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Table 2 Histologic comparison between congenital hepatic fibrosis (CHF) and its mimics

From: Congenital hepatic fibrosis and its mimics: a clinicopathologic study of 19 cases at a single institution

Histologic parameters CHF (n=13) Mimics (n=6) P-value
Biliary changes Bile ductular-like proliferation 13 3 <0.05
Dilated duct at the center 12 3 N.S.
Linear lining of ectatic bile ducts along the limiting plate 12 1 <0.01
Bile ducts crossing limiting plates 9 1 N.S.
Bile duct fusion and anastomosing 11 2 <0.05
VMC/VMC-like structures 6 0 N.S.
Cholangitis (lymphocytic or neutrophilic) 10 2 N.S.
Bile duct loss (% loss) 0 1 (68% loss) N.S.
Vascular changes Portal vein obliteration/hypoplasia 13 6 N.S.
Portal vein dilation/herniation 0 6 <0.001
Arterial hyperplasia 13 6 N.S.
Nodular regenerative hyperplasia 1 4 <0.05
Cholestasis Bile plugs in bile ducts and ductules 6 0 N.S.
Bile plugs in canaliculi (lobular cholestasis) 1 0 N.S.
Hepatocytic cholestasis 1 0 N.S.
Bile infarcts 0 0 N.S.
Cholate stasis 0 0 N.S.
Inflammation Portal inflammation 7 1 N.S.
Interface hepatitis 0 0 N.S.
Lymphoid aggregates 2 0 N.S.
Fibrosis Portal tract expansion with fibrosis 13 1 <0.05
Fibrosis stage: Ludwig (0-4) Stage 1 (portal fibrosis) 1 1 N.S.
Stage 2 (periportal fibrosis) 3 3
Stage 3 (bridging fibrosis) 6 1
Stage 4 (cirrhosis) 3 1