Clinical profile and treatment outcome of chronic pancreatitis in children: a long-term follow-up study of 156 cases

Abstract

Aim: There is a paucity of literature in pediatric chronic pancreatitis (CP) and most information is derived from adult literature. We, therefore, analyzed our experience of CP to look for clinical profile and long-term outcome.

Methods: From January 2003 to December 2015, 156 consecutive children (≤18 years) diagnosed as CP were included. Their clinical profile, management, and follow-up data were retrieved. Genetic markers (PRSS1, SPINK1, and CFTR) were studied in 40 idiopathic cases.

Results: The median age of the patients was 13 [inter-quartile range (IQR): 10–14] years (93 males) and 134 (86%) were idiopathic. Genetic mutations were found in 22/40 (55%) idiopathic cases. All but two presented with pain abdomen (episodic pain in 93.6%) and symptom duration was 12 (IQR: 6–24) months. There were two subsets; calcific (CCP) 68 (43.5%) and non-calcific (NCCP) 88 (56.5%). In CCP group, significantly more children had Cambridge grade 5 magnetic resonance cholangiopancreatography changes, low weight Z-score, and had continuous pain more compared to NCCP group. Over a median follow-up of 23 (IQR: 8–45.5) months, more children in CCP group had complications. Endoscopic therapy (done for persistent pain in 40) relieved pain in 52.5% of cases while medical therapy did so in 36% of cases.

Conclusion: Pediatric CP in Asia presents with episodic pain and genetic predisposition seems to be a major cause. There are two subsets; CCP and NCCP with former showing marked imaging changes, more often associated with malnutrition and complications. Endoscopic therapy for pain relief gives modest benefit but medical therapy is not encouraging.

Keywords:

• Idiopathic
• genetic mutations
• chronic-calcific
• malnutrition
• endoscopic therapy

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.