Diagnosis and management of cholestatic liver disease.

Cholestasis (slowing of bile flow) may be acute or chronic and affect any age group. In infants and children the causes often are congenital or inherited and as a result of improved management some affected children now survive to adulthood. Although jaundice is a hallmark of cholestasis it may be absent, particularly in adults with chronic cholestatic liver disease most of whom are entirely asymptomatic. A detailed history and physical are crucial to the diagnosis and noninvasive radiologic tests (ultrasound, computerized tomography scan, and magnetic resonance cholangiography) greatly facilitate diagnosis, particularly when the cause is extrahepatic. Only if sufficient portal tracts (>10) are present on liver biopsy examination can this test reliably evaluate damage to the small bile ducts. Therapy should address both the cause and the consequences of retained bile acids within the liver, and diminished delivery of bile to the gastrointestinal tract. Therapies should address symptoms, mostly pruritus and prevention, particularly osteoporosis and osteomalacia. Portal hypertension can be an early event in chronic cholestatic liver disease, sometimes occurring before the development of cirrhosis. Ursodeoxycholic acid improves the biochemical markers of cholestasis regardless of cause and may delay liver disease progression; only liver transplant is potentially curative.