Recurrent acute pancreatitis in patients with cystic fibrosis with normal pancreatic enzymes.

Ten adolescent and young adults with cystic fibrosis (CF) have had well-documented 
recurrent attacks of acute pancreatitis. The diagnosis of CF in each 
patient was delayed because they did not have pancreatic insufficiency. The 
diagnosis of CF was documented by the typical pulmonary involvement and 
elevated sweat sodium and chloride levels in all cases and a 
positive family history in six of the ten patients. Two patients 
were diagnosed as having acute pancreatitis before the diagnosis of CF 
was made, thus indicating that acute pancreatitis may be the presenting 
complaint in the young adult with CF. The diagnosis of acute 
pancreatitis was based on the presence of severe abdominal pain, usually 
with vomiting, tenderness in the mid- epigastrium, elevated serum and urinary 
amylase and serum lipase. Attacks were precipitated by fatty meals, alcohol 
ingestion; postcholecystectomy and tetracycline administration. In some patients no precipitating event 
could be elicited. Intravenous secretin- pancreozymin stimulation tests revealed a diminished 
bicarbonate secretion with little effect on the secretion of the zymogen 
enzymes. A mild attack of pancreatitis occurred after secretin-pancreozymin stimulation. The 
endocrine pancreatic function tested in four patients was normal as revealed 
by the glucose tolerance tests and determinations of serum insulin, growth 
hormone and free fatty acid. Transduodenal pancreatograms were performed in three 
patients; one showed a normal pancreatic duct, one showed duct obstruction 
and in the third patient a beady type of narrowing was 
found. The selenomethionine Se 75 uptake of the pancreas was noted 
only in the head of the pancreas. This suggests that loss 
of function occurs initially to a greater extent in the tail 
and body of the pancreas. Three patients died and showed characteristic 
lesions of CF. 