CLiC Cholestatic Liver Disease Consortium

PFIC (Progressive Familial Intrahepatic Cholestasis)

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PFIC1, liver biopsies at 2 and 8 years in one patient. No fibrosis.

PFIC2 in an infant. (Clinicopathological diagnosis only).

PFIC3 in an infant with profound intrahepatic cholangiopathy.

Coarse granular "Byler bile" in a dilated canaliculus

Compare canalicular bile in biliary atresia.

Compare canalicular bile in estrogen-induced cholestasis.

Compare canalicular bile in idiopathic neonatal hepatitis.

Byler bile in canaliculus, example #2.

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Alagille Syndrome

Alpha-1 Antitrypsin Deficiency

Bile Acid Synthesis Defects

Mitochondrial Hepatopathies

> PFIC (Progressive Familial Intrahepatic Cholestasis)