Summary
The possibility of malabsorption of triglycerides contained in the diets of children with cholestasis suggests a deficiency of essential fatty acids and, therefore, probable effects on eicosanoid metabolism. Children with either biliary atresia (BA) or syndromatic paucity of interlobular bile ducts (PILBD) were evaluated as to plasma and platelet total lipid fatty acid composition and synthesis of prostaglandins (PG) E1, PGE2, PGI2, PGF2, and thromboxane (TXB2) by whole blood incubated at 37°C for 10 min. In both diseases linoleate deficiency was present as shown by low 18:2 fatty acids in plasma lipids. The children with BA had lower plasma arachidonate than controls but normal eicosanoid synthesis except for excess PGI2. Those with PILBD had low platelet arachidonate and were severely deficient in TXB2 synthesis (<10% of controls). Three children with PILBD were fed a supplement of structured triglyceride (Captex 810) intended to provide as much as 10% of energy as linoleate for 2–3 months. Results for these three cases suggested that insufficient linoleate was absorbed to increase plasma linoleate and differences in eicosanoids could not be attributed to linoleate supplementation.
Similar content being viewed by others
References
Alagille, D., Odievre, M., Gautier, M. and Dommergues, J. P. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental and sexual development, and cardiac murmur.J. Pediatr. 86 (1975) 63–71
Alagille, D., Estrada, A., Hadchouel, M., Gautier, M., Odievre, M. and Dommergues, J. P. Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): Review of 80 cases.J. Pediatr. 110 (1987) 195–200
Alvarez, F., Cresteil, D., Lemonnier, F., Lemonnier, A. and Alagille, D. Plasma vitamin E levels in children with cholestasis.J. Pediatr. Gastroenterol. Nutr. 3 (1984) 390–393
Ascher, N. L. and Najarian, J. S. Hepatic transplantation and biliary atresia; early experience in 8 patients.World J. Surg. 8:1 (1984) 57–63
Chambaz, J., Ravel, D., Manier, M. C., Pepin, D., Mulliez, N. and Bereziat, G. Essential fatty acid interconversion in the human fetal liver.Biol. Neonate 47 (1985) 136–140
Chase, H. P. and Dupont, J. Abnormal levels of prostaglandins and fatty acids in blood of children with cystic fibrosis.Lancet 2 (1978) 236–238
Dahms, B. B., Petrelli, M., Wyllie, R., Henoch, M. S., Halpin, T. C., Morrison, S., Par, M. C. and Tavill, A. S. Arteriohepatic dysplasia in infancy and childhood: A longitudinal study of six patients.Hepatology 2 (1982) 350–358
Dupont, J. and Mathias, M. M. Effects of dietary fat on eicosanoid production in normal tissues. In Abraham, S. (ed.)Carcinogenesis and Dietary Fat, Kluwer Academic Publishers, Boston, 1989, pp. 29–52
Dupont, J., Amedee-Manesme, O., Alagille, D., Chambaz, J. and Pepin, D. Eicosanoid synthesis in Alagille syndrome.N. Engl. J. Med. 314 (1986) 718
Engelhardt, H. T. Jr. Allocating scarce medical resources and the availability of organ transplantation.N. Engl. J. Med. 311 (1984) 66–71
Gourley, G. R., Farrell, P. and Odell, G. B. Essential fatty acid deficiency after hepatic portoenterostomy for biliary atresia.Am. J. Clin. Nutr. 36 (1982) 1194–1199
Iwatsuke, S., Shaw, B. W. and Starzl, R. E. Liver transplantation for biliary atresia.World J. Surg. 8 (1984) 51–56
Kasai, M., Suzuki, H., Ohashi, E., Ohi, R., Chiba, T. and Okamoto, A. Technique and results of operative management of biliary atresia.World J. Surg. 2 (1978) 571
McCosh, E. J., Meyer, D. L. and Dupont, J. Radioimmunoassay of prostaglandins E1, E2, and F2 in unextracted plasma, serum and myocardium.Prostaglandins 12 (1976) 471–486
McKenna, M. C., Hubbard, V. S. and Bieri, J. B. Linoleic acid absorption from lipid supplements in patients with cystic fibrosis with pancreatic insufficiency and in control subjects.J. Pediatr. Gastroenterol. Nutr. 4 (1985) 45–51
Valencia-Mayoral, P., Weber, J., Cutz, E., Edwards, V. D. and Philipps, M. J. Possible defect in the bile secretory apparatus in arteriohepatic dysplasia (Alagille's syndrome): A review of observations on the ultrastructure of liver.Hepatology 4 (1984) 691–698
Author information
Authors and Affiliations
Additional information
Journal Paper No. J-12248 of the Iowa Agriculture and Home Economics Experiment Station, Ames, Project Number 2728.
Rights and permissions
About this article
Cite this article
Dupont, J., Amédée-Manesme, O., Pepin, D. et al. Eicosanoid synthesis in children with cholestatic disease. J Inherit Metab Dis 13, 212–218 (1990). https://doi.org/10.1007/BF01799688
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01799688