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P rogressive F amilial I ntrahepatic C holestasis (PFIC 1~3)

P rogressive F amilial I ntrahepatic C holestasis (PFIC 1~3). Definition. A heterogeneous group of disorders in which cholestasis of hepatocellular origin often presents in the neonatal period or first year of life

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P rogressive F amilial I ntrahepatic C holestasis (PFIC 1~3)

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  1. Progressive Familial Intrahepatic Cholestasis(PFIC 1~3)

  2. Definition • A heterogeneous group of disorders • in which cholestasis of hepatocellular origin often presents in the neonatal period or first year of life • and lead to death from liver failure at young ages (infancy to adolescence)

  3. Epidemiology • About 1/50,000 to 1/100,000 birth Male : Female = 1 : 1

  4. Treatment • UDCA • 모든 종류의 PFIC에서 초치료로 사용해 볼 수 있다. PFIC3에서 잘 듣는 경향. • Surgical Biliary diversion • PFIC1,2에서 도움이 될 수 있다. • Liver transplantation • 이후의 유일한 방법

  5. Prognosis • Portal HTN, Liver failure, Cirrhosis, HCC • Biliary stones, Drug induced cholestasis, Intrahepatic cholestasis of pregnancy

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