Book contents
- Frontmatter
- Contents
- Contributing Authors
- Preface to the Third Edition
- Preface to the First Edition
- SECTION I PATHOPHYSIOLOGY OF PEDIATRIC LIVER DISEASE
- SECTION II CHOLESTATIC LIVER DISEASES
- 9 Approach to the Infant with Cholestasis
- 10 Medical and Nutritional Management of Cholestasis in Infants and Children
- 11 Neonatal Hepatitis and Congenital Infections
- 12 Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts
- 13 Neonatal Jaundice and Disorders of Bilirubin Metabolism
- 14 Familial Hepatocellular Cholestasis
- 15 Alagille Syndrome
- 16 Diseases of the Gallbladder in Infancy, Childhood, and Adolescence
- SECTION III HEPATITIS AND IMMUNE DISORDERS
- SECTION IV METABOLIC LIVER DISEASE
- SECTION V OTHER CONDITIONS AND ISSUES IN PEDIATRIC HEPATOLOGY
- Index
- Plate section
- References
13 - Neonatal Jaundice and Disorders of Bilirubin Metabolism
from SECTION II - CHOLESTATIC LIVER DISEASES
Published online by Cambridge University Press: 18 December 2009
- Frontmatter
- Contents
- Contributing Authors
- Preface to the Third Edition
- Preface to the First Edition
- SECTION I PATHOPHYSIOLOGY OF PEDIATRIC LIVER DISEASE
- SECTION II CHOLESTATIC LIVER DISEASES
- 9 Approach to the Infant with Cholestasis
- 10 Medical and Nutritional Management of Cholestasis in Infants and Children
- 11 Neonatal Hepatitis and Congenital Infections
- 12 Biliary Atresia and Other Disorders of the Extrahepatic Bile Ducts
- 13 Neonatal Jaundice and Disorders of Bilirubin Metabolism
- 14 Familial Hepatocellular Cholestasis
- 15 Alagille Syndrome
- 16 Diseases of the Gallbladder in Infancy, Childhood, and Adolescence
- SECTION III HEPATITIS AND IMMUNE DISORDERS
- SECTION IV METABOLIC LIVER DISEASE
- SECTION V OTHER CONDITIONS AND ISSUES IN PEDIATRIC HEPATOLOGY
- Index
- Plate section
- References
Summary
Elevation of the serum bilirubin level is a common if not universal finding during the first week of life. This can be a transient phenomenon that will resolve spontaneously. Alternatively, hyperbilirubinemia may signify a serious or even potentially life-threatening condition. There are many causes of hyperbilirubinemia, and each has its own therapeutic and prognostic implications. Independent of the cause, elevated serum bilirubin levels may be potentially toxic to the newborn infant. This chapter begins with a review of perinatal bilirubin metabolism. Assessment, etiology, toxicity, and therapy for neonatal jaundice are then addressed. Finally, the diseases in which there is a primary disorder in the metabolism of bilirubin are reviewed regarding their clinical presentation, pathophysiology, diagnosis, and treatment. Other pertinent reviews have been published [1–3].
BILIRUBIN METABOLISM
Production and Circulation
In 1864, Städeler [4] used the term bilirubin, derived from Latin (bilis, “bile”; ruber, “red”), for the red-colored bile pigment. Bilirubin is formed from the degradation of heme-containing compounds (Figure 13.1). The largest source for the production of bilirubin is hemoglobin. However, other heme-containing proteins are also degraded to bilirubin, including the cytochromes, catalases, tryptophan pyrrolase, and muscle myoglobin [5].
The formation of bilirubin is initiated by cleaving the tetrapyrrole ring of protoheme (protoporphyrin IX), which results in a linear tetrapyrrole (biliverdin). The first enzyme system involved in the formation of bilirubin is microsomal heme oxygenase (HO). Two major forms of HO have been identified [6]. HO1, the inducible form, is located in the spleen and liver.
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- Chapter
- Information
- Liver Disease in Children , pp. 270 - 309Publisher: Cambridge University PressPrint publication year: 2007
References
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