TY - JOUR
T1 - Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden
AU - Schwarzenberg, Sarah Jane
AU - Bellin, Melena
AU - Husain, Sohail Z.
AU - Ahuja, Monika
AU - Barth, Bradley
AU - Davis, Heather
AU - Durie, Peter R.
AU - Fishman, Douglas S.
AU - Freedman, Steven D.
AU - Gariepy, Cheryl E.
AU - Giefer, Matthew J.
AU - Gonska, Tanja
AU - Heyman, Melvin B.
AU - Himes, Ryan
AU - Kumar, Soma
AU - Morinville, Veronique D.
AU - Lowe, Mark E.
AU - Nuehring, Neil E.
AU - Ooi, Chee Y.
AU - Pohl, John F.
AU - Troendle, David
AU - Werlin, Steven L.
AU - Wilschanski, Michael
AU - Yen, Elizabeth
AU - Uc, Aliye
N1 - Publisher Copyright:
Copyright © 2015 Elsevier Inc. All rights reserved.
PY - 2015
Y1 - 2015
N2 - Objective To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis. Study design 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. Results 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%). Conclusions Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.
AB - Objective To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis. Study design 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. Results 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%). Conclusions Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.
UR - http://www.scopus.com/inward/record.url?scp=84933041348&partnerID=8YFLogxK
U2 - 10.1016/j.jpeds.2014.11.019
DO - 10.1016/j.jpeds.2014.11.019
M3 - Article
C2 - 25556020
AN - SCOPUS:84933041348
VL - 166
SP - 890-896.e1
JO - Journal of Pediatrics
JF - Journal of Pediatrics
SN - 0022-3476
IS - 4
ER -