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9 - Approach to the Infant with Cholestasis

from SECTION II - CHOLESTATIC LIVER DISEASES

Published online by Cambridge University Press:  18 December 2009

By
Frederick J. Suchy M.D.
Edited by
Frederick J. Suchy ,
Ronald J. Sokol  and
William F. Balistreri
Frederick J. Suchy M.D.
Affiliation:
Professor and Chair, Department of Pediatrics, Mount Sinai School of Medicine of New York University, New York, New York; Pediatrician-in-Chief, Department of Pediatrics, Mount Sinai Hospital, New York, New York
Frederick J. Suchy
Affiliation:
Mount Sinai School of Medicine, New York
Ronald J. Sokol
Affiliation:
University of Colorado, Denver
William F. Balistreri
Affiliation:
University of Cincinnati
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Summary

Jaundice sometimes appears at birth, indicated by the dark yellow color of the countenance and arising from obstructions of the liver. Cases are generally incurable [1].

– Eli Ives of Yale University, America's first academic pediatrician, circa 1829

Cholestasis may be defined physiologically as a measurable decrease in bile flow, pathologically as the histologic presence of bile pigment in hepatocytes and bile ducts, and clinically as the accumulation in blood and extrahepatic tissues of substances normally excreted in bile (e.g., bilirubin, bile acids, and cholesterol). The process occurs as a result of impaired bile formation by the hepatocyte or from obstruction to the flow of bile through the intrahepatic and extrahepatic biliary tree [2, 3]. In the neonate, the clinical and laboratory features of the many liver diseases presenting with cholestasis are quite similar. An important focus of the pediatric hepatologist is to differentiate intrahepatic from extrahepatic cholestasis and, if possible, establish a specific diagnosis [4]. Strategies for the treatment of metabolic or infectious liver disease and for the surgical management of biliary anomalies require early diagnosis. Even when treatment is not available or effective, infants with progressive liver disease usually benefit from optimal nutritional support and medical management of medical complications of cholestasis and possibly cirrhosis until liver transplantation is done.

This chapter presents an overview of the approach to the infant with cholestatic liver disease. The diagnostic evaluation of these patients is emphasized.

Type
Chapter
Information
Liver Disease in Children , pp. 179 - 189
Publisher: Cambridge University Press
Print publication year: 2007

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