Table 1.
No. | Sex | Clinical characteristics |
At baseline |
12 wk after treatment |
|||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Age (yr) | BW (kg) | Diagnosis | Genetic diagnosis | 24-h proteinuria (g/m2) | Plasma albumin (g/l) | eGFR (ml/minper 1.73 m2) | BMI (kg/m2) | 24-h proteinuria (g/m2) | Plasma albumin (g/l) | eGFR (ml/minper 1.73 m2) | BMI (kg/m2) | ||
1 | F | 11.2 | 47.5 | Proteinuria | PAX2 | 2.35 | 38.7 | 72.8 | 25.1 | 1.45 | 43.0 | 73.2 | 24.8 |
2 | F | 13.8 | 43.7 | Proteinuria | NUP160 | 2.63 | 33.4 | 126.2 | 16.6 | 2.29 | 34.8 | 110.9 | 16.4 |
3 | M | 9.8 | 33.6 | Dent disease | CLCN5 | 1.64 | 47.5 | 102.7 | 18.4 | 1.26 | 51.6 | 97.6 | 18.1 |
4a | M | 11.9 | 42.2 | Alport syndrome | COL4A5 | 6.21 | 24.9 | 60.8 | 17.2 | - | - | - | - |
5 | M | 8.1 | 24 | Alport syndrome | COL4A5 | 2.42 | 34.2 | 113.1 | 15.6 | 1.75 | 35.3 | 112.5 | 15.3 |
6 | F | 14.2 | 43.4 | Alport syndrome | COL4A3 | 1.84 | 31.5 | 128.9 | 18.8 | 1.56 | 31.7 | 123.9 | 20.3 |
7 | M | 6.4 | 24.2 | Alport syndrome | COL4A5 | 1.23 | 38.0 | 103.1 | 15.7 | 1.22 | 37.0 | 100.7 | 15.5 |
8 | M | 8.4 | 28.2 | Alport syndrome | COL4A5 | 6.18 | 24.1 | 163.6 | 19.3 | 4.28 | 25.3 | 150.6 | 18.9 |
9b | F | 9.4 | 27 | FSGS | Negative | 1.28 | 34.9 | 63.3 | 13.4 | 0.55 | 41.1 | 60.8 | 14.2 |
BMI, body mass index; BW, body weight; eGFR, estimated glomerular filtration rate; F, female; FSGS, focal segmental glomerular sclerosis; M, male.
Patient 4 was loss to follow-up at the first 4 wk.
Patient 9 was with familial FSGS.