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Disaccharidase activities in small intestinal mucosa in patients with cystic fibrosis
Insights
Lactase deficiency in cystic fibrosis (CF) patients is not linked to the disease itself. Mucosal damage impacts disaccharidase activity similarly in both CF patients and controls, suggesting CF doesn't directly cause lactase deficiency.
Area of Science:
- Gastroenterology
- Pediatric Gastroenterology
- Biochemistry
Background:
- Cystic Fibrosis (CF) can affect nutrient absorption.
- Disaccharidase activity is crucial for carbohydrate digestion.
- Understanding enzyme activity in CF patients is important for management.
Purpose of the Study:
- To investigate disaccharidase activities in relation to mucosal morphology and age in CF patients.
- To determine if lactase deficiency in CF is disease-related.
- To compare enzyme activity patterns between CF patients and healthy controls.
Main Methods:
- Analysis of small intestinal biopsies from 63 CF patients and 177 healthy controls.
- Assay of disaccharidase activities (including lactase).
- Correlation of enzyme activity with mucosal morphology and patient age.
Main Results:
- No low lactase activity was observed in young CF patients or controls (<5 years) with normal mucosa.
- In older CF patients (>5 years), lactase activity varied (high/low), mirroring healthy controls.
- Mucosal atrophy equally reduced disaccharidase activity in both CF and control groups.
- CF patients with pancreatic insufficiency showed significantly higher disaccharidase activities than controls (P<0.001).
Conclusions:
- Lactase deficiency in CF patients is likely independent of the disease itself.
- Mucosal integrity is a primary determinant of disaccharidase activity, affecting CF patients and controls similarly.
- Elevated disaccharidase activity in CF patients with pancreatic insufficiency warrants further investigation.
Abstract:
The disaccharidase activities in small intestinal biopsies were related to the morphology of the mucosa and the ages of 63 patients with cystic fibrosis and 177 healthy control subjects of Caucasian origin. In patients with CF and in the healthy control subjects under 5 years of age with normal intestinal mucosa, no low lactase activity was found. In those patients with CF who were over 5 years of age, one group had high and one group had low lactase activity, as occurs in healthy Caucasian control subjects of the same age. This finding supports the view that in patients with CF, lactase deficiency is not related to the disease entity. In patients with or without CF who had the same degree of mucosal atrophy, the decrease of disaccharidase activities followed the same pattern, indicating that enzyme activities are affected to the same extent by the damage of the mucosa. In patients with CF with pancreatic insufficiency, the disaccharidase activities were significantly (P less than 0.001) higher when compared to those in control subjects of the same age and ethnic group, although the increase was not uniform in all patients with cystic fibrosis.