Table 1.

Clinical data of the patients with familial steroid-responsive INSa

Predominant Symptoms at Presentation Symptoms at Last Examination
Family No. Individual Gender Age at Onset (yr) PU (mg/m2/h) HU Creatinine (mg/dl)/GFR (ml/min/1,73 m2) Age (yr) at Renal Biopsy Creatinine (mg/dl)/GFR (ml/min/1,73 m2) PU (mg/m2/h)
aINS, idiopathic nephrotic syndrome; PU, proteinuria: nephrotic-range proteinuria is defined as >40 mg/m2 per h; HU, hematuria; 1, microhematuria; n.d., not determined; normal, normal renal function, exact values not available. All patients presented with edema during the acute phase. BP was normal in all patients at disease onset, as well as at last examination.
INS 1 II:2 m 0,7 125 1 0,3/155 n.d. 0,77/94 neg.
II:4 m 3,4 166 neg. 0,5/110 MCNS (5) 0,63/104 neg.
3 II:2 m 6,0 410 neg. 0,67/92 n.d. 0,68/121 neg.
II:3 m 3,7 280 neg. 0,4/130 n.d. 0,56/162 neg.
4 II:1 m 2,0 60 neg. 0,67/88 MCNS (14) 0,83/119 neg.
II:2 m 2,5 80 neg. 0,54/91 n.d. 0,76/110 neg.
6 II:1 m 5,5 250 neg. 0,5/130 MCNS (7,5) 0,68/133 13
II:2 f 4,2 700 n.d. 0,45/120 MCNS (5) 0,64/135 120
II:3 m 8,1 180 neg. 0,56/120 n.d. 0,44/173 neg.
15 II:2 m 2,5 500 1 0,3/196 MCNS (3,6) 0,77/112 neg.
II:3 m 3,8 330 neg. 0,3/170 MCNS (4) 1,08/86 neg.
20 II:2 m 10,0 80 neg. 0,49/175 n.d. 0,63/154 25
II:3 m 4,5 110 neg. 0,37/145 n.d. 0,38/188 neg.
23 II:1 f 3,0 60 neg. 0,37/167 n.d. normal neg.
II:3 m 5,5 70 neg. 0,59/85 n.d. normal neg.
24 II:1 m 2,1 130 neg. 0,4/157 MCNS (3) 0,6/128 neg.
II:3 f 4,0 220 neg. 0,3/179 n.d. 0,5/161 neg.
30 II:1 m 5,0 210 neg. 0,45/134 MCNS (7,5) 0,8/95 neg.
II:2 m 1,8 200 1 0,3/179 n.d. 0,5/125 neg.
34 II:2 m 3,1 160 neg. 0,6/110 n.d. normal neg.
II:3 m 3,5 240 neg. 0,6/193 n.d. normal neg.
39 II:1 m 14,0 110 neg. 0,5/158 n.d. 0,74/134 neg.
II:2 m 5,0 160 neg. 0,4/135 MCNS (5,5) 0,87/125 neg.
44 II:1 m 1,5 80 neg. 0,32/144 MCNS (5,5) normal neg.
II:3 f 2,0 120 neg. 0,38/130 MCNS (2,5) normal neg.
45 II:1 f 1,9 160 1 0.39/120 MCNS (3,5) 0,9/99 neg.
II:2 m 1,5 480 1 0,42/108 n.d. 0,76/96 neg.
48 II:1 f 1,9 >200 neg. 0,6/86 n.d. 0,68/135 neg.
II:2 f 2,0 >100 neg. 0,38/123 n.d. 0,73/115 neg.
II:4 m 2,8 >200 neg. 0,4/130 n.d. 0,39/175 neg.
51 II:3 m 5,3 200 neg. 0,34/186 n.d. 0,38/165 neg.
II:4 m 3,4 350 neg. 0,43/121 n.d. 0,45/122 neg.