A 30-year-old man has panacinar emphysema and transaminases two times the upper limit of normal. Liver biopsy shows round, eosinophilic globules within the cytoplasm of periportal hepatocytes that stain strongly with periodic acid-Schiff but persist after diastase digestion. The globules represent accumulation of:
- A Ubiquitinated cytokeratin 8 and 18 filaments
- B Copper complexed with metallothionein
- C Misfolded alpha-1 antitrypsin retained in hepatocytes ✓
- D Ferritin and hemosiderin within lysosomes
Correct answer: C. Misfolded alpha-1 antitrypsin retained in hepatocytes
Explanation
In homozygous PiZZ alpha-1 antitrypsin deficiency, the misfolded Z variant polymerizes and cannot exit the endoplasmic reticulum, forming PAS-positive, diastase-resistant globules in periportal hepatocytes. Ubiquitinated keratin filaments describe Mallory-Denk bodies of alcoholic steatohepatitis, which are also ubiquitin-positive but irregular rope-like inclusions rather than round globules. Copper accumulation occurs in Wilson disease and stains with rhodanine, not PAS.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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