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Failure to induce secretion in jejunal biopsies from children with cystic fibrosis

C J Taylor1, P S Baxter, J Hardcastle

  • 1Department of Paediatrics, Children's Hospital, Sheffield.

Gut
|July 1, 1988
PubMed

Insights

Jejunal biopsies from children with cystic fibrosis (CF) show a defect in chloride transport, failing to respond to common secretagogues. This jejunal defect may contribute to intestinal CF symptoms and offers diagnostic potential.

Area of Science:

  • Gastroenterology
  • Pediatrics
  • Cell Biology

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
  • Intestinal manifestations of CF are common but less understood than pulmonary issues.
  • Previous research identified chloride transport defects in CF epithelia.

Purpose of the Study:

  • To investigate the secretory activity of jejunal biopsies in children with CF.
  • To determine if CF affects jejunal chloride transport.
  • To explore the potential diagnostic value of jejunal biopsy analysis in CF.

Main Methods:

  • Utilized a modified Ussing chamber technique to study jejunal biopsies.
  • Challenged tissue samples with intestinal secretagogues: acetylcholine, prostaglandin E2, and dibutyryl cyclic AMP.
  • Assessed responses by measuring changes in short-circuit current (SCC).
  • Tested responses to calcium ionophore A23187 and sodium-linked glucose absorption.

Main Results:

  • CF jejunal biopsies failed to respond to acetylcholine, prostaglandin E2, and dibutyryl cyclic AMP.
  • Control tissues showed significant increases in SCC upon stimulation.
  • Calcium ionophore A23187 induced a small response in some CF tissues, unlike controls.
  • Both CF and control tissues demonstrated normal sodium-linked glucose absorption.

Conclusions:

  • The study confirms a defect in chloride transport in the jejunum of children with CF.
  • This jejunal transport defect may contribute to the gastrointestinal symptoms observed in CF patients.
  • The Ussing chamber technique is a valuable tool for studying CF defects and may have diagnostic applications.

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