Open AccessPulmonary langerhans cell histiocytosis — insight into the incidence of alfa-1-antitrypsin (A1ATD) deficiency alleles
Author(s) -
E Radzikowska,
Radosław Struniawski,
Joanna ChorostowskaWynimko,
Elżbieta Wiatr,
Kazimierz RoszkowskiŚliż
Publication year - 2017
Publication title -
advances in respiratory medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.288
H-Index - 18
eISSN - 2543-6031
pISSN - 2451-4934
DOI - 10.5603/arm.2017.0051
Subject(s) - medicine , alpha 1 antitrypsin deficiency , allele , gastroenterology , incidence (geometry) , genotyping , population , immunology , genotype , histiocytosis , pathology , disease , biology , genetics , physics , environmental health , optics , gene
The alpha-1 antitrypsin deficiency (A1ATD) is one of the three most common genetic disorders in Caucasians. It considerably increases the risk of progressive obstructive lung diseases, mostly chronic obstructive pulmonary disease. There is no data regarding prevalence of main, clinically most important A1ATD alleles PI*Z and PI*S in patients with pulmonary Langerhans cell histiocytosis (PLCH). PLCH is not only strongly linked to the cigarette smoking, but is also characterised by polycystic lung lesions. The goal of the study was to assess the incidence of A1ATD alleles in patients with PLCH.