case_images: PMC5733121_01
case_images: Liver biopsy. Severe inflammatory response comprised of neutrophils and lymphocytes with hepatocellular necrosis. No plasma cells or eosinophils and no bile duct damage or fibrosis. H&E stain, x400.
case_images: CRIHEP2017-9627452.001.jpg
case_images: PMC5733121_01_CRIHEP2017-9627452.001.jpg
case_images: fig1
case_images: A liver biopsy was performed which demonstrated severe acute and chronic hepatitis, destruction of the limiting plate, and hepatocyte necrosis, but initially without a specific etiology (Figure 1).
case_images: Immunohistochemistry for alpha-1-antitrypsin proteins. Diffuse strong positivity within hepatocytes and Kupffer cells indicated by the brown staining. IHC, x400.
case_images: CRIHEP2017-9627452.002.jpg
case_images: PMC5733121_01_CRIHEP2017-9627452.002.jpg
case_images: fig2
case_images: PAS-positive granules were alpha-1-antitrypsin proteins, immunohistochemistry for alpha-1-antitrypsin was performed, which was definitely positive (Figure 2). Phenotyping for alpha-1-antitrypsin was performed and the patient was heterozygous PiEM (normal PiMM).