CD. The presence of anti-endomisial antibody (IgA-EmA) was determined by indirect immunofluorescence and for the anti-transglutaminase antibody (IgA anti-h-tTG) by ELISA. Serologically positive patients were submitted to upper endoscopy with intestinal biopsy. Two individuals (2.63%) were positive for IgA-EmA and 5 (6.75%) for IgA anti-h-tTG; 1 (1.35%) had both tests positive. Histologic confirmation of CD occurred only in the IgA-EmA positive patients. In conclusion, data from the present study allows recommend the screening for CD in patients with CM using IgA-EmA test as the method of choice.

Importância da colonoscopia no rastreamento de pólipos e câncer colorretal em pacientes portadores de pólipos retais / Importance of the colonoscopy in the tracking of polyps and colorectal neoplasms in carrying patients of rectal polyps

O câncer colorretal é a terceira causa de morte nas regiões sul e sudeste do Brasil. Os tumores crescem lentamente seguindo a seqüência adenoma-carcinoma. A colonoscopa permite o diagnóstico e a ressecção dos pólipos. Atualmente discute-se a colonoscopia para pacientes portadores de pólipos retais não-neoplásicos e pólipos pequenos, além dos adenomas. Objetivo: verificar a prevalência dos pólipos colônicos nos pacientes portadores de pólipos retais, correlacionar o tipo histológico e o tamanho do pólipo retal com o achado de neoplasia proximal. Pacientes e método: estudo transversal, entre 2000 e 2003, com pacientes portadores de pólipos retais submetidos a colonoscopia. Dividiram-se os pacientes por faixa etária, tipo e tamanho do pólipo retal. Pólipos e câncer foram considerados achados positivos na colonoscopia. Foram excluídas síndromes polipóides genéticas e câncer retal. Resultados: examinaram-se 1.715 pacientes dos quais 74 (4,31por cento) tinham pólipos retais. O estudo histopatológico mostrou que 54,1por cento eram adenomatosos, 23por cento hiperplásicos, 12,2por cento inflamatórios e 10,8por cento, excrescências de mucosa. A maioria dos pólipos foi encontrada em pacientes acima de 40 anos, havendo significância estatística no grupo dos neoplásicos (p<0,0001). Não houve diferença estatística quando comparados os achados positivos na colonoscopia entre os grupos de pólipos retais adenomatosos e hiperplásicos (p=0,052). O tamanho do pólipo retal não foi estatisticamente significante para achados positivos na colonoscopia. Conclusões: prevalência de pólipos proximais em portadores de pólipos retais foi 49,1por cento. Prevalência de neoplasia proximal foi de 42,5por cento e 11,7por cento nos portadores de pólipos retais neoplásicos e não-neoplásicos, respectivamente. O tamanho do pólipo retal não foi preditivo para achado de neoplasia proximal.

Uso de ciclosporina A, microemulsão de fabricação nacional, no tratamento da sindrome nefrótica na infância: resultados preliminares / Brazilian cyclosporine a microemulsion for treating idiopathic nephrotic syndrome in children: preliminary results

Objetivo :Determinar a efetividade e segurança da ciclosporina A (CsA), microemulsão de fabricação nacional, no tratamento da síndrome nefrótica primária (SNP) na infância. Métodos :Estudo de coorte não controlado, avaliação clínico-laboratorial e biópsia renal. Para significância estatística fixou-se a=0,01. Resultados :27 crianças com SNP foram acompanhadas por seis meses e 22 (81,5 por cento), por 12 meses, sendo 17 (63 por cento) delas do sexo masculino, com idade no início da administração de CsA entre 2,5 e 9,9 anos (mediana em 6,5). Houve remissão em 9/11 (81,2 por cento) dos pacientes córtico-dependentes (CD) e em 8/16 (50por cento) dos córtico-resistentes (CR)- A dose cumulativa mediana de corticoesteróide (CE) foi 154,75 mg/kg no mês I, 86,50 mg/kg no mês 6 (p<0,000 1) e 70,86 no mês 12 (p=0, 1 16). A ciciosporinemia média foi 134,03 ±45,20 (mediana 126) no mês 6 e 93,47± 1 7,68 no mês 12 (p< 0,0001). No mês 1, 69,2 (por cento) dos pacientes necessitaram de internamente, 23,1 (por cento) no mês 6 (p = 0,0022) e 11,1(porcento) no mês 12 (p = 0,2744). As médias da albumina sérica foram 2,1 ±0,7g/l, 3,5±0,9 (p<0,000 1) e 3,5± 1,0 (p=0,93 1). A proteinúria de 24 horas foi 4,1 8±2,47g/l (mediana 3,20), 1,34±2,20 (p<0,000 1) e 1,44± 1,57 (p-=O, 102). As diferenças na distribuição no número de recaídas, na prevalência de hipertensão arterial e na média da creatinina (entre 0,58±0,16mg/dl e 0,60±0,13) não foram estatisticamente significativas. Conclusões : Observou-se benefício significativo no uso de CsA em pacientes co m SNP, CD e CR. Houve remissão da protenúria em 63 ( por cento) dos pacientes aos seis meses de tratamento e em 75 (por cento) dos que completaram 12 meses de tratamento. A função manteve-se estável no período.Observou-se beneficio significativo no uso de CsA em pacientes com SNP, CD e CR.Houve remissão da protenúria em 63 por cento dos pacientes aos seis meses de tratamento e em 75 por cento dos que completaram 12 meses de tratamento . A função renal manteve-se estável no período.

