Table 4.

Clinical characteristics of COL4A, other genetic cause, and no proven genetic cause subgroups

Clinical characteristic	COL4A (n=12)	Other Genetic (n=12)	No Proven Genetic (n=169)
% Male	42	67	58
Mean age of onset of kidney disease (95% CI)	36 (29 to 42)	26 (17 to 37)	34 (32 to 37)
% with hematuria	60	25	29
% of patients with family history of kidney disease	46	44	12.2
% Partial remission	29	30	26
% Complete remission	0	0	20
% No remission	14	20	15
% Unknown status of remission	57	50	39
% ESKD	46	44	50
% with only global glomerulosclerosis on light microscopy	11	50	9
% Glomerular basement membrane abnormalities on electron microscopy	56	25	27
% with >50% podocyte foot process effacement on electron microscopy	100	75	72
No. of patients where pathology report with electron microscopy description is available	9	4	113
Mean age at ESKD (95% CI)	58 (49 to 69)	43 (31 to 55)	62 (58 to 66)
% Kidney transplant in patients with ESKD	100	75	54
% of recurrence of disease after kidney transplant (within the first year)	0	0	13

Patients in the COL4A, podocyte/kidney development (called other), and no proven genetic basis subgroups had disease onset at 36 years (95% CI, 29 to 42), 26 years (95% CI, 17 to 37), and 34 years (95% CI, 32 to 37). The estimated mean survival/age at ESKD was 58 years (95% CI, 49 to 69), 43 years (95% CI, 31 to 55), and 62 years (95% CI, 58 to 66). The only statistically significant P values were in comparing COL4A with the no proven genetic basis subgroup for hematuria (P=0.02), glomerular basement membrane abnormalities (P=0.03), and >50% effacement (P=0.03). Number of patients where the pathology report was available is indicated. Percentages represent of available data. Absolute numbers are presented in Supplemental Table 9. 95% CI, 95% confidence interval.