Abstract
Background
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).
Methods
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.
Results
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).
Conclusions
In children with RHD, kidney size at diagnosis predicts the likelihood of developing ESRD.
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Supplemental Figure 1
Identification and selection of cases. Cases with the diagnosis of renal dysplasia or RHD using ICD-9 and 10 codes were identified from the BCCH Nephrology datasets from 1983–2013. A total of 202 cases of RHD were selected from the initial group of 429 patients coded as RHD using the established entry criteria. 1MCDK = multicystic dysplastic kidney, 2 abnormal parenchyma on renal ultrasound scan with normal size, VUR = vesicoureteral reflux, HN = hydronephrosis. (PDF 29 kb)
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Matsell, D.G., Cojocaru, D., Matsell, E.W. et al. The impact of small kidneys. Pediatr Nephrol 30, 1501–1509 (2015). https://doi.org/10.1007/s00467-015-3079-5
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DOI: https://doi.org/10.1007/s00467-015-3079-5