RECENT ADVANCES IN NEONATALGASTROENTEROLOGY 00955108/02 $15.00 .00

UPDATE ON THE ETIOLOGIESAND MANAGEMENT OF

NEONATAL CHOLESTASIS

Saul J. Karpen, MD, PhD

Discriminating between benign and serious causes of jaundice is acommon task faced by most pediatricians and neonatologists in theirdaily practice. Because a substantial proportion of all newborn infantsare visibly jaundiced at some point during the rst week of life, identi-fying those with serious underlying disease is sometimes problematicand requires a combination of insight, experience, and heightened con-cern.14, 34, 50, 78 The principal issue facing the clinician is the ability todistinguish between hyperbilirubinemia resulting from a variety ofcauses and those with underlying liver disease. This is critical becausethe earlier an infant is recognized as having serious cholestatic liverdisease, the more likely a correct diagnosis can be made, and institutionof appropriate therapy begun. Making it even more difcult for theprimary practitioner is the fact that most newborn infants are not sched-uled to be seen in the ofce between 2 weeks and 2 months of age, atime when many cholestatic liver diseases begin to present.

An exploration of all causes of jaundice in infancy is beyond thescope of this article, and has been recently covered in detail.26 Rather,this article gives an overview of the causes of neonatal cholestasis,dened as a marked impairment in bile ow.14, 98, 100, 101 A brief overviewof the determinants of bile ow assists in the analysis of clinical and

From the Department of Pediatrics, and Texas Childrens Liver Center, Baylor College ofMedicine, Houston, Texas

CLINICS IN PERINATOLOGY

VOLUME 29 NUMBER 1 MARCH 2002 159