Table 4.

Genotype/phenotype correlation in FHHNC, subgroups separately

GenotypeCL/CL (n = 23)PL/PL (n = 29)PL/CL (n = 12)PL/X (n = 5)
Age at onset (yr; mean [95% CI])2.2 (1.1 to 3.3)5.7 (3.3 to 8.2)a4.9 (2.3 to 7.6)5.9 (2.6 to 9.2)
Loss of GFR/yr (ml/min per 1.73 m2; mean [95% CI])7.3 (5.0 to 9.6)3.3 (1.6 to 4.9)b3.0 (1.1 to 4.9)a2.9 (1.6 to 4.1)
Renal function at end of study
    GFR >60 ml/min per 1.73 m29% (2/22)55% (16/29)b5/124/5
    GFR <60 ml/min per 1.73 m250% (11/22)17% (5/29)b3/121/5
    ESRD41% (9/22)18% (8/29)b4/120/5
ESRD at 15 yr54% (7/13)25% (5/20)1/10
ESRD at 20 yr70% (7/10)36% (5/14)3/6
  • a P < 0.05 versus CL/CL.

  • b P < 0.01 versus CL/CL (ANOVA with Bonferroni multiple comparison test).