Table 1.
Factor deficiencies and other bleeding disorders reported in patients with NS
| Type of deficiency | Single factor deficiency | Multiple factor deficiency | Total |
|---|---|---|---|
| FI | 2 | 1 | 3 |
| FII | 0 | 1 | 1 |
| FV | 0 | 5 | 5 |
| FVII | 6 | 6 | 12 |
| FVIII | 18 | 12 | 30 |
| FIX | 4 | 7 | 11 |
| FX | 0 | 5 | 5 |
| FXI | 57 | 30 | 87 |
| FXII | 19 | 16 | 35 |
| FXIII | 1 | 0 | 1 |
| vWD | 4 | 6 | 10 |
| Platelet-relateda | 42 | 5 | 47 |
| Totalb | 153 | 42 | 195 |
Notes:
a
Thrombocytopenia: 20 (two were transient after birth); ITP + cyclooxygenase deficiency: 1; Bernard–Soulier syndrome: 1; platelet aggregation abnormality: 19; platelet storage pool deficiency: 1.
b
Total is the number of patients with factor deficiencies, and for multiple factor deficiencies patients could have more than one deficiency type.
Abbreviations: ITP, idiopathic thrombocytopenia; FI, factor I; FII, factor II; FV, factor V; FVII, factor VII; FVIII, factor VIII; FIX, factor IX; FX, factor X; FXI, factor XI, FXII, factor XII; FXIII, factor XIII; NS, Noonan syndrome; vWD, von Willebrand disease.