Article
[Clinical aspects, results of diagnostic pulmonary function tests and biochemical parameters in children with homozygote alpha-1-antitrypsin deficiency].
Pneumologie (Stuttgart, Germany) - 1 Oct 1990
Paul K, Heck B, Schulz V, Ebert W, Trefz G, Feist D
Abstract excerpt
The clinical course and pulmonary function tests of individuals with severe Alpha-1-Antitrypsin (Alpha-1-AT) deficiency reveal a marked interindividual variability. 4 patients with PI type ZZ and 2 patients with PI type SZ had been identified by neonatal cholestasis. None had pulmonary symptoms a...
Topics
- Adolescent
- Child
- Female
- Humans
- Lung
- Male
- Metabolism, Inborn Errors
- Phenotype
- Protease Inhibitors
- Respiratory Function Tests
- alpha 1-Antitrypsin
- alpha 1-Antitrypsin Deficiency
