ty -jour t1-丘脑贫血的肺主要患者接受常规输血JF-欧洲呼吸杂志JO -EUR RESSIR J SP -1389 LP -1394 VL -9 IS -9 IS -7 AU -TAI -TAI，dy au -Wang，wang，yt au -yt au -lou，j lou，j lou，jAu -Wang，Wy Au -Mak，Kh au -Cheng，HK Y1-1996/07/01 UR- http：//www.qdcxjkg.com/content.com/content/9/7/7/1389.Abstract n2-渐进的组织铁沉积中的渐进式组织铁沉积多种输血在β-甲性贫血中很常见，肺铁沉积可能会导致实质性损害。这项研究的目标是：1）确定丘脑症主要患者的主要肺功能障碍；2）证明实质性疾病（如果存在）处于肺泡膜膜的水平。招募了14名Thalassya的主要患者（13名非吸烟者）接受定期输血，没有任何慢性呼吸道疾病病史。在预定输血之前进行肺功能测试和超声心动图。选择了三名肺功能最严重的患者进行肺的高分辨率计算机断层扫描（CT）。一名患者的阻塞性模式在一秒钟内强迫呼气量为71％的强迫生命力（FEV1/FVC）的百分比。四名患者表现出限制性模式，如总肺容量（TLC）所定义的小于80％的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 -