PT  - JOURNAL ARTICLE
AU  - Mohamed Badawy
AU  - Atef Farouk
AU  - Hamdy Mohammdien
TI  - Clinical features of alpha one antitrypsin deficiency in non cystic fibrosis bronchiectasis
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - P2277
VI  - 40
IP  - Suppl 56
4099  - http://erj.ersjournals.com/content/40/Suppl_56/P2277.short
4100  - http://erj.ersjournals.com/content/40/Suppl_56/P2277.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - Setting: It's important to identify manifestation of alpha1-antitrypsin deficiency (A1ATD) in bronchiectasis to improve patients care and outcome. Objective: To clinically evaluate A1ATD in patients with non cystic fibrosis bronchiectasis.Material and methods: Patients with non cystic fibrosis bronchiectasis were diagnosed clinically and confirmed radiologically. They fulfilled the inclusion criteria and divided into group (A) bronchiectasis with hyperinflation (30cases) and group (B) bronchiectasis without hyperinflation (30 cases). All patients were subjected to history taking, pulmonary function tests, and quantitative measurements of serum A1AT by radio-immunoassay. Results: Mean age of both groups was (50±8.58) and (36.87±11.35) respectively (p=0.001). There were significant difference in gender distribution (p=0.006), and smoking history (p=0.001). Heamoptysis presented in 12 cases (40%), and 20 cases (66.67%) in both groups respectively (p=0.04). Dyspnea presented in 27 cases (90%) and 19 cases (63%) for group A&B with (P=0.02). There were no significant difference in sinusitis, hepatological symptoms, clubbing and family history. There were significant difference in cyanosis, oedema of lower limb, wheeze, radiological findings and spirometeric tests (P value 0.01, 0.004, 0.001, 0.001, 0.001) respectively. Three cases (5%) of A1ATD were diagnosed among all patients' one case (1.5%) in group (A) of MZ allele and two brother cases (3.5%) in group (B) of SZ allele without statistical significance. Conclusion: A1ATD is seldom found in patients with bronchiectasis even with concomitant hyperinflation. Inheritance could influence an individual risk of A1ATD for developing bronchiectasis.