Table 2.

Liver Biopsy Finding in PFIC Subtypes.

	PFIC-1 (N = 2)	PFIC-2 (N = 19)	PFIC-3 (N = 3)
Liver biopsy
(1) Giant cell transformation	–	16	0
(2) Bile ductular proliferation	–	0	3
(3) Cholestasis	2	19	2
(4) Fibrosis grading (F)	F0—2	F0—2, F1—3, F2—6, F3—3, F4—5	F3—1, F4—2
Immunostaining	BSEP+	BSEP absent: 11	MDR-3 absent in all 3 cases
		BSEP reduced: 8