Diagnosing Primary Sclerosing Cholangitis in Children

Abstract

Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease in children caused by chronic inflammatory process affecting either intrahepatic or extrahepatic bile ducts. Although it is infrequent, incidence is increasing worldwide, may be due to more awareness, understanding and expertise in managing children with chronic liver disease (CLD). In the developing world like Pakistan where resources and expertise are limited, very few tertiary centers are equipped to manage CLD in children. This case report is about a teenage child who presented to us with decompensated CLD in the form of jaundice and ascites. In our center, after a much needed workup he was commenced on appropriate treatment for PSC. After six months of treatment, he has managed to clear jaundice. The liver synthetic functions have improved with normal coagulation profile. His MELD (model for end-stage liver disease) score, which has come down from 19 to 9, delays liver transplant (LT) for years, which remains the best available treatment. MELD is a scoring system to assess the severity of CLD and remains an important tool to determine the outcome and ranking for receipt of an LT. Subsequently, he developed colitis and colonoscopy confirmed lymphocytic colitis (LC), which is a rare association of PSC.