Alpha 1 antitrypsin deficiency in non cystic fibrosis bronchiectasis

[Association between alpha 1 antitrypsin deficiency and cystic fibrosis severity].

OBJECTIVE To ascertain the distribution of alpha 1 antitrypsin genotypes and correlate it with the severity of pulmonary disease in patients with cystic fibrosis. METHOD A clinical and laboratory cross sectional study of 70 patients at the Universidade Estadual de Campinas teaching hospital. Cystic fibrosis diagnoses was confirmed by both clinical and laboratory methods. The severity of cysti...

Non-cystic fibrosis bronchiectasis.

Bronchiectasis is characterized by irreversible widening of the medium-sized airways, with inflammation, chronic bacterial infection and destruction of the bronchial walls. Exercise or inspiratory muscle training may improve quality of life and exercise endurance in people with non-cystic fibrosis bronchiectasis. Prolonged-use antibiotics improve clinical response rates, but may not reduce exac...

Non-cystic Fibrosis Bronchiectasis

Non-cystic fibrosis bronchiectasis.

Bronchiectasis is a chronic debilitating condition with considerable phenotypic diversity. A vicious cycle of infection and inflammation exists in damaged airways with patients suffering from persistent cough, purulent sputum production, recurrent chest infections and general malaise. The associated burden of disease in terms of increased morbidity, reduced quality of life and the socioeconomic...

Emphysema and bronchiectasis secondary to alpha-1 antitrypsin deficiency.

A 47-year-old Caucasian male presented to the chest clinic with a 4-week history of exertional dyspnea. A chest radiograph showed mild hyperinflation without any focal pathology and spirometry showed a mild obstructive defect. In view of symptoms being disproportionate to spirometric and radiologic abnormalities, a thoracic CT scan was obtained. It revealed that there was evidence of bronchiect...