Cystic fibrosis "factor(s)": present also in sera of Schwachman's pancreatic insufficiency.

Abstract

Using an in vivo closed-loop technique in rat jejunum, we have confirmed previous in vitro studies that cystic fibrosis (CF) serum inhibits water, sodium, and glucose absorption and transmural potential difference. CF heterozygote serum or sera form children with diarrhoeal disorders not associated with exocrine pancreatic insufficiency had no effect of transport. Sera from patients with Shwachman's syndrome and exocrine pancreatic insufficiency had identical effects to CF serum. These results challenge the specificity of the CF "factor."