title: A rare case of alpha 1-antitrypsin deficiency associated with hypogammaglobulinemia and recurrent pulmonary thrombosis.
abstract: 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.
authors.affiliation: Department of Internal Medicine, Allergy and Immunology, St. Luke's Hospital, Chesterfield, MO, USA.
authors.forename: Raghav
authors.initials: R
authors.lastname: Gupta
authors.affiliation: Department of Internal Medicine, Allergy and Immunology, St. Luke's Hospital, Chesterfield, MO, USA.
authors.forename: Srilekha
authors.initials: S
authors.lastname: Sridhara
authors.affiliation: Department of Pulmonology, Allergy and Immunology, St. Luke's Hospital, Chesterfield, MO, USA.
authors.forename: John A
authors.initials: JA
authors.lastname: Wood
keywords: Alpha 1-antitrypsin deficiency; hypogammaglobulinemia; recurrent pulmonary thrombosis; serpinopathies