Progressive Familial Intrahepatic Cholestasis (PFIC) | PFIC Liver

Treatment of PIFC

Treatment of PIFC isn’t curative therapy; it just reduces complications.

Your doctor will prefer surgical interventions in late cases.

Medical therapy

1. Ursodeoxycholic acid helps fat digestion, decreases the cholesterol level, and helps the gall stones’ destruction.
2. Cholestyramine prevents the absorption of bile acids from the gut. It can treat itching by reducing the deposition of bile in the body.
3. Fat-soluble vitamins supplements (A, D, E, and K)

Surgical:

Partial External Biliary Diversion (PEBD):

This surgery aims to reduce bile levels in the liver by using a part of the intestine -about 11 cm- as a tube for bile drainage.

Indications of this surgery:

PIFC patients without liver cirrhosis and resistant to medical therapy.

Liver transplantation:

Most cases of PIFC develop liver cirrhosis and liver cell failure, which requires liver transplantation. It has good outcomes.

Prognosis of PIFC

Without liver transplantation, this disease leads to liver failure and liver cancer, which ends in death.