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J Pediatr. 2017 Jul;186:95-100. doi: 10.1016/j.jpeds.2017.03.063. Epub 2017 May 10.

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

Author information

1
Department of Pediatrics, Seattle Children's Hospital, Seattle, WA.
2
Department of Pediatrics, Children's Hospital, of Pittsburgh, Pittsburgh, PA.
3
Department of Pediatrics, Medical College of Wisconsin, Milwaukee, WI.
4
Department of Biostatistics, University of Iowa, College of Public Health, Iowa City, IA.
5
Department of Pediatrics, Hadassah Hebrew University Hospital, Jerusalem, Israel.
6
Department of Pediatrics, University of Texas Southwestern Medical School, Dallas, TX.
7
Department of Pediatrics, University of Minnesota Masonic Children's Hospital, Minneapolis, MN.
8
Department of Pediatrics, University of Utah, Salt Lake City, UT.
9
Department of Pediatrics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.
10
Department of Pediatrics, Sydney Children's Hospital, University of New South Wales, Sydney, Australia.
11
Department of Pediatrics, Montreal Children's Hospital, McGill University, Montreal, Québec, Canada.
12
Department of Pediatrics, Baylor College of Medicine, Houston, TX.
13
Department of Pediatrics, University of California San Francisco, San Francisco, CA.
14
Department of Pediatrics, Hospital for Sick Children, Toronto, ON, Canada.
15
Department of Pediatrics, Nationwide Children's Hospital, Columbus, OH.
16
Department of Pediatrics, Harvard Medical School, Boston, MA.
17
University of Iowa Carver College of Medicine, Stead Family Department of Pediatrics, Iowa City, IA. Electronic address: aliye-uc@uiowa.edu.

Erratum in

Abstract

OBJECTIVES:

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).

STUDY DESIGN:

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).

RESULTS:

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).

CONCLUSIONS:

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.

KEYWORDS:

children; genetic; pediatric; risk; young

PMID:
28502372
PMCID:
PMC5506853
DOI:
10.1016/j.jpeds.2017.03.063
[Indexed for MEDLINE]
Free PMC Article

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