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Deficiency of alpha1-antitrypsin and bronchiectasis
Annals of Clinical Research
|April 1, 1978
Summary
Alpha-1 antitrypsin deficiency, a genetic condition, was found in 10% of bronchiectasis patients, a significantly higher rate than in controls. This suggests a potential link between alpha-1 antitrypsin deficiency and the development of bronchiectasis.
Area of Science:
- Pulmonology
- Genetics
Background:
- Bronchiectasis is a chronic respiratory condition characterized by permanent enlargement of the airways.
- Alpha-1 antitrypsin deficiency is an inherited disorder that can cause lung and liver disease.
Purpose of the Study:
- To investigate the prevalence of alpha-1 antitrypsin deficiency in patients with bronchiectasis.
- To determine if there is a statistically significant association between alpha-1 antitrypsin deficiency and bronchiectasis.
Main Methods:
- Screening of 60 consecutive bronchiectasis patients for alpha-1 antitrypsin deficiency.
- Genotyping (PiMZ) to identify carriers of the deficiency.
- Comparison of PiMZ frequency in patients versus a control group.
Main Results:
- Six cases (10%) of alpha-1 antitrypsin deficiency (PiMZ genotype) were identified in the bronchiectasis cohort.
- The frequency of PiMZ in bronchiectasis patients was significantly higher (p < 0.005) than in the control group (2.7%).
- Two patients with bronchiectasis also had cystic fibrosis.
Conclusions:
- Alpha-1 antitrypsin deficiency is more prevalent in patients with bronchiectasis than in the general population.
- Proteolytic damage associated with alpha-1 antitrypsin deficiency may contribute to the pathogenesis of bronchiectasis.