Volume 59, Issue 5, Pages 1654-1662 (May 2001) Progression of autosomal-dominant polycystic kidney disease in children1  Godela M. Fick-Brosnahan, Zung Vu Tran, Ann M. Johnson, John D. Strain, Patricia A. Gabow  Kidney International  Volume 59, Issue 5, Pages 1654-1662 (May 2001) DOI: 10.1046/j.1523-1755.2001.0590051654.x Copyright © 2001 International Society of Nephrology Terms and Conditions

Figure 1 Ultrasound findings and gene linkage analysis (GLA) results in the study children (1985 to 1999). Abbreviations are: US, ultrasound; GLA, gene linkage analysis; NA, not available; +, positive; -, negative. Kidney International 2001 59, 1654-1662DOI: (10.1046/j.1523-1755.2001.0590051654.x) Copyright © 2001 International Society of Nephrology Terms and Conditions

Figure 2 Ultrasound findings in 185 autosomal dominant polycystic kidney disease (ADPKD) children at their first visit and at follow-up. Abbreviations are: US, ultrasound; GLA, gene linkage analysis. Kidney International 2001 59, 1654-1662DOI: (10.1046/j.1523-1755.2001.0590051654.x) Copyright © 2001 International Society of Nephrology Terms and Conditions

Figure 3 Relationship between mean renal volume (MRV; cm3) and age (years) in 182 affected (solid line; ultrasound-positive) and 127 unaffected (dashed line) children who had one to four visits before the age of 20 years. The regression model that gave the best fit for the data was the growth model. The equation for the affected children is MRV = e3.679+0.103×age, with R2 = 0.61; for the unaffected children MRV = e3.399+0.095×age, with R2 = 0.77. The β coefficients of the growth curves differ significantly (1.38 vs. 1.05; P < 0.001) between the affected and unaffected children, with the curve for the affected children being steeper, indicating that renal volume increases faster over time in the affected than the unaffected children. Kidney International 2001 59, 1654-1662DOI: (10.1046/j.1523-1755.2001.0590051654.x) Copyright © 2001 International Society of Nephrology Terms and Conditions

Figure 4 (A) Scatter plot of individual MRV in affected children according to age. The growth model is represented by the black dots, whereas the actual data are plotted in gray. (B) Scatter plot of individual MRV in unaffected children according to age. The growth model is represented by the black dots, whereas the actual data are plotted in gray. Kidney International 2001 59, 1654-1662DOI: (10.1046/j.1523-1755.2001.0590051654.x) Copyright © 2001 International Society of Nephrology Terms and Conditions

Figure 5 (A) Relationship between MRV normalized for height and age in 46 unaffected boys (solid line) and 81 unaffected girls (dashed line). The equation for boys is: MRV = e3.937+0.051×age, with R2 = 0.45; for girls MRV = e3.862+0.049×age, with R2 = 0.57. The curves and the β coefficients (0.97 vs. 0.81 for boys vs. girls) are not significantly different, indicating that the height-normalized MRV of unaffected boys and girls are similar and increase at a similar rate (the same is true when MRV are not normalized for height). (B) Relationship between MRV normalized for height and age in 90 affected boys and 92 affected girls. The equation for boys is: MRV = e4.246+0.048×age, with R2 = 0.27; for girls MRV = e4.089+0.065×age, with R2 = 0.34. The curves and the β coefficients (0.67 vs. 0.58 for boys vs. girls) are not significantly different, indicating that the height-normalized MRV of affected boys and girls are similar and increase at a similar rate (the same is true when MRV are not normalized for height). Kidney International 2001 59, 1654-1662DOI: (10.1046/j.1523-1755.2001.0590051654.x) Copyright © 2001 International Society of Nephrology Terms and Conditions

Figure 6 Mean renal volume according to age in 108 ADPKD children who had two or more visits before age 20 years, separated into curves for 30 children with early severe disease (—) and 78 children who did not have early severe disease (--). For the “early severe” children MRV = e3.863+0.131×age, R2 = 0.89. For the children with mild disease MRV = e3.395+0.107×age, R2 = 0.79. The β coefficients of the curves were significantly different (1.74 vs. 1.40, P < 0.05), with the curve of the early severe children being significantly steeper, indicating significantly faster renal growth. Kidney International 2001 59, 1654-1662DOI: (10.1046/j.1523-1755.2001.0590051654.x) Copyright © 2001 International Society of Nephrology Terms and Conditions

Figure 7 Mean renal volume according to age in 33 ADPKD children with blood pressures above the 75th percentile at the first and last visit (HBP 75; —) and 37 ADPKD children with blood pressures below the 75th percentile at both visits (NBP; --). For the HBP 75 children MRV = e3.765+0.107×age, with R2 = 0.65; for the NBP children MRV = e3.570+0.099×age, with R2 = 0.70. The β coefficients of the curves were significantly different (1.22 vs. 1.11, P < 0.05), with the curve of the HBP 75 children being significantly steeper, indicating significantly faster renal growth than the NBP children. Kidney International 2001 59, 1654-1662DOI: (10.1046/j.1523-1755.2001.0590051654.x) Copyright © 2001 International Society of Nephrology Terms and Conditions