Table I.

Haemorrhagic events and FVIII consumption in patients with Glu113Asp, Arg593Cys and the patient with Arg593Cys over the 6-year period.

Patient/family	Age	Blood group	Observ. period (years)	FVIII:C (%)		Spontaneous bleeding			Other bleeds	Treatment
				1st	Chr	Joints	Soft tissue	Mucosae		FVIII (IU/kg)	Other
1/1	13	A	6	11.9	9.8	-	-	-	Trauma, GI (ulcer)	140	BT
2/2	12	A	6	8.3	7.6	-	-	-	-	0	0
3/2	2.5	A	2.5	6.5	4.2	-	-	-	-	0	0
4/3	44	A	6	8.2	6.7	-	-	-	2 traumas, tooth extraction	279	0
5/3	38	O	6	5.8	3.1	-	-	-	Tonsillectomy	1,190	Resuturing
6/4	18	ND	6	8.9	6.9	-	-	-	Trauma	0	0
7/4	12	ND	6	9.1	7.1	-	-	-	Trauma	0	0
8/5	31	O	6	7.1	5.1	-	-	-	-	0	0
9/6	34	O	7	6.2	5.2	1	-	-	Tooth extraction	218	0
10/6	31	O	6	6.2	5.1	-	-	-	-	0	0
11/7	20	ND	6	7.6	7.1	-	-	-	-	0	0
12/7	51	A	6	7.3	6.5	-	-	1	3 traumas	431	0
13/8#	18	A	6	6.6	5.7	4	11	-	6 traumas, appendectomy, radiosynovect.	1,229	NS 624 mg, AF DDAVP$
14/9	5	A	5	7.3	4.6	-	-	-	5 traumas, urol. surgery, haemato-tympanon	1,067	DDAVP 2x
15/10	54	O	6	8.1	7.9	-	-	-	Tooth extraction	272	0
16/11	36	O	6	8.2	8.2	-	-	-	-	0	0
17/12	34	A	6	12.2	10.5	-	-	-	-	0	0
18/13†	48	O	6	14.1	13.5	-	-	-	-	0	0

Observ.: observation; FVIII:C: factor VIII coagulant activity; IU: International Unit; GI: gastrointestinal; BT: blood transfusion; 1st: one-stage assay; Chr: chromogenic assay; ND: not determined.

^#Development of inhibitors;

^$from 4/2008-2/2010 treatment with NovoSeven (NS) and antifibrinolytic (AF), from then on with DDAVP (negative inhibitors);

^†patient with a single mutation.