Related Experiment Video
Updated: Mar 27, 2026
Bile Duct Ligation in Mice: Induction of Inflammatory Liver Injury and Fibrosis by Obstructive Cholestasis
Published on: February 10, 2015
Primary Sclerosing Cholangitis with Inflammatory Bowel Disease in Korean Children
Jisun Yoon1, Seak Hee Oh1, Hyun Jin Kim1
1Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.
Insights
In children, primary sclerosing cholangitis (PSC) is always associated with inflammatory bowel disease (IBD). This study highlights the significant incidence of PSC in pediatric IBD patients, emphasizing the need for concurrent management.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Autoimmune Diseases
Background:
- Primary sclerosing cholangitis (PSC) is a rare chronic liver disease often linked with inflammatory bowel disease (IBD).
- Understanding the association between PSC and IBD in pediatric populations is crucial for early diagnosis and management.
- Pediatric PSC presents unique challenges due to its rarity and potential long-term complications.
Purpose of the Study:
- To evaluate the incidence and clinical characteristics of PSC in children.
- To investigate the association between PSC and IBD in a pediatric cohort.
- To assess the long-term outcomes of pediatric patients with PSC and IBD.
Main Methods:
- Retrospective analysis of 13 pediatric patients (<18 years) diagnosed with PSC between 1989 and 2013.
- Evaluation of the incidence of PSC among 600 Crohn disease and 210 ulcerative colitis patients.
- Review of clinical findings, disease progression, and long-term outcomes.
Main Results:
- All 13 pediatric PSC patients had concurrent IBD (12 ulcerative colitis, 1 Crohn disease).
- The cumulative incidence of PSC was 5.7% in ulcerative colitis and 0.2% in Crohn disease.
- Over a median follow-up of 77.3 months, 15.4% developed liver cirrhosis, and 15.4% developed colorectal cancer; no cholangiocarcinoma was observed.
Conclusions:
- Pediatric PSC is consistently associated with IBD, with a notable incidence in ulcerative colitis.
- The findings suggest PSC is not as rare in children with IBD as previously thought.
- Clinicians should consider PSC in pediatric IBD patients and IBD in pediatric PSC patients for comprehensive care.
Purpose:
Primary sclerosing cholangitis (PSC) is a rare condition that can be associated with inflammatory bowel disease (IBD). The aim of this study was to evaluate PSC and its association with IBD in children.
Methods:
We retrospectively enrolled 13 pediatric patients (<18 years) with PSC treated at Asan Medical Center between June 1989 and December 2013. Clinical findings and long-term outcomes were investigated. During the same period, the incidence of PSC among IBD patients was evaluated among 600 Crohn disease (CD) and 210 ulcerative colitis (UC) patients.
Results:
All 13 study patients diagnosed with PSC also presented with IBD. Eleven boys and two girls with a median age of 15.0 years old (9.0-17.8 years) were included. The cumulative incidence of PSC for UC was 5.7% (12 of 210) and 0.2% for CD (1 of 600), respectively. PSC occurred during follow-up for IBD for five patients (38.5%) whereas, IBD developed during follow-up for PSC for two patients (15.4%), and was diagnosed during the initial work-up for PSC for 6 patients (46.2%). For the 77.3 month median follow-up period, 9/13 patients (69.2%), neither the clinical symptoms nor blood test results worsened. Two cases (15.4%) developed liver cirrhosis and underwent liver transplantation. Among 13 PSC patients with IBD, two (15.4%) developed colorectal cancer, and no one developed cholangiocarcinoma.
Conclusion:
All patients with PSC in this study had associated IBD. The incidence of PSC was not rare compared to reports in adults. PSC should be considered during the management of IBD and vice versa in children.
Related Concept Videos
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...
01:30Inflammatory Bowel Disease II: Crohn's Disease
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
01:27Inflammatory Bowel Disease I: Ulcerative Colitis
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
01:21Inflammatory Bowel Disease V: Surgical Management
Here are some common surgical interventions for IBD:
01:30Inflammatory Bowel Disease III: Diagnostic Studies and Management I-Nutritional Therapy
Diagnostic studies
A colonoscopy is the definitive screening test, distinguishing ulcerative colitis from other colon diseases with similar symptoms. During a colonoscopy test, inflamed mucosa with exudate ulcerations can be observed, and biopsies are taken to determine the histologic characteristics of the...