Table 1.
Characteristics of 95 SRNS patients who never received intensified immunosuppressive therapy. Data are given as N (% (normal-approximated 95% confidence intervals)), mean ± standard deviation or median (interquartile range). For incompletely reported items, number of informative patients is given in italics. MCD, minimal-change disease; MesPGN, mesangio-proliferative glomerulonephritis; FSGS, focal segmental glomerulosclerosis; eGFR, estimated glomerular filtration rate; CKD, chronic kidney disease, CKD stage 5, kidney failure, and/or start of kidney replacement therapy
| Total | Non-Genetic | Genetic | Unknown | |
|---|---|---|---|---|
| N = 95 | N = 45 | N = 39 | N = 11 | |
| Characteristics at disease onset | ||||
| Age (years) | 6.1 ± 5.6 | 6.7 ± 5.7 | 4.9 ± 5.2 | 7.8 ± 6.5 |
| Age groups | ||||
| 3 mo to < 1 yr | 22 (23 (15; 32) %) | 9 (20 (8; 32) %) | 11 (28 (14; 42) %) | 2 (18 (0; 41) %) |
| 1 to < 6 yrs | 32 (34 (24; 43) %) | 13 (29 (16; 42) %) | 16 (41 (26; 56) %) | 3 (27 (1; 54) %) |
| 6 to < 12 yrs | 22 (23 (15; 32) %) | 13 (29 (16; 42) %) | 7 (18 (6; 30) %) | 2 (18.(0; 41%) |
| ≥ 12 yrs | 19 (20 (12; 28) %) | 10 (22 (10; 34) %) | 5 (13 (2; 23) %) | 4 (36 (8; 65) %) |
| Serum albumin (g/l) | 31.5 ± 11.7 | 32.1 ± 10.9 | 32.1 ± 12.5 | 27.5 ± 13.1 |
| Proteinuria | 82 | 38 | 33 | 11 |
| Nephrotic range | 67 (82 (73; 90) %) | 31 (82 (69; 94) %) | 27 (82 (69; 95) %) | 9 (82 (59; 100) %) |
| Non-nephrotic range | 13 (16 (8; 24) %) | 7 (18 (6; 31) %) | 4 (12 (1; 23) %) | 2 (18 (0; 41) %) |
| No proteinuria | 2 (2 (0; 6) %) | 0 | 2 (6 (0; 14) %) | 0 |
| Edema | ||||
| Severe | 5 (5 (1; 10) %) | 4 (9 (1; 17) %) | 1 (3 (0; 8) %) | 0 |
| Moderate | 13 (14 (7; 20) %) | 5 (11 (2; 20) %) | 7 (18 (6; 30) %) | 1 (9 (0; 26) %) |
| Mild | 17 (18 (10; 26) %) | 11 (24 (12; 37) %) | 4 (10 (1; 20) %) | 2 (18 (0; 41) %) |
| None | 60 (63 (54; 73) %) | 25 (56 (41; 70) %) | 27 (69 (55; 84) %) | 8 (73 (46; 99) %) |
| Kidney function | 76 | 37 | 31 | 8 |
| eGFR(ml/min*1.73m2/d) | 97.8 (68.9; 136.5) | 97.3 (68.3; 144.6) | 103.8 (69.0; 138.4) | 89.6 (72.5; 107.8) |
| CKD Stage | 76 | 37 | 31 | 8 |
| CKD 1 | 43 (57 (46; 68) %) | 20 (54 (38; 70) %) | 19 (61 (44; 78) %) | 4 (50 (15; 85) %) |
| CKD 2 | 19 (25 (15; 35) %) | 11 (30 (15; 44) %) | 5 (16 (3; 29) %) | 3 (38 (4; 71) %) |
| CKD 3 | 12 (16 (8; 24) %) | 5 (14 (3; 25) %) | 6 (19 (6; 33) %) | 1 (13 (0; 35) %) |
| CKD 4 | 2 (3 (0; 6) %) | 1 (3 (0; 8) %) | 1 (3 (0; 9) %) | 0 |
| Hypertension | 13 (14 (7; 21) %) | 7 (16 (5; 26) %) | 3 (8 (0; 16) %) | 3 (27 (10; 54) %) |
| Hematuria | 26 (28 (19; 37) %) | 11 (24 (12; 37) %) | 11 (28 (14; 42) %) | 4 (40 (11; 69) %) |
| Histopathological diagnosis | 67 | 32 | 26 | 9 |
| MCD | 9 (13 (5; 21) %) | 4 (13 (1; 25) %) | 3 (12 (0; 24) %) | 2 (22 (0; 49) %) |
| MesPGN | 8 (12 (4; 20) %) | 4 (13 (1; 25) %) | 4 (15 (2; 29) %) | 0 (0%) |
| FSGS | 40 (60 (48; 72) %) | 20 (63 (46; 80) %) | 17 (65 (47; 84) %) | 3 (33 (3; 64) %) |
| Other | 10 (15 (6; 24) %) | 4 (13 (1; 25) %) | 2 (8 (0; 18) %) | 4 (44 (12; 77) %) |
| Family history | 75 | 33 | 31 | 11 |
| Positive | 40 (53 (42; 65) %) | 13 (39 (23; 56) %) | 19 (61 (44; 78) %) | 8 (73 (46; 99) %) |
| Negative | 35 (47 (35; 58) %) | 20 (61 (44; 77) %) | 12 (39 (22; 56) %) | 3 (27 (1; 54) %) |
| Follow-up information | ||||
| Duration of observation (years) | 3.5 (1.7; 7.2) | 3.3 (2.3; 6.1) | 4.8 (1.4; 8.4) | 2.5 (1.0; 5.1) |
| Kidney failure during observation | ||||
| No. of patients | 18 (19 (11; 27) %) | 2 (4 (0; 10) %) | 13 (33 (19; 48) %) | 2 (18 (0; 41) %) |
| Time to kidney failure (years) | 2.3 (1.3; 4.1) | 2.1 (1.7; 3.0) | 3.2 (1.3; 9.1) | 1.4 (0.9; 1.9) |