#2357 Kidney volume based on multi-dimensional orthogonal ultrasound measurements is more accurate than kidney length in evaluation of kidney growth in paediatric ADPKD

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Abstract Background and Aims Monitoring kidney growth is crucial in paediatric autosomal dominant polycystic kidney disease (ADPKD). While kidney length is often used as a surrogate for kidney growth, its accuracy in reflecting dynamic volume changes, especially during growth spurts, is unclear. In addition to kidney length, this study investigated the value of measuring kidney width and depth on ultrasound to estimate total kidney volume (TKV) in a cohort of paediatric ADPKD patients, focusing on the contribution of each dimension to overall kidney growth during childhood. Method All 2D ultrasound measurements of patients included in the international ADPedKD registry extracted in November 2024 were evaluated. A total of 1506 patients and 6648 entries (see Fig. 1) were analysed. Of these, only a subset of 611 patients with 1625 evaluations (∼2.7 evaluations per patient) had completed data for length, width, and depth of both kidneys, allowing for TKV estimation using the 2D ellipsoid formula. Regression analysis was performed to assess the growth rates of kidney length, width and depth, averaged over both kidneys, as well as estimated TKV before and after the age of 10 years. Results The 611 patients (309 females, 302 males) with complete imaging data had proven genotypes of PKD1 (n = 292), PKD2 (n = 29), GANAB (n = 1), and no genotyping in the remaining cases (n = 289). Regression analysis demonstrated a marked acceleration in TKV growth after age 10 years (30.5 mL/yr) compared to before (10.9 mL/yr), representing a nearly threefold increase. However, when looking at kidney length growth rates, this substantial increase in TKV was not present at all; they even decreased slightly in the second decade (0.33 vs 0.31 cm/yr). In contrast, both width and depth exhibited similar increased growth rates after age 10, with width increasing from 0.09 to 0.18 cm/yr and depth increasing from 0.09 to 0.14 cm/yr. Although these increases were smaller in magnitude than the change in TKV, they demonstrate that width and depth contribute significantly to the observed volumetric growth acceleration in ADPKD patients after 10 years of age. Conclusion Our results indicate that, in paediatric ADPKD patients, incorporating kidney width and depth measurements in routine ultrasound assessments provides crucial kidney growth information not adequately captured by kidney length alone. This allows to provide a kidney volume estimation and as such a more accurate and comprehensive assessment of disease progression.

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