Table 4.
Baseline characteristics of patients with class 1 ADPKD: development and validation sets
| Class | Men/Women (n/n) | Median Age (yr) | Median eGFR (ml/min per 1/.73 m2)a | Median HtTKV (ml/m)b | Family History of ADPKD—Yes/No/Unknown (n/n/n) | Genetic Analysis, n (%) | NMD, n (%) | PKD1, n (%) | PKD2, n (%) | Median Follow-Up SCr (mg/dl)c | Median Follow-Up (yr) |
|---|---|---|---|---|---|---|---|---|---|---|---|
| Development set | |||||||||||
| 1A | 40 (7/33) | 50d (44–58) | 84 (64–97) | 249 (214–280) | 23/12/5 | 16 (40) | 5 (31) | 7 (44) | 4 (25) | 6 (3–11) | 5 (3–10) |
| 1B | 88 (23/65) | 46e (36–53) | 78 (62–97) | 433 (322–565) | 67/14/7 | 58 (66) | 6 (10) | 37 (64) | 15 (26) | 8 (3–10) | 6 (3–9) |
| 1C | 122 (46/76) | 44f (36–50) | 71 (47–98) | 701 (514–1037) | 109/8/5 | 73 (60) | 3 (4) | 58 (80) | 12 (16) | 7 (4–14) | 6 (4–10) |
| 1D | 77 (40/37) | 41g (34–49) | 60 (36–96) | 1195 (843–1544) | 62/8/7 | 43 (56) | 0 (0) | 39 (91) | 4 (9) | 5 (3–12) | 6 (4–11) |
| 1E | 49 (28/21) | 36 (29–43) | 46 (26–94) | 1874 (1118–2609) | 37/9/3 | 20 (41) | 0 (0) | 20 (100) | 0 (0) | 6 (4–11) | 5 (3–8) |
| Subtotal | 376 (144/232) | 44 (35–51) | 71 (44–97) | 651 (431–1195) | 298/51/27 | 210 (56)h | 14 (7)i | 161 (77)i | 35 (16)i | 6 (4–11) | 6 (4–10) |
| Internal validation set | |||||||||||
| 1A | 16 (6/10) | 43d (34–54) | 97 (77–114) | 226 (193–247) | 10/3/3 | 10 (63) | 2 (20) | 5 (50) | 3 (27) | 5 (3–10) | 5 (4–11) |
| 1B | 37 (10/27) | 47e (40–55) | 77 (49–100) | 453 (342–527) | 32/4/1 | 23 (62) | 1 (4.3) | 18 (78.3) | 4 (17.4) | 7 (4–11) | 5 (4–9) |
| 1C | 48 (20/28) | 45f (35–55) | 69 (31–94) | 778 (536–1278) | 41/5/2 | 24 (50) | 2 (8) | 18 (75) | 4 (17) | 8 (5–16) | 7 (4–11) |
| 1D | 41 (25/16) | 42g (32–49) | 67 (30–91) | 1212 (822–1836) | 32/6/3 | 25 (61) | 1 (4) | 22 (88) | 2 (8) | 6 (3–13) | 5 (4–9) |
| 1E | 20 (13/7) | 34 (27–40) | 82 (60–91) | 1406 (920–2214) | 17/2/1 | 16 (80) | 0 (0) | 15 (94) | 1 (6) | 7 (5–14) | 8 (5–11) |
| Subtotal | 162 (74/88) | 43 (33–51) | 77 (46–98) | 725 (416–1306) | 132/20/10 | 98 (60)h | 6 (6)i | 78 (80)i | 14 (14)i | 7 (4–13) | 6 (4–11) |
| External validation set | |||||||||||
| 1A | 10 (2/8) | 40d (34–42) | 105 (92–106) | 231 (220–248) | 8/1/1 | 9 (90) | 2 (22) | 5 (56) | 2 (22) | 6 (5–6) | 9 (7–9) |
| 1B | 41 (17/24) | 36e (31–42) | 98 (83–112) | 354 (274–397) | 33/3/5 | 41 (100) | 5 (12) | 20 (49) | 16 (39) | 5 (4–6) | 9 (7–10) |
| 1C | 55 (21/34) | 35f (27–42) | 93 (77–114) | 537 (403–633) | 44/3/8 | 54 (98) | 4 (7.4) | 45 (83.3) | 5 (9.3) | 5 (4–6) | 9 (8–9) |
| 1D | 39 (15/24) | 32g (22–40) | 90 (77–111) | 759 (467–1016) | 34/1/4 | 39 (100) | 2 (5) | 37 (95) | 0 (0) | 5 (3–6) | 9 (8–9) |
| 1E | 28 (12/16) | 25 (18–31) | 93 (79–123) | 906 (643–1334) | 20/3/5 | 28 (100) | 1 (3.5) | 26 (94) | 1 (3.5) | 6 (5–6) | 9 (8–10) |
| Subtotal | 173 (67/106) | 34 (25–40) | 94 (80–112) | 492 (356–744) | 139/11/23 | 171 (99)h | 14 (8)i | 133 (78)i | 24 (14)i | 5 (4–6) | 9 (8–10) |
Median values are expressed with interquartile ranges in parentheses. NMD, no mutation detected; SCr, Serum creatinine.
Estimated using Chronic Kidney Disease Epidemiology equation. Class 1 patients classified based on percentage increase rate based on HtTKV ellipsoid.
Calculated by ellipsoid formula.
Does not include baseline SCr.
Subclass 1A versus 1B, P=0.01 (development set); P=NS (internal validation set); P=NS (external validation set).
Subclass 1B versus 1C, P=NS (development set); P=NS (internal validation set); P=NS (external validation set).
Subclass 1C versus 1D, P=NS (development set); P=NS (internal validation set); P=NS (external validation set).
Subclass 1D versus 1E, P=0.001 (development set); P=0.04 (internal validation set); P=0.01 (external validation set).
Percentage is based on total number of patients for each class.
Percentage is based on patients with genetic analysis within each class.