Table 4.
Summary of the clinical and pathologic characteristics of the patients with steroid-resistant nephrotic syndrome included in the study, divided by genetic group
| Characteristics | Podocytopathies (n=19) | Phenocopies (n=18) | Negative (n=17) | Undefined (n=10) |
|---|---|---|---|---|
| Age at onset, yr | 4 [1–7] | 5 [3–10] | 5 [3–8] | 5 [3–13] |
| Sex (male) | 10/19 (53%) | 9/18 (50%) | 10/17 (59%) | 3/10 (30%) |
| Ethnicity (European) | 19/19 (100%) | 15/18 (83%) | 17/17 (100%) | 9/10 (90%) |
| Histopathological findings (FSGS) | 13/16 (81%) | 11/16 (69%) | 11/17 (65%) | 8/10 (80%) |
| Remission | ||||
| Complete | 0/19 (0%) | 0/18 (0%) | 8/17 (47%) | 0/0 (0%) |
| Partial | 1/19 (5%) | 11/18 (61%) | 5/17 (29%) | 0/0 (0%) |
| None | 18/19 (95%) | 7/18 (39%) | 4/17 (24%) | 10/10 (0%) |
| Response to CNIs | 1/8 (13%) | 0/6 (0%) | 5/10 (50%) | 0/6 (0%) |
| Response to RASi | 1/15 (7%) | 10/18 (56%) | 7/15 (47%) | 0/8 (0%) |
| ESKD at 10 yr | 11/15 (73%) | 3/10 (30%) | 3/6 (50%) | 8/10 (80%) |
| Post-transplant recurrence | 0/11 (0%) | 0/0 (0%) | 1/3 (33%) | 3/7 (43%) |
| Length of follow-up, yr | 8 [5–12] | 7 [2–10] | 6 [5–10] | 8 [4–12] |
Continuous variables are presented as median [interquartile range] and categorical variables are presented as n (%). CNIs, calcineurin inhibitors; RASi, inhibitors of the renin-angiotensin-aldosterone system.