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纯合子β地中海贫血的肺功能异常

Pulmonary function abnormalities in homozygous beta-thalassemia.

作者信息

Hoyt R W, Scarpa N, Wilmott R W, Cohen A, Schwartz E

出版信息

J Pediatr. 1986 Sep;109(3):452-5. doi: 10.1016/s0022-3476(86)80116-0.

Abstract

Pulmonary function tests were performed on 19 patients with homozygous beta-thalassemia ranging in age from 10 to 29 years. These included patients who had and had not received transfusions. None of the 19 subjects had completely normal pulmonary function. Residual volume (in 16 of 19 patients), ratio of residual volume to total lung capacity (12 of 19), and airway resistance (16 of 19) were abnormally increased; maximum expiratory flow (15 of 19) and peak flow (10 of 19) were abnormally reduced. Single-breath carbon monoxide diffusion was normal in 16 of 19. These results indicate that mild to moderate small airway obstruction and hyperinflation are common in thalassemia and that patient age, transfusion history, and iron accumulation are not important factors in the genesis of these pulmonary abnormalities.

摘要

对19例年龄在10至29岁之间的纯合子β地中海贫血患者进行了肺功能测试。这些患者包括接受过和未接受过输血的患者。19名受试者中没有一人的肺功能完全正常。残气量(19例中的16例)、残气量与肺总量之比(19例中的12例)以及气道阻力(19例中的16例)异常增加;最大呼气流量(19例中的15例)和峰值流量(19例中的10例)异常降低。单次呼吸一氧化碳弥散在19例中的16例中正常。这些结果表明,轻至中度小气道阻塞和肺过度充气在thalassemia中很常见,并且患者年龄、输血史和铁蓄积不是这些肺部异常发生的重要因素。

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