Early-Onset Acute Recurrent and Chronic Pancreatitis Is Associated with PRSS1 or CTRC Gene Mutations

Matthew J Giefer; Mark E Lowe; Steven L Werlin; Bridget Zimmerman; Michael Wilschanski; David Troendle; Sarah Jane Schwarzenberg; John F Pohl; Joseph Palermo; Chee Y Ooi; Veronique D Morinville; Tom K Lin; Sohail Z Husain; Ryan Himes; Melvin B Heyman; Tanja Gonska; Cheryl E Gariepy; Steven D Freedman; Douglas S Fishman; Melena D Bellin; Bradley Barth; Maisam Abu-El-Haija; Aliye Uc

doi:10.1016/j.jpeds.2017.03.063

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Early-Onset Acute Recurrent and Chronic Pancreatitis Is Associated with PRSS1 or CTRC Gene Mutations

Journal article

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Early-Onset Acute Recurrent and Chronic Pancreatitis Is Associated with PRSS1 or CTRC Gene Mutations

Matthew J Giefer, Mark E Lowe, Steven L Werlin, Bridget Zimmerman, Michael Wilschanski, David Troendle, Sarah Jane Schwarzenberg, John F Pohl, Joseph Palermo, Chee Y Ooi, …

The Journal of pediatrics, Vol.186, pp.95-100

07/2017

DOI: 10.1016/j.jpeds.2017.03.063

PMCID: PMC5506853

PMID: 28502372

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Abstract

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). 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). 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). 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.

genetic

risk

children

young

pediatric

Details

Title: Subtitle: Early-Onset Acute Recurrent and Chronic Pancreatitis Is Associated with PRSS1 or CTRC Gene Mutations
Creators: Matthew J Giefer - Department of Pediatrics, Seattle Children's Hospital, Seattle, WA
Mark E Lowe - Department of Pediatrics, Children's Hospital, of Pittsburgh, Pittsburgh, PA
Steven L Werlin - Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI
Bridget Zimmerman - Department of Biostatistics, University of Iowa, College of Public Health, Iowa City, IA
Michael Wilschanski - Department of Pediatrics, Hadassah Hebrew University Hospital, Jerusalem, Israel
David Troendle - Department of Pediatrics, University of Texas Southwestern Medical School, Dallas, TX
Sarah Jane Schwarzenberg - Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, MN
John F Pohl - Department of Pediatrics, University of Utah, Salt Lake City, UT
Joseph Palermo - Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Chee Y Ooi - Department of Pediatrics, Sydney Children's Hospital, University of New South Wales, Sydney, Australia
Veronique D Morinville - Department of Pediatrics, Montreal Children's Hospital, McGill University, Montreal, Québec, Canada
Tom K Lin - Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Sohail Z Husain - Department of Pediatrics, Children's Hospital, of Pittsburgh, Pittsburgh, PA
Ryan Himes - Department of Pediatrics, Baylor College of Medicine, Houston, TX
Melvin B Heyman - Department of Pediatrics, University of California San Francisco, San Francisco, CA
Tanja Gonska - Department of Pediatrics, Hospital for Sick Children, Toronto, ON, Canada
Cheryl E Gariepy - Department of Pediatrics, Nationwide Children's Hospital, Columbus, OH
Steven D Freedman - Department of Pediatrics, Harvard Medical School, Boston, MA
Douglas S Fishman - Department of Pediatrics, Baylor College of Medicine, Houston, TX
Melena D Bellin - Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, MN
Bradley Barth - Department of Pediatrics, University of Texas Southwestern Medical School, Dallas, TX
Maisam Abu-El-Haija - Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Aliye Uc - University of Iowa Carver College of Medicine, Stead Family Department of Pediatrics, Iowa City, IA
Resource Type: Journal article
Publication Details: The Journal of pediatrics, Vol.186, pp.95-100
DOI: 10.1016/j.jpeds.2017.03.063
PMID: 28502372
PMCID: PMC5506853
NLM abbreviation: J Pediatr
ISSN: 0022-3476
eISSN: 1097-6833
Publisher: Elsevier Inc
Grant note: 2UL1 TR000442 / Clinical and Translational Science Award R21 DK096327; U01 DK108334 / National Institutes of Health (http://dx.doi.org/10.13039/100000002) REDCap
Language: English
Date published: 07/2017
Academic Unit: Stead Family Department of Pediatrics; Biostatistics; Radiation Oncology; Gastroenterology, Hepatology, Pancreatology, and Nutrition
Record Identifier: 9983997312702771

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