I ntrahepatic C holestasis PFIC 13 Definition A heterogeneous group of disorders in which cholestasis of hepatocellular origin often presents in the neonatal period or first year of life ID: 934760
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Slide1
Progressive Familial Intrahepatic Cholestasis(PFIC 1~3)
Slide2DefinitionA heterogeneous group of disordersin 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)
Slide3EpidemiologyAbout1/50,000 to 1/100,000 birthMale : Female = 1 : 1
Slide4Slide5Slide6Slide7TreatmentUDCA 모든 종류의 PFIC에서 초치료로 사용해 볼 수 있다. PFIC3에서 잘 듣는 경향
.
Surgical Biliary diversion
PFIC1,2
에서 도움이 될 수 있다
.
Liver transplantation
이후의 유일한 방법
Slide8PrognosisPortal HTN, Liver failure, Cirrhosis, HCCBiliary stones, Drug induced cholestasis, Intrahepatic cholestasis of pregnancy