Ayres J G, Pope F M, Reidy J F, Clark T J
Thorax. 1985 Apr;40(4):300-5. doi: 10.1136/thx.40.4.300.
Twenty patients with the Ehlers-Danlos syndrome, (10 type I, six type II, and four type IV) were studied to assess the frequency of respiratory abnormalities in this condition. Five patients (25%) had had at least one episode of haemoptysis, but none had any defect of coagulation. There was a high frequency of recurrent sinusitis, notably in those with the type I syndrome. Two patients had bullous lung disease, one of whom (type IV) had had three pneumothoraces and subsequent pleurodesis; he also had tracheomegaly (the Mounier-Kuhn abnormality). Minor skeletal abnormalities such as pectus excavatum were common, particularly in patients with type IV disease. Three patients had the straight back syndrome. There were no consistent spirometric or lung volume abnormalities, but eight patients (40%) had a raised gas transfer coefficient (Kco), possibly due to an increased intrapulmonary vascular volume. Two other patients had very low values of Kco that were unexplained.
对20例埃勒斯-当洛综合征患者（10例Ⅰ型、6例Ⅱ型和4例Ⅳ型）进行了研究，以评估该疾病中呼吸异常的发生率。5例患者（25%）至少有一次咯血发作，但均无凝血缺陷。复发性鼻窦炎的发生率很高，尤其是在Ⅰ型综合征患者中。2例患者患有大疱性肺病，其中1例（Ⅳ型）发生过3次气胸并随后进行了胸膜固定术；他还患有气管扩张（穆尼耶-库恩异常）。轻度骨骼异常如漏斗胸很常见，尤其是在Ⅳ型疾病患者中。3例患者患有直背综合征。肺功能测定或肺容量没有一致的异常，但8例患者（40%）的气体转移系数（Kco）升高，可能是由于肺内血管容量增加。另外2例患者的Kco值非常低，原因不明。
