Fahim Ahmed, Wilmot Rachel, Hart Simon Paul
Department of Cardiovascular and Respiratory Studies, Castle Hill Hospital, Cottingham, UK.
J Coll Physicians Surg Pak. 2013 Mar;23(3):224-5.
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.
一名47岁的白人男性因劳力性呼吸困难4周就诊于胸科门诊。胸部X线片显示轻度肺过度充气，无任何局灶性病变，肺功能检查显示轻度阻塞性缺陷。鉴于症状与肺功能和放射学异常不相称，遂行胸部CT扫描。结果显示有支气管扩张和以肺底部分布为主的轻度肺气肿的证据。随后，他被确诊为严重的α1抗胰蛋白酶缺乏症。该病例说明了在患有以肺底部分布为主的肺气肿和支气管扩张的患者中考虑α1抗胰蛋白酶缺乏症的重要性。虽然肺底部肺气肿是α1抗胰蛋白酶缺乏症公认的肺部表现，但伴有非常轻度肺气肿的支气管扩张极为罕见。
