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Sclerosing cholangitis in children with inflammatory bowel disease
J C Ong1, E V O'Loughlin, K R Kamath
1Department of Gastroenterology, Royal Alexandra Hospital for Children, Sydney, NSW.
Insights
Primary sclerosing cholangitis (PSC) in children with inflammatory bowel disease (IBD) is rare. Yearly liver function tests in IBD patients can help detect PSC early.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Autoimmune Diseases
Background:
- Primary sclerosing cholangitis (PSC) is infrequently diagnosed in pediatric patients with inflammatory bowel disease (IBD).
- This study focuses on characterizing PSC in a pediatric cohort with co-existing IBD.
Observation:
- Four pediatric patients with IBD presented with subtle liver disease symptoms.
- Abnormal liver function tests, including elevated transaminases and alkaline phosphatase, were noted in all patients.
- Radiological and histological findings revealed bile duct anomalies consistent with PSC.
Findings:
- Three patients exhibited typical "onion skin" bile duct fibrosis and cholangiographic abnormalities.
- One patient with normal cholangiography was diagnosed with small duct PSC based on liver biopsy.
- Early stages of PSC in children with IBD may present with minimal clinical signs.
Implications:
- Routine annual liver function testing is recommended for all children diagnosed with IBD.
- Abnormal liver tests in pediatric IBD patients warrant investigation for PSC.
- Early detection and diagnosis of PSC in children can be achieved through biochemical monitoring, liver biopsy, and cholangiography.
Background:
Primary sclerosing cholangitis (PSC) with inflammatory bowel disease (IBD) has been rarely reported in children.
Aim:
To describe the clinical presentation, sequential liver function test abnormalities, radiological bile duct anomalies and liver histology in four children with PSC and IBD.
Methods:
Over a period of 18 years, four of 130 patients with IBD developed abnormal liver function tests. Three of the four patients had ulcerative colitis and the other Crohn's disease. All four patients had baseline and follow-up liver function tests, percutaneous transhepatic cholangiography and a needle biopsy of the liver.
Results:
The four patients at presentation had minimal symptoms or signs of liver disease. All had elevation of serum transaminases, gamma glutamyl transferase and/or alkaline phosphatase. Three had the typical onion skin fibrosis of bile ducts. Percutaneous transhepatic cholangiography demonstrated irregularity and beading of the hepatic and common bile ducts in three patients. The other with normal cholangiography had fibrosing cholangitis on liver biopsy and was considered to have small duct disease.
Conclusions:
We conclude that yearly biochemical assessment of liver function should be performed on all children with IBD, and if abnormal should raise the suspicion of PSC. The latter diagnosis can be confirmed by liver biopsy and cholangiography.