Related Experiment Video
Updated: Dec 16, 2025
Isolation of Neonatal Extrahepatic Cholangiocytes
Published on: June 5, 2014
Diagnosing Primary Sclerosing Cholangitis in Children
Iqtadar Seerat1, Muhammad Atique2, Eitzaz Ud Din Khan3
1Pediatric Gastroenterology & Hepatology, Pakistan Kidney and Liver Institute and Research Center, Lahore, PAK.
Insights
Primary sclerosing cholangitis (PSC) in children is a rare, increasing liver disease. Early treatment improved jaundice and liver function, delaying the need for liver transplant.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Autoimmune Diseases
Background:
- Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease in children.
- Its incidence is increasing globally, potentially due to enhanced awareness and diagnostic capabilities.
- Managing pediatric chronic liver disease (CLD) is challenging, especially in resource-limited settings.
Observation:
- A case report details a teenage patient presenting with decompensated CLD, including jaundice and ascites.
- The patient received treatment for PSC at a tertiary care center.
- Following treatment, jaundice resolved, and liver synthetic functions improved.
Findings:
- The patient's MELD score decreased significantly from 19 to 9, indicating improved CLD severity.
- This improvement potentially postpones the need for liver transplantation (LT), the current best treatment.
- The patient later developed colitis, diagnosed as lymphocytic colitis (LC), a rare association with PSC.
Implications:
- This case highlights the importance of timely diagnosis and treatment of PSC in pediatric patients.
- Effective management can improve clinical outcomes and delay LT.
- The rare co-occurrence of PSC and LC warrants further investigation into their potential relationship.
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.
Related Concept Videos
01:29Chronic Pancreatitis II: Collaborative Care
Assessment:
01:17Chronic Bowel Disorders: Introduction
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
01:26Endoscopic Procedures V: ERCP
Patient...