[PiSZ-phenotype alpha 1-antitrypsin deficiency: a rare cause of bronchiectasis].
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.
Veloso
S
S
Servicio de Medicina Interna, Hospital Universitario Juan XXIII, Institut d'Estudis Avancats, Universidad Rovira i Virgili, Tarragona, Spain.
Aguilar
X
X
Paniagua
M J
MJ
Vidal
F
F
Richart
C
C
D001987:Bronchiectasis / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000209:etiology*; D005260:Female; D006801:Humans; D008875:Middle Aged; D010641:Phenotype; D013902:Radiography, Thoracic; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications / Q000175:diagnosis