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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., (Copyright © 2018 Elsevier Inc. All rights reserved.)

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., (© 2018 Wiley Periodicals, Inc.)

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.

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.

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.

Background: The portal hypertension in cirrhotic patients is the main cause of this illness complication, that are clinically translated to visible collateral circulation in the abdominal wall, ascites and esophageal varices., Aim: To evaluate if the portal system echodoppler is able to estimate the presence of esophageal varices, gastric varices and congestive gastropathy in patients with hepatic cirrhosis., Patients and Methods: One hundred and eighty six patients of the gastroenterology and hepatology ambulatory of the Clinical Hospital of the Federal University of Paraná, Curitiba, PR, Brazil, had been selected for evaluation. Of those, 145 had completed all the stages of the evaluation and 133 had been enclosed in the final analysis. All had been submitted to high digestive endoscopy for evaluation of esophagogastric varices and congestive gastropathy and then to Doppler ultrasound of the portal system with study of the systolic peak speed of the portal vein, diameter of the portal and splenic vein and spleen size, presence of the umbilical vein recanalization and hepatofugal flow., Results: The patients with esophagogastric varices had significant difference of the spleen size when compared to patients without these change. However, none of the Doppler ultrasound parameters showed good accuracy and specificity in this group of cirrhotic patients. Congestive gastropathy patients had their diagnosis predict with significant manner not only by the portal and splenic vein diameter but also by the spleen size. Similarly to that described above, they do not have a good accuracy and specificity. These evaluations were validated by the construction of ROC (Receiver Operating Characteristic) curves, whose areas below the curves had always been less than 0,8., Conclusion: There was not a good correlation of the Doppler ultrasound parameters of the portal system to the presence of the main endoscopic alterations (esophagogastric varices and congestive gastropathy) in patients with hepatic cirrhosis.

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.

Background: Inflammatory bowel disease patients have shown greater reduction of the bone mineral density compared to healthy people., Aim: To evaluate the bone mineral density in a population of patients with inflammatory bowel disease., Methods: Ninety patients from 20 to 50 years old, of the Inflammatory Bowel Disease Ambulatory of the Gastroenterology Service of the Clinics Hospital, Curitiba, PR, Brazil, were selected for the evaluation. From those, 76 completed all the stages of the evaluation. The densitometry was made from lumbar column and right femur with a dual-energy x-ray absorptiometry (Hologyc QDR 1000/W) device., Results: The inflammatory bowel disease patients had a significant reduction of the bone mineral density in all the evaluated parts, femur neck, total femur and lumbar column. The analysed variables, disease activity index, usage of corticoids, the lack of physical activities, the index body mass and previous surgeries did not have influence in the results., Conclusion: Reduced bone mineral density was founded in inflammatory bowel disease patients of the Clinics Hospital, mainly in the Crohn's disease patients, as described in literature. None analyzed variables had significant correlation to the bone mineral density.

Background: The sustained response rates of chronic hepatitis C therapy to interferons alpha 2A and 2B associated to ribavirin are described as approximately 40%. Nevertheless, studies on the sustained response rates in a setting of frequent changes of type of interferon as well as of supplies of interferons and ribavirin are lacking., Aims: To evaluate the prevalence of sustained response and to determine the predictors of response to chronic hepatitis C therapy, in a cohort of outpatients at Curitiba, PR, south Brazil, in a setting of heterogeneous therapy regarding type and supply of the medications., Population and Methods: A cohort of 87 chronic hepatitis C patients submitted to therapy with interferon alpha 2 and ribavirin were followed from August 1999 to August 2002. The prevalence of sustained response and the influence of gender, age, genotype and severity of liver fibrosis in the response were evaluated., Results: A sustained response prevalence of 32.1% was found, positively associated to genotypes 2 and 3., Conclusions: Despite the frequent changes on product type and/or procedence, the sustained response prevalence rate found in this study was similar to that described in the literature.

Background: Specialized intestinal metaplasia can be categorized according endoscopic and histological findings in long segment Barrett, short segment Barrett and specialized intestinal metaplasia of cardia. Barrett's esophagus is an acquired disease that is found in about 10%-13% of patients undergoing endoscopy for symptoms of gastroesophageal reflux disease and it is well established as predisposing to esophageal adenocarcinoma. The columnar epithelium with goblet cells replaces the normal squamous epithelium., Objective: To determine the prevalence and clinical-demographic characteristics of specialized intestinal metaplasia of distal esophagus in the gastroesophageal reflux disease., Methods: From April to October 2002, 402 patients referred to upper endoscopy due gastroesophageal reflux disease were evaluated through of a symptom questionnaire about clinical and demographic features and submitted to upper endoscopy with four-quadrant biopsies 1 cm below escamocolumnar junction., Results: Eighteen point four percent of patients had specialized intestinal metaplasia, 0.5% long segment Barrett esophagus, 3.2% short segment Barrett's esophagus and 14.7% specialized intestinal metaplasia of cardia. Patients with Barrett's esophagus showed a tendency to be male and specialized metaplasia of cardia to be female. All patients with Barrett's esophagus were white. There was not association between symptoms of gastroesophageal reflux disease and specialized intestinal metaplasia, but patients with Barrett's esophagus showed a tendency to have symptoms over 5 years and had more hiatal hernia and esophagitis. The use of alcohol and tobacco was not related to the presence of specialized intestinal metaplasia., Conclusions: Barrett's esophagus was more related to the male gender, gastroesophageal reflux disease symptoms for 5 years or longer, more intense esophagitis and hiatal hernia, but was not related to the use of tobacco and alcohol.

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.