title: Pediatric chronic pancreatitis is associated with genetic risk factors and substantial disease burden.
abstract: OBJECTIVE
  To determine the clinical presentation, diagnostic variables, risk factors, and disease burden in children with chronic pancreatitis.
  
  
  METHODS
  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.
  
  
  RESULTS
  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%).
  
  
  CONCLUSIONS
  Chronic pancreatitis occurs at a young age with distinct clinical features. Genetic and obstructive risk factors are common, and disease burden is substantial.
authors.affiliation: University of Minnesota Children's Hospital, Minneapolis, Minnesota, USA.
authors.forename: Sarah Jane
authors.initials: SJ
authors.lastname: Schwarzenberg
authors.affiliation: University of Minnesota Children's Hospital, Minneapolis, Minnesota, USA.
authors.forename: Melena
authors.initials: M
authors.lastname: Bellin
authors.affiliation: Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
authors.forename: Sohail Z
authors.initials: SZ
authors.lastname: Husain
authors.affiliation: University of Iowa Carver College of Medicine, Iowa City, IA, USA.
authors.forename: Monika
authors.initials: M
authors.lastname: Ahuja
authors.affiliation: University of Texas Southwestern Medical School, Dallas, TX, USA.
authors.forename: Bradley
authors.initials: B
authors.lastname: Barth
authors.affiliation: University of Iowa Carver College of Medicine, Iowa City, IA, USA.
authors.forename: Heather
authors.initials: H
authors.lastname: Davis
authors.affiliation: Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Peter R
authors.initials: PR
authors.lastname: Durie
authors.affiliation: Baylor College of Medicine, Houston, TX, USA.
authors.forename: Douglas S
authors.initials: DS
authors.lastname: Fishman
authors.affiliation: Harvard Medical School, Boston, MA, USA.
authors.forename: Steven D
authors.initials: SD
authors.lastname: Freedman
authors.affiliation: Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Cheryl E
authors.initials: CE
authors.lastname: Gariepy
authors.affiliation: Seattle Children's Hospital, Seattle, WA, USA.
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Giefer
authors.affiliation: Hospital for Sick Children, Toronto, ON, Canada.
authors.forename: Tanja
authors.initials: T
authors.lastname: Gonska
authors.affiliation: University of California at San Francisco, San Francisco, CA, USA.
authors.forename: Melvin B
authors.initials: MB
authors.lastname: Heyman
authors.affiliation: Baylor College of Medicine, Houston, TX, USA.
authors.forename: Ryan
authors.initials: R
authors.lastname: Himes
authors.affiliation: Nationwide Children's Hospital, Columbus, OH, USA.
authors.forename: Soma
authors.initials: S
authors.lastname: Kumar
authors.affiliation: Montreal Children's Hospital, McGill University, Montreal, QC, Canada.
authors.forename: Veronique D
authors.initials: VD
authors.lastname: Morinville
authors.affiliation: Children's Hospital of Pittsburgh, Pittsburgh, PA, USA.
authors.forename: Mark E
authors.initials: ME
authors.lastname: Lowe
authors.affiliation: University of Iowa Carver College of Medicine, Iowa City, IA, USA.
authors.forename: Neil E
authors.initials: NE
authors.lastname: Nuehring
authors.affiliation: 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.
authors.forename: Chee Y
authors.initials: CY
authors.lastname: Ooi
authors.affiliation: University of Utah, Salt Lake City, UT, USA.
authors.forename: John F
authors.initials: JF
authors.lastname: Pohl
authors.affiliation: University of Texas Southwestern Medical School, Dallas, TX, USA.
authors.forename: David
authors.initials: D
authors.lastname: Troendle
authors.affiliation: Medical College of Wisconsin, Milwaukee, WI, USA.
authors.forename: Steven L
authors.initials: SL
authors.lastname: Werlin
authors.affiliation: Hadassah Hebrew University Hospital, Jerusalem, Israel.
authors.forename: Michael
authors.initials: M
authors.lastname: Wilschanski
authors.affiliation: University of California at San Francisco, San Francisco, CA, USA.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Yen
authors.affiliation: University of Iowa Carver College of Medicine, Iowa City, IA, USA.
authors.forename: Aliye
authors.initials: A
authors.lastname: Uc
mesh_terms: D002648:Child; D002760:Cholangiopancreatography, Endoscopic Retrograde; D003430:Cross-Sectional Studies; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004247:DNA / Q000235:genetics; D004252:DNA Mutational Analysis; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D015994:Incidence; D008297:Male; D009154:Mutation; D050500:Pancreatitis, Chronic / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics*; D015995:Prevalence; D012307:Risk Factors; D014481:United States / Q000453:epidemiology
chemical_list: C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D004247:DNA