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Background: Infants with a solitary functioning kidney (SFK) are at risk for chronic kidney injury (CKI). Lack of compensatory kidney growth (CKG) is associated with CKI, but measuring CKG is challenging since it is typically reported relative to normal kidneys. This study aims to (1) standardize SFK growth in infants, (2) investigate the relationship between standardized kidney length and clinical outcomes, and (3) use these results to develop a risk-based prediction model and local clinical pathway for SFK care. Methods: This was a quality improvement study of 166 infants with an SFK. Linear regression was used to assess kidney growth from 0 to 180 days of life. Univariate binary regression analysis was used to identify kidney length to body length thresholds associated with the development of CKI, defined as the composite outcome of chronic kidney disease (eGFR < 60 mL/min/1.73 m2), hypertension, or proteinuria. Results: Kidneys grew in length from 0 to 180 days, and growth was constant when standardized to body length. Over follow-up, infants with a baseline kidney length to body length ≤ 0.088 were more likely to experience CKI than the rest of the cohort (27 vs. 8%, p = 0.04). Kidney length to body length ≤ 0.088 was also significantly associated with CKI development (OR 4.17, 95% CI 1.14–15.28, p = 0.04). Conclusions: In this study, kidney length to body length ratio was a stable CKG metric over 0–180 days, and a baseline ratio ≤ 0.088 was a risk factor for CKI. Results will aid in developing a practical, point-of-care risk assessment tool, and overarching risk-stratified clinical pathway for infants with an SFK. A higher resolution version of the Graphical abstract is available as Supplementary information [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]