Hashemieh Mozhgan, Radfar Mitra, Azarkeivan Azita, Hosseini Tabatabaei Seyed Mohammad Taghi, Nikbakht Sedigheh, Yaseri Mehdi, Sheibani Kourosh
Imam Hossein Medical Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Research Center of Iranian Blood Transfusion Organization, Thalassemia Clinic, Tehran, Iran.
Int J Hematol Oncol Stem Cell Res. 2017 Apr 1;11(2):133-138.
In recent years, the success in management of thalassemic patients, has allowed for some previously unrecognized complications including renal abnormalities to emerge. This prospective study aimed to investigate kidney iron overload by means of MRI T2* and also renal function based on laboratory tests for early markers of glomerular and tubular dysfunction among adult Iranian transfusion-dependent thalassemia major patients. Two-hundred and two patients with transfusion-dependent β-thalassemia major were included in this study in Zafar Adult Thalassemia Center, Tehran, Iran. For all patients, kidney MRI T2* as well as evaluation of BUN, creatinine, uric acid, calcium, phosphorus, sodium (Na), potassium (K), total protein, albumin, cystatin C, serum ferritin β2-microglobulin, NAG (N-acetyl-beta-D-Glucosaminidase), and urine protein were performed. One-hundred and fourteen female and 88 male transfusion-dependent β-thalassemia major patients with mean age of 30.1 ± 9.4 participated in the present study. We found that 77.7% of our patients had kidney hemosiderosis based on MRI T2*. Also, 67 patients (33.2%) had elevation of serum cystatin C, and 104 patients (51.5%) had reduced estimated glomerular filtration rate (e-GFR). Increased urinary excretion of NAG and hypercalciuria were found in 50% and 79.2% of participants, respectively. Renal hemosiderosis and asymptomatic renal dysfunction are prevalent among transfusion- dependent β-thalassemia major patients which necessitate regular screening with early markers of glomerular and tubular dysfunction. Further studies in order to investigate the correlation between renal hemosiderosis and early markers of kidney dysfunction among these patients are recommended.
近年来，地中海贫血患者管理方面的成功使得一些以前未被认识到的并发症出现，包括肾脏异常。这项前瞻性研究旨在通过MRI T2* 研究肾脏铁过载情况，并基于实验室检测评估成年伊朗重型输血依赖型地中海贫血患者肾小球和肾小管功能障碍早期标志物的肾功能。伊朗德黑兰扎法尔成人地中海贫血中心纳入了202例重型输血依赖型β地中海贫血患者。对所有患者进行了肾脏MRI T2* 检查以及血尿素氮、肌酐、尿酸、钙、磷、钠（Na）、钾（K）、总蛋白、白蛋白、胱抑素C、血清铁蛋白、β2微球蛋白、NAG（N-乙酰-β-D-氨基葡萄糖苷酶）和尿蛋白评估。114例女性和88例男性重型输血依赖型β地中海贫血患者参与了本研究，平均年龄为30.1±9.4岁。我们发现，基于MRI T2*，77.7%的患者存在肾脏含铁血黄素沉着。此外，67例患者（33.2%）血清胱抑素C升高，104例患者（51.5%）估算肾小球滤过率（e-GFR）降低。分别有50%和79.2% 的参与者NAG尿排泄增加和高钙尿症。重型输血依赖型β地中海贫血患者中肾脏含铁血黄素沉着和无症状性肾功能障碍很常见，这需要用肾小球和肾小管功能障碍的早期标志物进行定期筛查。建议进一步开展研究以调查这些患者中肾脏含铁血黄素沉着与肾功能障碍早期标志物之间的相关性。
