Table 3. Progression of nephropathy to chronic renal failure (CRF) and end-stage renal disease (ESRD) in 54 MYH9-RD patients according to the NMMHC-IIA region affected by mutation.
CRF | ESRD | |||||
---|---|---|---|---|---|---|
NMMHC-IIA region (n. of patients) | Affected patients (%) [median follow-up, mo.] | Rate per 100 person year (95%CI) | p value | Affected patients (%) [median follow-up, mo.] | Rate per 100 person year (95%CI) | p value |
SH1 helix (R702) (n=23) |
17 (74%) [48] |
19.76 (13.8-28.6) |
p=0.02 | 14 (61%) [48] |
18.33 (12.43-27.20) |
p=0.001 |
Coiled-coil (n=31) |
19 (61%) [36] |
8.72 (6.04-12.49) |
9 (29%) [40] |
3.35 (1.55-7.96) |
Abbreviation: mo.= months.
Additional information:
For the mutations in SH1 helix, the age at onset of nephropathy, IRC and ESRD was 19 (SD 9), 23 (16) and 24 (9) years, respectively.
For the mutations in the coiled-coil, the age at onset of nephropathy, IRC and ESRD was 31 (SD 15) 38 (8) and 42 (23) years, respectively.