Symptomatic pancreatitis in patients with cystic fibrosis.
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.
Gooding
Ian
I
Department of Gastroenterology, Hammersmith Hospital, Du Cane Road, London, UK. ian_gooding@yahoo.co.uk
Bradley
Emma
E
Puleston
Joanne
J
Gyi
Khin-Ma
KM
Hodson
Margaret
M
Westaby
David
D
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
D019005:Cystic Fibrosis Transmembrane Conductance Regulator