Analysis of INSPPIRE-2 Cohort: Risk Factors and Disease Burden in Children with Acute Recurrent or Chronic Pancreatitis

Aliye Uc, Gretchen A. Cress, Fuchenchu Wang, Maisam Abu-El-Haija, Kate M. Ellery, Douglas S. Fishman, Cheryl E. Gariepy, Tanja Gonska, Tom K. Lin, Quin Y. Liu, Megha Mehta, Asim Maqbool, Brian A. McFerron, Veronique D. Morinville, Chee Y. Ooi, Emily R. Perito, Sarah Jane Schwarzenberg, Zachary M. Sellers, Jose Serrano, Uzma ShahDavid M. Troendle, Michael Wilschanski, Yuhua Zheng, Ying Yuan, Mark E. Lowe

Research output: Contribution to journalArticlepeer-review

5 Scopus citations


Objectives: The objective of this study is to investigate risk factors and disease burden in pediatric acute recurrent pancreatitis (ARP) and chronic pancreatitis (CP). Methods: Data were obtained from INternational Study group of Pediatric Pancreatitis: In search for a cuRE-2 (INSPPIRE-2), the largest multi-center prospective cohort study in pediatric patients with ARP or CP. Results: Of 689 children, 365 had ARP (53%), 324 had CP (47%). CP was more commonly associated with female sex, younger age at first acute pancreatitis (AP) attack, Asian race, family history of CP, lower BMI%, genetic and obstructive factors, PRSS1 mutations and pancreas divisum. CFTR mutations, toxic-metabolic factors, medication use, hypertriglyceridemia, Crohn disease were more common in children with ARP. Constant or frequent abdominal pain, emergency room (ER) visits, hospitalizations, medical, endoscopic or surgical therapies were significantly more common in CP, episodic pain in ARP. A total of 33.1% of children with CP had exocrine pancreatic insufficiency (EPI), 8.7% had diabetes mellitus. Compared to boys, girls were more likely to report pain impacting socialization and school, medical therapies, cholecystectomy, but no increased opioid use. There was no difference in race, ethnicity, age at first AP episode, age at CP diagnosis, duration of disease, risk factors, prevalence of EPI or diabetes between boys and girls. Multivariate analysis revealed that family history of CP, constant pain, obstructive risk factors were predictors of CP. Conclusions: Children with family history of CP, constant pain, or obstructive risk factors should raise suspicion for CP.

Original languageEnglish (US)
Pages (from-to)643-649
Number of pages7
JournalJournal of pediatric gastroenterology and nutrition
Issue number5
StatePublished - Nov 1 2022

Bibliographical note

Funding Information:
Sources of Funding: Research reported in this publication was supported by National Institute of Diabetes and Digestive and Kidney Diseases and National Cancer Institute of the National Institutes of Health under award numbers 2U01 DK108334, 2U01 DK108328, R01 DK118752. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

Funding Information:
M.E.L. is on the Board of Directors of the National Pancreas Association and receives royalties from Millipore Inc and UpToDate. T.G. received a research grant from Vertex Pharmaceuticals, and she is a consultant for Cystic Fibrosis Foundation (CFF). A.U. is a member of American Board of Pediatrics, Subboard of Pediatric Gastroenterology, Associate Editor of Pancreatology, and consultant for CFF. S.J.S. is a consultant for UpToDate, Nestle, Abbvie, and the Cystic Fibrosis Foundation, and she has a grant from Gilead. V.D.M. is an Associate Editor for JPGN Reports. The remaining authors report no conflicts of interest.

Publisher Copyright:
© 2022 Lippincott Williams and Wilkins. All rights reserved.


  • acute recurrent pancreatitis
  • children
  • chronic pancreatitis
  • diabetes
  • exocrine pancreatic insufficiency


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