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[PiSZ-phenotype alpha 1-antitrypsin deficiency: a rare cause of bronchiectasis]
S Veloso1, X Aguilar, M J Paniagua
1Servicio de Medicina Interna, Hospital Universitario Juan XXIII, Institut d'Estudis Avancats, Universidad Rovira i Virgili, Tarragona, Spain.
Archivos De Bronconeumologia
|May 25, 2002
Abstract:
Panacinar emphysema is the most characteristic pulmonary disease in patients with alpha1-antitrypsin deficiency (AAT). Bronchiectasis can also arise with AAT deficiency, although the association is much less common and no clear cause-effect relationship has yet been established. We report the case of a woman who presented with bronchiectasis and PiSZ-phenotype ATT deficiency, without emphysema. A review of the literature showed that the case is exceptional.