First Department of Pediatrics, Aristotle University of Thessaloniki, 'Hippokration' General Hospital, Thessaloniki, Greece.
Acta Haematol. 2010;123(3):148-52. doi: 10.1159/000287238. Epub 2010 Feb 24.
There are limited studies on renal involvement in beta-thalassemia, mainly involving patients on deferoxamine, reporting both glomerular and tubular dysfunction. The aim of the present study was to investigate renal involvement in young thalassemia patients, using both conventional and early markers of renal dysfunction, and to correlate findings to iron chelation therapy. Forty-two patients aged 4-23 years were studied and, for analysis purposes, were divided into two groups based on chelation therapy (group A receiving deferasirox and group B receiving deferoxamine and deferiprone combination therapy). In addition to conventional renal biochemistries, creatinine clearance, estimated glomerular filtration rate, serum cystatin C (Cys C), fractional excretion of sodium, tubular phosphorus reabsorption and urine calcium, protein, beta(2)-microglobulin (beta(2)-MG) and glucose levels were measured. A considerable number of patients demonstrated impaired renal function with elevated Cys C levels (36%), glomerular dysfunction with proteinuria (24%) and tubulopathy with hypercalciuria (35.5%) and elevated excretion of beta(2)-MG (33.5%). Renal involvement seems to be present even in young patients with beta-thalassemia, therefore, routine use of early markers of renal dysfunction is recommended. Further studies are needed in order to investigate the role of new chelators in tubular function parameters.
有关β-地中海贫血的肾脏受累的研究较少,主要涉及接受去铁胺治疗的患者,报告既有肾小球功能障碍也有肾小管功能障碍。本研究旨在通过使用常规和早期肾功能障碍标志物,来研究年轻地中海贫血患者的肾脏受累情况,并将结果与铁螯合疗法相关联。研究了 42 名年龄在 4-23 岁的患者,并根据螯合疗法(A 组接受地拉罗司,B 组接受去铁胺和地拉罗司联合治疗)将其分为两组进行分析。除了常规的肾脏生化指标外,还测量了肌酐清除率、估计肾小球滤过率、血清胱抑素 C(Cys C)、钠的分数排泄率、管状磷重吸收率和尿钙、蛋白质、β2-微球蛋白(β2-MG)和葡萄糖水平。相当数量的患者表现出肾功能受损,Cys C 水平升高(36%)、蛋白尿伴肾小球功能障碍(24%)、高钙尿症伴肾小管病(35.5%)和β2-MG 排泄增加(33.5%)。即使是年轻的β-地中海贫血患者,肾脏受累似乎也很常见,因此,建议常规使用早期肾功能障碍标志物。需要进一步研究以探讨新型螯合剂在肾小管功能参数中的作用。