Cooper D M, Mansell A L, Weiner M A, Berdon W E, Chetty-Baktaviziam A, Reid L, Mellins R B
Am Rev Respir Dis. 1980 Apr;121(4):639-46. doi: 10.1164/arrd.1980.121.4.639.
We evaluated lung function in 17 children with thalassemia major in stable condition receiving blood transfusions at regular intervals and subcutaneous desferoxamine daily. Total lung capacity (TLC) was below 2 SD of normal values for height in 7 of the 17 children and arterialized capillary PO2 was below the normal range in 15. We studied lung mechanics in 4 children with reduced TLC and found static and dynamic compliance below 2 SD of normal values for height in 3, and lung recoil at TLC above normal values and specific upstream conductance (Gus/TLC) above 2 SD of normal values in all 4. Although these alterations in lung function have been described in patients with pulmonary fibrosis, we found no fibrosis in autopsy specimens of lung from 8 other patients with thalassemia. The rate constant of carbon monoxide diffusion (kCO) was above the predicted mean in 14 of 15 children. These findings can be explained by a decrease in the growth of airspace relative to the vascular bed and major airways during childhood.
我们评估了17名重度地中海贫血患儿的肺功能，这些患儿病情稳定，定期接受输血治疗，且每日皮下注射去铁胺。17名患儿中，有7名的肺总量（TLC）低于同身高正常数值的2个标准差，15名的动脉化毛细血管氧分压低于正常范围。我们研究了4名TLC降低患儿的肺力学，发现其中3名的静态和动态顺应性低于同身高正常数值的2个标准差，所有4名患儿在TLC时的肺回缩高于正常数值，比气道传导率（Gus/TLC）高于正常数值的2个标准差。尽管肺功能的这些改变已在肺纤维化患者中有所描述，但我们在另外8名地中海贫血患者的肺尸检标本中未发现纤维化。15名患儿中有14名的一氧化碳弥散速率常数（kCO）高于预测平均值。这些发现可以通过儿童期气腔相对于血管床和主要气道生长减少来解释。
