[Primary sclerosing cholangitis in children and adolescents]. / Colangite esclerosante primária em crianças e adolescentes.

Primary sclerosing cholangitis is a disease usually with a poor prognosis with a mean survival of 10-12 years, starting from the time of diagnosis. Among the different populations studied, males predominate in a ratio 2:1 with ages ranging from 20-78 (mean 42 years). This article describes four patients with this syndrome, a child of eight and three adolescents, thirteen and fourteen years old. It is possibly of autoimmune etiology, characterized by stenosis, dilatation and fibrosis involving the intrahepatic and extrahepatic biliary tree. We analyzed laboratory assays, clinical, immunological, laboratory assays, histological and cholangiographic aspects. In conclusion, in this age group perhaps of the small population sample there was no sex predominance, and 50% progressed without jaundice and without hepatomegaly. The serum levels of gamma glutamyltransferases and especially alkaline phosphatase were elevated compared to aminotransferases without increase of the serum auto antibodies. Only one patients displayed the typical histological appearance of primary sclerosing cholangitis. The diagnosis in al was confirmed by typical cholangiographic signs, restricted only to the intrahepatic biliary tree, identified in all those included in this study.

[Diffuse primary malignant mesothelioma in abdominal cavity]. / Mesotelioma maligno primário difuso em cavidade abdominal.

Two cases of diffuse malignant mesothelioma of abdominal cavity were analysed. These tumors arise from the peritoneum and are also found in the parietal and visceral pleura, pericardium and in vaginal tunic. All of them, infra or supra-diaphragmatic, are associated with asbestos exposure in at least 80% of cases. It is difficult to explain how inhaled asbestos induces peritoneal neoplasms. This aspects become very important in the diagnostic, basically why it is done at laparotomy or laparoscopy. When was proceed the biopsy of the lesions, and occasionally by identification of malignant mesothelial cells in ascitic fluid. In this two cases exposed considerations about the advanced phase of diagnostic are made, the diagnostic was performed in the majority of the collected cells, showing the advanced stage of the disease. At that time of diagnosis we observed poor evolution. We call attention to the importance of precancer diagnosis, the best chance to treatment options, always based on surgical resections, radiation or chemotherapy alone or combined. If the radical surgery is not possible, this patients must be treated by chemotherapy or radiotherapy, defined after complete staging of the disease.