Table 2.

Comparison of clinical characteristics at time of diagnosis and prospective kidney survival of patients with ADCK4-related SRNS versus patients with NPHS2- and WT1-related glomerulopathy from the PodoNet Registry8

ADCK4 SRNSNPHS2 SRNSWT1 SRNSd
n2614066
Age at first reported manifestation, years14.1 (10.8–17.0)3.4 (1.1–6.6)d 2.0 (0.7–5.4)d
Asymptomatic, incidental diagnosis26.9%22.9%28.1%
Edema (none/mild/moderate/severe)54/42/4/0%48/17/16/19%47/19/24/10%
Proteinuria (subnephrotic/nephrotic range)57.1/43.9%14.9/85.1%d 19.4/80.6%c
Hematuria25.0%44.0%26.3%
Hypertension30.8%15.7%41.5%b
CKD stage 3–546.1%a13.6%a,d 23.4%a,b
including RTT:26.9%a2.9%a,d 15.6%a
Age at start of RRT, years16.1 (13.7–18.0)a12.9 (7.6–19.4)a,d 10.9 (2.3–17.0)a,d
Histopathological diagnosis
FSGS/global glomerulosclerosis61.5%49.3%45.0%
Diffuse mesangial sclerosis00.7%33.3%d
Mesangioproliferative GN012.9%4.5%
Minimal change GN010.7%1.5%
Other05.0%3.0%
No data/ not performed38.5%21.4%13.7%
Neurologic abnormalities (seizures, NI, behavioral problems)24.0%5.0%c6.1%bb
Congenital organ abnormalitiesCAKUT 4.0%CAKUT 0%CAKUT 42.2%d
Other 8.0%Other 5.7%Other 4.5%
  • Data are given as median (interquartile range) or percentage. Sixty-one of the patients with WT1 mutations were previously described by Lipska et al. (2014)9

  • a Percentages given are relative to all observation with information on a specific variable.

  • b P<0.05.

  • c P<0.01.

  • d P<0.001.