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- David Brumbaugh, MD*
- Cara Mack, MD*
- *Digestive Health Institute, Children’s Hospital of Colorado, University of Colorado Anschutz Medical Campus, Denver, CO.
Author Disclosure
Drs Brumbaugh and Mack have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.
- AIH:
- autoimmune hepatitis
- ALT:
- alanine aminotransferase
- AST:
- aspartate aminotransferase
- A1AT:
- alpha-1 antitrypsin
- BA:
- biliary atresia
- BRIC:
- benign recurrent intrahepatic cholestasis
- BSEP:
- bile salt excretory protein
- CDC:
- choledochal cyst
- ERCP:
- endoscopic retrograde cholangiopancreatography
- GGT:
- gamma glutamyltransferase
- MCT:
- medium chain triglycerides
- MRCP:
- magnetic resonance cholangiopancreatography
- PFIC:
- progressive familial intrahepatic cholestasis
- PN:
- parenteral nutrition
- PSC:
- primary sclerosing cholangitis
Education Gaps
Awareness of telltale signs and performance of appropriate diagnostic testing can help clinicians identify neonatal cholestasis in time to ameliorate its potentially catastrophic outcomes.
The success of the Kasai procedure to restore bile flow is directly related to patient age: at less than 60 days after birth, two-thirds of patients benefit from the procedure; however, at 90 days after birth, chances for bile drainage diminish markedly.
Objectives
After completing this article, readers should be able to:
Understand the metabolism of bilirubin, the differences between conjugated and unconjugated bilirubin, and the relationship of conjugated hyperbilirubinemia to cholestasis.
Delineate the causes of cholestasis in the newborn and know how to evaluate the cholestatic neonate.
Manage the infant who has prolonged cholestasis.
Understand the causes of conjugated hyperbilirubinemia in the older child and adolescent and know how to assess children who have conjugated hyperbilirubinemia.
Introduction
Central to human digestive health are both the production of bile by hepatocytes and cholangiocytes in the liver and the excretion of bile through the biliary tree. By volume, conjugated bilirubin is a relatively small component of bile, the yellowish-green liquid that also contains cholesterol, phospholipids, organic anions, metabolized drugs, xenobiotics, and bile acids. In most cases, the elevation of serum-conjugated bilirubin is a biochemical manifestation of cholestasis, which is the pathologic reduction in bile formation or flow.
Complex mechanisms exist for the transport of bile components from serum into hepatocytes across the basolateral cell surface, for the trafficking of bile components through the hepatocyte, and finally for movement of these bile components across the apical cell surface into the bile canaliculus, which is the smallest branch of the biliary …
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Jump to section
- Article
- Education Gaps
- Objectives
- Introduction
- Diagnosis
- Recognition of the Cholestatic Newborn
- Initial Approach to the Cholestatic Infant
- Differential Diagnosis of Neonatal Cholestasis
- Management of the Infant Who Has Prolonged Cholestasis
- Approach to the Child and Adolescent Who Has Conjugated Hyperbilirubinemia
- Gallstone Disease
- Choledochal Cyst
- Other Causes of Obstructive Jaundice
- Infectious Hepatitis
- Autoimmune Disease of the Liver and Biliary System
- Drug- and Toxin-Induced Cholestasis
- Wilson Disease
- Benign Recurrent Intrahepatic Cholestasis
- References
- Suggested Reading
- Figures & Data
- Info & Metrics
- Comments
- Quiz