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Exocrine pancreatic insufficiency in syndromic paucity of interlobular bile ducts
S K Chong1, J Lindridge, C Moniz
1Department of Child Health, King's College Hospital, London, England.
Insights
Paucity of interlobular bile ducts (PILBD) in children can cause failure to thrive. Pancreatic insufficiency may contribute to chronic diarrhea and poor weight gain in these patients, potentially benefiting from pancreatic extract supplementation.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Endocrinology
Background:
- Severe failure to thrive is a significant issue in children with paucity of interlobular bile ducts (PILBD).
- PILBD can be syndromic or nonsyndromic, impacting overall child development.
- Chronic diarrhea is a notable symptom in a subset of children with PILBD.
Purpose of the Study:
- To investigate pancreatic exocrine function in children with PILBD.
- To determine if pancreatic insufficiency contributes to failure to thrive and chronic diarrhea in PILBD.
- To evaluate the efficacy of pancreatic supplementation in affected children.
Main Methods:
- Pancreatic secretions were measured via duodenal aspirate after secretin-pancreozymin stimulation in 13 children with PILBD.
- Studies included children with and without chronic diarrhea, and a control group.
- Pancreatic secretion parameters (volume, bicarbonate, lipase) were analyzed.
Main Results:
- Children with PILBD and chronic diarrhea showed significantly reduced duodenal aspirate volume, bicarbonate concentration and output, and lipase concentrations compared to controls.
- These reductions were statistically significant (p < 0.05 for volume and output, p < 0.02 for bicarbonate, p < 0.005 for lipase).
- Five children with PILBD and diarrhea experienced improved stool frequency and weight gain after pancreatic supplementation.
Conclusions:
- Pancreatic insufficiency may be a contributing factor to poor weight gain in children with PILBD and diarrhea.
- Pancreatic extract supplementation may benefit children with PILBD experiencing chronic diarrhea and failure to thrive.
- Early identification and management of pancreatic insufficiency can improve outcomes for children with PILBD.
Abstract:
Severe failure to thrive is an important feature in children with both syndromic and nonsyndromic paucity of interlobular bile ducts (PILBD). Thirteen children age 7 months-11 years with PILBD had pancreatic secretions in duodenal aspirate measured for 40 min after secretin-pancreozymin stimulation, six of these children had chronic diarrhoea. Studies were also performed in seven children age 2-12 years who presented with failure to thrive or nonspecific diarrhoea that subsequently resolved. The results of the control and the PILBD children were similar except in the six with chronic diarrhoea. These children had significant reductions in total volume of duodenal aspirate (p less than 0.05), bicarbonate concentration (p less than 0.02) and output (p less than 0.05), and in lipase concentrations (p less than 0.005). Five of these children have had a reduction in stool frequency and more rapid weight gain since receiving pancreatic supplementation. In children with PILBD and diarrhoea, pancreatic insufficiency may be a contributory factor to poor weight gain. These children may benefit from pancreatic extract supplementation.