Mohd Zikre Nurwahida, Muhamad Nor A, Eng Caroline S Y, Zailanalhuddin Nur E, Lai Charles D, Foo Jen C, Yap Suet L, Ariffin Hany, Abu Bakar Karmila
Paediatric Unit, Faculty of Medicine, University Malaya, Kuala Lumpur, Malaysia.
Sector for Evidence-Based Healthcare, National Institutes of Health, Ministry of Health, Kuala Lumpur, Malaysia.
Front Pediatr. 2021 Nov 22;9:754813. doi: 10.3389/fped.2021.754813. eCollection 2021.
Thalassemia is the commonest hemoglobinopathy in Southeast Asia. Kidney dysfunction is an underreported sequelae in children with thalassemia. We conducted a retrospective study to identify the prevalence of and predisposing factors for kidney dysfunction in children with transfusion-dependent thalassemia (TDT). Abnormal kidney function was defined as children with a glomerular filtration rate (GFR) of <90 ml/min/1.73 m or a decline in GFR of >20 ml/min/1.73 m or presence of nephrotic range proteinuria within 3 years of commencing regular (every ≤6 weeks) red cell transfusion. Data analyzed were age at diagnosis of thalassemia, number of transfusion-years, iron chelation therapy, serum ferritin, and pre-transfusion hemoglobin levels. Eighty-one children were studied. Mean age was 11.72 ± 5.275 years. Thirty out of 81 (37%) demonstrated abnormal kidney function. Evidence of glomerular hyperfiltration was seen in 29/81 patients (25.85%) at their last clinic visit. This fraction was doubled [48/81 (59.3%)] when the cohort was tracked back by 3 years from the last clinic encounter. Age at diagnosis (RR, 1.157; 95% CI, 1.014-1.319; = 0.03) and duration of receiving transfusions (RR, 0.984; 95% CI, 0.974-0.994; = 0.001) were associated with increased risk of developing abnormal kidney function. Abnormal kidney function in children with TDT may be overlooked by medical personnel without active screening measures. Children receiving regular red cell transfusions require systematic surveillance to enable early detection of kidney dysfunction and timely implementation of appropriate therapeutic interventions.
地中海贫血是东南亚最常见的血红蛋白病。肾功能障碍是地中海贫血患儿中报告不足的后遗症。我们进行了一项回顾性研究,以确定依赖输血的地中海贫血(TDT)患儿肾功能障碍的患病率和易感因素。肾功能异常定义为肾小球滤过率(GFR)<90 ml/min/1.73 m²的儿童,或GFR下降>20 ml/min/1.73 m²的儿童,或在开始定期(每≤6周)红细胞输血3年内出现肾病范围蛋白尿的儿童。分析的数据包括地中海贫血诊断时的年龄、输血年数、铁螯合治疗、血清铁蛋白和输血前血红蛋白水平。研究了81名儿童。平均年龄为11.72±5.275岁。81名儿童中有30名(37%)表现出肾功能异常。在最后一次门诊就诊时,81名患者中有29名(25.85%)出现肾小球高滤过的证据。当从最后一次门诊就诊时间回溯3年追踪该队列时,这一比例翻倍[48/81(59.3%)]。诊断时的年龄(RR,1.157;95%CI,1.014 - 1.319;P = 0.03)和接受输血的持续时间(RR,0.984;95%CI,0.974 - 0.994;P = 0.001)与肾功能异常风险增加相关。如果没有积极的筛查措施,医务人员可能会忽略TDT患儿的肾功能异常。接受定期红细胞输血的儿童需要系统监测,以便早期发现肾功能障碍并及时实施适当的治疗干预措施。