Table 1.

Liver histological and ultrastructural features in the spectrum of PFIC2 and zebrafish abcb11b mutant.

Histological features
	Patient A	Patient B	Zebrafish abcb11b mutant
Lobular structure	Largely preserved	Distorted	Largely preserved
Hepatocyte swelling	Yes	Yes	Yes
Hepatocyte necrosis	Yes	Yes	Yes
Giant cells	Yes	Yes	Not evident
Fibrosis	Moderate	Severe	Mild
Inflammation	Mild	Mild	Not evident
Bile duct paucity or ductular reaction	No	No	No
Ultrastructural changes
Amorphous bile-like granular deposits	Yes	Yes	Yes
Bile canaliculi dilatation, effacement of microvilli	Yes	Yes	Yes
Glycogen depletion	Yes	Yes	Yes
Mitochondria pleomorphism	Yes	No	Yes
Swelling of ER	Yes	Yes	Not evident