Table 2

Complement studies of families 1 and 2 and patient 3

	F1: I1	F1: I2	P1		F2: I1	F2: I2	P2	P3
	F1: I1	F1: I2	In1	In2	F2: I1	F2: I2	P2	P3
Families are indicated by F1 for patient 1 (P1) and F2 for patient 2 (P2). Two results are given for patient 1 corresponding to investigations 3 months after disease onset (In1) and at the time of end stage renal disease (In2). Plasma concentrations of factor B, C3, and C4 antigens were measured by nephelometry (Dade Behring, Paris La Defense, France). Plasma concentrations of factor I were determined by ELISA using an anti-factor I polyclonal antibody (Calbiochem, Meudon, France).
CH50 (70–130%)	115	96	89	111	100	78	87	75
C3 (660–1250 mg/l)	876	1230	601	716	1060	456	624	990
Factor B (90–320 mg/l)	147	177	55	107	153	61	76	123
C4 (93–320 mg/l)	198	84	186	311	145	140	154	256
H ag (70–130%)	111	115	76	102	136	85	126	107
Factor I ag (70–130%)	40	120	40	48	133	80	109	36
FH gene			No mutation				No mutation	No mutation
FI gene	G456X	G456	G456X		D506	D506V	D506V	W528X