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. Author manuscript; available in PMC: 2018 Nov 13.
Published in final edited form as: Hum Mutat. 2013 Dec 12;35(2):236–247. doi: 10.1002/humu.22476

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.

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