The effect of tamoxifen in intermediate alpha 1-antitrypsin deficiency associated with the phenotype PiSZ
- PMID: 6603815
The effect of tamoxifen in intermediate alpha 1-antitrypsin deficiency associated with the phenotype PiSZ
Abstract
Three patients, one with lung disease and two with liver disease, with intermediate alpha 1-antitrypsin (AAT) deficiency phenotype PiSZ, (plasma AAT concentration approximately 1/3 of normal) were treated with the anti-oestrogen drug, tamoxifen, for 2-24 months. Patients responded with 50-70% increases in plasma AAT concentrations. Progress of emphysema appeared to be retarded in one case but no improvement in liver function was recorded in the other two patients.
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