TY - JOUR T1 - 欧洲呼吸杂志JO - - EUR呼吸杂志SP - 1389 LP - 1394 VL - 9 - 7 AU - 大，DY AU - 王，YT AU - 娄，J在接受定期输血JF重型地中海贫血患者的肺Au - Wang，Wy Au - Mak，KH Au - Cheng，HK Y1 - 1996/07/01 UR - //www.qdcxjkg.com/content/9/7/1389.abstract n2 - 渐进式组织铁沉积在β-地中海贫血中，多种输血常见，肺铁沉积可能导致实质损伤。本研究的目标是：1）确定患有地中海贫血患者的主要肺功能障碍;2）证明实质疾病（如果存在）在肺泡膜的水平。招募了十四名中西血症的主要患者（13个非助剂），招募了常规输血和没有任何慢性呼吸道疾病史的患者。在预定的输血之前进行肺功能测试和超声心动图。选择了三项具有最受限制的肺功能患者，用于肺的高分辨率计算机断层扫描（CT）。一名患者具有阻塞性的模式，其强制呼气量为一秒钟，百分比为71％的强制生命能力（FEV1 / FVC）。四名患者证明了限制性图案，如肺部总肺容量（TLC）所定义的，常规FEV1 / FVC％预测的80％。 Twelve patients had pulmonary transfer factors for carbon monoxide (TL,CO) below 80% pred, even after correction for the anaemia, indicating parenchymal disease. Eight of these 12 patients had alveolocapillary membrane defect, as demonstrated by a gas transfer factor of the pulmonary membrane (Tm) less than 80% pred. Mean resting arterial oxygen saturation was 95 +/- 2 (range 92-98) %. Eleven patients had oxygen desaturation of 5% or more during exercise on a bicycle ergometer, consistent with interstitial lung disease. There was no clinical or echocardiographic evidence of heart failure. Percentage predicted TLC was inversely correlated with age (r = -0.547; p = 0.043). Both percentage predicted TLC and TL,CO were not correlated with iron burden or desferoxamine ratio. High resolution CT in the three selected patients showed no evidence of pulmonary fibrosis. We conclude that thalassaemia major patients have a predominant restrictive lung dysfunction with pulmonary parenchymal disease and alveolocapillary membrane block. The restrictive and interstitial lung disease could not be accounted for by iron loading or pulmonary fibrosis in our patients. ER -