Table 1.

Key characteristics at time of diagnosis and response to intensified immunosuppressive therapy stratified by age at disease onset

Age at Disease OnsetNo. of Patients (%)Genetic Cause Identified N (%)Nephrotic-Range Proteinuria (%)Hypertension (%)Serum Albumin (g/dl)eGFRa (ml/min per 1.73 m2)Response to IITESRD at Last Observation (%)Post-Tx Recurrence N/NTx (%)
Partial Remission N/Ninfo (%)Complete Remission N/Ninfo (%)
<3 mo98 (6.3)55 (66.3)92.710.21.7 (0.9)62 (41–80)2/11 (18.2)3/11 (27.3)60.92/34 (5.9)
3–12 mo106 (6.9)31 (35.6)89.717.02.1 (1.0)89 (53–132)3/33 (9.1)16/33 (48.5)37.80/21 (0.0)
1–5 yr786 (50.9)119 (21.8)93.314.32.0 (0.9)105 (78–147)97/505 (19.2)231/505 (45.7)21.516/86 (18.6)
6–11 yr350 (22.7)40 (15.6)88.116.62.6 (1.0)105 (76–145)49/208 (23.6)73/208 (35.1)27.211/36 (30.6)
≥12 yr204 (13.2)24 (16.1)89.827.92.6 (1.0)93 (64–129)29/128 (22.7)42/128 (32.8)28.63/26 (11.5)
  • Data are given as number (%), mean (SD), or median (interquartile range). IIT, immunosuppressive therapy; Ninfo, number of patients with reported information; Tx, transplant; NTx, number of patients who received a kidney transplant.

  • a Median (interquartile range)