Management Pruritis		Pruritis is a common complication of chronic cholestasis, often appearing as early as 3 months of age. It can significantly impact on the quality of life; in fact, in severe cases, it can be an indication for liver transplant. It is difficult to manage, primarily because the etiology is obscure. Bile acid levels do not consistently correlate with or accurately predict pruritis. Nevertheless, treatment with the bile acid-binding resin, cholestyramine, has been tried in the past. Its complications include steatorrhea, acidosis, diarrhea and intestinal obstruction. Some research has implicated endogenous opioids in pruritis, leading to the use of opioid-antagonists like naloxone. Withdrawal-like symptoms and "breakthrough" phenomenon - a worsening of the pruritis after an initial improvement - have limited their usefulness. Recently the serotonin neurotransmitter system has been studied in pruritis, leading to the use of ondansetron in a few patients. Phenobarbital, 5-10 mg/kg/day, stimulates bile acid-independent bile flow and decreases bile acid levels. It has commonly been used in the past. However inconsistent results and its sedative side-effects have limited its efficacy. Rifampin, 10 mg/kg/day, another microsomal enzyme inducer, has also been tried. It does not cause sedation, however prolonged use can lead to hepatotoxicity. Antihistamines should always be used, but are seldom effective alone. Ursodeoxycholic acid (UDCA) or ursodiol, 15-30 mg/kg/day, a naturally occurring bile acid which alters bile acid composition and increases bile flow, has successfully been used at many pediatric GI centers. Finally external biliary diversion may be used as a last resort before liver transplant. Return to top of page