Bronchiectasis is defined as a condition chracterized by the damage of the localized, irreversible dilation of part of the bronchial tree and the walls of the large airways of the lung as a result of the destruction of the lung muscles and elastic tissues. Bronchiectasis can be present alone, but in most cases, it is a disease coexisted with patient of chronic obstructive pulmonary disease (COPD).
1. Congenital bronchiectasis
In cystic fibrosis, mutations of the CFTR gene lead to diffuse bronchiectasis (DB).and the disease is associated with other diseases including rheumatoid arthritis (RA). In the study to to determine whether the frequency of CFTR mutations was higher in patients with RA-associated DB and to determine whether a causal relationship could be established between the variant and the disease by evaluating its cosegregation with DB within families, found that the frequency of CFTR mutations was higher in family members with RA-DB or DB only than in unaffected relatives (p<0.005 for each comparison) and in unrelated healthy controls (p<0.001 for each comparison) but not in family members with RA only. CFTR mutations were more frequent in family members with RA-DB than in those with RA only (OR 5.30, 95% CI 2.48 to 11.33; p<5×10(-5)). They cosegregated with RA-DB in the families (sib-TDT=10.82, p=0.005)(8).
Alpha-1-antitrypsin (A1AT) is the main inhibitor of neutrophil elastase, and severe alpha-1-antitrypsin deficiency (A1ATD) is a genetic risk factor for early-onset emphysema. In the study to investigate the adults with chronic obstructive pulmonary disease (COPD) (n=348), asthma (n=71), and bronchiectasis (n=35)with the control was 1435 healthy blood donors, showed that The high prevalence of A1ATD phenotypes/allele in our population has confirmed the necessity of screening for A1ATD in patients with COPD(9). Other study indicated that clinically significant bronchiectasis (radiologic bronchiectasis in 4 or more bronchopulmonary segments together with symptoms of regular sputum production) occurred in 20 subjects (27%). AAT-deficient index cases had higher airway disease scores (P < 0.05), more severe emphysema (P < 0.001), and greater impairment of physiology (P < 0.001) and health status (P < 0.05) than nonindex cases. Airway disease scores correlated with health status, and bronchial wall thickening correlated with FEV(1)(10).
The long-term course of a patient with bronchiectasis and bronchiolitis obliterans, both of which developed as late complications of a smoke inhalation injury. Sequential chest X-rays obtained during the observation period showed gradual progression of bronchiectasis from the saccular to the cystic type, according to the study by Department of Medicine, School of Medicine, Keio University(11).
In the study to to determine causative factors in 150 adults with bronchiectasis (56 male, 94 female) identified using high-resolution computerized tomography, found that immotile cilia syndrome (primary ciliary dyskinesia) and genetic anomalies that are associated with a humoral or cellular immunodeficiency are common causes of bronchiectasis., including immune defects (12 cases), cystic fibrosis (4), Young’s syndrome (5), ciliary dysfunction (3), aspiration (6), panbronchiolitis (1), congenital defect (1), ABPA (11), rheumatoid arthritis (4), and early childhood pneumonia, pertussis, or measles (44)(12). In other study, researchers at the indicated that Broncholithiasis is a rare condition in which calcified material erodes into the tracheobronchial tree. Most are caused from a fungal, nocardial, mycobacterial, or silicosis-related granulomatous lymphadenitis. Over time, the peribronchial lymph nodes become calcified; thereafter, with the normal repetitive motions of respiration, circulation, and deglutition, the calcifications erode into the lumen of the airway(13).
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