PHILIP E. PALMER, M.D.; GHERARDO J. GHERARDI, M.D.; JOSEPH M. BALDWIN, M.D.; HUBERT J. WOLFE, M.D.
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Liver disease associated with alpha-1-antitrypsin deficiency, classically described in protease inhibitor (Pi)-type ZZ phenotype children, characteristically shows variable fibrosis or cirrhosis and distinctive periodic acid-Schiff-positive (diastase-resistant) globules, containing alpha-1-antitrypsin, within periportal hepatocytes. In adults, liver involvement may occur without lung disease, and severely affected heterozygotes (non-ZZ individuals) have been reported. However, the significance of alpha-1-antitrypsin liver disease in adults remains undefined (1, 2).
Case 1, a 73-year-old man, presented with biochemical evidence of liver disease in 1971 during hospitalization for unrelated medical problems. Total bilirubin was 1.0 mg/dl (normal < 1.0), alkaline phosphatase 145 IU (normal, 9 to 35), serum
PALMER PE, GHERARDI GJ, BALDWIN JM, et al. Adult Liver Disease in SZ Phenotype Alpha-1-Antitrypsin Deficiency. Ann Intern Med. 1978;88:59–60. doi: https://doi.org/10.7326/0003-4819-88-1-59
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Published: Ann Intern Med. 1978;88(1):59-60.
Gastroenterology/Hepatology, Liver Disease.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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