Department of Pediatrics, University College of Medical Sciences, Delhi. Correspondence to: Dr Neha Panwar, Senior Resident, Department of Pediatrics, University College of Medical Sciences, Delhi 110 095.
Department of Pediatrics, University College of Medical Sciences, Delhi.
Indian Pediatr. 2022 Jun 15;59(6):451-454. Epub 2022 Apr 26.
To evaluate pulmonary functions in children with transfusion-dependent thalassemia, and its reversal (lung dysfunction) using intensive intravenous chelation with desferrioxamine (DFO) (4 weeks).
This descriptive study enrolled 77 children with transfusion-dependent thalassemia. Pulmonary function test (PFT) and iron load (serum ferritin (SF) and T2* MRI of heart and liver) were done. PFT included spirometry, total lung capacity (TLC) by helium dilution test and diffusion capacity by carbon monoxide (DLCO). Follow-up PFT was available for 13 children with moderate to severe lung dysfunction given intravenous DFO.
50 (68.8%) patients had lung dysfunction, most commonly diffusional impairment (48; 96%), and reduced TLC (11; 22%); and none had obstructive pattern. 9 (81.8%) patients with restrictive defect had moderate to severely deranged DLCO. PFT and T2* MRI values were inversely correlated with serum ferritin. Among 13 patients receiving intensive chelation for 4 weeks, significant improvement was noticed in forced expiratory volume in one minute/ forced vital capacity ratio (DFEV1/FVC) (P=0.009), DDLCO (P=0.006) and DSF (P=0.01).
Pulmonary dysfunction is common in children with multi-transfused thalassemia, and routine screening by PFT needs to be part of the management guidelines.
评估依赖输血的地中海贫血患儿的肺功能，并使用去铁胺（DFO）进行强化静脉螯合治疗以逆转（肺功能障碍）。
本描述性研究纳入了 77 名依赖输血的地中海贫血患儿。进行了肺功能测试（PFT）和铁负荷（血清铁蛋白（SF）和心脏和肝脏的 T2* MRI）。PFT 包括肺活量测定、氦稀释试验测定的总肺活量（TLC）和一氧化碳扩散能力（DLCO）。对 13 名中至重度肺功能障碍的患儿进行了静脉 DFO 治疗，并进行了随访 PFT。
50 名（68.8%）患者存在肺功能障碍，最常见的是弥散功能障碍（48 例；96%）和 TLC 降低（11 例；22%）；无阻塞模式。9 名（81.8%）存在限制性缺陷的患者的 DLCO 中度至严重异常。PFT 和 T2* MRI 值与血清铁蛋白呈负相关。在接受 4 周强化螯合治疗的 13 名患者中，用力呼气量/用力肺活量比值（DFEV1/FVC）（P=0.009）、DLCO（P=0.006）和 DSF（P=0.01）显著改善。
肺功能障碍在多次输血的地中海贫血患儿中很常见，常规的 PFT 筛查需要成为管理指南的一部分。
