title: Symptomatic pancreatitis in patients with cystic fibrosis.
abstract: OBJECTIVE
  A small group of cystic fibrosis (CF) patients develop symptomatic pancreatitis. The clinical characteristics of these cases are not well documented in the literature. Most cases are in pancreatic sufficient (PS) patients, but it is not clear whether pancreatitis does occur in pancreatic insufficient (PI) patients. There is no information on how the group with PS and pancreatitis differs from the group with PS that does not develop pancreatitis.
  
  
  METHODS
  The Royal Brompton Hospital database of adult CF patients was searched to identify all patients with symptomatic pancreatitis. Clinical details were taken from the case notes. PS pancreatitis patients were then compared with an age- and sex-matched PS control group drawn from the database.
  
  
  RESULTS
  Sixteen patients (9 males) had suffered symptomatic pancreatitis, representing 1.6% of the total database. The mean age at CF diagnosis was 18.7 years, and at presentation with pancreatitis it was 28.8 years. Twelve were PS at diagnosis of CF. At presentation with pancreatitis, seven patients were PS and at the most recent follow-up or death, two remained PS. There was a median of three hospital admissions with pancreatitis. Eight cases developed pancreatic or hepatobiliary complications. In the comparison of pancreatitis patients with controls, there was no difference in survival but pancreatitis patients were significantly more likely to develop PI status. Mild CF transmembrane conductance regulator mutations in general, and R117H in particular, were found more often in pancreatitis patients.
  
  
  CONCLUSIONS
  Symptomatic pancreatitis is a significant problem in 1-2% of patients with CF. These patients are PS at birth but are more likely to develop late PI status than PS patients without pancreatitis. R117H may be associated with this phenotype.
authors.affiliation: Department of Gastroenterology, Hammersmith Hospital, Du Cane Road, London, UK. ian_gooding@yahoo.co.uk
authors.forename: Ian
authors.initials: I
authors.lastname: Gooding
authors.forename: Emma
authors.initials: E
authors.lastname: Bradley
authors.forename: Joanne
authors.initials: J
authors.lastname: Puleston
authors.forename: Khin-Ma
authors.initials: KM
authors.lastname: Gyi
authors.forename: Margaret
authors.initials: M
authors.lastname: Hodson
authors.forename: David
authors.initials: D
authors.lastname: Westaby
mesh_terms: D000293:Adolescent; D000328:Adult; 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; D004739:England / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009017:Morbidity / Q000639:trends; D009154:Mutation; D010195:Pancreatitis / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology; D010641:Phenotype; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D013997:Time Factors; D055815:Young Adult
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator