STUDY OF Α1- ANTITRYPSIN PHENOTYPES FREQUENCIES IN PATIENTS WITH PRIMARY ANTIBODY DEFICIENCY
作者： F Zandieh , M Gharagozlou , A Aghamohammadi , J Ghaffari , N Rezaei
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摘要: Primary antibody deficiencies are the most frequent primary immunodeficiency disorders. Bronchiectasis as a feature of these disorders may be developed due to some factors such α-1antitrypsin deficiency. In order determine prevalence two common α-1-antitrypsin deficiency alleles (PI*Z and PI*S) in Iranian patients with deficiency, this study was performed. The PI*M, PI*S, PI*Z allele combinations determined 40 (with without bronchiectasis) compared 60 healthy control subjects. Phenotyping performed by isoelectric focusing. phenotype frequencies among were follow: M 92.5%, S 2.5% Z 5%. There not any significant difference distribution or phenotypes between Moreover, no found bronchiectasis. We did find evidence support an association small, controlled study. Larger studies will required clarify relationship genotype susceptibility bronchiectasis
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