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PubMed 25556020


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Title: Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden.

Authors: Sarah Jane Schwarzenberg, Melena Bellin, Sohail Z Husain, Monika Ahuja, Bradley Barth, Heather Davis, Peter R Durie, Douglas S Fishman, Steven D Freedman, Cheryl E Gariepy, Matthew J Giefer, Tanja Gonska, Melvin B Heyman, Ryan Himes, Soma Kumar, Veronique D Morinville, Mark E Lowe, Neil E Nuehring, Chee Y Ooi, John F Pohl, David Troendle, Steven L Werlin, Michael Wilschanski, Elizabeth Yen, Aliye Uc

Journal, date & volume: J. Pediatr., 2015 Apr , 166, 890-6.e1

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/25556020


Abstract
To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis.We performed a cross-sectional study of data from the International Study Group of Pediatric Pancreatitis: In Search for a Cure, a registry of children with acute recurrent pancreatitis and chronic pancreatitis. Between-group differences were compared using Wilcoxon rank-sum test.Among 170 subjects in the registry, 76 (45%) had chronic pancreatitis; 57% were female, 80% were white; median age at diagnosis was 9.9 years. Pancreatitis-predisposing genetic mutations were identified in 51 (67%) and obstructive risk factors in 25 (33%). Toxic/metabolic and autoimmune factors were uncommon. Imaging demonstrated ductal abnormalities and pancreatic atrophy more commonly than calcifications. Fifty-nine (77%) reported abdominal pain within the past year; pain was reported as constant and receiving narcotics in 28%. Children with chronic pancreatitis reported a median of 3 emergency department visits and 2 hospitalizations in the last year. Forty-seven subjects (70%) missed 1 day of school in the past month as the result of chronic pancreatitis; 26 (34%) missed 3 or more days. Children reporting constant pain were more likely to miss school (P = .002), visit the emergency department (P = .01), and experience hospitalizations (P = .03) compared with children with episodic pain. Thirty-three children (43%) underwent therapeutic endoscopic retrograde pancreatography; one or more pancreatic surgeries were performed in 30 (39%).Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.