Cromoendoscopia com azul de metileno para diagnóstico de esôfago de Barrett / Methylene blue chromoendoscopy for Barrett's esophagus diagnosis

RACIONAL: O esôfago de Barrett é uma condição na qual a mucosa escamosa esofágica é substituída por metaplasia intestinal especializada, que predispõe o paciente ao desenvolvimento de adenocarcinoma esofágico. Este é precedido por displasia e carcinoma precoce; o rastreamento dessas lesões faz-se através de endoscopias digestivas periódicas com biopsias randomizadas. A incidência aumentada desse, tem despertado interesse no desenvolvimento de novas técnicas endoscópicas, como a cromoendoscopia com azul de metileno, para melhorar a identificação do esôfago de Barrett e suas complicações. OBJETIVO: Determinar se as biopsias dirigidas pela cromoendoscopia com azul de metileno oferecem vantagem em relação ao método convencional na detecção do esôfago de Barrett. MATERIAL E MÉTODO: Estudaram-se 45 pacientes com diagnóstico prévio de esôfago de Barrett, todos submetidos a dois exames de endoscopia digestiva alta com biopsias, em intervalo de 4 semanas, um convencional e outro com aplicação do corante, no período entre abril e outubro de 2002. RESULTADOS: Os resultados histológicos das biopsias de todos os exames foram comparados. Observou-se sensibilidade de 62,5 por cento, especificidade de 15,4 por cento, valor preditivo positivo de 57,7 por cento e valor preditivo negativo de 18,2 por cento. Não houve diferença significativa quanto ao número de biopsias. O tempo de duração da técnica de cromoendoscopia foi significativamente maior quando comparado ao da técnica convencional. CONCLUSAO: Não se observou vantagem na utilização da cromoendoscopia em relação à técnica randomizada no diagnóstico do esôfago de Barrett.

[Methylene blue chromoendoscopy for Barrett's esophagus diagnosis]. / Cromoendoscopia com azul de metileno para diagnóstico de esôfago de Barrett.

BACKGROUND: Barrett's esophagus is a condition in which the normal stratified squamous epithelium of the esophagus is replaced by specialized intestinal metaplasia, that carries an increased risk for the development of adenocarcinoma of the esophagus. Dysplasia and initial stage carcinoma generally precede this neoplasm; the increased risk has led to attempts at early detection of these lesions through periodic upper endoscopy and biopsies. The continued rise in the incidence of adenocarcinoma of the esophagus has fueled resurgent interest in the use of a variety of endoscopic techniques, like methylene blue chromoendoscopy, to improve the diagnosis of Barrett's esophagus and associated complications. AIM: To determine if methylene blue chromoendoscopy directed biopsies offer advantage over the conventional technique in the detection of Barrett's esophagus. MATERIAL AND METHOD: Forty-five patients with previous diagnosis of Barrett's esophagus were enrolled in this study; all of them underwent upper endoscopy with random biopsies and upper endoscopy with methylene-blue directed biopsies within 4 weeks, in the period between April and October, 2002. RESULTS: Results of histologic examination of random biopsies and those of methylene blue directed biopsies were compared. Sensitivity, specificity, positive and negative predictive values were 62,5%, 15,4%, 57,7% and 18,2%, respectively. No significant differences were found in the total number of biopsy specimens. Differences were found in the total amount of time spent during each procedure (chromoendoscopy x conventional technique), with chromoendoscopy being significantly longer. CONCLUSION: We concluded that methylene blue chromoendoscopy offered no advantage over the conventional method in the diagnosis of Barrett's esophagus.