Hepatocellular Cholestasis and Pruritus: Beyond Clinical Treatment

Abstract

Hepatocellular cholestasis in infants can be caused by hepatitis, alfa-1 antitrypsin deficiency, inborn errors of bile acid synthesis, drugs, or total parenteral nutrition. Occasionally, Alagille Syndrome and Progressive Familiar Intrahepatic Cholestasis (PFIC) may result in intrahepatic cholestasis, with an incidence of 1 in 90 000. The affected patients present in infancy or childhood with intermittent painless jaundice. The most apparent and debilitating symptom associated with this disorder is pruritus, which may be out of all proportion to the hyperbilirubinemia and often leads to self-mutilation from scratching. This results in a very poor quality of life, and treatment is very difficult because traditional drugs used to treat itching are typically ineffective [1] [2] [3].