PMC5733121_01
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.
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fig1
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).
Immunohistochemistry for alpha-1-antitrypsin proteins. Diffuse strong positivity within hepatocytes and Kupffer cells indicated by the brown staining. IHC, x400.
CRIHEP2017-9627452.002.jpg
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fig2
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).