Table 1.

Details of renal survival analysesa

VariablePatients (Families)% Renal Survival 50 yr% Renal Survival 60 yrMedian Age at ESRDRobust P value (df)
a ESRD, end-stage renal disease; ACE, angiotensin-converting enzyme.
b P value from standard Cox model with nonrobust variance estimate (few families to reliably estimate robust variance).
Overall324 (80)64.327.954
    Gender
        men14761.832.1530.57
        women17766.424.855(1)
    ACE
        II4470.040.0540.26
        ID10859.413.452(2)
        DD6451.322.152
    Largest families
        12680.733.5560.013b
        132076.848.060(3)
        17 & 17A1738.512.843
        1251991.742.856
    Mutation type
        truncating219 (54)65.127.8540.40
        in-frame40 (11)44.824.950(2)
        missense65 (15)75.031.056
    Nucleotide location median
        <7812162 (36)55.818.9530.025
        >=7812162 (44)74.539.756(1)
    Mutation type and location
        truncating
            <7812140 (30)60.321.8540.094
            >=781279 (24)74.139.757(1)
        in-frame
            <781210 (3)13.00.0500.021
            >=781230 (8)63.343.753(1)
        missense
            <781212 (4)42.90.0470.058
            >=781253 (11)81.639.357(1)