APPARENT TOTAL ALPHA1-ANTITRYPSIN DEFICIENCY - REPORT OF A CASE

  • 1 January 1976
    • journal article
    • research article
    • Vol. 12  (7) , 678-685
Abstract
A 29 yr old female, with chronic renal failure and chronic bilateral emphysema, was admitted with severe uremia and septicemia secondary to multiple abscesses in the right kidney. Her condition improved after right nephrectomy. Pulmonary function studies showed marked obstructive and restrictive lung disease consistent with the diagnosis of primary emphysema. On biochemical and histological examination, the liver was normal. .alpha.1-antitrypsin could not be demonstrated in the patient''s serum at normal pH by any of the known techniques, but protein molecules with .alpha.1-antitrypsin antigenicity were found at pH 4.8. This suggests a pH-dependent structural difference in .alpha.1-antitrypsin protein. Starch gel electrophoresis gave a multibanding pattern not previously described. A new form of apparent total .alpha.1-antitrypsin deficiency is postulated.

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