重型β地中海贫血、去铁胺的使用与近端肾小管损伤

Major beta-thalassemia, use of desferiexamine and renal proximal tubular damage.

作者信息

Jafari H M, Vahidshahi K, Kosaryan M, Karami H, Reza Mahdavi M, Ehteshami S

机构信息

Department of Pediatrics, Mazandaran University of Medical Sciences, Sari, Iran.

出版信息

Bratisl Lek Listy. 2011;112(5):278-81.

PMID:21682083

Abstract

BACKGROUND AND PURPOSE

Thalassemia is a quantitative hereditary hemoglobinopathy, which is one of common hereditary diseases in the Mediterranean region including Iran. Homozygotic beta-thalassemia patients have manifestations and complications such as severe anemia and multiorgan dysfunction. Recently there have been some concerns about renal complications in â-thalassemia but some controversies have been demonstrated. The aim of this study was to investigate the renal function of major â-thalassemia patients in comparison with control group.

METHODS AND MATERIALS

This was a historical cohort. Study population consisted of major beta-thalassemia patients who were under a management plan of thalassemia ward of Bo Ali sina hospital sari, IRAN (case group ) and uninvolved brothers or sisters of the patients (the control group). Two groups (case and control) were matched. Renal function of patients was investigated by measurement of renal and serum markers. Demographic and therapeutic data of patients were extracted from medical records. The data were analyzed using SPSS 11.

FINDINGS

Eighty-four samples were studied in total, while 42 of them were from the case group and the rest from the control group. The case and control groups were matched according to gender and age. Mean age of patients was 21.3 +/- 5.2 years. Desferiexamin was dosed in amount of 70 +/- 19 mg/kg. There was no significant difference in the measures of 24-hour urine protein (beta-2 microglobin and fraction excretion of Na and K between case and control groups. Significant differences were however in the measures of Bun serum and creatinin as well as K (from both urine and serum). Gender, Hb, and level of ferritin related significantly with differences.

CONCLUSION

This study demonstrates no evidence of proximal tubular damage in major â-thalassemia patients. Nevertheless, the patients with severe anemia, high dose of desferal and high level of ferritin yielded an increase in the levels of Bun, serum K, and in some cases in uric acid (Tab. 4, Ref. 33).

摘要

背景与目的

地中海贫血是一种定量遗传性血红蛋白病，是包括伊朗在内的地中海地区常见的遗传性疾病之一。纯合子β地中海贫血患者有严重贫血和多器官功能障碍等表现及并发症。最近，人们对β地中海贫血的肾脏并发症有所关注，但也存在一些争议。本研究的目的是比较重型β地中海贫血患者与对照组的肾功能。

方法与材料

这是一项历史性队列研究。研究人群包括伊朗萨里市博阿利·西纳医院地中海贫血病房管理计划下的重型β地中海贫血患者（病例组）以及患者未患病的兄弟姐妹（对照组）。两组（病例组和对照组）进行了匹配。通过测量肾脏和血清标志物来研究患者的肾功能。从病历中提取患者的人口统计学和治疗数据。使用SPSS 11对数据进行分析。

研究结果

共研究了84个样本，其中42个来自病例组，其余来自对照组。病例组和对照组在性别和年龄上进行了匹配。患者的平均年龄为21.3±5.2岁。去铁胺的剂量为70±19mg/kg。病例组和对照组之间24小时尿蛋白（β2微球蛋白以及钠和钾的分数排泄）的测量值没有显著差异。然而，血尿素氮、肌酐以及钾（尿液和血清中的）的测量值存在显著差异。性别、血红蛋白和铁蛋白水平与差异显著相关。