Book contents
- Liver Disease in Children
- Liver Disease in Children
- Copyright page
- Contents
- Contributors
- Preface
- Section I Pathophysiology of Pediatric Liver Disease
- Section II Cholestatic Liver Disease
- Section III Hepatitis and Immune Disorders
- Section IV Metabolic Liver Disease
- Chapter 24 Laboratory Diagnosis of Inborn Errors of Liver Metabolism
- Chapter 25 α1-Antitrypsin Deficiency
- Chapter 26 Cystic Fibrosis Liver Disease in Children
- Chapter 27 Inborn Errors of Carbohydrate Metabolism
- Chapter 28 Copper Metabolism and Copper Storage Disorders in Children
- Chapter 29 Iron Storage Disorders in Children
- Chapter 30 Heme Biosynthesis and the Porphyrias in Children
- Chapter 31 Tyrosinemia in Children
- Chapter 32 Lysosomal Storage Disorders in Children
- Chapter 33 Disorders of Bile Acid Synthesis and Metabolism in Children
- Chapter 34 Inborn Errors of Fatty Acid Oxidation
- Chapter 35 Mitochondrial Hepatopathies
- Chapter 36 Non-Alcoholic Fatty Liver Disease in Children
- Chapter 37 Peroxisomal Disorders in Children
- Chapter 38 Urea Cycle Disorders in Children
- Section V Other Considerations and Issues in Pediatric Hepatology
- Index
- References
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
Book contents
- Liver Disease in Children
- Liver Disease in Children
- Copyright page
- Contents
- Contributors
- Preface
- Section I Pathophysiology of Pediatric Liver Disease
- Section II Cholestatic Liver Disease
- Section III Hepatitis and Immune Disorders
- Section IV Metabolic Liver Disease
- Chapter 24 Laboratory Diagnosis of Inborn Errors of Liver Metabolism
- Chapter 25 α1-Antitrypsin Deficiency
- Chapter 26 Cystic Fibrosis Liver Disease in Children
- Chapter 27 Inborn Errors of Carbohydrate Metabolism
- Chapter 28 Copper Metabolism and Copper Storage Disorders in Children
- Chapter 29 Iron Storage Disorders in Children
- Chapter 30 Heme Biosynthesis and the Porphyrias in Children
- Chapter 31 Tyrosinemia in Children
- Chapter 32 Lysosomal Storage Disorders in Children
- Chapter 33 Disorders of Bile Acid Synthesis and Metabolism in Children
- Chapter 34 Inborn Errors of Fatty Acid Oxidation
- Chapter 35 Mitochondrial Hepatopathies
- Chapter 36 Non-Alcoholic Fatty Liver Disease in Children
- Chapter 37 Peroxisomal Disorders in Children
- Chapter 38 Urea Cycle Disorders in Children
- Section V Other Considerations and Issues in Pediatric Hepatology
- Index
- References
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.
- Type
- Chapter
- Information
- Liver Disease in Children , pp. 593 - 610Publisher: Cambridge University PressPrint publication year: 2021
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