Table 3.

Clinical course in nephropathic cystinosis dialysis patients versus the matched dialysis population

Nephropathic cystnosis Patients (n = 57)Matched Pediatric RRT Population (n = 171)Unadjusted P Value/Adjusted P Valuea
Median height SDS at start of RRT (95% CI)−2.61 (−3.28 to −1.94)−1.62 (−2.13 to −1.67)0.009/0.002
    between 1995 and 2000−2.81 (−3.81 to −1.81)−1.85 (−2.41 to −1.30)0.05
    between 2001 and 2009−2.46 (−3.27 to −1.64)−1.55 (−2.16 to −0.95)0.03
Laboratory valuesn = 60n = 180
    albumin g/L, mean (95% CI)36.7 (32.7 to 40.9)37.3 (36.1 to 38.4)0.73/0.88
    Hb g/dl, mean (95% CI)10.4 (9.1 to 11.7)10.7 (10.2 to 11.2)0.37/0.33
    phosphate mmol/L, mean (95% CI)1.84 (1.42 to 2.26)1.83 (1.72 to 1.95)0.89/0.83
    PTH pg/ml, median (95% CI)56 (34 to 91)140 (101 to 189)0.001/0.003
    Blood pressure
    % hypertensive42.3%51.7%0.06/0.04
    SDS systolic blood pressure, mean (95% CI)1.13 (0.64 to 1.56)1.55 (1.13 to 1.97)0.27/0.28
    SDS diastolic blood pressure, mean (95% CI)0.78 (0.11 to 1.44)0.95 (0.44 to 1.46)0.53/0.51
  • SDS, standard deviation score; PTH, parathyroid hormone; RRT, renal replacement therapy; CI, confidence interval; Hb, hemoglobin.

  • a Adjusted for age, sex, calendar year of start of RRT, duration of RRT, and dialysis modality.