Acute pancreatitis (AP) is increasingly recognized in children, with an incidence approaching that of adults. Most children with pancreatitis have a single, mild, acute episode that resolves without complications. However, a subset of children with AP develops recurrent episodes (defined as acute recurrent pancreatitis or ARP) and some progress to chronic pancreatitis (CP). Few studies have been performed to characterize ARP and CP in children. We recognized that the lack of a well-phenotyped pediatric cohort presented a major obstacle in understanding the pathophysiology of CP and its sequelae. To address this knowledge gap, we have formed INSPPIRE (INternational Study Group of Pediatric Pancreatitis: In search for a cuRE), composed of 18 participating sites. Results from 301 pediatric patients enrolled into our study revealed a high frequency of pancreatitis-associated gene mutations and substantial disease burden in children with CP. The objective of this application is to determine the natural history of pediatric CP, identify risk factors and genetic modifiers for its onset and sequelae and develop approaches to improve clinical outcomes. The overall hypothesis of this proposal is that pediatric CP has unique characteristics that determine disease onset and sequelae.
Our specific aims are: 1) Characterize pediatric CP, determine predictors of disease severity and progression from ARP to CP; 2) Define the role of genetic mutations in early onset CP and identify disease modifiers; 3) Determine the efficacy of early pain management using cognitive-behavioral intervention on pediatric CP. Our long-term goal is to develop diagnostic modalities, prognostic factors and innovative treatment approaches for pediatric ARP and CP.

Public Health Relevance

Chronic pancreatitis is an emerging, but poorly understood condition in children that carries a significant socioeconomic burden. The objective of this application is to determine the natural history of pediatric chronic pancreatitis, identify risk factors and genetic modifiers for its onset and sequelae and develop approaches to improve clinical outcomes. Our long-term goal is to develop diagnostic modalities, prognostic factors and better treatment approaches for pediatric chronic pancreatitis.

Agency
National Institute of Health (NIH)
Institute
National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Type
Research Project--Cooperative Agreements (U01)
Project #
5U01DK108334-04
Application #
9566165
Study Section
Special Emphasis Panel (ZDK1)
Program Officer
Unalp-Arida, Aynur
Project Start
2015-09-28
Project End
2020-08-31
Budget Start
2018-09-01
Budget End
2019-08-31
Support Year
4
Fiscal Year
2018
Total Cost
Indirect Cost
Name
University of Iowa
Department
Pediatrics
Type
Schools of Medicine
DUNS #
062761671
City
Iowa City
State
IA
Country
United States
Zip Code
52242
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Abu-El-Haija, Maisam; Uc, Aliye; Werlin, Steven L et al. (2018) Nutritional Considerations in Pediatric Pancreatitis: A Position Paper from the NASPHAN Pancreas Committee and ESPHAN Cystic Fibrosis/Pancreas Working Group. J Pediatr Gastroenterol Nutr 67:131-143
Scheers, Isabelle; Palermo, Joseph J; Freedman, Steven et al. (2018) Recommendations for Diagnosis and Management of Autoimmune Pancreatitis in Childhood: Consensus From INSPPIRE. J Pediatr Gastroenterol Nutr 67:232-236
Uc, Aliye; Perito, Emily R; Pohl, John F et al. (2018) INternational Study Group of Pediatric Pancreatitis: In Search for a CuRE Cohort Study: Design and Rationale for INSPPIRE 2 From the Consortium for the Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer. Pancreas 47:1222-1228

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