Table 1.
Kidney survival of genetically defined NPH cohorts
| Patient characteristics | NPHP1 | NPHP3 | NPHP4 | NPHP11 | Unsolved |
|---|---|---|---|---|---|
| Total number of participants | 116 | 101 | 81 | 85 | 44 |
| Participants with ESKD (%) | 70% | 81% | 100% | 45% | 57% |
| Mean age at onset of ESKD (years/ range) | 12.5 (5.3–27.6) | 7.7 (0–47) | 17.1 (6–54) | 11.9 (0–32) | 11.1 (2.1–18.6) |
| Median age and interquartile range (IQR) for the onset of ESKD (years) | 13.5 (10.5–16.5) | 4.0 (0.25–12.0) | 16.0 (11.0–25.0) | 19.0 (8.7–28.0) | 15.4 (9.7–n.a.) |
ESKD, end-stage kidney disease; IQR, interquartile range; n.a., not assessed; NPH, nephronophthisis.