Date: | 2017 Jul |
PMID: | |
Category: | 2 |
Authors: | Matthew J Giefer 1, Mark E Lowe 2, Steven L Werlin 3, Bridget Zimmerman 4, Michael Wilschanski 5, David Troendle 6, Sarah Jane Schwarzenberg 7, John F Pohl 8, Joseph Palermo 9, Chee Y Ooi 10, Veronique D Morinville 11, Tom K Lin 9, Sohail Z Husain 2, Ryan Himes 12, Melvin B Heyman 13, Tanja Gonska 14, Cheryl E Gariepy 15, Steven D Freedman 16, Douglas S Fishman 12, Melena D Bellin 7, Bradley Barth 6, Maisam Abu-El-Haija 9, Aliye Uc 17 |
Abstract: |
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Objectives: To assess whether the age of onset was associated with unique features or disease course in pediatric acute recurrent pancreatitis (ARP) or chronic pancreatitis (CP).
Study Design: Demographic and clinical information on children with ARP or CP was collected at INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE) centers. The Cochran-Armitage trend test and Jonckheere-Terpstra test were used to examine for differences between pediatric age groups (<6, 6-11, and ≥12 years).
Results: Between September 2012 and March 2016, 342 children with ARP or CP were enrolled; 129 (38%) were <6 years of age at the time of first diagnosis of acute pancreatitis, 111 (32%) were 6-11 years of age, and 102 (30%) were ≥12 years of age. Early-onset disease was associated with mutations in cationic trypsinogen (PRSS1) (P < .01), chymotrypsin C (CTRC) (P = .01), family history of acute pancreatitis (P = .02), family history of CP (P < .01), biliary cysts (P = .04), or chronic renal failure (P = .02). Later-onset disease was more commonly present with hypertriglyceridemia (P = .04), ulcerative colitis (P = .02), autoimmune diseases (P < .0001), or medication use (P < .01). Children with later-onset disease also were more likely to visit the emergency department (P < .05) or have diabetes (P < .01).
Conclusions: Early-onset pancreatitis is associated strongly with PRSS1 or CTRC mutations and family history of pancreatitis. Children with later-onset disease are more likely to have nongenetic risk factors. Future studies are needed to investigate whether the disease course, response to therapy, or clinical outcomes differ relative to the timing of disease onset.
Acknowledgements:
The content of this article is solely the responsibility of the authors and does not necessarily represent the official views of the National Cancer Institute, the National Institute of Health, or the National Institute of Diabetes and Digestive and Kidney Diseases.
The Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer (CPDPC) Research Consortia is supported and funded by grants from the National Cancer Institute and the National Institute of Diabetes and Digestive and Kidney Diseases under the following award numbers:
Project Number: | Awardee Organization |
U01DK108326 | Baylor College of Medicine |
U01DK108314 | Cedars-Sinai Medical Center |
U01DK108332 | Indiana University |
U01DK108323 | Kaiser Foundation Research Institute |
U01DK108288 | Mayo Clinic |
U01DK108327 | Ohio State University |
U01DK108300 | Stanford University |
U01DK108320 | University of Florida |
U01DK108306 | University of Pittsburgh |
U01DK108328 | University of Texas MD Anderson Cancer Center |
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