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首页> 外文期刊>Transfusion medicine >Pulmonary functions in Egyptian children with transfusion‐dependent β‐thalassemia
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Pulmonary functions in Egyptian children with transfusion‐dependent β‐thalassemia

机译：埃及儿童的肺功能依赖于输血依赖性β-zhalassemia

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SUMMARY Background In β‐thalassemia, there are varying degrees of ineffective haematopoiesis, intermittent haemolysis and iron overload. Excess iron is deposited in organs such as the heart, the liver, the endocrine glands and the lungs. Objectives To evaluate the pulmonary functions in asymptomatic beta thalassemic children on regular transfusion therapy and their relation to iron overload. Methods The study included 50 transfusion‐dependent β‐thalassemic children and 50 apparently healthy children as control. All children had undergone pulmonary function tests (spirometry, lung volumes and diffusion capacities). In addition, test to determine the mean serum ferritin of the last 2?years and pre‐transfusion haemoglobin and chest radiograph and echocardiography were performed for the thalassemic children only. Results A total of 70% of the thalassemic children had diffusion impairment, whereas 34% of them had associated restrictive abnormality. Thalassemic children with serum ferritin 2500?ng?mL ?1 had significantly lower values of forced vital capacity (FVC), forced expiratory volume at one second (FEV1), peak expiratory flow (PEFR), total lung capacity (TLC) and diffusing capacity of carbon monoxide (DLCO) ( P ??0·05). Only diffusion impairment had a significant positive correlation with serum ferritin level. Restrictive impairment had significant positive correlations with age, duration of blood transfusion and serum ferritin level and a significant negative correlation with duration of chelation ( P ??0·05). Having a serum ferritin 2500?ng?mL ?1 was the only predicting factor for diffusion impairment and the strongest predicting factor for restrictive dysfunction. Conclusion Despite being asymptomatic, the majority of thalassemic children in this study suffered from diffusion impairment either alone or in combination with restrictive dysfunction. These pulmonary dysfunctions correlated significantly with body iron stores measured by serum ferritin.
机译：发明内容β-地中海贫血中的背景，有不同程度的无效血液，间歇溶血和铁过载。过量的铁沉积在心脏，肝脏，内分泌腺和肺等器官中。目的是评估无症状β的肺功能对常规输血治疗的肺功能及其与铁过载的关系。方法该研究包括50例药物依赖性β-丘脑血症儿童，50名明显健康的儿童作为控制。所有儿童都经历了肺功能测试（肺活量，肺体积和扩散能力）。此外，试验确定最后2年的平均血清铁蛋白及其预输血血红蛋白和胸部射线影子，仅对丘脑血症儿童进行。结果总计70％的丘脑患儿具有扩散障碍，而34％的34％具有相关的限制性异常。患有血清铁蛋白的血清血液＆ 2500？ng？mlα1具有显着较低的强制生气能力（FVC）的值，初期呼气量（FEV1），峰值呼气流（PEFR），总肺容量（TLC）和一氧化碳（DLCO）的扩散能力（p≤α0·05）。只有扩散障碍与血清铁蛋白水平具有显着的正相关性。限制性损伤与年龄，输血持续时间和血清铁蛋白水平具有显着的阳性相关性，以及与螯合持续时间的显着的负相关性（p？＆ 0·05）。具有血清铁蛋白＆ 2500？ng？mlα1是扩散障碍的唯一预测因素和限制功能障碍的最强预测因素。结论尽管是无症状的，但这项研究中的大多数脑神经儿童均遭受单独或与限制性功能障碍组合的扩散障碍。这些肺功能障碍与血清铁蛋白测量的身体铁储物有显着相关。

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