Activated FVII levels in factor VII Padua (Arg304Gln) coagulation disorder and in true factor VII deficiency: a study in homozygotes and heterozygotes

Figures & data

Table 1. Main coagulation parameters in six homozygotes with FVII Padua and in three cases homozygous for ‘true’ factor VII deficiency

		Factor VII activity (%)
	PT (second)	Rabbit brain	Human placenta	Recombinant human	Ox-brain	Factor VII antigen	Activated FVII levels (mU/ml)	Comments
FVII Padua
Case 1	30·2	8	36	30	105	95	5
Case 2	30·0	9	32	20	110	110	5·5
Case 3	32·5	10	36	32	100	105	12
Case 4	34·0	11	34	29	100	110	12
Case 5	31·0	8	33	30	90	120	10
Case 6	31·6	7	29	25	95	100	6
True FVII deficiency
Case 1	48	1	1·2	1	1	Traces	Traces	Unassailable
Case 2	42	1·5	1·5	2	1·5	Traces	Traces	Unassailable
Case 3	50	1	1	1	1	Traces	Traces	Unassailable
Normal values	13–15	80–120	80–120	80–120	80–120	80–120	36–135

Table 2. Comparison of levels of activated FVII observed in the different groups of patients investigated

Defect	No. of cases (M/F)	Mean value	Range	Comments
Homozygous FVII Padua	6 (1/5)	8·4	5–12	P>0·01 with all other groups for which calculation could be carried out
Heterozygous FVII Padua	14 (8/6)	41·0	25–90
True FVII deficiency	3 (1/2)	Traces	…	Unassailable traces
Heterozygous for True deficiency	11 (6/5)	36·2	20–75
Normal controls	21 (10/11)	64·8	36–135