K Hallberg1, A Reims, B Strandvik. 1. Dept. of Paediatrics, Göteborg University, Queen Silvia Children's Hospital, Sweden.
Abstract
BACKGROUND: Abnormality in chloride transport across epithelial tissues is a basic defect in cystic fibrosis (CF). Our aim was to compare the induced chloride secretion in duodenum in CF patients with different mutations. METHODS: Duodenal biopsies from 9 patients were investigated in a modified Ussing chamber and the secretory response to prostaglandin E2 (PGE2) and acetylcholine (ACh) were measured. RESULTS: PGE2 and ACh induced no changes in chloride secretion in the AF508 homozygotes. In heterozygotes the induced change in chloride secretion corresponded to the severity of the known mutations. CONCLUSION: The secretory response in duodenum in CF is influenced by the patients genotype and mainly related to sweat chloride secretion.
BACKGROUND: Abnormality in chloride transport across epithelial tissues is a basic defect in cystic fibrosis (CF). Our aim was to compare the induced chloride secretion in duodenum in CF patients with different mutations. METHODS: Duodenal biopsies from 9 patients were investigated in a modified Ussing chamber and the secretory response to prostaglandin E2 (PGE2) and acetylcholine (ACh) were measured. RESULTS:PGE2 and ACh induced no changes in chloride secretion in the AF508 homozygotes. In heterozygotes the induced change in chloride secretion corresponded to the severity of the known mutations. CONCLUSION: The secretory response in duodenum in CF is influenced by the patients genotype and mainly related to sweat chloride secretion.