Laboratory data for the 6 families
Family . | Individual . | Fibrinogen activity, g/L . | Fibrinogen antigen, g/L . | PT, % . | aPTT, seconds . | Arg134 genotype* . |
|---|---|---|---|---|---|---|
| A | 1 | 1.1 | 1.26 | 81 | 30.7 | +/- |
| 2 | 0.88 | 1.2 | 85 | 30.1 | +/- | |
| 3 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| 4 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| B | 5 | 1.01 | 1.25 | 91 | 29.1 | +/- |
| 6 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| C | 7 | 1.59 | 1.62 | 93 | 25.9 | +/- |
| 8 | 1.21 | 1.31 | 81 | 30.7 | +/- | |
| 9 | 3.09 | nd | 90 | 28.3 | +/- | |
| 10 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| 11 | 2.73 | 2.51 | 91 | 27.1 | +/+ | |
| 12 | 3.22 | 2.8 | 95 | 29.3 | +/+ | |
| 13 | 1.04 | 1.3 | 88 | 27.2 | +/- | |
| 14 | 1.25 | 1.28 | 96 | 26.4 | +/- | |
| 15 | 3.3 | nd | 68 | 37.4 | +/+ | |
| 16 | 1.13 | 1.27 | 90 | 29.6 | +/- | |
| 17 | 0.92 | 1.03 | 80 | 30.6 | +/- | |
| 18 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| 19 | 0.82 | 1.0 | 86 | 30 | +/- | |
| 20 | 0.94 | 1.1 | 88 | 27.2 | +/- | |
| D | 21 | 1.19 | 1.2 | 84 | 28.9 | +/- |
| 22 | 3.5 | 2.83 | 94 | 27.2 | +/+ | |
| 23 | 3.0 | nd | 93 | 31 | +/+ | |
| 24 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| E | 25 | 0.99 | 1.3 | 90 | 26.6 | +/- |
| 26 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| F | 27 | < 0.1 | < 0.05 | no clot | no clot | -/- |
| 28 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| 29 | < 0.1 | < 0.05 | no clot | no clot | -/- |
Family . | Individual . | Fibrinogen activity, g/L . | Fibrinogen antigen, g/L . | PT, % . | aPTT, seconds . | Arg134 genotype* . |
|---|---|---|---|---|---|---|
| A | 1 | 1.1 | 1.26 | 81 | 30.7 | +/- |
| 2 | 0.88 | 1.2 | 85 | 30.1 | +/- | |
| 3 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| 4 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| B | 5 | 1.01 | 1.25 | 91 | 29.1 | +/- |
| 6 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| C | 7 | 1.59 | 1.62 | 93 | 25.9 | +/- |
| 8 | 1.21 | 1.31 | 81 | 30.7 | +/- | |
| 9 | 3.09 | nd | 90 | 28.3 | +/- | |
| 10 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| 11 | 2.73 | 2.51 | 91 | 27.1 | +/+ | |
| 12 | 3.22 | 2.8 | 95 | 29.3 | +/+ | |
| 13 | 1.04 | 1.3 | 88 | 27.2 | +/- | |
| 14 | 1.25 | 1.28 | 96 | 26.4 | +/- | |
| 15 | 3.3 | nd | 68 | 37.4 | +/+ | |
| 16 | 1.13 | 1.27 | 90 | 29.6 | +/- | |
| 17 | 0.92 | 1.03 | 80 | 30.6 | +/- | |
| 18 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| 19 | 0.82 | 1.0 | 86 | 30 | +/- | |
| 20 | 0.94 | 1.1 | 88 | 27.2 | +/- | |
| D | 21 | 1.19 | 1.2 | 84 | 28.9 | +/- |
| 22 | 3.5 | 2.83 | 94 | 27.2 | +/+ | |
| 23 | 3.0 | nd | 93 | 31 | +/+ | |
| 24 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| E | 25 | 0.99 | 1.3 | 90 | 26.6 | +/- |
| 26 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| F | 27 | < 0.1 | < 0.05 | no clot | no clot | -/- |
| 28 | < 0.1 | < 0.05 | no clot | no clot | -/- | |
| 29 | < 0.1 | < 0.05 | no clot | no clot | -/- |
Normal laboratory levels are follows: fibrinogen activity, 1.6-3.5 g/L; fibrinogen antigen, 1.6-3.5 g/L; PT, 80%-100%; aPTT, 25-31 seconds.
PT indicates prothrombin time; aPTT, activated partial thromboplastin time; nd, not determined.
Arg134 genotypes are shown as follows: +/+, homozygous normal; +/-, heterozygous Arg134Xaa; -/-, homozygous Arg134Xaa