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β-地中海贫血轻型与肾小管功能障碍:二者是否存在关联?

β-Thalassemia minor & renal tubular dysfunction: is there any association?

机构信息

Social Determinants of Health Research Center, Health Research Institute, Babol University of Medical Sciences, Babol, Iran.

Students Research Committee, Babol University of Medical Sciences, Babol, IR, Iran.

出版信息

BMC Nephrol. 2021 Dec 7;22(1):404. doi: 10.1186/s12882-021-02602-9.

DOI:10.1186/s12882-021-02602-9
PMID:34872508
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8650370/
Abstract

OBJECTIVE

Beta(β)-thalassemia is one of the most common hereditary hematologic disorders. Patients with thalassemia minor (TM) are often asymptomatic and the rate of renal dysfunction is unknown in these patients. Due to the high prevalence of renal dysfunction in Iran, the current study aimed to determine renal tubular dysfunction in patients with beta-TM.

METHODS

In this case-control study, 40 patients with TM and 20 healthy subjects were enrolled and urinary and blood biochemical analysis was done on their samples. Renal tubular function indices were determined and compared in both groups. Data was analyzed by SPSS software, version 20.0.

RESULTS

The fraction excretion (FE) of uric acid was 8.31 ± 3.98% in the case and 6.2 ± 34.71% in the control group (p = 0.048). Also, FE of potassium was significantly higher in patients with TM (3.22 ± 3.13 vs. 1.91 ± 0.81; p = 0.036). The mean Plasma NGAL level was 133.78 ± 120.28 ng/mL in patients with thalassemia and 84.55 ± 45.50 ng/mL in the control group (p = 0.083). At least one parameter of tubular dysfunction was found in 45% of patients with thalassemia.

CONCLUSION

Based on the results of this study, the prevalence of tubular dysfunction in beta-thalassemia minor patients is high. Due to the lack of knowledge of patients about this disorder, periodic evaluation of renal function in TM patients can prevent renal failure by early diagnosis.

摘要

目的

β-地中海贫血是最常见的遗传性血液病之一。轻度地中海贫血(TM)患者通常无症状,这些患者的肾功能障碍发生率尚不清楚。由于伊朗肾功能障碍的高患病率,本研究旨在确定β-TM 患者的肾小管功能障碍。

方法

在这项病例对照研究中,纳入了 40 名 TM 患者和 20 名健康受试者,并对他们的样本进行了尿液和血液生化分析。在两组中确定并比较了肾小管功能指标。数据由 SPSS 软件(版本 20.0)进行分析。

结果

病例组尿酸的分数排泄(FE)为 8.31±3.98%,对照组为 6.2±34.71%(p=0.048)。此外,TM 患者的钾 FE 明显更高(3.22±3.13 比 1.91±0.81;p=0.036)。地中海贫血患者的平均血浆 NGAL 水平为 133.78±120.28ng/mL,对照组为 84.55±45.50ng/mL(p=0.083)。至少有一个肾小管功能障碍参数在 45%的地中海贫血患者中发现。

结论

根据这项研究的结果,β-地中海贫血轻度患者肾小管功能障碍的患病率较高。由于患者对这种疾病缺乏了解,定期评估 TM 患者的肾功能可以通过早期诊断来预防肾衰竭。

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Hematology. 2019 Dec;24(1):426-438. doi: 10.1080/16078454.2019.1599096.
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Iron overload across the spectrum of non-transfusion-dependent thalassaemias: role of erythropoiesis, splenectomy and transfusions.非输血依赖型地中海贫血症中的铁过载:红细胞生成、脾切除术及输血的作用
Br J Haematol. 2017 Jan;176(2):288-299. doi: 10.1111/bjh.14373. Epub 2016 Dec 5.
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β-Thalassemia.β地中海贫血
Genet Med. 2017 Jun;19(6):609-619. doi: 10.1038/gim.2016.173. Epub 2016 Nov 3.
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Novel Tubular Biomarkers Predict Renal Progression in Type 2 Diabetes Mellitus: A Prospective Cohort Study.新型肾小管生物标志物预测2型糖尿病患者的肾脏进展:一项前瞻性队列研究。
J Diabetes Res. 2016;2016:3102962. doi: 10.1155/2016/3102962. Epub 2016 Sep 8.
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