Article
[Substitution therapy in alpha 1-protease inhibitor deficiency (alpha 1-antitrypsin deficiency)].
Schweizerische Rundschau fur Medizin Praxis = Revue suisse de medecine Praxis - 26 Nov 1991
Stössel U, Brändli O
Abstract excerpt
Deficiency of alpha 1-protease inhibitor is a dominant autosomally inherited error of metabolism leading to destruction of alveolar septa by proteolytic enzymes mainly released by neutrophils often before the fifth decade. Diagnosis and determination of phenotype are achieved by serologic tests. Affected individuals have to be informed about the accelerated evolution of the lung disease by smoking and the...
Topics
- Female
- Humans
- Infusions, Intravenous
- Metabolism, Inborn Errors
- Middle Aged
- Pedigree
- Phenotype
- alpha 1-Antitrypsin
- alpha 1-Antitrypsin Deficiency