Open AccessA rare case of alpha 1-antitrypsin deficiency associated with hypogammaglobulinemia and recurrent pulmonary thrombosis
Author(s) -
Raghav Gupta,
Srilekha Sridhara,
John A. Wood
Publication year - 2014
Publication title -
annals of thoracic medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.639
H-Index - 33
eISSN - 1817-1737
pISSN - 1998-3557
DOI - 10.4103/1817-1737.124442
Subject(s) - medicine , hypogammaglobulinemia , concomitant , alpha 1 antitrypsin deficiency , thrombosis , alpha (finance) , serine protease , immunology , protease , gastroenterology , enzyme , surgery , antibody , biochemistry , construct validity , chemistry , patient satisfaction
Alpha 1-antitrypsin (AAT) belongs to the family of serpins (serine protease inhibitors). Loop sheet polymerization is the pathology behind serpinopathies which encompasses AAT, anti-thrombin III and neuroserpin deficiency. To the best of our knowledge, we report the first case of alpha 1-antitrypsin deficiency associated with hypogammaglobulinemia and recurrent pulmonary thrombosis without any concomitant use of drugs.