Evaluation of Alpha-1 antitrypsin level in the serum of children with idiopathic bronchiectasis

Bronchiectasis is a clinical syndrome characterized by chronic cough, sputum production, recurrent respiratory infections, and permanent bronchial dilation.  The association between the level of alpha-1 antitrypsin (AAT) and bronchiectasis is controversial. In this study, we aimed to investigate this association in children with idiopathic bronchiectasis.

  The study was conducted on 20 patients with idiopathic bronchiectasis as the case group (mean age 15.9±2.1) and 20 healthy individuals as the control group (mean age 14.9±2.6). Serum AAT level was measured using nephelometric analysis (g/L). Other criteria including sex, parent consanguinity, number of hospitalization, age of the first symptom were evaluated in both groups related to AAT level.

The mean serum level of AAT in the case and control groups were 1.3±0.29; 1.5 ±0.59, respectively with statistical significance (P=0.001). There was a significant difference between the two groups in the AAT level distribution, according to AAT normal range (p=0.01). The case group had a more positive attitude toward consanguinity than the control group (66.7% versus 33.3%; P<0.001). The results showed that 80% of patients had the first symptom of disease under 1 year of age, 6.6% 1- 5 years, 6.6% 5- 10 years, and 6.6% in more than 10 years old. In the case group, 53.3% had a history of medical hospitalization for one time, 26.7% two times, while 20% of the patients had no medical hospitalization.

Decreased AAT serum level and high consanguinity rates may be considered as two risk factors for idiopathic bronchiectasis occurrence in children.