摘要
多次输血导致的进行性组织铁沉积在地中海贫血中很常见,肺部铁沉积可导致实质损害。本研究的目的是:1)确定重型地中海贫血患者的主要肺功能障碍;2)显示实质疾病,如果存在,是在肺泡毛细血管膜水平。研究招募了14例地中海贫血患者(13例非吸烟者),定期输血,无任何慢性呼吸系统疾病史。计划输血前行肺功能检查及超声心动图检查。选择3例肺功能最受限的患者进行肺高分辨率CT检查。1例患者为阻塞性,1秒用力呼气量占用力肺活量(FEV1/FVC)的百分比为71%。4例患者表现出限制性模式,即总肺活量(TLC)小于80%,FEV1/FVC%正常。12例患者的一氧化碳肺转移因子(TL,CO)在80%以下,即使在贫血纠正后,表明实质疾病。12例患者中有8例存在肺泡毛细血管膜缺损,表现为肺膜气体转移因子(Tm)小于80%。 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.