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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