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地中海贫血患者的肾功能障碍。

Renal dysfunction in patients with thalassaemia.

机构信息

U.T. Southwestern Medical Center, Dallas, TX, USA.

出版信息

Br J Haematol. 2011 Apr;153(1):111-7. doi: 10.1111/j.1365-2141.2010.08477.x. Epub 2011 Feb 21.

Abstract

Little is known about the effects of thalassaemia on the kidney. Characterization of underlying renal function abnormalities in thalassaemia is timely because the newer iron chelator, deferasirox, can be nephrotoxic. We aimed to determine the prevalence and correlates of renal abnormalities in thalassaemia patients, treated before deferasirox was widely available, using 24-h collections of urine. We calculated creatinine clearance and urine calcium-to-creatinine ratio and measured urinary β(2) -microglobulin, albumin, and protein. We used multivariate modelling to identify clinical, therapeutic, and laboratory predictors of renal dysfunction. One-third of thalassaemia patients who were not regularly transfused had abnormally high creatinine clearance. Regular transfusions were associated with a decrease in clearance (P = 0·004). Almost one-third of patients with thalassaemia had hypercalciuria, and regular transfusions were associated with an increase in the frequency and degree of hypercalciuria (P < 0·0001). Albuminuria was found in over half of patients, but was not consistently associated with transfusion therapy. In summary, renal hyperfiltration, hypercalciuria, and albuminuria are common in thalassaemia. Higher transfusion intensity is associated with lower creatinine clearance but more frequent hypercalciuria. The transfusion effect needs to be better understood. Awareness of underlying renal dysfunction in thalassaemia can inform decisions now about the use and monitoring of iron chelation.

摘要

目前对于地中海贫血症对肾脏的影响知之甚少。由于新型铁螯合剂地拉罗司可能具有肾毒性,因此及时对地中海贫血症患者的潜在肾功能异常进行特征描述非常重要。本研究旨在通过 24 小时尿液收集,确定在广泛应用地拉罗司之前接受治疗的地中海贫血症患者的肾脏异常的患病率和相关因素。我们计算了肌酐清除率和尿钙/肌酐比值,并测量了尿 β(2)-微球蛋白、白蛋白和蛋白。我们使用多元模型来确定肾功能障碍的临床、治疗和实验室预测因素。三分之一未定期输血的地中海贫血症患者的肌酐清除率异常升高。定期输血与清除率降低相关(P = 0.004)。近三分之一的地中海贫血症患者存在高钙尿症,且定期输血与高钙尿症的频率和程度增加相关(P < 0.0001)。超过一半的患者存在蛋白尿,但与输血治疗并不一致。总之,在地中海贫血症中,肾脏高滤过、高钙尿症和蛋白尿很常见。更高的输血强度与肌酐清除率降低有关,但与更频繁的高钙尿症有关。需要更好地了解输血的影响。了解地中海贫血症患者潜在的肾功能障碍可以为现在有关铁螯合作用的应用和监测的决策提供信息。

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