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. 2022 Jul 23;188(10):2920–2931. doi: 10.1002/ajmg.a.62906

FIGURE 3.

FIGURE 3

(a) Liver echography in different years: a. Year 2004, b. Year 2007, c. Year 2009, d. Year 2013, e. Year 2015, and f. Year 2016. In this figure it is shown the evolution of liver involvement in our patients in a time span of 12 years, seen on ultrasound scan. He developed a progressive cirrhosis, with a micro‐ and macronodular pattern, predominantly in right liver sections. Portal vein and its intrahepatic branches are dilated. Irregularity in the Glisson's capsule contour. in figure “f” it is possible to observe a massive ascites. The intra and extra‐hepatic biliary tree is not dilated. (b) a, b Abdominal CT: severe reduction in the dimension of the liver, predominantly involving the right sections of the liver, with a clear micro and macronodular pattern, in relation to the cirrhotic involution of the liver. Portal vein and its branches are markedly dilated, with an arterialization of the hepatic parenchyma; activation of the paraumbilical and periesophageal collateral pathways. Extra‐ and intrahepatic biliary tree is in the range of normality. it is possible to observe an important splenomegaly with homogeneous densitometry. Presence of free abdominal fluid. The diaphragmatic crus are bilaterally thickened. Levoscoliosis of the spine.