Table 4.

Baseline characteristics of patients with class 1 ADPKD: development and validation sets

ClassMen/Women (n/n)Median Age (yr)Median eGFR (ml/min per 1/.73 m2)aMedian HtTKV (ml/m)bFamily History of ADPKD—Yes/No/Unknown (n/n/n)Genetic Analysis, n (%)NMD, n (%)PKD1, n (%)PKD2, n (%)Median Follow-Up SCr (mg/dl)cMedian Follow-Up (yr)
Development set
  1A40 (7/33)50d (44–58)84 (64–97)249 (214–280)23/12/516 (40)5 (31)7 (44)4 (25)6 (3–11)5 (3–10)
  1B88 (23/65)46e (36–53)78 (62–97)433 (322–565)67/14/758 (66)6 (10)37 (64)15 (26)8 (3–10)6 (3–9)
  1C122 (46/76)44f (36–50)71 (47–98)701 (514–1037)109/8/573 (60)3 (4)58 (80)12 (16)7 (4–14)6 (4–10)
  1D77 (40/37)41g (34–49)60 (36–96)1195 (843–1544)62/8/743 (56)0 (0)39 (91)4 (9)5 (3–12)6 (4–11)
  1E49 (28/21)36 (29–43)46 (26–94)1874 (1118–2609)37/9/320 (41)0 (0)20 (100)0 (0)6 (4–11)5 (3–8)
  Subtotal376 (144/232)44 (35–51)71 (44–97)651 (431–1195)298/51/27210 (56)h14 (7)i161 (77)i35 (16)i6 (4–11)6 (4–10)
Internal validation set
  1A16 (6/10)43d (34–54)97 (77–114)226 (193–247)10/3/310 (63)2 (20)5 (50)3 (27)5 (3–10)5 (4–11)
  1B37 (10/27)47e (40–55)77 (49–100)453 (342–527)32/4/123 (62)1 (4.3)18 (78.3)4 (17.4)7 (4–11)5 (4–9)
  1C48 (20/28)45f (35–55)69 (31–94)778 (536–1278)41/5/224 (50)2 (8)18 (75)4 (17)8 (5–16)7 (4–11)
  1D41 (25/16)42g (32–49)67 (30–91)1212 (822–1836)32/6/325 (61)1 (4)22 (88)2 (8)6 (3–13)5 (4–9)
  1E20 (13/7)34 (27–40)82 (60–91)1406 (920–2214)17/2/116 (80)0 (0)15 (94)1 (6)7 (5–14)8 (5–11)
  Subtotal162 (74/88)43 (33–51)77 (46–98)725 (416–1306)132/20/1098 (60)h6 (6)i78 (80)i14 (14)i7 (4–13)6 (4–11)
External validation set
  1A10 (2/8)40d (34–42)105 (92–106)231 (220–248)8/1/19 (90)2 (22)5 (56)2 (22)6 (5–6)9 (7–9)
  1B41 (17/24)36e (31–42)98 (83–112)354 (274–397)33/3/541 (100)5 (12)20 (49)16 (39)5 (4–6)9 (7–10)
  1C55 (21/34)35f (27–42)93 (77–114)537 (403–633)44/3/854 (98)4 (7.4)45 (83.3)5 (9.3)5 (4–6)9 (8–9)
  1D39 (15/24)32g (22–40)90 (77–111)759 (467–1016)34/1/439 (100)2 (5)37 (95)0 (0)5 (3–6)9 (8–9)
  1E28 (12/16)25 (18–31)93 (79–123)906 (643–1334)20/3/528 (100)1 (3.5)26 (94)1 (3.5)6 (5–6)9 (8–10)
  Subtotal173 (67/106)34 (25–40)94 (80–112)492 (356–744)139/11/23171 (99)h14 (8)i133 (78)i24 (14)i5 (4–6)9 (8–10)
  • Median values are expressed with interquartile ranges in parentheses. NMD, no mutation detected; SCr, Serum creatinine.

  • a Estimated using Chronic Kidney Disease Epidemiology equation. Class 1 patients classified based on percentage increase rate based on HtTKV ellipsoid.

  • b Calculated by ellipsoid formula.

  • c Does not include baseline SCr.

  • d Subclass 1A versus 1B, P=0.01 (development set); P=NS (internal validation set); P=NS (external validation set).

  • e Subclass 1B versus 1C, P=NS (development set); P=NS (internal validation set); P=NS (external validation set).

  • f Subclass 1C versus 1D, P=NS (development set); P=NS (internal validation set); P=NS (external validation set).

  • g Subclass 1D versus 1E, P=0.001 (development set); P=0.04 (internal validation set); P=0.01 (external validation set).

  • h Percentage is based on total number of patients for each class.

  • i Percentage is based on patients with genetic analysis within each class.