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Chronic Pancreatitis

Pediatric and Adult Cohorts Show Similarities in Disease Progress Despite Different Risk Factors

Schwarzenberg, Sarah J.*; Uc, Aliye; Zimmerman, Bridget; Wilschanski, Michael§; Wilcox, C. Mel||; Whitcomb, David C.¶,#,**,††; Werlin, Steven L.‡‡; Troendle, David§§; Tang, Gong||||; Slivka, Adam; Singh, Vikesh K.¶¶; Sherman, Stuart##; Shah, Uzma***; Sandhu, Bimaljit S.†††; Romagnuolo, Joseph††; Rhee, Sue‡‡‡; Pohl, John F.§§§; Perito, Emily R.‡‡‡; Ooi, Chee Y.||||||; Nathan, Jaimie D.¶¶¶; Muniraj, Thiruvengadam###; Morinville, Veronique D.****; McFerron, Brian††††; Mascarenhas, Maria‡‡‡‡; Maqbool, Asim‡‡‡‡; Liu, Quin§§§§; Lin, Tom K.¶¶¶; Lewis, Michele||||||||; Husain, Sohail Z.¶¶¶¶; Himes, Ryan####; Heyman, Melvin B.‡‡‡; Guda, Nalini*****; Gonska, Tanja†††††; Giefer, Matthew J.‡‡‡‡‡; Gelrud, Andres§§§§§; Gariepy, Cheryl E.||||||||||; Gardner, Timothy B.¶¶¶¶¶; Freedman, Steven D.#####; Forsmark, Christopher E.******,††††††; Fishman, Douglas S.####; Cote, Gregory A.*****; Conwell, Darwin‡‡‡‡‡‡; Brand, Randall E.; Bellin, Melena*; Barth, Bradley§§; Banks, Peter A.§§§§§§; Anderson, Michelle A.||||||||||||; Amann, Stephen T.¶¶¶¶¶¶; Alkaade, Samer######; Abu-El-Haija, Maisam¶¶¶; Abberbock, Judah N.||||; Lowe, Mark E.*******; Yadav, Dhiraj

Journal of Pediatric Gastroenterology and Nutrition: April 2019 - Volume 68 - Issue 4 - p 566–573
doi: 10.1097/MPG.0000000000002279
Original Articles: Pancreatology

Objectives: The aim of the present study was to investigate the natural history of chronic pancreatitis (CP); patients in the North American Pancreatitis Study2 (NAPS2, adults) and INternational Study group of Pediatric Pancreatitis: In search for a cuRE (INSPPIRE, pediatric) were compared.

Methods: Demographics, risk factors, disease duration, management and outcomes of 224 children and 1063 adults were compared using appropriate statistical tests for categorical and continuous variables.

Results: Alcohol was a risk in 53% of adults and 1% of children (P < 0.0001); tobacco in 50% of adults and 7% of children (P < 0.0001). Obstructive factors were more common in children (29% vs 19% in adults, P = 0.001). Genetic risk factors were found more often in children. Exocrine pancreatic insufficiency was similar (children 26% vs adult 33%, P = 0.107). Diabetes was more common in adults than children (36% vs 4% respectively, P < 0.0001). Median emergency room visits, hospitalizations, and missed days of work/school were similar across the cohorts. As a secondary analysis, NAPS2 subjects with childhood onset (NAPS2-CO) were compared with INSPPIRE subjects. These 2 cohorts were more similar than the total INSPPIRE and NAPS2 cohorts, including for genetic risk factors. The only risk factor significantly more common in the NAPS2-CO cohort compared with the INSPPIRE cohort was alcohol (9% NAPS2-CO vs 1% INSPPIRE cohorts, P = 0.011).

Conclusions: Despite disparity in age of onset, children and adults with CP exhibit similarity in demographics, CP treatment, and pain. Differences between groups in radiographic findings and diabetes prevalence may be related to differences in risk factors associated with disease and length of time of CP.

