Abstract Background: Hirschsprung’s Disease (HD) is characterized by intestinal sub-occlusion and the absence of enteric ganglion cells. A rectal biopsy examination is performed to confirm the diagnosis. In a recent study, we demonstrated that the analysis of 60 sections of rectal mucosa and submucosa stained by H&E may ensure a 90% diagnostic accuracy. Although the need to analyze so many sections makes the process of reading the slides more time-consuming, this encouraged us to study their distribution in the healthy rectal submucosa, to simplify the diagnosis. Objectives: To develop a method that facilitates HD diagnosis by studying the distribution of ganglion cells in the submucosal plexus. Methods: Using the calretinin technique, we studied the distribution of plexuses in 60 fragments of rectal submucosa from 19 cadavers. After the study, the reading method created was used for diagnosis in 47 cases of suspected HD, using H&E staining. The accuracy was verified by comparing the results obtained with H&E to those obtained with the acetylcholinesterase technique, the golden standard in our laboratory. Results: The study of submucosal plexus distribution showed that just by examining the submucosal region every 20 µm, approximately, it is possible to locate a ganglionic plexus, and we have already been able to diagnose HD with 93% accuracy. Conclusion: The study of ganglion cell distribution enabled the creation of a simplified method for reading the slides. The method applied achieved good accuracy and it can be used as an alternative method in HD diagnosis.

ABSTRACT Purpose: To evaluate the caliber of an arterial micro-anastomosis in the young growing animal using a continuous suture technique. Additionally, late morphological changes and blood flows distal to the anastomosis were evaluated. Methods: Seventy-four Wistar rats were submitted to laparotomy to access the aorta for blood flow measurement. The aorta was sectioned using microsurgery technique and an end-to-end anastomosis with continuous suture. After a period of six months to one year, the anastomosis was checked. Results: Regarding the size of the aortas, comparing the pre- and postoperative values, there was an increase of 13.33% in adult animals and 25% in young animals, without any difference in the blood flows. Conclusions: The arteries of young rats show signs of growth at the site of the anastomosis performed with continuous suture.

RESUMO Objetivo: O tratamento cirúrgico das malformações anorretais (MAR) e da doença de Hirschsprung (DH) leva a alterações do hábito intestinal e incontinência fecal com prejuízo da qualidade de vida. Os objetivos foram criar e validar o Questionário para o Índice de Continência Fecal (ICF), baseado no Holschneider Criteria, bem como o Questionário para Avaliar a Qualidade de Vida Relativa à Continência Fecal em Crianças e Adolescentes (QQVCFCA), baseado no Fecal Incontinence Quality of Life. Métodos: Os questionários foram aplicados em 71 crianças operadas, em duas etapas. A validade foi testada por meio da comparação do QQVCFCA e um questionário genérico de qualidade de vida (SF-36) e entre o QQVCFCA e o ICF. Um grupo de 59 crianças normais foi usado como controle. Resultados: Nas duas etapas, 45,0% (32/71) e 42,8% (21/49) dos pacientes apresentaram incontinência fecal. Verificou-se que o QQVCFCA apresentou correlação significativa com o SF-36 e o ICF (correlação de Pearson 0,57) e mostrou que a qualidade de vida é diretamente proporcional à melhoria da continência fecal. A qualidade de vida no paciente com incontinência fecal está ainda comprometida globalmente, em comparação com os indivíduos controles (p<0,05; teste t de Student). Não houve ainda diferença significativa entre os resultados de crianças com MAR e crianças com DH. Conclusões: O QQVCFCA e o ICF são instrumentos úteis para a avaliação da qualidade de vida e da incontinência fecal nesses grupos de crianças. Crianças operadas de MAR e DH apresentam comprometimentos semelhantes da qualidade de vida.

