Skip to main content
NCBI home page
Thorax logoLink to Thorax
. 1995 Apr;50(4):424–425. doi: 10.1136/thx.50.4.424

Bronchiectasis and homozygous (P1ZZ) alpha 1-antitrypsin deficiency in a young man.

W Rodriguez-Cintron 1, K Guntupalli 1, A E Fraire 1
PMCID: PMC474302  PMID: 7785020

Abstract

Pulmonary complications of alpha 1-antitrypsin deficiency are most commonly manifested by panlobular emphysema. This report describes histologically proven bronchiectasis in a 21 year old man with massive haemoptysis and homozygous deficiency of alpha 1-antitrypsin. Neither panlobular emphysema nor cirrhosis of the liver were present. Bronchiectasis must be considered part of the spectrum of the pulmonary pathology that may be encountered in individuals with alpha 1-antitrypsin deficiency.

Full text

PDF
424

Images in this article

424Image
on p.424

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Gadek J. E., Fells G. A., Zimmerman R. L., Rennard S. I., Crystal R. G. Antielastases of the human alveolar structures. Implications for the protease-antiprotease theory of emphysema. J Clin Invest. 1981 Oct;68(4):889–898. doi: 10.1172/JCI110344. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Hubbard R. C., Brantly M. L., Sellers S. E., Mitchell M. E., Crystal R. G. Anti-neutrophil-elastase defenses of the lower respiratory tract in alpha 1-antitrypsin deficiency directly augmented with an aerosol of alpha 1-antitrypsin. Ann Intern Med. 1989 Aug 1;111(3):206–212. doi: 10.7326/0003-4819-111-3-206. [DOI] [PubMed] [Google Scholar]
  3. Kagan E., Soskolne C. L., Zwi S., Hurwitz S., Maier G. M., Ipp T., Rabson A. R. Immunologic studies in patients with recurrent bronchopulmonary infections. Am Rev Respir Dis. 1975 Apr;111(4):441–451. doi: 10.1164/arrd.1975.111.4.441. [DOI] [PubMed] [Google Scholar]
  4. Larsson C. Natural history and life expectancy in severe alpha1-antitrypsin deficiency, Pi Z. Acta Med Scand. 1978;204(5):345–351. doi: 10.1111/j.0954-6820.1978.tb08452.x. [DOI] [PubMed] [Google Scholar]
  5. Longstreth G. F., Weitzman S. A., Browning R. J., Lieberman J. Bronchiectasis and homozygous alpha1-antitrypsin deficiency. Chest. 1975 Feb;67(2):233–235. doi: 10.1378/chest.67.2.233. [DOI] [PubMed] [Google Scholar]
  6. MOLLER B., KRISTENSEN H. S. ON THE TREATMENT OF TETANUS. Acta Med Scand. 1965 Jan;177:1–6. doi: 10.1111/j.0954-6820.1965.tb01796.x. [DOI] [PubMed] [Google Scholar]
  7. Scott J. H., Anderson C. L., Shankar P. S., Stavrides A. Alpha1-antitrypsin deficiency with diffuse bronchiectasis and cirrhosis of the liver. Chest. 1977 Apr;71(4):535–538. doi: 10.1378/chest.71.4.535. [DOI] [PubMed] [Google Scholar]
  8. Stockley R. A. Chronic bronchitis: the antiproteinase/proteinase balance and the effect of infection and corticosteroids. Clin Chest Med. 1988 Dec;9(4):643–656. [PubMed] [Google Scholar]
  9. Stockley R. A. Chronic bronchitis: the antiproteinase/proteinase balance and the effect of infection and corticosteroids. Clin Chest Med. 1988 Dec;9(4):643–656. [PubMed] [Google Scholar]
  10. Tobin M. J., Cook P. J., Hutchison D. C. Alpha 1 antitrypsin deficiency: the clinical and physiological features of pulmonary emphysema in subjects homozygous for Pi type Z. A survey by the British Thoracic Association. Br J Dis Chest. 1983 Jan;77(1):14–27. doi: 10.1016/0007-0971(83)90002-5. [DOI] [PubMed] [Google Scholar]

Articles from Thorax are provided here courtesy of BMJ Publishing Group

RESOURCES