Alpha-1 Antitrypsin Deficiency in Iranian Patients with Chronic Obstructive Pulmonary Disease

Abstract

BackgroundAlpha-1 antitrypsin deficiency is a genetic disease which affects both lung and liver. This disease is a recognized factor for chronic obstructive pulmonary disease (COPD). However its importance as the cause of COPD in a country such as Iran is unclear.ObjectivesThis study was conducted to find out the role of α-1 antitrypsin deficiency as a cause of COPD in Iranian patients.Materials and MethodsThe serum concentration of α-1 antitrypsin was determined and the genotype of α-1 antitrypsin was also evaluated by PCR-RFLP in 130 patients with COPD and 50 normal healthy blood donors.ResultsNo α-1 antitrypsin deficient case was found in normal healthy people and COPD patients.ConclusionsOur results clarify that deficiency of α-antitrypsin is not a major cause of COPD in Iranian patients.

Pathological Mechanism and Targeted Drugs of COPD

“…The signaling pathway of this kind of factor often resides in the oxidants’ activation of airway cells, and then involves in COPD. Regarding the structural destruction of lung tissue caused by protease-antiprotease imbalance, due to α1-antitrypsin 24 is the most active one, its inactivation is the most critical one. And the large amount of oxidants released by noxious gases, oxidative stress also makes the antiprotease inactivated and finally the protease-antiprotease imbalance occurs.…”

Case-finding for alpha1-antitrypsin deficiency in Kazakh patients with COPD

“…In China, electrophoretic analysis of 748 normal subjects and 414 COPD patients did not detect any S or Z mutations in either COPD group [ 28 ], nor were any S or Z variants identified in a Korean study on 56 male emphysematous patients over 50 years old [ 29 ]. Likewise, in Iran, an investigation of 130 COPD patients detected no S and Z variants [ 30 ]. While in India, genotyping of 200 COPD patients identified two SS (1%) and one ZZ patient (0.5%) [ 31 ].…”