SUPPLEMENTARY FILES

Supplemental figure 1. Flow chart

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Supplemental Figure 2. Distribution of liver stiffness values according to the individual

components of Anali score without gadolinium: dysmorphy (Supplemental Fig. 2a), portal

hypertension (Supplemental Fig.2b), intrahepatic bile duct dilatation (Supplemental Fig. 2c).

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Supplemental table 1. Graphic description of Anali score without gadolinium

Component of Anali score

without gadolinium

Grade Example

Intrahepatic bile duct

dilatation

0: 3 mm

1: 3.1-4.9 mm

2: 5 mm

Dysmorphy 0: absent

1: present

Portal hypertension 0: absent

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1: present

Anali score without gadolinium =

1 x IHBD dilatation + 2 x Dysmorphy + 1 x Portal hypertension

Notes: The grade of IHBD dilation is defined by the greatest IHBD dilatation. Dysmorphy was

defined by the presence of an increase of the modified caudate/right lobe ratio evaluated

with use of the right portal vein to set the lateral boundary of caudate lobe. Portal

hypertension is defined by the presence of portosystemic shunts including left gastric,

paraumbilical, splenorenal and splenogastrorenal shunts with or without splenomegaly as

defined by splenic index higher to 480 cm3.

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Supplemental table 2. Correlations between liver stiffness or Anali score without

gadolinium and biochemical variables.

Liver Stiffness Anali score without gadolinium

Spearman’s P Spearman’s P

Total bilirubin 0.35 <0.001 0.24 <0.01

AST 0.52 <0.001 0.34 <0.001

ALP 0.48 <0.001 0.33 <0.001

GGT 0.50 <0.001 0.40 <0.001

Albumin -0.31 <0.001 -0.26 <0.01

Platelets -0.18 <0.001 -0.09 n.s.

Abbreviations: AST, aspartate aminotransferase; ALP, alkaline phosphatase; GT, gamma-glutamyl

transpeptidase.

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Supplemental table 3. Results of Cox regression analysis for each component of ANALI

score without gadolinium and LS.

Cox regression model including LS and dysmorphy

Variable HR 95%CI p

LS 1.02 1.01-1.04 0.007

Dysmorphy 5.27 2.01-13.85 0.001

Cox regression model including LS and IHBD dilatation

Variable HR 95%CI p

LS 1.03 1.01-1.05 0.001

IHBD dilatation 2.35 1.46-3.78 < 0.001

Cox regression model including LS and portal hypertension

Variable HR 95%CI p

LS 1.02 1.00-1.04 0.035

Portal hypertension 2.83 1.32-6.06 0.007

Cox regression model including LS, dysmorphy, IHBD dilatation and portal hypertension

Variable HR 95%CI p

LS 1.02 1.01-1.04 0.011

Dysmorphy 3.32 1.16-9.46 0.025

IHBD dilatation 1.77 1.05-2.97 0.033

Abbreviations: LS, liver stiffness; HR, hazard ratio; CI, confidence interval; IHBD, intrahepatic

bile duct

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