Table 3.

Within-family disease variability in affected relatives of patients with protein-truncating PKD1 mutations and mild kidney disease by imaging

Patient ID	Age (yr)	htTKV (ml/m)	Mayo Clinic Imaging Class	eGFR (ml/min per 1.73 m2)	No. of Affected Relatives Requiring KRT
					Before Age 55	After Age 65
Disease discordance by imaging in one or more relative pair
TOR308.2	57	927	1B	65	0	0
TOR308.1	25	637	1E	97
TOR17.1	51	623	1B	48	4	0
TOR17.13	28	655	1D	102
TOR17.12	23	712	1E	133
Disease concordance by imaging in one or more relative pair
TOR094.1	22	264	1B	93	2	0
TOR094.2	19	211	1B	91
TOR139.1	20	183	1A	127	3	0
TOR139.2	22	175	1A	125
TOR198.3	39	291	1B	105	1	0
TOR198.4	35	316	1B	93
TOR173.1	54	587	1B	86	0	2
TOR173.2	28	493	1C	115
Disease discordance by imaging and age at KRT in one or more relative pair
TOR094.1	22	264	1B	93	2	0
TOR139.1	22	183	1B	127	3	0
TOR198.3	39	291	1B	105	1	0
TOR420.1	39	275	1B	109	1	0
TOR431.1	25	254	1B	102	2	0
TOR438.1	25	296	1B	122	2	0
TOR48.9	35	352	1B	96	3	0
TOR595.1	32	344	1B	85	1	0
TOR663.1	39	324	1B	85	1	0
TOR833.1	36	221	1A	90	1	0
TOR841.1	18	237	1B	113	1	0
TOR845.1	34	378	1B	83	1	0
TOR968.1	59	371	1B	64	1	0
Disease concordance by imaging and age at KRT in one or more relative pair
TOR619.2	43	342	1B	79	0	1
Uninformative for assessment of within-family disease variability
TOR242.2	31	326	1B	119	0	0
TOR291.3	46	589	1B	105	0	0
TOR388.1	46	463	1B	103	0	0
TOR674.1	33	348	1B	115	0	0
TOR803.1	35	389	1B	93	0	0
TOR895.1	28	321	1B	120	0	0

htTKV, height-adjusted total kidney volume.