FIFTEEN YEAR PROSPECTIVE STUDY OF PANCREATITIS IN A POPULATION OF PATIENTS WITH CYSTIC FIBROSIS

Abstract

In Cystic Fibrosis (CF) pancreatic damage begins in utero. Pancreatic insufficiency (PI) is usually present at birth. In 15% of patients, the progression of pancreatic disease does not occur or is retarded. These patients are pancreatic sufficient (PS) and are susceptible to acute recurrent pancreatitis. The aim of this study is to investigate the frequency, natural history and outcome of pancreatitis in a CF population. Pancreatic function was assessed in 96 CF patients at diagnosis by measurement of serum pancreatic isoamylase activity (Phadebas PIA test, PIA normal values 40- 120 u/L). Fifteen CF patients (16%) who had pancreatic sufficiency (PS) (PIA 60-250 u/L) were followed up for episodes of pancreatic pain, evaluated with serial measurements of serum amylase, serum PIA and ultrasound of the pancreas. At the final phase, exocrine pancreatic function was monitored by serial fecal human pancreatic elastase (ELISA, Schebo-Tech, E1 normal values>200μg/gr stool). Clinical status was assessed by Schwachman score. Six of 15 patients with PS (40%) manifested recurrent, multiple episodes of acute pancreatic pain, marked elevation of pancreatic enzymes (PIA up to 2400 u/L) and evidence of pancreatitis on ultrasound. The episodes decreased gradually and serum PIA was stabilized at 60 to 90u/L. Gradual decline of fecal elastase to less than 200 (144, 170, 180) μg/gr stool was demonstrated in three while the remaining 12 PS patients had fecal elastase ranging from 257 to 2068μg/gr stool. Schwachman score in PS patients remained high throughout the observation period. It is concluded that acute recurrent pancreatitis occured in 6% of a CF population, exclusively in PS patients (40%). A subgroup of PS patients (20%) among those with recurrent pancreatitis developed PI and the need for pancreatic enzyme supplementation. Monitoring of exocrine pancreatic function in PS patients, especially in those with burnt out recurrent pancreatitis, is recommended.