输血依赖性地中海贫血儿童肾小球小管功能:一项前瞻性队列研究

IF 0.2 Q4 UROLOGY & NEPHROLOGY
H. Puspitasari, L. Rahmartani, Diashati Ramadhani Mardiasmo, P. Wahidiyat, E. L. Hidayati
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引用次数: 0

摘要

引言:地中海贫血是印度尼西亚最常见的血红蛋白病。慢性贫血、铁过载和铁螯合剂治疗是导致输血依赖性地中海贫血(TDT)儿童肾功能障碍的因素。尿中性粒细胞明胶酶相关脂质运载蛋白(uNGAL)和尿白蛋白-肌酐比值(uACR)是肾小球和肾小管损伤的标志物。目的:分析TDT患儿的肾小球管功能。患者和方法:这项前瞻性队列研究招募了40名年龄≤18岁的TDT儿童,他们来自印度尼西亚雅加达Cipto Mangunkusumo综合医院地中海贫血输血门诊。在入组期间(T0)获得病史、治疗和人体测量。在入组(T0)和1个月(T1)、2个月(T2)和3个月(T3)随访时采集血液和尿液样本。分析的数据包括铁螯合剂、铁蛋白、血红蛋白、估计肾小球滤过率(eGFR)、uNGAL和uACR水平。结果:我们在超过30%的参与者中观察到肾脏过度滤过。输血量是eGFR(β=0.974,P=0.022)和uNGAL(β=0.872,P=0.015)的最主要因素。发现蛋白尿的比例较低,疾病持续时间是主要因素(β=0.946,P=0.000)。除铁酮(DFP)和去铁罗克斯(DFX)与所有肾脏生物标志物的相关性相当。结论:本研究结果表明,TDT儿童肾小球高滤过是肾小球功能损害的主要早期迹象。此外,在我们的研究中也发现了一小部分蛋白尿。因此,这两种生物标志物都应该是TDT儿童长期肾脏随访的一部分。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Glomerulotubular function in transfusion-dependent thalassemia children: a prospective cohort study
Introduction: Thalassemia is the most common hemoglobinopathy in Indonesia. Chronic anemia, iron overload, and treatment with iron chelating agents are factors that contribute to kidney dysfunction in children with transfusion-dependent thalassemia (TDT). Urine neutrophil gelatinase-associated lipocalin (uNGAL) and urine albumin-creatinine ratio (uACR) are markers of glomerular and tubular damage. Objectives: To analyze the glomerulotubular function in TDT children. Patients and Methods: This prospective cohort study recruited 40 TDT children aged ≤18 years old from thalassemia transfusion outpatient clinic, Dr. Cipto Mangunkusumo general hospital, Jakarta, Indonesia between February and June 2021. Disease history, treatment, and anthropometry were obtained during enrollment (T0). Blood and urine samples were taken at enrollment (T0) and at 1 (T1), 2 (T2), and 3 (T3) month follow-up appointments. Data analyzed were iron chelating agent, ferritin, hemoglobin, estimated glomerular filtration rate (eGFR ), uNGAL, uACR levels. Results: We observed kidney hyperfiltration in more than 30% of the participants. Transfusion volume was the most dominant factor for eGFR (β=-0.974, P=0.022) and uNGAL (β=0.872, P=0.015). A low-proportion of albuminuria was found with duration of disease as the predominant factor (β=-0.946, P=0.000). Deferiprone (DFP) and deferasirox (DFX) showed comparable association with all kidney biomarkers. Conclusion: The findings of this study indicate glomerular hyperfiltration in TDT children as the main early sign of glomerulotubular function impairment. In addition, a small proportion of proteinuria was also found in our study. Thus, both biomarkers should be part of long term kidney follow up in TDT children.
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来源期刊
Journal of Renal Injury Prevention
Journal of Renal Injury Prevention UROLOGY & NEPHROLOGY-
CiteScore
1.60
自引率
0.00%
发文量
36
期刊介绍: The Journal of Renal Injury Prevention (JRIP) is a quarterly peer-reviewed international journal devoted to the promotion of early diagnosis and prevention of renal diseases. It publishes in March, June, September and December of each year. It has pursued this aim through publishing editorials, original research articles, reviews, mini-reviews, commentaries, letters to the editor, hypothesis, case reports, epidemiology and prevention, news and views and renal biopsy teaching point. In this journal, particular emphasis is given to research, both experimental and clinical, aimed at protection/prevention of renal failure and modalities in the treatment of diabetic nephropathy. A further aim of this journal is to emphasize and strengthen the link between renal pathologists/nephropathologists and nephrologists. In addition, JRIP welcomes basic biomedical as well as pharmaceutical scientific research applied to clinical nephrology. Futuristic conceptual hypothesis that integrate various fields of acute kidney injury and renal tubular cell protection are encouraged to be submitted.
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