Department of Pediatrics, Division of Nephrology, BC Children's Hospital, 4480 Oak Street, ACB K4-151, Vancouver, British Columbia, Canada.
Pediatr Nephrol. 2011 Apr;26(4):605-12. doi: 10.1007/s00467-010-1737-1. Epub 2010 Dec 29.
With increasing life expectancy and the need for lung transplantation in the cystic fibrosis (CF) population, there are increasing reports of chronic kidney disease (CKD). However, values for baseline or longitudinal glomerular filtration rate (GFR) as measured by exogenous clearance markers are lacking in this population. Retrospective cross-sectional study in 2 to 18-year-olds cared for at a single CF center who had a GFR measured by plasma disappearance of Technetium-99 m diethylenetriaminepentaacetic acid (mGFR). The primary outcome was evidence of renal dysfunction as defined by CKD stage II or below (mGFR <90 ml/min/1.73 m(2), persistent abnormalities in urinary sediment, abnormal renal imaging). Of 63 patients evaluated, four had apparent renal dysfunction, one demonstrated decreased mGFR, and three others had persistent microscopic hematuria. The mean mGFR was substantially higher (140 ± 24 ml/min/1.73 m(2)) than expected or previously reported for healthy children. We did not demonstrate the presence of significant renal impairment after limited aminoglycoside exposure in the first decade following diagnosis with CF. However, we did document the presence of glomerular hyperfiltration in a significant proportion of our CF patients.
随着预期寿命的延长和囊性纤维化 (CF) 人群中肺移植的需求增加，慢性肾脏病 (CKD) 的报告也越来越多。然而，在该人群中，缺乏外源性清除标志物测量的基线或纵向肾小球滤过率 (GFR) 值。对在单一 CF 中心接受治疗的 2 至 18 岁患者进行的回顾性横断面研究，这些患者的 GFR 通过血浆中 Technetium-99 m 二乙三胺五乙酸的消失来测量 (mGFR)。主要结局是根据 CKD 分期 II 或以下定义的肾功能障碍的证据（mGFR<90ml/min/1.73m(2)，尿沉渣持续异常，肾脏影像学异常）。在评估的 63 名患者中，有 4 名患者出现明显的肾功能障碍，1 名患者的 mGFR 降低，另有 3 名患者持续出现镜下血尿。mGFR 的平均值（140±24ml/min/1.73m(2)）明显高于预期或以前报道的健康儿童。在 CF 诊断后的第一个十年中，我们在有限的氨基糖苷类药物暴露后，并未证明存在明显的肾功能损害。然而，我们确实记录了相当一部分 CF 患者存在肾小球高滤过。
