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Relationship between bile acid malabsorption and pancreatic insufficiency in cystic fibrosis
Insights
Children with cystic fibrosis (CF) and pancreatic insufficiency exhibit significantly increased bile acid (BA) loss in stools. This malabsorption is linked to pancreatic dysfunction and correlates with fat loss, suggesting impaired digestion.
Area of Science:
- Gastroenterology
- Pediatric Medicine
- Biochemistry
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including the pancreas.
- Pancreatic insufficiency in CF leads to maldigestion and malabsorption of nutrients.
- Bile acids (BA) are crucial for fat digestion and absorption.
Purpose of the Study:
- To quantify bile acid (BA) loss in children with cystic fibrosis (CF) and pancreatic insufficiency.
- To investigate the relationship between BA loss, fat malabsorption, and pancreatic enzyme status.
- To explore the effect of pancreatic enzyme supplements and dietary modifications on BA and fat excretion.
Main Methods:
- Measurement of fecal bile acid (BA) and fat excretion in children with CF (with and without pancreatic insufficiency) and healthy controls.
- Analysis of BA and fat loss in CF patients before and after pancreatic enzyme supplementation.
- Evaluation of BA patterns in various gastrointestinal conditions, including CF and ileal resection.
Main Results:
- Significantly elevated fecal BA loss (751.1 ± 48.3 mg/m2/24h) was observed in CF children with pancreatic insufficiency compared to controls (109.8 ± 9.8 mg/m2/24h).
- A strong correlation was found between increased bile acid (BA) and fat malabsorption in CF patients.
- Pancreatic enzyme supplementation reduced fat loss but did not significantly alter BA excretion.
Conclusions:
- Increased fecal bile acid (BA) loss is a common finding in children with cystic fibrosis (CF) and pancreatic insufficiency.
- The mechanism of increased BA loss in CF is not fully understood but is related to pancreatic insufficiency.
- Further research is needed to confirm if unhydrolyzed triglycerides interfere with intestinal BA absorption in CF.
Abstract:
Bile acid loss (mg/m2 24h) in the stools of 43 cystic fibrosis (CF) children with pancreatic insufficiency was 751-1 +/- 48-3, while that of six without clinical evidence of pancreatic disease (133-4 +/- 15-9) did not differ from values in 25 controls (109-8 +/- 9-8). There was a good correlation between the degree of bile acid (BA) and fat sequestration. Concomitant changes in bile acid and fat loss were observed in the one group of six patients studied on and off pancreatic enzymes as well as in a second group of seven children treated with pancreatic supplements and maintained on a normal diet followed by a low fat diet supplemented with medium chain triglycerides. Administration of NA bicarbonate led to a significant decrease in fat loss (15-8 +/- 2-7 leads to 10-3 +/- 1-9) without any simultaneous change in bile acid excretion (533-1 +/- 58-3 leads to 500-4 +/- 58-6). Qualitative bile acid patterns in controls, in infants after an ileal resection, and in patients with CF or with coeliac disease showed that the percentage of primary BA followed closely the total amount excreted except in situations where antibiotics were administered. The exact mechanism for the increased loss of BA in CF is unknown. It is found in all age groups and is related to the presence and degree of pancreatic insufficiency. The possibility that unhydrolysed triglycerides may interfere with the intestinal absorption of bile acid needs further confirmation.
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