Progressive Familial Intrahepatic Cholestasis Follow-up

Updated: Apr 26, 2017
  • Author: Andrew J Wehrman, MD; Chief Editor: Carmen Cuffari, MD  more...
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Follow-up

Transfer

Transfer patients with progressive familial intrahepatic cholestasis (PFIC) to an appropriate transplant center, as the disease will progress to end-stage liver disease (ESLD).

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Complications

Complications are those of chronic cholestasis and include the following:

  • Fat malabsorption
  • Fat-soluble vitamin deficiency
  • Pruritus
  • Hyperlipidemia
  • Growth failure
  • Progression to ESLD in patients without successful biliary diversions
  • Hepatocellular carcinoma in PFIC2
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Prognosis

Progressive familial intrahepatic cholestasis results in ESLD if not diagnosed before the development of cirrhosis. Early diagnosis and biliary diversion may prevent significant morbidity and mortality from ESLD. Hepatocellular carcinoma is also a major risk in patients with PFIC2.

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Patient Education

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