Abstract Objective: Surgical treatment of anorectal malformations (ARMs) and Hirschsprung's disease (HD) leads to alterations in bowel habits and fecal incontinence, with consequent quality of life impairment. The objectives were to create and validate a Questionnaire for the Fecal Incontinence Index (FII) based on the Holschneider score, as well as a Questionnaire for the Assessment of Quality of Life Related to Fecal Incontinence in Children and Adolescents (QQVCFCA), based on the Fecal Incontinence Quality of Life. Methods: The questionnaires were applied to 71 children submitted to surgical procedure, in two stages. Validity was tested by comparing the QQVCFCA and a generic quality of life questionnaire (SF-36), and between QQVCFCA and the FII. A group of 59 normal children was used as control. Results: At two stages, 45.0% (32/71) and 42.8% (21/49) of the patients had fecal incontinence. It was observed that the QQVCFCA showed a significant correlation with the SF-36 and FII (Pearson's correlation 0.57), showing that the quality of life is directly proportional to improvement in fecal incontinence. Quality of life in patients with fecal incontinence is still globally impaired, when compared with control subjects (p<0.05, Student's t-test). There were also significant differences between the results of children with ARMs and children with HD. Conclusions: QQVCFCA and FII are useful tools to assess the quality of life and fecal incontinence in these groups of children. Children with ARMs submitted to surgical procedure and HD have similar quality of life impairment.

OBJECTIVE: Intestinal ischemia-reperfusion injury occurs in several clinical conditions and after intestinal transplantation. The aim of the present study was to investigate the phenomena of apoptosis and cell proliferation in a previously described intestinal ischemia-reperfusion injury autograft model using immunohistochemical markers. The molecular mechanisms involved in ischemia-reperfusion injury repair were also investigated by measuring the expression of the early activation genes c-fos and c-jun, which induce apoptosis and cell proliferation. MATERIALS AND METHODS: Thirty adult male Wistar rats were subjected to surgery for a previously described ischemia-reperfusion model that preserved the small intestine, the cecum and the ascending colon. Following reperfusion, the cecum was harvested at different time points as a representative segment of the intestine. The rats were allocated to the following four subgroups according to the reperfusion time: subgroup 1: 5 min; subgroup 2: 15 min; subgroup 3: 30 min; and subgroup 4: 60 min. A control group of cecum samples was also collected. The expression of c-fos, c-jun and immunohistochemical markers of cell proliferation and apoptosis (Ki67 and TUNEL, respectively) was studied. RESULTS: The expression of both c-fos and c-jun in the cecum was increased beginning at 5 min after ischemia-reperfusion compared with the control. The expression of c-fos began to increase at 5 min, peaked at 30 min, and exhibited a declining tendency at 60 min after reperfusion. A progressive increase in c-jun expression was observed. Immunohistochemical analyses confirmed these observations. CONCLUSION: The early activation of the c-fos and c-jun genes occurred after intestinal ischemia-reperfusion injury, and these genes can act together to trigger cell proliferation and apoptosis.

A aplasia de medula é uma das mais raras (<1%) e sérias complicações após o transplante hepático por insuficiência hepática aguda grave viral não A, não B e não C. Esta condição clínica, que acomete simultaneamente o tecido hepático e o hematopoético, foi descrita pela primeira vez em 1987, por Stock, e a fisiopatologia relacionada é uma condição imunomediada, provavelmente secundária à infecção viral desconhecida, e associada a grave prognóstico. A recuperação espontânea da aplasia medular adquirida habitualmente é muito rara e 50%-70% dos pacientes respondem ao tratamento imunossupressor com ciclosporina A (CsA) e glubulina antitimocítica (ATG), mesmo após o transplante hepático. Além do tratamento imunossupressor, outra opção é o transplante de medula óssea (TMO). Apresentamos o caso de uma criança com aplasia medular grave após transplante hepático, por insuficiência hepática aguda grave, que recebeu tratamento imunossupressor com CsA e ATG e evoluiu com recuperação completa das três séries do hemograma.

Aplastic anemia (AA) is one of the rarest (<1%) and most serious complications of liver transplantation for fulminant non-A, non-B and non-C hepatitis. It was first described in 1987 by Stock; the mechanism involved is an immunologically mediated condition secondary to an unknown viral infection. The disease is associated with a dismal prognosis. Spontaneous recovery from acquired AA is very rare however some patients (50-70%) recover after immunosuppressive therapy, such as Cyclosporin A (CsA) and Antithymocyte globulin (ATG), even after liver transplantation. Another treatment option is bone marrow transplantation. We report on a child who developed AA following liver transplantation for fulminant viral hepatitis that was treated with intensive immunosuppression including CsA and ATG and achieved complete recovery.