PMC6759724_01
Abdominal MRI findings. MRI examination was performed in the patient at age 2 years. It showed hepatosplenomegaly, portal tract edema, and a slightly low signal intensity of the liver on T2-weighted imaging (A). MRI combined with diffusion-weighted imaging (DWI) examination was performed in the patient at age 5 years (B-D). The nodules showed a slightly high signal intensity on T2-weighted imaging (B, white arrows). The nodules showed restricted diffusion on DWI (C, white arrows). Enlarged retroperitoneal lymph nodes showed restricted diffusion on DWI (D, gray arrows).
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F1
What's more, these nodules and lymph nodes had diffusion restriction on diffusion-weighted imaging (DWI) (Figure 1).
Histopathology images of liver stained by hematoxylin-eosin. Liver histology in the patient at age 2 years (A,B). It showed a typical picture of cirrhosis with regenerative nodules of hepatocytes separated by fibrous septa (A, x100). It also shows hydropic degeneration, microvesicular fatty droplets (white arrows), bile duct proliferation (black arrows), and inflammatory cells infiltration (B, x200). Liver histology in the patient at age 4 years (C,D). It showed lower hydropic degeneration rate and fewer inflammatory cells infiltration.
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F2
However, we didn't observe the portal area in this specimen (Figure 2).