Aspects of bile acid metabolism in cystic fibrosis.

Previous reports have indicated that cystic fibrosis (CF) patients with pancreatic enzyme 
insufficiency have a raised faecal bile acid output. In this study, 
18 out of 29 CF patients and 2 out of the 
4 non-CF patients with pancreatic enzyme insufficiency had raised faecal bile 
acid levels. In the CF patients no correlation was found between 
faecal bile acid and faecal fat excretion, but an inverse relation 
was shown between faecal bile acid values and age. Those CF 
patients with overt liver disease tended to have the lowest faecal 
bile acid values. Duodenal spiration in 5 CF patients and in 
one non-CF patient with pancreatic enzyme insufficiency (Shwachman-Diamond syndrome), produced very 
small fluid volumes. Duodenal fluid mean total bile acid concentrations were 
within normal limits. Estimation of serum bile acids in these 6 
patients showed that 3 patients had raised serum bile acid values. 
It is suggested that excessive faecal bile acid loss may produce 
a contraction of the bile acid pool, and lead eventually to 
a reduction of intraduodenal bile acid concentrations. Measures which curtail faecal 
bile acid loss may have a particular significance in the management 
of CF. 