Table 1.

Clinical data of patients with steroid-resistant nephrotic syndromea

Family No.IndividualGenderSymptoms at Disease OnsetRenal Biopsy (Age at Biopsy, yr)Age at ESRF (yr)Age at Transplant (yr)
Age at Onset (yr)PU (mg/m2/h)HUCreatinin (mg/dl)/GFR (ml/min)
a INS, idiopathic nephrotic syndrome; PU, proteinuria; HU, hematuria; ESRF, end-stage renal failure; ND, not determined; normal, normal renal function, exact values not available. All patients presented with edema during initial clinical examination except INS 28 II:1/II:2, INS 29 II:1/II:2, INS 43 II:1/II:2, INS 92 II:1/II:2 and INS 72 II:1. Both siblings in INS11 received transplants twice because of rejection.
b Proteinuria indicated (mg/h).
SRINS families
    INS11II:1M3.3>400.85/166FSGS (11.5)14.415.3; 38
II:2M2>400.9/130FSGS (6.0)1414; 36
16II:1F3243+0.5/178FSGS (3.0)5.36.3
II:2F0.5313+0.2/194FSGS (2.0)4.25
18II:1M3NDNDNDFSGS(7.5)17.818.5
II:2F3.5>130NormalFSGS (8.0)23
28II:1M4404+0.7/105FSGS (10.5)13
II:2M7153+0.4/172MCNS (7.0)
29II:2F946b0.44/116FSGS (16.0)
II:1M13.42820.9/96FSGS (24.0)
37II:1F2.5>40NormalFSGS (2.9)13.915.8
II:2M0.1>40NDFSGS (6.0)9.510.5
41II:1M1>40+Normal88.5
II:2M142b+0.7/normal9.29.3
43II:1M5.5183+0.5/normalMCNS (5.5)11.211.8
II:2F2.5175b0.2/normalMCNS (2.6)11
46II:1M11.881b+0.44/112unspecific (13.8)
II:2F3.5357+0.77/90FSGS (3.8 and 8.8)10.510.5
50II:1M0.1>40Normal<7.97.9
II:2F0.1>400.3/normalFSGS (4.9)10
67II:1M3.8>40+0.1/normalFSGS (5.75)
II:2M1.81583bNDFSGS (3.5)3.84.9
92II:1F0.196NormalFSGS (1.0)6.17.2
II:2M0.1250b0.2/130FSGS (1.0)
Sporadic SRINS patients
72II:1M16.6217+0.5/90FSGS (16.7)20.1
73II:1F<76250.3/201MCNS (4.4)7.4
74II:1M5625b+0.9/62FSGS (5)
76II:1F2276+0.5/172FSGS (8.6)99.8
83II:1F9.11208b+0.5/178MCNS (7.9)10.712.5
86II:1F1.3>417+NormalFSGS (7.4)
90II:1M0.1229+0.2/normalMCNS (1)