Table 2.
LIC Severity | Class A | Class B | Class C | Class D | Class E |
---|---|---|---|---|---|
Number of patients | 18 | 16 | 20 | 12 | 8 |
Sex, M/F | 6/12 | 8/8 | 13/7 | 7/5 | 3/5 |
Age, yr | |||||
Median (LQR–UQR) | 11 (7–16) | 12 (8–15) | 7 (4–12) | 10 (4–14) | 6 (4–8) |
Range | 1.00–18 | 0–18 | 0–17 | 1–19 | 3–15 |
Systolic BP, mm Hg | |||||
Median (LQR–UQR) | 103 (89–116) | 104 (86–113) | 100 (87–116) | 108 (92–111) | 96 (93–113) |
Range | 81–127 | 80–140 | 71–138 | 85–126 | 82–118 |
Systolic BP, mm Hg, SDS | |||||
n (%) | 18 (100) | 16 (100) | 18 (90) | 10 (83) | 7 (87.5) |
Median (LQR–UQR) | 0.00 (−0.70 to 0.50) | 0.00 (−0.68 to 0.57) | 0.00 (−0.70 to 0.60) | 0.00 (−0.70 to 0.50) | −0.05 (−0.73 to 0.50) |
Range | −4.3 | −2.30 to 2.00 | −2.30 to 2.00 | −2.30 to 2.00 | −2.30 to 2.00 |
Diastolic BP, mm Hg | |||||
Median (LQR–UQR) | 55 (52–64) | 59 (54–68) | 56 (51–59) | 60 (60–64) | 54 (50–63) |
Range | 40–70 | 47–78 | 47–76 | 56–67 | 48–71 |
Diastolic BP, mm Hg, SDS | |||||
n (%) | 18 (100) | 16 (100) | −0.18 (90) | 10 (83.3) | 7 (87.5) |
Median (LQR–UQR) | 0.25 (−0.60 to 0.30) | −0.25 (−0.60 to 0.30) | −0.30 (−0.60 to 0.30) | −0.20 (−0.60 to 0.30) | −0.25 (−0.60 to 0.30) |
Range | −3.8 | −1.60 to 2.20 | −1.60 to 2.20 | −1.60 to 2.20 | −1.60 to 2.20 |
Hypertension, n (%) | 3 (16.7) | 1 (6.25) | 2 (10.0) | 3 (25.0) | 1 (12.5) |
TKV, ml | |||||
Median (LQR–UQR) | 179 (101–272) | 220 (133–348) | 158 (106–288) | 285 (159–532) | 209 (184–284) |
Range | 58–371 | 60–508 | 58–630 | 76–901 | 147–706 |
hTKV, ml/m | |||||
Median (LQR–UQR) | 124 (89–161) | 146 (112–195) | 126 (103–182) | 193 (124–318) | 180 (164–217) |
Range | 74–198 | 89–286 | 91–319 | 103–551 | 141–445 |
Genetics | |||||
PKD1 % (n) | 19 (14) | 22 (16) | 27 (20) | 15 (11) | 11 (8) |
Truncating % (n) | 29 (4) | 25 (4) | 15 (3) | 27 (3) | 38 (3) |
Nontruncating % (n) | 71 (10) | 75 (12) | 85 (17) | 73 (8) | 63 (5) |
PKD2 % (n) | 5 (4) | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
Truncating % (n) | 75 (3) | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
Nontruncating % (n) | 25 (1) | 0 (0) | 0 (0) | 0 (0) | 0 (0) |
GANAB % (n) | 0 (0) | 0 (0) | 0 (0) | 1.35 (1) | 0 (0) |
Serum creatinine, mg/dl | |||||
Median (LQR–UQR) | 0.56 (0.46–0.71) | 0.52 (0.39–0.60) | 0.44 (0.30–0.59) | 0.49 (0.29–0.75) | 0.37 (0.32–0.55) |
Range | 0.30–0.96 | 0.27–0.99 | 0.18–0.74 | 0.18–1.43 | 0.21–0.65 |
Height, cm | |||||
Median (LQR–UQR) | 148 (125–167) | 153 (119–168) | 126 (104–156) | 148 (110–165) | 122 (105–136) |
Range | 78–188 | 67–198 | 55–198 | 73–177 | 103–159 |
Weight, kg | |||||
Median (LQR–UQR) | 32 (22–53) | 41 (19–55) | 25 (16–42) | 35 (21–54) | 24 (17–29) |
Range | 11–77 | 8–76 | 4–98 | 10–97 | 14–42 |
BMI, kg/m 2 | |||||
Median (LQR–UQR) | 16 (14–18) | 18 (15–19) | 17 (15–19) | 18 (17–20) | 16 (14–17) |
Range | 13–26 | 13–23 | 13–28 | 15–31 | 13–18 |
BMI, kg/m 2 , SDS | |||||
n (%) | 18 (100) | 15 (94) | 17 (85) | 11 (92) | 7 (88) |
Median (LQR–UQR) | −0.4 (−1.1 to 0.5) | −0.6 (−1.2 to 0.5) | −0.4 (−1.1 to 0.5) | −0.3 (−1.1 to 0.6) | −0.5 (−1.1 to 0.7) |
Range | −2.6 to 2.2 | −2.6 to 2.2 | −2.6 to 2.2 | −2.6 to 2.2 | −2.5 to 2.2 |
eGFR (CKiD) or CKD-EPI, ml/min per 1.73 m 2 | |||||
Median (LQR–UQR) | 107 (95–118) | 119 (112–130) | 126 (110–144) | 120 (113–133) | 131 (104–150) |
Range | 81–129 | 82–155 | 94–166 | 48–299 | 101–205 |
eGFR (FAS-Age), ml/min per 1.73 m 2 | |||||
Median (LQR–UQR) | 103 (93–120) | 115 (111–127) | 114 (110–133) | 105 (99–122) | 118 (95–124) |
Range | 81–131 | 92–161 | 95–156 | 51–363 | 95–165 |
LIC, Leuven Imaging Classification; M, male; F, female; LQR, lower quartile range; UQR, upper quartile range; GANAB, glucosidase IIα unit; SDS, SD score; n, number of patients; TKV, total kidney volume; htTKV, height-corrected total kidney volume; BMI, body mass index; CKiD, revised/bedside Schwartz equation; CKD-EPI, Chronic Kidney Disease Epidemiology Collaboration formula; FAS-Age, full-age spectrum–age formula.