Related Experiment Videos
Abnormal pancreatic electrolyte secretion in cystic fibrosis. Reliability as a diagnostic marker
Insights
Cystic fibrosis (CF) patients often show significantly reduced pancreatic enzyme and bicarbonate secretion. However, some CF children exhibit near-normal function, suggesting pancreatic function varies in cystic fibrosis.
Area of Science:
- Pediatric Gastroenterology
- Genetics
- Pancreatic Physiology
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, including the pancreas.
- Pancreatic insufficiency is a common complication of CF, impacting digestion and nutrient absorption.
Purpose of the Study:
- To quantitatively assess pancreatic exocrine function in children with CF.
- To investigate the relationship between pancreatic function and clinical characteristics in CF patients.
Main Methods:
- Utilized continuous intravenous infusion of cholecystokinin and secretin for pancreatic function tests.
- Collected duodenal juice quantitatively using an unabsorbable marker (bromosulfthalein).
- Analyzed enzyme, bicarbonate, and ion secretion in 65 CF patients and 11 controls.
Main Results:
- Most (79%) CF patients had severely reduced trypsin secretion.
- Nearly all (96.5%) CF children showed impaired bicarbonate secretion compared to controls.
- Some CF patients had near-normal enzyme outputs; better function correlated with younger age and male sex.
Conclusions:
- Pancreatic exocrine function is significantly impaired in most children with CF, particularly trypsin and bicarbonate secretion.
- While impaired, pancreatic function in CF can vary, with some patients showing near-normal levels.
- A linear correlation between acinar and tubular secretion suggests bicarbonate secretion may not be the primary genetic defect in CF pancreatic dysfunction.
Abstract:
We performed pancreatic function tests on sixty-five cystic fibrosis (CF), and eleven control children. The technique used continuous IV infusion of cholecystokinin and secretin, with duodenal juice collection over a 90 min period, and was made quantitative by continuous duodenal infusion and distal collection of an unabsorbable marker (bromosulphthalein). Some CF patients had near normal pancreatic enzyme outputs, some had impaired but measurable levels, but most (79%) had almost absent trypsin secretion. CF children with better pancreatic function, were younger and more likely to be male. All controls showed a large increase in bicarbonate concentration and secretion rate per kilogram body weight during the test, but most children with CF (96.5%) did not. Because two of our CF patients had water and bicarbonate secretion within the control range, this finding does not exclude the diagnosis of CF. Sodium, potassium and chloride ion secretion in CF patients was lower than controls but overlap occurred. We found a linear correlation between acinar and tubular secretion in CF patients which indicates that there is probably not a primary genetic defect in pancreatic bicarbonate secretion in CF.