Management

General management:

• nutrition - problem is that of intestinal bile salt deficiency:
  • calorie and protein intake must be maintained but fat reduced. Additional fat supplied by medium chain triglycerides e.g. Portagen or coconut oil
  • fat soluble vitamins - A, D, E, K - may need to be supplemented if signs of deficiency
• pruritus - options:
  • external biliary drainage
  • cholestyramine
  • phototherapy
  • ursodeoxycholic acid
  • rifampicin
• hepatic osteodystrophy may require vitamin D, phosphate or calcium; oestrogens in postmenopausal women

Extrahepatic cholestasis often requires surgical management.

Note that the use of clofibrate therapy in the hyperlipidaemia associated of cholestasis is associated with a raised serum cholesterol.