title: The etiology of acute recurrent pancreatitis in children: a challenge for pediatricians.
abstract: OBJECTIVE
  To assess specific etiologies of acute recurrent pancreatitis at a single Italian pediatric cystic fibrosis (CF) center.
  
  
  METHODS
  We studied, retrospectively, 78 young patients (39 female subjects; mean age at diagnosis, 8.8 ± 5.1 years) affected by acute recurrent episodes of pancreatitis, remained etiologically undiagnosed at first-level assessment. All patients were submitted to endoscopic retrograde cholangiopancreatography to exclude biliopancreatic malformations and tested for CF by a sweat chloride test. Most patients also were studied for the research of CFTR, PRSS1, and SPINK1 gene mutations.
  
  
  RESULTS
  A high percentage of family history for chronic pancreatitis was observed (20.5%). The sweat test identified 8 subjects (10.3%) with classic CF (2 patients) or at risk for CF (6 patients). Genetic analysis showed mutations in CFTR, SPINK1, and PRSS1 genes in 39.6%, 7.1%, and 4.5% of patients, respectively. A biliopancreatic malformation was diagnosed in 15 patients (19.2%). We also observed biliary lithiasis (5 patients [6.5%]), congenital pancreatic polycystosis (2 patients), a case of dyslipidemia, and 1 patient with a posttransplantation, drug-induced pancreatitis.
  
  
  CONCLUSIONS
  Recurrent pancreatitis in children has several etiologies. Genetic testing confirms the high frequency of CFTR mutations. This suggests that it is of some value to identify patients with late-onset CF and CFTR-related disorders.
authors.affiliation: Unit of Cystic Fibrosis, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. lucidi@opbg.net
authors.forename: Vincenzina
authors.initials: V
authors.lastname: Lucidi
authors.forename: Federico
authors.initials: F
authors.lastname: Alghisi
authors.forename: Luigi
authors.initials: L
authors.lastname: Dall'Oglio
authors.forename: Maria Rosaria
authors.initials: MR
authors.lastname: D'Apice
authors.forename: Lidia
authors.initials: L
authors.lastname: Monti
authors.forename: Paola
authors.initials: P
authors.lastname: De Angelis
authors.forename: Stefano
authors.initials: S
authors.lastname: Gambardella
authors.forename: Adriano
authors.initials: A
authors.lastname: Angioni
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Novelli
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D002352:Carrier Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D002760:Cholangiopancreatography, Endoscopic Retrograde; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis* / Q000235:genetics; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005260:Female; D005787:Gene Frequency; D020022:Genetic Predisposition to Disease / Q000235:genetics; D005820:Genetic Testing / Q000379:methods*; D005838:Genotype; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D010195:Pancreatitis / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics; D010372:Pediatrics / Q000379:methods*; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D014357:Trypsin / Q000235:genetics; D014359:Trypsin Inhibitor, Kazal Pancreatic
chemical_list: D002352:Carrier Proteins; C486642:SPINK1 protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D014359:Trypsin Inhibitor, Kazal Pancreatic; C489303:PRSS1 protein, human; D014357:Trypsin