Department of Pediatrics, Medical College and Hospital, Kolkata. Correspondence to: Dr Mihir Sarkar, 88, College Street, Department of Pediatrics, Medical College and Hospital, Kolkata, West Bengal 700 073.
Department of Pediatrics, Medical College and Hospital, Kolkata.
Indian Pediatr. 2022 Jul 15;59(7):531-534. doi: 10.1007/s13312-022-2554-x. Epub 2022 May 20.
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)检查。PFT 包括肺活量测定、氦稀释试验测定的总肺活量(TLC)和一氧化碳弥散量(DLCO)。对 13 名中重度肺功能障碍的患儿进行静脉内 DFO 治疗,可获得随访的 PFT。
50 名(68.8%)患儿存在肺功能障碍,最常见的是弥散功能障碍(48 例;96%)和 TLC 降低(11 例;22%);没有阻塞性模式。9 名(81.8%)有限制性缺陷的患儿有中重度 DLCO 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,PFT 常规筛查应成为管理指南的一部分。