Matsell Douglas G, Cojocaru Dan, Matsell Eli W, Eddy Allison A
Department of Pediatrics, British Columbia Children's Hospital, Vancouver, BC, Canada,
Pediatr Nephrol. 2015 Sep;30(9):1501-9. doi: 10.1007/s00467-015-3079-5. Epub 2015 Mar 21.
Small kidneys due to renal hypodysplasia (RHD) result from a decrease in nephron number. The objectives of this study were to identify clinical variables that determine long-term renal outcome in children with RHD and to define the role of kidney size as a predictor of developing end-stage renal disease (ESRD).
This was a single-center retrospective cohort analysis. The primary outcome was development of ESRD. We identified 202 RHD cases, with 25 (12%) reaching ESRD at mean age of 8.9 (±6.6) years.
Children with RHD with a known genetic syndrome had the smallest kidneys while those with posterior urethral valves (PUV) had the largest kidneys at diagnosis. Cases with bilateral RHD were most likely to develop ESRD. Younger gestational age (OR 0.8, CI 0.69-0.99, p = 0.05), smaller kidney size at diagnosis (OR 0.13, CI 0.03-0.47, p = 0.002), lower best-estimated glomerular filtration rate (eGFR) (OR 0.74, CI 0.58-0.93, p = 0.01), proteinuria (OR 1.03, CI 1.01-1.05, p < 0.001) and high blood pressure (OR 1.02, CI 1.01-1.04, p = 0.01) were associated with development of ESRD, while kidney size at diagnosis was independently associated with ESRD (HR 0.03, CI 0.01-0.72, p = 0.043).
In children with RHD, kidney size at diagnosis predicts the likelihood of developing ESRD.
肾发育不全（RHD）导致的小肾脏是由于肾单位数量减少所致。本研究的目的是确定决定RHD患儿长期肾脏预后的临床变量，并明确肾脏大小作为终末期肾病（ESRD）发生预测指标的作用。
这是一项单中心回顾性队列分析。主要结局是ESRD的发生。我们确定了202例RHD病例，其中25例（12%）在平均年龄8.9（±6.6）岁时发展为ESRD。
已知患有遗传综合征的RHD患儿的肾脏最小，而后尿道瓣膜（PUV）患儿在诊断时的肾脏最大。双侧RHD病例最有可能发展为ESRD。较小的孕周（OR 0.8，CI 0.69 - 0.99，p = 0.05）、诊断时较小的肾脏大小（OR 0.13，CI 0.03 - 0.47，p = 0.002）、较低的最佳估计肾小球滤过率（eGFR）（OR 0.74，CI 0.58 - 0.93，p = 0.01）、蛋白尿（OR 1.03，CI 1.01 - 1.05，p < 0.001）和高血压（OR 1.02，CI 1.01 - 1.04，p = 0.01）与ESRD的发生相关，而诊断时的肾脏大小与ESRD独立相关（HR 0.03，CI 0.01 - 0.72，p = 0.043）。
在RHD患儿中，诊断时的肾脏大小可预测发生ESRD的可能性。
