Current problems of sclerosing cholangitis in children
https://doi.org/10.31146/1682-8658-ecg-231-11-184-193
Abstract
Sclerosing cholangitis (SC) is a rare chronic disease characterized by inflammation and progressive obliterative fibrosis of the intrahepatic and/or extrahepatic bile ducts. Diagnosis is based on a cholangiogram showing dilatation of the bile ducts, narrowing and obliteration of the biliary tree, and histologically the presence of inflammatory lesions of the bile ducts leading to periductal fibrosis. In children, the most common SC is associated with prominent autoimmune manifestations, overlapping with those of autoimmune hepatitis (AIH); this form is known as autoimmune sclerosing cholangitis, ASC. Conversely, primary SC (PSC), a condition in which the term “primary” indicates that the etiology and pathogenesis are unknown, is rare in pediatrics. Secondary SC (SSC) defines cholangiopathy associated with an identifiable etiology, such as immunodeficiency, infection, or hematologic disorder. ASC and PSC are closely associated with inflammatory bowel diseases (IBD). ASC responds biochemically well to immunosuppressants and ursodeoxycholic acid (UDCA). Primary forms are treated exclusively with oral UDCA, whereas in secondary forms, drug treatment depends on the underlying etiology. Despite treatment, SC often progresses to biliary cirrhosis and end-stage liver disease requiring liver transplantation. The disease may recur after transplantation. To improve the prognosis of this disabling liver disease, a better understanding of the pathogenetic mechanisms and improved treatment methods are needed.
About the Authors
G. V. Volynets
Veltischev Research and Clinical Institute for pediatrics and pediatric surgery at the Pirogov Russian National Research Medical University; N. I. Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
A. S. Potapov
National Medical Research Center for Children’s Health; Sechenov University
Russian Federation
Russian Federation
A. I. Khavkin
Research Clinical Institute of Childhood, Ministry of Health of the Moscow Region
Russian Federation
Russian Federation
A. V. Nikitin
Veltischev Research and Clinical Institute for pediatrics and pediatric surgery at the Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
T. A. Skvortsova
Veltischev Research and Clinical Institute for pediatrics and pediatric surgery at the Pirogov Russian National Research Medical University
Russian Federation
Russian Federation
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Volynets G.V., Potapov A.S., Khavkin A.I., Nikitin A.V., Skvortsova T.A. Current problems of sclerosing cholangitis in children. Experimental and Clinical Gastroenterology. 2024;(11):184-193. (In Russ.) https://doi.org/10.31146/1682-8658-ecg-231-11-184-193
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