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Chapter 33 - Disorders of Bile Acid Synthesis and Metabolism in Children

from Section IV - Metabolic Liver Disease

Published online by Cambridge University Press:  19 January 2021

By
Kenneth D.R. Setchell  and
James E. Heubi
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Edited in association with
Jorge A. Bezerra ,
Cara L. Mack  and
Benjamin L. Shneider
Frederick J. Suchy
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Ronald J. Sokol
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
William F. Balistreri
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Jorge A. Bezerra
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Cara L. Mack
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Benjamin L. Shneider
Affiliation:
Texas Children’s Hospital, Houston
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Summary

The importance of bile acid synthesis and metabolism to normal physiology and their role in pathophysiological states is well recognized. For such small and relatively simple molecules, bile acids have amazingly diverse properties and functions. Bile acid biosynthesis represents one of the major pathways for regulating cholesterol homeostasis – each day approximately 0.5 g of cholesterol is metabolized to bile acids [1]. These molecules are essential for providing the major driving force for the promotion and secretion of bile and therefore are key elements in the development and maintenance of an efficient enterohepatic circulation. Bile acids are essential for facilitating the solubilization and absorption of fats and fat-soluble vitamins from the small bowel, although in the large bowel these molecules if in excess are potentially harmful in that they are carthartic, membrane damaging, and promoters of colonic disease. More recently, bile acids have become of interest because of their hormone-like actions of relevance to obesity, glucose and insulin regulation where they are now regarded as important molecules that signal through orphan receptors to regulate metabolism. With regard to bile acid biosynthesis, comprehensive reviews on the topic have been published previously [1, 2], and in the fourth edition of this textbook a detailed description of the pathways for bile acid synthesis was described.

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Liver Disease in Children , pp. 593 - 610
Publisher: Cambridge University Press
Print publication year: 2021

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References

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