Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden

Sarah Jane Schwarzenberg; Melena Bellin; Sohail Z Husain; Monika Ahuja; Bradley Barth; Heather Davis; Peter R Durie; Douglas S Fishman; Steven D Freedman; Cheryl E Gariepy; Matthew J Giefer; Tanja Gonska; Melvin B Heyman; Ryan Himes; Soma Kumar; Veronique D Morinville; Mark E Lowe; Neil E Nuehring; Chee Y Ooi; John F Pohl; David Troendle; Steven L Werlin; Michael Wilschanski; Elizabeth Yen; Aliye Uc

doi:10.1016/j.jpeds.2014.11.019

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Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden

Journal article

Open access

Peer reviewed

Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden

Sarah Jane Schwarzenberg, Melena Bellin, Sohail Z Husain, Monika Ahuja, Bradley Barth, Heather Davis, Peter R Durie, Douglas S Fishman, Steven D Freedman, Cheryl E Gariepy, …

The Journal of pediatrics, Vol.166(4), pp.890-896.e1

04/2015

DOI: 10.1016/j.jpeds.2014.11.019

PMCID: PMC4380827

PMID: 25556020

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https://www.ncbi.nlm.nih.gov/pmc/articles/4380827View

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Abstract

To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis. 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. 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%). Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.

Mutation

United States - epidemiology

Genetic Predisposition to Disease

Prevalence

Cross-Sectional Studies

Humans

Pancreatitis, Chronic - diagnosis

Risk Factors

Pancreatitis, Chronic - epidemiology

Cholangiopancreatography, Endoscopic Retrograde

Male

Pancreatitis, Chronic - genetics

Incidence

DNA - genetics

DNA Mutational Analysis

Cystic Fibrosis Transmembrane Conductance Regulator - genetics

Female

Child

Details

Title: Subtitle: Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden
Creators: Sarah Jane Schwarzenberg - University of Minnesota Children's Hospital, Minneapolis, Minnesota, USA
Melena Bellin - University of Minnesota Children's Hospital, Minneapolis, Minnesota, USA
Sohail Z Husain - Children's Hospital of Pittsburgh, Pittsburgh, PA, USA
Monika Ahuja - University of Iowa Carver College of Medicine, Iowa City, IA, USA
Bradley Barth - University of Texas Southwestern Medical School, Dallas, TX, USA
Heather Davis - University of Iowa
Peter R Durie - Hospital for Sick Children, Toronto, ON, Canada
Douglas S Fishman - Baylor College of Medicine, Houston, TX, USA
Steven D Freedman - Harvard Medical School, Boston, MA, USA
Cheryl E Gariepy - Nationwide Children's Hospital, Columbus, OH, USA
Matthew J Giefer - Seattle Children's Hospital, Seattle, WA, USA
Tanja Gonska - Hospital for Sick Children, Toronto, ON, Canada
Melvin B Heyman - University of California at San Francisco, San Francisco, CA, USA
Ryan Himes - Baylor College of Medicine, Houston, TX, USA
Soma Kumar - Nationwide Children's Hospital, Columbus, OH, USA
Veronique D Morinville - Montreal Children's Hospital, McGill University, Montreal, QC, Canada
Mark E Lowe - Children's Hospital of Pittsburgh, Pittsburgh, PA, USA
Neil E Nuehring - University of Iowa Carver College of Medicine, Iowa City, IA, USA
Chee Y Ooi - Discipline of Paediatrics, School of Women's and Children's Health, Medicine, University of New South Wales and Sydney Children's Hospital Randwick Sydney, Australia
John F Pohl - University of Utah, Salt Lake City, UT, USA
David Troendle - University of Texas Southwestern Medical School, Dallas, TX, USA
Steven L Werlin - Medical College of Wisconsin, Milwaukee, WI, USA
Michael Wilschanski - Hadassah Hebrew University Hospital, Jerusalem, Israel
Elizabeth Yen - University of California at San Francisco, San Francisco, CA, USA
Aliye Uc - University of Iowa Carver College of Medicine, Iowa City, IA, USA
Resource Type: Journal article
Publication Details: The Journal of pediatrics, Vol.166(4), pp.890-896.e1
DOI: 10.1016/j.jpeds.2014.11.019
PMID: 25556020
PMCID: PMC4380827
NLM abbreviation: J Pediatr
ISSN: 0022-3476
eISSN: 1097-6833
Publisher: United States
Grant note: R01 DK097820 / NIDDK NIH HHS DK096327 / NIDDK NIH HHS UL1 TR000442 / NCATS NIH HHS R21 DK096327 / NIDDK NIH HHS P30 DK054759 / NIDDK NIH HHS
Language: English
Date published: 04/2015
Academic Unit: Stead Family Department of Pediatrics; Radiation Oncology; Gastroenterology, Hepatology, Pancreatology, and Nutrition
Record Identifier: 9984046925802771

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