Abu-Ekteish F M, Al-Rimawi H S, Al-Ali M K, Shehabi I M
Department of Pediatrics, Jordan University of Science & Technology, Irbid, Jordan.
Chron Respir Dis. 2007;4(1):19-22. doi: 10.1177/1479972306070376.
Lung function abnormality is a known complication of thalassemia, but the results of studies in pulmonary function have been inconsistent. This study was conducted to describe the type of lung impairment in thalassemic children. Pulmonary function tests were conducted in 40 children with beta-thalassemia major, 23 males and 17 females. Tests included spirometry, total lung capacity (TLC), single breath diffusing capacity of the lung for carbon monoxide (DL(CO)) and arterial blood gases. Serum ferritin level was measured in all children to study its relationship to lung function impairment. A predominantly restrictive pattern was seen in 14 patients (35%). These patients had a significant reduction in RV, FVC, TLC and PEF with an FEV1/FVC ratio of more than 75%. Obstructive airway disease was found in six patients (15%), with an FEV1/FVC ratio less than 75%, increased RV and reduced FEF(25%-75%). Impairment of diffusion was found in 10 patients (25%), with DL(CO) reduced to less than 80% of the predicted value. Arterial blood gases results showed that no patient was hypoxic. No correlation was found between the severity of restrictive or obstructive disease and the serum ferritin level. There was a significant linear correlation between age and serum ferritin level (P < 0.019). Patients with thalassemia have a predominantly restrictive lung dysfunction pattern. This may be due to pulmonary parenchymal pathology, although the reason for the obstructive pattern seen in a small proportion of patients remains obscure.
肺功能异常是地中海贫血已知的并发症，但肺功能研究结果并不一致。本研究旨在描述地中海贫血患儿的肺损伤类型。对40例重型β地中海贫血患儿进行了肺功能测试，其中男性23例，女性17例。测试包括肺活量测定、肺总量（TLC）、单次呼吸一氧化碳肺弥散量（DL(CO)）和动脉血气分析。测定了所有患儿的血清铁蛋白水平，以研究其与肺功能损害的关系。14例患者（35%）表现为以限制性为主的模式。这些患者的残气量（RV）、用力肺活量（FVC）、肺总量（TLC）和呼气峰流速（PEF）显著降低，第一秒用力呼气容积/用力肺活量（FEV1/FVC）比值超过75%。6例患者（15%）发现存在阻塞性气道疾病，FEV1/FVC比值低于75%，RV增加，25%-75%用力呼气流量（FEF(25%-75%)）降低。10例患者（25%）发现存在弥散功能损害，DL(CO)降至预测值的80%以下。动脉血气分析结果显示，无患者存在低氧血症。未发现限制性或阻塞性疾病的严重程度与血清铁蛋白水平之间存在相关性。年龄与血清铁蛋白水平之间存在显著的线性相关性（P < 0.019）。地中海贫血患者主要表现为限制性肺功能障碍模式。这可能是由于肺实质病变所致，尽管一小部分患者出现阻塞性模式的原因仍不清楚。
