Definition: PFIC Type 3 (progressive familial intrahepatic cholestasis)

Progressive familial intrahepatic cholestasis (PFIC-3) is an inherited gene mutation (MDR3) that affects the canalicular phosphatidylcholine transporter.  Unlike the other PFIC mutations, this one has markedly elevated GGT levels and extensive bile duct proliferation with periportal fibrosis.

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