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依赖输血的地中海贫血患儿及成人的肾小球和肾小管功能

Glomerular and Tubular Functions in Children and Adults with Transfusion-Dependent Thalassemia.

作者信息

Annayev Agageldi, Karakaş Zeynep, Karaman Serap, Yalçıner Altan, Yılmaz Alev, Emre Sevinç

机构信息

İstanbul University İstanbul Faculty of Medicine, Department of Pediatric Hematology and Oncology, İstanbul, Turkey.

Düzen Laboratories, İstanbul, Turkey.

出版信息

Turk J Haematol. 2018 Mar 1;35(1):66-70. doi: 10.4274/tjh.2017.0266. Epub 2017 Jul 28.

DOI:10.4274/tjh.2017.0266
PMID:28753129
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5843777/
Abstract

This study aimed at assessing renal functions in patients with transfusion-dependent thalassemia (TDT). Fifty patients and 30 controls were enrolled in this prospective study. Serum levels of electrolytes and albumin were measured by a spectrophotometer. Serum levels of cystatin-C and urinary levels of β2-microglobulin were measured by nephelometric method. Thirty-eight patients were receiving deferasirox and 8 were on deferiprone. Serum electrolytes and albumin levels of the patients were found to be within normal ranges. Urinary β2-microglobulin and serum cystatin-C levels were significantly higher in patients than controls. They did not significantly differ between the subgroup of patients on deferiprone and the control group, whereas they were found to be higher in patients using deferasirox compared to controls. Urinary β2-microglobulin levels significantly increased in patients who were receiving high-dose deferasirox compared to those who were receiving a daily dose of 15-20 mg/kg or controls. Subclinical renal injury may be present in TDT patients.

摘要

本研究旨在评估依赖输血的地中海贫血(TDT)患者的肾功能。本前瞻性研究纳入了50例患者和30例对照。采用分光光度计测量血清电解质和白蛋白水平。采用散射比浊法测量血清胱抑素-C水平和尿β2-微球蛋白水平。38例患者接受地拉罗司治疗,8例患者接受去铁酮治疗。患者的血清电解质和白蛋白水平在正常范围内。患者的尿β2-微球蛋白和血清胱抑素-C水平显著高于对照组。接受去铁酮治疗的患者亚组与对照组之间无显著差异,而与对照组相比,使用地拉罗司的患者上述指标更高。与接受每日剂量15 - 20mg/kg的患者或对照组相比,接受高剂量地拉罗司治疗的患者尿β2-微球蛋白水平显著升高。TDT患者可能存在亚临床肾损伤。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1874/5843777/f9e6afc212e3/TJH-35-66-g6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1874/5843777/20653f6c711a/TJH-35-66-g5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1874/5843777/f9e6afc212e3/TJH-35-66-g6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1874/5843777/20653f6c711a/TJH-35-66-g5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1874/5843777/f9e6afc212e3/TJH-35-66-g6.jpg

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本文引用的文献

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Ann Hematol. 2016 Feb;95(3):375-81. doi: 10.1007/s00277-015-2561-2. Epub 2015 Nov 23.
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Frequency of glomerular dysfunction in children with Beta thalassaemia major.重型β地中海贫血患儿肾小球功能障碍的发生率
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Iron overload in β-thalassemia intermedia: an emerging concern.
接受螯合剂治疗的铁过载β地中海贫血患者肾脏并发症的标志物:一项系统评价
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Renal function in β-thalassemia major patients treated with two different iron-chelation regimes.β-重型地中海贫血患者接受两种不同铁螯合治疗方案的肾功能。
BMC Nephrol. 2021 Dec 20;22(1):418. doi: 10.1186/s12882-021-02630-5.
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Comparing different markers of tubular dysfunction in transfusion-dependent thalassemia patients.比较依赖输血的地中海贫血患者不同的管状功能障碍标志物。
Int Urol Nephrol. 2022 Feb;54(2):421-428. doi: 10.1007/s11255-021-02914-7. Epub 2021 Jun 24.
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The multifaceted role of iron in renal health and disease.铁在肾脏健康和疾病中的多方面作用。
Nat Rev Nephrol. 2020 Feb;16(2):77-98. doi: 10.1038/s41581-019-0197-5. Epub 2019 Sep 25.
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β-中间型地中海贫血中的铁过载:一个新出现的问题。
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