ABSTRACT
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.
