Cell organelles and intracellular trafficking
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A 37-year-old man who has never smoked is evaluated for progressive exertional dyspnea. Chest CT shows emphysema that is most pronounced at the lung bases. He also has persistently elevated aminotransferases. His serum alpha-1 antitrypsin concentration is 32 mg/dL (reference, 100–220). A liver biopsy shows diastase-resistant, periodic acid–Schiff-positive globules within hepatocytes.