tailieunhanh - Diseases of the Gallbladder and Bile Ducts - part 2

nhìn thấy trong cả hai cản trở và PSC. Tuy nhiên, tắc nghẽn ống dẫn lớn từ các nguyên nhân khác, thay đổi sự mất mát của các ống dẫn mật interlobular và teo trong biểu mô ống động mạch không xảy ra | 34 Section 1 Anatomy pathophysiology and epidemiology of the biliary system Table Staging of primary sclerosing cholangitis. Source Wiesner et al. 55 . Stage Designation Features 1 Portal Ductabnormalities 2 Periportal Ductular proliferation 3 Septal Bridging fibrous septa 4 Cirrhosis Nodular architecture seen in both obstruction and PSC. However in large duct obstruction from other causes loss of interlobular bile ducts and atrophic changes in ductal epithelium do not occur. The presence of numerous eosinophils in the portal inflammatory infiltrate also favors PSC. In pediatric patients with PSC overlap of clinical and histopathologic features with autoimmune hepatitis may occur 56 . Although alkaline phosphatase is usually elevated in adults with PSC normal alkaline phosphatase levels may be seen in children with the disease in one study of 32 children with PSC 15 had normal alkaline phosphatase levels at presentation 56 . Most pediatric patients with PSC will also have ulcerative colitis 55 although this figure is less than the commonly quoted 70 in adults. The cholangiogram may show very subtle irregularity of bile ducts without overt stricture formation and predominance of intrahepatic disease is common in childhood PSC. Concentric periductal fibrosis is rarely seen in biopsies from children instead the most notable feature is the loss of interlobular bile ducts which often seem to vanish without a trace. The portal tracts may contain a dense mononuclear inflammatory infiltrate with piecemeal necrosis and scattered plasma cells further resembling autoimmune hepatitis. A high index of suspicion on the part of the gastroenterologist and the pathologist is often necessary to make the diagnosis of PSC in the pediatric patient. Secondary sclerosing cholangiopathies Other causes of biliary strictures are intrahepaticartery chemotherapy immunodeficiency syndromes and Langerhans cell histiocytosis. Hepatic artery infusion of floxuridine for treatment of hepatic .