Influence of Congenital Heart Defect Types over Cardiopulmonary Bypass, Aortic Cross-Clamping, and Intensive Care Unit Length of Stay and Their Association with Genetic Factors

Aim: Surgical correction of congenital heart defects (CHD) often requires interruption of blood flow through cardiopulmonary bypass (CPB) and aortic cross-clamping (ACC), for which duration(s) are considered to be prognostic factors, along with intensive care unit (ICU) length of stay (ICULOS). The aim of this study was to evaluate these surgical prognostic factors in pediatric patients with different types of CHD regarding their type of lesion and associated genetic factors. Study Design: Cross-sectional cohort study with 307 pediatric patients. Place and Duration of Study: Pediatric Intensive Care Unit (ICU) of Hospital da Criança Santo Antônio, in Porto Alegre/RS, Brazil, from 2006-2009 (3 years) Methodology: After inclusion criteria, we studied 266 pediatric patients admitted for the first time in a reference cardiac pediatric ICU from Southern Brazil following cardiac surgery. Intraoperative prognostic factors such as duration of CPB, ACC and ICULOS, in addition to dysmorphological and cytogenetic examinations were compiled and analyzed. P-values of <0.05 were considered significant. Results: CPB time was associated to four outflow tract defects (Tetralogy of Fallot [ToF], transposition of the great arteries [TGA], double outlet right ventricle, and truncus arteriosus [TA]), atrioventricular septal defect, and hypoplastic left heart syndrome (HLHS) (P < 0.001). ACC duration was associated with three outflow tract defects (ToF, TGA, and TA) and HLHS (P < 0.001). Moreover, CPB and ACC times showed an association with cyanotic and complex heart defects, as well as prolonged ICULOS (P < 0.001). There was no relationship between these prognostic factors and syndromic aspects or cytogenetic findings. Conclusions: CHD type has an impact over CPB and ACC duration and ICULOS, whereas genetic factors are not associated with those prognostic factors.

Sildenafil delays the intestinal transit of a liquid meal in awake rats

Sildenafil slows down the gastric emptying of a liquid test meal in awake rats and inhibits the contractility of intestinal tissue strips. We studied the acute effects of sildenafil on in vivo intestinal transit in rats. Fasted, male albino rats (180-220 g, N = 44) were treated (0.2 mL, iv) with sildenafil (4 mg/kg) or vehicle (0.01 N HCl). Ten minutes later they were fed a liquid test meal (99m technetium-labeled saline) injected directly into the duodenum. Twenty, 30 or 40 min after feeding, the rats were killed and transit throughout the gastrointestinal tract was evaluated by progression of the radiotracer using the geometric center method. The effect of sildenafil on mean arterial pressure (MAP) was monitored in a separate group of rats (N = 14). Data (medians within interquartile ranges) were compared by the Mann-Whitney U-test. The location of the geometric center was significantly more distal in vehicle-treated than in sildenafil-treated rats at 20, 30, and 40 min after test meal instillation (3.3 (3.0-3.6) vs 2.9 (2.7-3.1); 3.8 (3.4-4.0) vs 2.9 (2.5-3.1), and 4.3 (3.9-4.5) vs 3.4 (3.2-3.7), respectively; P < 0.05). MAP was unchanged in vehicle-treated rats but decreased by 25 percent (P < 0.05) within 10 min after sildenafil injection. In conclusion, besides transiently decreasing MAP, sildenafil delays the intestinal transit of a liquid test meal in awake rats.

Examining life-course influences on chronic disease: the importance of birth cohort studies from low- and middle- income countries. An overview

The objectives of this overview are to describe the past and potential contributions of birth cohorts to understanding chronic disease aetiology; advance a justification for the maintenance of birth cohorts from low- and middle-income countries (LMIC); provide an audit of birth cohorts from LMIC; and, finally, offer possible future directions for this sphere of research. While the contribution of birth cohorts from affluent societies to understanding disease aetiology has been considerable, we describe several reasons to anticipate why the results from such studies might not be directly applied to LMIC. More than any other developing country, Brazil has a tradition of establishing, maintaining and exploiting birth cohort studies. The clear need for a broader geographical representation may be precipitated by a greater collaboration worldwide in the sharing of ideas, fieldwork experience, and cross-country cohort data comparisons in order to carry out the best science in the most efficient manner. This requires the involvement of a central overseeing body - such as the World Health Organization - that has the respect of all countries and the capacity to develop strategic plans for `global' life-course epidemiology while addressing such issues as data-sharing. For rapid progress to be made, however, there must be minimal bureaucratic entanglements.

Doença de Wilson: apresentaçäo com hepatite aguda

Apresentam-se dois casos de doença de Wilson que se manifestaram por hepatite aguda. No primeiro caso, o quadro clínico foi acompanhado por anemia hemolítica, hipofosfatasia com fosfatase alcalina que atingiu o mínimo de 7U/dl (normal para a idade: 57-303U/dl) e síndrome de Fanconi com hiperfosfatúria, hiperuricosúria e eliminaçäo aumentada de glicinina, glutamina, alanina e lisina. O segundo caso apresentava já quadro histológico de cirrose hepática e evoluiu para hepatite fulminante complicada por infecçäo do líquido ascítico. Nos dois casos foi iniciada terapêutica com penicilamina, imediatamente após o diagnóstico, verificando-se a regressäo das principais mainifestaçöes no primeiro caso. No segundo caso a evoluçäo foi desfavorável, verificando-se o aparecimento de sepse e insuficiência renal que näo respondeu à terapêutica com hemofiltraçäo, vindo a falecer três semanas após o início da terapêutica. Discutem-se os problemas de diagnóstico diferencial da terapêutica e prognóstico desta forma de apresentaçäo, com mençäo especial das indicaçöes atuais para transplante

Neoplasias das vias biliares supraampulares. Analise de 16 casos. / Supra-ampullary neoplasms of the biliary tract. Analysis of 16 cases

Apresenta-se uma serie de 16 doentes com neoplasia das vias biliares supra-ampulares (NVB), entre 60 com tumores das vias biliares observados num Servico de Medicina Interna no periodo de cinco anos (19751979). A distribuicao foi igual nos dois sexos e nove dos 16 doentes tinham mais de 60 anos. O quadro clinico iniciou-se gradualmente, por sintomas inespecificos, em 10 doentes; foi subito em seis. Ictericia foi o primeiro sinal em tres, mas estava sempre presente a data do internamento. O diagnostico foi, em geral, feito cerca de cinco meses apos o inicio das queixas e foi obtido por visualizacao direta das vias biliares: colangiografia percutanea transhepatica (CPT) e/ou colangiografia endoscopica retrogada (CPRE). Foi possivel a resseccao do tumor apenas em tres doentes. O tipo histologico dos tumores foi conhecido em 12: 11 adenocarcinomas (seis indeferenciados) e um linfoma. Encontraram-se metastases regionais apenas em cinco casos, com um tempo medio de evolucao superior a seis meses. A lenta evolucao destes tumores justifica uma atitude diagnostica mais agressiva para antecipar a terapeutica cirurgica