Ozyoruk Derya, Misirlioglu Emine D
Departments of *Pediatric Hematology Oncology †Pediatric Allergy and Immunology, Health of Ministry Children's Hospital, Şanliurfa, Turkey.
J Pediatr Hematol Oncol. 2015 Nov;37(8):605-10. doi: 10.1097/MPH.0000000000000425.
Thalassemia major (TM) is a chronic disease requiring regular transfusions that may result in generalized iron loading, such as in the heart, the liver, endocrine organs, and the lungs. We aimed to determine pulmonary function abnormalities in children with TM in our center.
In this study, pulmonary function tests (PFTs) of 49 patients with TM who received regular blood transfusion and had no history of chronic respiratory disease were evaluated. The relationship between PFTs and the age, the body surface area, pretransfusional hemoglobin, and serum ferritin was evaluated.
Among the β-TM patients included in this study, 61% were male and 39% were female, with a mean age of 10.8±3 years (range, 5 to 17 y). The patients' mean level of ferritin was 3873±2011 ng/dL (range, 676 to 9476 ng/dL). A reduced forced vital capacity (FVC) was found in 33 patients (67%). A reduced forced expiratory volume in 1 second (FEV1) was found in 15 patients (30%). A forced expiratory volume in 1 second to forced vital capacity (FEV1/FVC) ratio of >80% was found in all patients. The peak expiratory flow (PEF) was decreased in 23 patients (46.9%). The forced mid-expiratory flow between 25% and 75% of the exhaled vital capacity (MEF25%-75%) was decreased in 5 patients (10%). FVC and FEV1 values in patients with a high ferritin level (>2500 ng/dL) were decreased compared with patients with a low ferritin level (<2500 ng/dL) (P=0.04, 0.03). FVC, FEV1, and PEF parameters were negatively correlated with the age and the body surface area. Age was a predictor of FVC (β=-0.450, P<0.001), FEV1 (β=-0.419, P<0.001), and PEF (β=-0.505, P<0.001), and hemoglobin was a predictor of FEV1/FVC (β=0.366, P=0.01) and MEF25%-75% (β=0.323, P=0.003).
Our results concluded that the respiratory system should be evaluated by PFTs even in asymptomatic patients with high serum ferritin levels during the adolescent period annually to prevent the squeal of pulmonary disease in TM. Patients who have abnormal PFTs should be reevaluated for compliance with chelation therapy and the transfusion program.
重型地中海贫血（TM）是一种需要定期输血的慢性疾病，这可能导致全身铁负荷增加，如在心脏、肝脏、内分泌器官和肺部。我们旨在确定本中心TM患儿的肺功能异常情况。
在本研究中，对49例接受定期输血且无慢性呼吸道疾病史的TM患者进行了肺功能测试（PFTs）评估。评估了PFTs与年龄、体表面积、输血前血红蛋白和血清铁蛋白之间的关系。
本研究纳入的β-TM患者中，61%为男性，39%为女性，平均年龄为10.8±3岁（范围为5至17岁）。患者的铁蛋白平均水平为3873±2011 ng/dL（范围为676至9476 ng/dL）。33例患者（67%）的用力肺活量（FVC）降低。15例患者（30%）的1秒用力呼气容积（FEV1）降低。所有患者的1秒用力呼气容积与用力肺活量（FEV1/FVC）比值均>80%。23例患者（46.9%）的呼气峰值流速（PEF）降低。5例患者（10%）的呼出肺活量25%至75%之间的用力呼气中期流速（MEF25%-75%）降低。铁蛋白水平高（>2500 ng/dL）的患者与铁蛋白水平低（<2500 ng/dL）的患者相比，FVC和FEV1值降低（P=0.04，0.03）。FVC、FEV1和PEF参数与年龄和体表面积呈负相关。年龄是FVC（β=-0.450，P<0.001）、FEV1（β=-0.419，P<0.001）和PEF（β=-0.505，P<0.001）的预测因素，血红蛋白是FEV1/FVC（β=0.366，P=0.01）和MEF25%-75%（β=0.323，P=0.003）的预测因素。
我们的结果表明，即使是青春期血清铁蛋白水平高且无症状的患者，每年也应通过PFTs评估呼吸系统，以预防TM患者肺部疾病的发生。PFTs异常的患者应重新评估其对螯合治疗和输血方案的依从性。
