Youngchaiyud P, Suthamsmai T, Fucharoen S, Udompanich V, Pushpakom R, Wasi P
Department of Medicine, Siriraj Hospital, Bangkok, Thailand.
Birth Defects Orig Artic Ser. 1987;23(5A):361-70.
Pulmonary function tests were performed on 30 patients with beta-thalassemia/Hb E. Only one patient had normal pulmonary function. Arterial hypoxemia at rest was present in 22 of 30 patients (73.3%). Abnormal VC, FEV1, FEV1/FVC, and end tidal FEV1 were found in 29 (96.7%), 17 (56.7%), 6 (20.0%), and 17 (56.7%) patients, respectively. Single-breath carbon monoxide diffusing capacity was abnormal in one out of 11 patients (9.1%). These results suggest the presence of abnormal ventilatory function included restrictive, obstructive, and combined defects. The hypoxemia in thalassemia was probably due to ventilation/perfusion mismatch and a diffusion defect.
对30例β地中海贫血/Hb E患者进行了肺功能测试。只有1例患者肺功能正常。30例患者中有22例（73.3%）存在静息动脉低氧血症。分别有29例（96.7%）、17例（56.7%）、6例（20.0%）和17例（56.7%）患者的肺活量（VC）、第一秒用力呼气容积（FEV1）、FEV1/用力肺活量（FVC）和呼气末FEV1异常。11例患者中有1例（9.1%）单次呼吸一氧化碳弥散量异常。这些结果提示存在包括限制性、阻塞性和混合性缺陷在内的通气功能异常。地中海贫血中的低氧血症可能是由于通气/灌注不匹配和弥散障碍所致。
