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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.

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/1874/5843777/20653f6c711a/TJH-35-66-g5.jpg

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