case_images: PMC10834036_01
case_images: Trend in liver enzymes over time. AST, aspartate aminotransferase; ALT, alanine aminotransferase; ALP, alkaline phosphatase; ULN, upper limit of normal.
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case_images: PMC10834036_01_crg-2024-0018-0001-536117_F01.jpg
case_images: F1
case_images: Remaining pertinent laboratory studies included alkaline phosphatase (ALP) of 293 U/L, alanine aminotransferase (ALT) of 53 U/L, aspartate aminotransferase (AST) of 91 U/L (Fig. 1), total bilirubin 0.8 mg/dL with direct bilirubin 0.3 mg/dL, prothrombin time 16.7 s, INR 1.33, albumin 2.9 g/dL, and ferritin 199 mug/L.
case_images: Magnetic resonance imaging of the liver, (a) axial and (b) coronal view showing atrophic right lobe, enlarged left lateral segment.
case_images: crg-2024-0018-0001-536117_F02.jpg
case_images: PMC10834036_01_crg-2024-0018-0001-536117_F02.jpg
case_images: F2
case_images: Magnetic resonance imaging of the liver (Fig. 2) showed atrophic right lobe, enlarged lateral segment of left lobe, and caudate lobe suggestive of chronic liver disease.
case_images: Hepatic changes in prolidase deficiency. a Inflamed portal area with pale periportal hepatocytes indicative of chronic cholestasis (H&E, x200). b Small portal area with a slit-like portal vein (arrow) (H&E, x400). c Perisinusoidal fibrosis was present near central veins (Masson trichrome, x200). d Regenerative nodularity, demonstrated by curved sinusoidal spaces (arrows). (Reticulin, x200). e Abnormal staining of sinusoidal endothelial cells for CD34 (anti-CD34, x100). f Periportal hepatocytes expressing keratin 7 consistent with chronic cholestasis (anti-keratin 7, x200).
case_images: crg-2024-0018-0001-536117_F03.jpg
case_images: PMC10834036_01_crg-2024-0018-0001-536117_F03.jpg
case_images: F3
case_images: At age 24, she had a trans-jugular liver biopsy (Fig. 3) which showed mild predominantly lobular hepatitis associated with focal consolidation with hemorrhage, sinusoidal dilatation and mild perisinusoidal and periportal fibrosis, copper accumulation suggestive of chronic cholestasis.
case_images: PMC10834036_02
case_images: PMC10834036_03