Progressive Familial Intrahepatic Cholestasis Clinical Presentation

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

History

The following may be noted in the history of a patient with progressive familial intrahepatic cholestasis (PFIC)

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Physical

See the list below:

  • Pruritus

    • Scratching

    • Cutaneous mutilation

    • Irritability in infants

  • Jaundice

    • Scleral icterus

    • Cutaneous jaundice

  • Hepatomegaly

  • Splenomegaly

  • Altered anthropometrics

  • Reduced height

  • Reduced weight for height

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Causes

Progressive familial intrahepatic cholestasis is a genetically determined autosomal recessive disorder. Consanguinity is a major risk factor. [9]

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