Clinical, morphological characteristics, and outcome in patients with chronic pancreatitis (CP) due to hereditary pancreatitis (HP) and cystic fibrosis (CF)

C. P. Choudari, G. A. Lehman, S. Sherman, T. F. Imperiale, E. L. Fogel, N. J. Nicki, W. B. Silverman, M. F. Catalano, J. E. Geenen, M. J. Schmalz, K. Block

Research output: Contribution to journalArticle

Abstract

Chronic pancreatitis is an unusual cause of chronic abdominal pain in children, and can lead to long-term complications. We compared the initial clinical manifestations and follow-up of patients (pts) with HP and those with CF, in an attempt to characterize the clinical course of these two conditions. Methods: The medical records of 17 pts with HP and 15 with CF were reviewed along with telephone follow-up. The diagnosis of HP was made by identifying a mutation in the trypsinogen gene in 7 and strong family history in 10. All CF pts were sweat chloride positive or homozygous by gene testing. Results: HP (n=17) CF (n=15) Sex: M:F 7:10 10:5 Onset of symptoms (<5:6-15; 16-20;>20 yrs) 2;8;4;3 5;4;3;3 Abdominal pain 17 (100%) 15 (100%) CP (grade I, II, III) 4;4;9 6;6;2 Pancreas divisum 0 (0%) 2 (13%) Pancreatic stricture*6 (35%) 3 (20%) Pancreatic duct stone*7 (41%) 3 (20%) Pseudocyst*2 (12%) 0 (0%) Endoscopic treatment 11 (65%) 12 (80%) Surgical intervention 7 (41%) 2 (13%) Cancer (age 50 & 60 yrs) 2 0 Follow-up (mean months) 19 32 Pain-free 9 (52%) 7 (46%) > 50% improvement 3 (18%) 4 (27%) Recurrent symptoms 5 (30%) 4 (27%) Death 1 (6%) 0 (0%)*p = 0.008 (Combined stricture, stone, and pseudocyst) Summary: 1) Majority of pts (>80%) in both groups were symptomatic by 20 yrs of age. 2) Severe CP on ductography and complications of stricture formation, stone disease and pseudocyst were more common in HP pts (p=0.008). 3) Surgical intervention was more common in HP pts (41% vs 13%). 4) 70% of the HP and 73% of the CF group are improved or asymptomatic following therapy with short-term follow-up. Conclusion: The initial clinical manifestations were similar in both groups, but HP was more frequently associated with complications and the need for surgical intervention.

Original languageEnglish (US)
Pages (from-to)AB134
JournalGastrointestinal endoscopy
Volume47
Issue number4
StatePublished - Dec 1 1998

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Gastroenterology

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