Evaluation of Alpha-1 Antitrypsin Level in the Serum of Children With Idiopathic Bronchiectasis
Abstract
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 hospitalizations, 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 one year of age, 6.6% 1-5 years, 6.6% 5-10 years, and 6.6% in more than ten 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.
2. Cortese R, Mennitti MC, Mariani F, et al. Bronchiectasis in patients with alpha1-antitrypsin deficiency: prevalence and characteristics. Eur Respiratory Soc 2016; 48: PA737.
3. Chalmers JD, Chang AB, Chotirmall SH, Dhar R, McShane PJ. Bronchiectasis (Primer). Nat Rev Dis Primers 2018; 4 (1): 45.
4. Tsang K, Tipoe G. Bronchiectasis: not an orphan disease in the East. Int J Tuberc Lung Dis 2004; 8 (6): 691-702.
5. McShane PJ, Naureckas ET, Tino G, Strek ME. Non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med 2013; 188 (6): 647-56.
6. Shoemark A, Ozerovitch L, Wilson R. Aetiology in adult patients with bronchiectasis. Respir Med 2007; 101(6): 1163-70.
7. Stroh G, Scrodin MD. Bronchiectasis in alpha-1 antitrypsin deficiency: a single-center review. Chest 2018; 154 (4): 7A.
8. Mulgrew AT, Taggart CC, Lawless MW, et al. Z α1-antitrypsin polymerizes in the lung and acts as a neutrophil chemoattractant. Chest 2004; 125 (5): 1952-1957.
9. Chan ED, Kaminska AM, Gill W, et al. Alpha-1-antitrypsin (AAT) anomalies are associated with lung disease due to rapidly growing mycobacteria and AAT inhibits Mycobacterium abscessus infection of macrophages. Scand J Infect Dis 2007; 39 (8): 690-696.
10. Greulich T, Nell C, Herr C, et al. Results from a large targeted screening program for alpha-1-antitrypsin deficiency: 2003 - 2015. Orphanet J Rare Dis 2016; 11(1): 75.
11. Badawy MS, El Qarn AF, Mohamadeen HA. Alpha 1 antitrypsin deficiency in non-cystic fibrosis bronchiectasis. Egypt J Chest Dis Tuberc 2013; 62 (2): 311-317.
12. Costa A.F.C.E., Farinha I. Bronchiectasis in alpha1-antitrypsin deficiency. ERJ 2019; 54: PA4359.
13. Parr DG, Guest PG, Reynolds JH, Dowson LJ, Stockley RA. Prevalence and impact of bronchiectasis in α1-antitrypsin deficiency. Am J Respir Crit Care Med 2007; 176 (12): 1215-1221.
14. Lonni S, Chalmers JD, Goeminne PC, et al. Etiology of non-cystic fibrosis bronchiectasis in adults and its correlation to disease severity. Ann Am Thorac Soc 2015; 12 (12): 1764-1770.
15. Amorim A, Gamboa F, Sucena M, et al. Recommendations for aetiological diagnosis of bronchiectasis. Rev Port Pneumol 2016; 22 (4): 222-235.
16. Satırer O, Yesil AM, Emiralioglu N, et al. A review of the etiology and clinical presentation of non-cystic fibrosis bronchiectasis: a tertiary care experience. Respir Med 2018; 137: 35-39.
17. Karadag B, Karakoc F, Ersu R, Kut A, Bakac S, Dagli E. Non-cystic-fibrosis bronchiectasis in children: a persisting problem in developing countries. Respiration 2005; 72 (3): 233-238.
18. Doğru D, Nik-Ain A, Kiper N, et al. Bronchiectasis: the consequence of late diagnosis in chronic respiratory symptoms. J Trop Pediatr 2005; 51 (6): 362-365.
19. Nag VK, Nandan D, Singh S, Arora SK, Tsanglao W, Arya N. Pulmonary tuberculosis: a predominant cause of childhood bronchiectasis in India. J. Commun. Dis 2015; 47 (1): 14-18.
20. Kim H-Y, Kwon J-W, Seo J, et al. Bronchiectasis in children: 10-year experience at a single institution. Allergy Asthma Immunol Res 2011; 3 (1): 39-45.
| Files | ||
| Issue | Vol 59, No 11 (2021) | |
| Section | Original Article(s) | |
| DOI | https://doi.org/10.18502/acta.v59i11.7775 | |
| Keywords | ||
| Alpha-1 antitrypsin Idiopathic bronchiectasis Nephelometry | ||
| Rights and permissions | |
|
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
