A 32-year-old man has progressive dyspnea with reduced breath sounds at both lung bases. Serum ALT is mildly elevated. Liver biopsy shows periodic acid-Schiff positive, diastase-resistant eosinophilic globules within periportal hepatocytes. Which protein accumulates in these hepatocytes?

• A Fibrinogen degraded by hepatocyte proteases
• B Misfolded alpha-1 antitrypsin retained in the endoplasmic reticulum ✓
• C Ceruloplasmin bound to excess copper
• D Immunoglobulin light chains phagocytosed by hepatocytes

Explanation

In PiZZ alpha-1 antitrypsin deficiency, the Z mutant protein misfolds and is retained within hepatocyte endoplasmic reticulum, forming PAS positive, diastase resistant globules concentrated in periportal hepatocytes. The same protein causes panacinar, basally predominant emphysema because circulating antiprotease levels are deficient. Ceruloplasmin accumulation points to Wilson disease, which shows copper granules rather than PASD globules.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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