title: Emphysema and bronchiectasis secondary to alpha-1 antitrypsin deficiency.
abstract: A 47-year-old Caucasian male presented to the chest clinic with a 4-week history of exertional dyspnea. A chest radiograph showed mild hyperinflation without any focal pathology and spirometry showed a mild obstructive defect. In view of symptoms being disproportionate to spirometric and radiologic abnormalities, a thoracic CT scan was obtained. It revealed that there was evidence of bronchiectasis and mild emphysema in basal distribution. Subsequently, he was confirmed to have severe α1-Antitrypsin deficiency. This case illustrates the importance of considering α1-Antitrypsin deficiency in patients with combination of emphysema and bronchiectasis in a basal distribution. Although basal emphysema is well-recognized pulmonary manifestation of α1-Antitrypsin deficiency, it is extremely unusual to have bronchiectasis with very mild degree of emphysema.
authors: Department of Cardiovascular and Respiratory Studies, Castle Hill Hospital, Cottingham, UK. ahmedfahim@doctors.org.uk
authors: Ahmed
authors: A
authors: Fahim
authors: Rachel
authors: R
authors: Wilmot
authors: Simon Paul
authors: SP
authors: Hart
mesh_terms: D001797:Blood Protein Electrophoresis; D001987:Bronchiectasis / Q000150:complications / Q000000981:diagnostic imaging* / Q000188:drug therapy; D001993:Bronchodilator Agents / Q000008:administration & dosage; D004417:Dyspnea / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011656:Pulmonary Emphysema / Q000150:complications / Q000000981:diagnostic imaging*; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed; D019896:alpha 1-Antitrypsin Deficiency / Q000097:blood* / Q000150:complications
chemical_list: D001993:Bronchodilator Agents