Quintero J, Swenson E W, Arborelius M, Paulin S
Eur J Respir Dis. 1980 Aug;61(4):181-94.
We have studied nine male patients (age 18 to 68 years) with radiographic and physiologic evidence of an abnormally small lung on one side (three right and six left). All had had a childhood pneumonia or bronchiolitis and eight had chronic or recurrent bronchitis and exertional dyspnea. Radiography showed two of the small lungs to be hypolucent while seven were hyperlucent. Bronchography revealed evidence of bilateral chronic bronchitis in all with saccular bronchiectasis in three. Angiography showed strikingly diminished vascularity to the smaller lung. Spirometry revealed airway obstruction in seven of the patients. All had pulmonary arterial hypertension. Radiospirometry showed that the small lung had on the average 30% of the total ventilation but only 15% of the perfusion. Washout of 133 Xe was extremely slow in radiolucent regions. We suggest the name "crippled lung" syndrome for this entity because it is purely descriptive and encompasses several clinical variants. It also avoids the pitfalls of etiologic implication (acquired-congenital). Clinical or subclinical bronchitis seems to be common in these patients and the prime goal in therapy must be to combat the tendency towards airway infection.
我们研究了9例男性患者(年龄18至68岁),其一侧肺脏在影像学和生理学上显示异常小(右侧3例,左侧6例)。所有患者童年时均患过肺炎或细支气管炎,8例有慢性或复发性支气管炎及劳力性呼吸困难。影像学检查显示,2例小肺透亮度减低,7例透亮度增加。支气管造影显示所有患者均有双侧慢性支气管炎证据,3例有囊状支气管扩张。血管造影显示较小肺脏的血管显著减少。肺量计检查显示7例患者存在气道阻塞。所有患者均有肺动脉高压。放射性肺量计检查显示,小肺平均占总通气量的30%,但仅占总灌注量的15%。在透亮度增加区域,133Xe的洗脱极其缓慢。我们建议将此病症命名为“残废肺”综合征,因为它纯粹是描述性的,涵盖了几种临床变异情况。它还避免了病因暗示(后天性-先天性)的陷阱。临床或亚临床支气管炎在这些患者中似乎很常见,治疗的主要目标必须是对抗气道感染的倾向。