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Eicosanoid synthesis in children with cholestatic disease
J Dupont1, O Amédée-Manesme, D Pepin
1Department of Food and Nutrition, Iowa State University, Ames 50011.
Journal of Inherited Metabolic Disease
|January 1, 1990
Summary
Children with cholestasis show essential fatty acid deficiency, impacting eicosanoid metabolism. Supplementation trials did not significantly improve linoleate absorption or eicosanoid synthesis in these patients.
Area of Science:
- Biochemistry
- Pediatric Gastroenterology
- Nutrition Science
Background:
- Cholestasis in children can lead to malabsorption of dietary triglycerides, potentially causing essential fatty acid deficiencies.
- Essential fatty acids are crucial precursors for eicosanoids, which play vital roles in various physiological processes.
Purpose of the Study:
- To investigate the impact of cholestasis on essential fatty acid composition and eicosanoid synthesis in children.
- To evaluate the efficacy of linoleate supplementation in addressing these deficiencies.
Main Methods:
- Analysis of plasma and platelet fatty acid composition in children with biliary atresia (BA) and paucity of interlobular bile ducts (PILBD).
- Measurement of prostaglandin (PG) and thromboxane (TXB2) synthesis in whole blood samples.
- Assessment of linoleate absorption following structured triglyceride supplementation in a subset of PILBD patients.
Main Results:
- Both BA and PILBD groups exhibited linoleate deficiency (low 18:2 fatty acids).
- PILBD patients showed significantly reduced thromboxane B2 (TXB2) synthesis (<10% of controls).
- Linoleate supplementation in three PILBD cases did not improve plasma linoleate levels or alter eicosanoid synthesis.
Conclusions:
- Essential fatty acid deficiency and altered eicosanoid metabolism are evident in pediatric cholestasis.
- Current linoleate supplementation strategies may be insufficient to correct these metabolic derangements in affected children.