Department of Pediatric Hematology/Oncology/Hemostaseology, University Medical Center Mainz, Langenbeckstraße 1, 55131, Mainz, Germany.
Institute of Clinical Chemistry and Laboratory Medicine, University Medical Center Mainz, Mainz, Germany.
Pediatr Nephrol. 2017 Oct;32(10):1915-1925. doi: 10.1007/s00467-017-3673-9. Epub 2017 Apr 28.
Considering the improved outcome, a better understanding of the late effects in Wilms tumor survivors (WT-S) is needed. This study was aimed at evaluating renal function and determining the prevalence of clinical and subclinical renal dysfunction in a cohort of WT-S using a multimodal diagnostic approach.
Thirty-seven WT-S were included in this prospective cross-sectional single center study. To evaluate renal function, glomerular filtration rate (GFR) and urinary protein excretion were assessed. Additionally, kidney sonomorphology and blood pressure were analyzed.
All examined WT-S (mean age 28.7 years, mean follow-up 24.8 years) had been treated with a combination of surgery and chemotherapy; 59.5% had received adjuvant radiotherapy. Impaired glomerular renal function was detected in a considerable proportion of WT-S, with age-adjusted cystatin-based GFR estimation below age norm in 55.9%. A lower cystatin-based estimated GFR (eGFR) correlated with longer follow-up time and higher irradiation dose. In 5 patients (13.5%) albuminuria was identified. Analysis of sonomorphology detected compensatory contralateral renal hypertrophy in 83.3% of WT-S. Chronic kidney disease (CKD) ≥ stage II was present in 55.9% of WT-S. Blood pressure measurements revealed arterial hypertension in 15 (40.5%) WT-S (newly diagnosed n=10). In 24.3% both CKD ≥ stage II and arterial hypertension were determined.
Even though WT-S are believed to carry a low risk for end-stage renal disease, in this study, a remarkable number of WT-S presented with previously unidentified subclinical signs of renal function impairment and secondary morbidity. Therefore, it is important to continue regular follow-up, especially after transition into adulthood.
考虑到预后改善,有必要更好地了解威尔姆斯瘤幸存者(WT-S)的晚期效应。本研究旨在采用多模态诊断方法评估一组 WT-S 的肾功能,并确定临床和亚临床肾功能障碍的患病率。
本前瞻性横断面单中心研究纳入 37 名 WT-S。为了评估肾功能,评估了肾小球滤过率(GFR)和尿蛋白排泄。此外,还分析了肾脏超声形态和血压。
所有接受检查的 WT-S(平均年龄 28.7 岁,平均随访 24.8 年)均接受手术和化疗联合治疗;59.5%接受了辅助放疗。相当一部分 WT-S 肾小球肾功能受损,年龄校正后基于半胱氨酸的 GFR 估计值低于年龄正常值的 55.9%。较低的基于半胱氨酸的估计肾小球滤过率(eGFR)与随访时间较长和照射剂量较高相关。5 名患者(13.5%)存在白蛋白尿。超声形态学分析发现 83.3%的 WT-S 存在代偿性对侧肾肥大。55.9%的 WT-S 存在慢性肾脏病(CKD)≥Ⅱ期。血压测量显示 15 名 WT-S(新诊断 10 名)存在动脉高血压。24.3%的患者同时存在 CKD≥Ⅱ期和动脉高血压。
尽管 WT-S 被认为发生终末期肾病的风险较低,但在本研究中,相当数量的 WT-S 出现了以前未识别的亚临床肾功能损害和继发性发病率的迹象。因此,继续定期随访非常重要,特别是在进入成年期后。