PMC7793769_01
Laboratory data. (A) Direct bilirubin (DB), aspartate aminotransferase (AST), and alanine aminotransferase (ALT). (B) Procollagen type III peptide (P-III-P) and type IV collagen.
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However, his cholestasis progressively worsened (Figure 1A), and acholic stools were observed. DB, AST, and ALT levels peaked at 2 months of age and improved gradually thereafter (Figure 1A). The levels of serum markers of liver fibrosis, including procollagen type III peptide (P-III-P, 622 ng/mL, reference range: 3.6 to 9.5 ng/mL) and type IV collagen (1,154 ng/mL, reference range: <150 ng/mL), increased at 3 months of age; however, they gradually decreased thereafter (Figure 1B).
Light microscopic images of the liver biopsy specimen obtained at 1.5 months of age. (A,B) Hematoxylin and eosin staining demonstrated inflammatory cell infiltration and moderate intrahepatic cholestasis. (A) The scale bar represents 200 mum. (B) The scale bar represents 50 mum. (C) Higher-magnification images of hematoxylin and eosin-stained liver specimens demonstrating intrahepatic cholestasis. The scale bar represents 20 mum. (D) Most of the infiltrating inflammatory cells were CD68-positive macrophages. The scale bar represents 50 mum. (E) Small numbers of CD20-positive B lymphocytes were also observed. The scale bar represents 50 mum. (F,G) Azan staining demonstrated mild pericellular fibrosis. (F) The scale bar represents 200 mum. (G) The scale bar represents 50 mum.
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Liver pathology tests showed intrahepatic cholestasis with a few bile plugs within the bile ducts (Figures 2A-C). Moderate inflammatory cell infiltration was also observed, mainly consisting of CD68-positive macrophages and small numbers of CD20-positive B lymphocytes (Figures 2D,E). Azan staining showed mild pericellular fibrosis surrounding hepatocytes, with slight fibrosis in the portal vein area (Figures 2F,G).