Introduction


















Cholestasis is defined physiologically as a reduction in bile flow. The clinical marker is usually an elevation of the direct bilirubin - > 1 mg% if the total bilirubin is < 5 mg% or > 20% of the total bilirubin if the latter is > 5 mg%.

Cholestatic jaundice is an uncommon clinical problem in pediatrics, with an incidence of just 1 in every 2500 infants. Compare this to the prevalence of physiologic and breast milk jaundice, both characterized by an increased indirect bilirubin, with a combined incidence of 1 in every 12 infants. However, despite its infrequent occurrence, its causes are much more likely to be serious, requiring prompt diagnosis and early intervention.

The American Academy of Pediatrics (AAP) and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) have recommended that any infant who is jaundiced at 2 weeks of age have a total and direct bilirubin measured. The only exception is the healthy, breastfed infant with a normal physical exam and history; in such infants, evaluation for cholestasis can be delayed until 3 weeks, if still jaundiced.

It has also been regularly reported that improved morbidity and mortality result from diagnosis and treatment of the various causes of cholestasis before 45 days of age. However, in spite of the above, infants are often not diagnosed with cholestatic jaundice until after 60 days of age. Therefore, it is imperative that the pediatrician be alert to the appearance of jaundice in any young infant and initiate an appropriate workup.

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