Table 1. Clinical features of all nephrotic patients enrolled in the study that included (overall 48): (a) 8 pts with circulating auto-antibodies; (b) 5 pts with familial trait of nephritic syndrome; (c) 3 pts with NPHS1-NPHS2 mutations; (d) 32 pts with primary nephritic syndrome.
Drug sensitivity FSGS/Mes | |||||||
---|---|---|---|---|---|---|---|
Age at onset (years) | Histology | ||||||
n | Sex | mean (range) | Steroids | Cyclosporin | IgM | ESRF | |
Nephrotic syndrome with familial trait/NPHS1-2 mut | 8 | 3F/5M | 6·2 (2–8) | 1S/2D/2R | 2S/2R | 2/2 | 2 |
Sporadic nephrotic syndrome | 52 | 17F/35M | 7·1 (1–36) | 16S/21D/15R | 23S/5R | 28/9 | 11 |
FSGS, focal segmental glomerulosclerosis; Mes IgM, mesangial proliferation with deposits of IgM; ESRF, end stage renal failure; S, sensitive; D, dependent; R, resistant; NPHS1, gene encoding for nephrin; NPHS2, gene encoding for podocin.