*University of Minnesota Masonic Children's Hospital, Minneapolis, MN

University of Iowa, Stead Family Children's Hospital

Department of Biostatistics, University of Iowa, Iowa City, IA

§Hadassah Hebrew University Hospital, Jerusalem, Israel

||Department of Medicine, University of Alabama Birmingham, Birmingham, AL

Department of Medicine

#Department of Cell Biology & Physiology

**Department of Human Genetics, University of Pittsburgh, Pittsburgh, PA

††Palmetto Health, Columbia Gastroenterology Associates, Columbia, SC

‡‡Medical College of Wisconsin, Milwaukee, WI

§§University of Texas Southwestern Medical School, Dallas, TX

||||Department of Biostatistics, Graduate School of Public Health, University of Pittsburgh, Pittsburgh, PA

¶¶Department of Medicine, Johns Hopkins Medical Institutions, Baltimore, MD

##Department of Medicine, Indiana University, Indianapolis, IN

***Massachusetts General Hospital for Children, Harvard Medical School, Boston, MA

†††Richmond Gastroenterology Associates, St. Mary's Hospital, Richmond, VA

‡‡‡University of California San Francisco Benioff Children's Hospital, San Francisco, CA

§§§University of Utah, Salt Lake City, UT

||||||School of Women's and Children's Health, Medicine, University of New South Wales and Sydney Children's Hospital Randwick Sydney, Australia

¶¶¶Cincinnati Children's Hospital Medical Center, Cincinnati, OH

###Yale University, New Haven, CT

****Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada

††††Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN

‡‡‡‡Children's Hospital of Philadelphia, Philadelphia, PA

§§§§Cedars-Sinai Medical Center, Los Angeles, CA

||||||||Department of Medicine, Mayo Clinic, Jacksonville, FL

¶¶¶¶Children's Hospital of Pittsburgh, Pittsburgh, PA

####Baylor College of Medicine, Houston, TX

*****Aurora Health Care, St. Luke's Medical Center, Milwaukee, WI

†††††Hospital for Sick Children, Toronto, Ontario, Canada

‡‡‡‡‡Seattle Children's Hospital, Seattle, WA

§§§§§GastroHealth, Miami, FL

||||||||||Nationwide Children's Hospital, Columbus, OH

¶¶¶¶¶Department of Medicine, Dartmouth Hitchcock Medical Center, Lebanon, NH

#####Harvard Medical School, Boston, MA

******Department of Medicine, University of Florida, Gainesville, FL

††††††Department of Medicine, Medical University of South Carolina, Charleston, SC

‡‡‡‡‡‡Department of Medicine, Ohio State University, Columbus, OH

§§§§§§Department of Medicine, Brigham and Women's Hospital, Boston, MA

||||||||||||Department of Medicine, University of Michigan, Ann Arbor, MI

¶¶¶¶¶¶Digestive Health Specialists, Tupelo, MS

######Department of Medicine, Saint Louis University, St. Louis, MO

*******Washington University School of Medicine; St. Louis, MO.

Address correspondence and reprint requests to Sarah J. Schwarzenberg, MD, University of Minnesota Masonic Children's Hospital, East Building, 6th Floor 2450 Riverside Avenue Minneapolis, MN 55454 (e-mail:

Received 2 November, 2018

Accepted 29 December, 2018

Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (

NAPS2: This research was supported by the National Institute of Health under award numbers DK061451 (D.C.W.); DK077906 (D.Y.), UO1 DK108327 (D.C.), UO1 DK108320 (C.E.F.), U01 DK108306 (D.C.W., D.Y.), and UL1 RR024153 and UL1TR000005 (PI—S.E.R.), INSPPIRE (R21 DK096327, U01 DK108334, R01 DK097820), CTSA (2UL1 TR000442), and National Pancreas Foundation (A.U.).

The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.

M.L. is on the Board of Directors of the National Pancreas Foundation; he receives royalties from Millipore Inc and UpToDate. T.G. and M.W. received a research grant from Vertex Pharmaceuticals. S.H. owns equity in PrevCon. J.P. is on the speaker's bureau for Medical Education Resources, Inc.; M.B. is a consultant for AbbVie Inc and ARIEL Precision Medicine. S.J.S. is a consultant for AbbVie, Inc. A.U. is a member of American Board of Pediatrics, Subboard of Pediatric Gastroenterology. The other authors declare no conflicts of interest.

Mark E. Lowe and Dhiraj Yadav contributed equally to the manuscript.

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,