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Symptomatic pancreatitis in patients with cystic fibrosis
- Source :
- The American journal of gastroenterology. 104(6)
- Publication Year :
- 2009
-
Abstract
- Objectives 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.
- Subjects :
- Adult
Male
medicine.medical_specialty
Pancreatic disease
Time Factors
Adolescent
Cystic Fibrosis
Cystic Fibrosis Transmembrane Conductance Regulator
Cystic fibrosis
Gastroenterology
Young Adult
Internal medicine
medicine
Humans
In patient
Genetic Predisposition to Disease
Young adult
Child
Retrospective Studies
Cholangiopancreatography, Endoscopic Retrograde
Hepatology
business.industry
Respiratory disease
Infant
Retrospective cohort study
Middle Aged
medicine.disease
Survival Rate
Phenotype
England
Pancreatitis
Child, Preschool
Mutation
Case note
Female
Morbidity
business
Follow-Up Studies
Subjects
Details
- ISSN :
- 15720241
- Volume :
- 104
- Issue :
- 6
- Database :
- OpenAIRE
- Journal :
- The American journal of gastroenterology
- Accession number :
- edsair.doi.dedup.....5e6405ad1fc7c4ec9c3dfd09381fb456