Table 3.
FIX inhibitor risks in Argentine patients with HB.
| Mutation Type | HB1 [%] | Cases2 N=5 | Controls3 N=50 | IOR4(CI95) | IP5(CI95) [%] | P value6 |
|---|---|---|---|---|---|---|
| Missense | 61.5 | 0 | 32 | 0.05(0.003-0.99) | 0.4(0.02-3.04) | 0.0097** |
| Splicing defect | 7.7 | 0 | 4 | 0.94(0.04-19.9) | 2.9(0.15-24.8) | 1.0000 |
| Frameshift indel & Leiden | 7.7 | 0 | 4 | 0.94(0.04-19.9) | 2.9(0.15-24.8) | 1.0000 |
| Nonsense | 13.5 | 3 | 6 | 11(1.51-79.88) | 14.3(4.3-21) | 0.0267* |
| All-large deletions | 9.6 | 2 | 4 | 7.67(0.97-60.2) | 14.3(3.0-27.5) | 0.0864 |
| Entire F9 deletions | 3.8 | 2 | 1 | 32.7(2.26-471) | 45.0(6.6-75.4) | 0.0194* |
1
HB [%]: Artificially made unbiased HB population excluding three patients of the Inhibitor Molecular Protocol to estimate the natural mutation type frequencies in Argentine patients with HB.
2
Cases: Long-lasting inhibitor positive cases (high and low responders).
3
Controls: Inhibitor negative or transient.
4
IOR: Inhibitor Likelihood Odds Radio (Mutation positive/Mutation negative), (CI95): Confidence Interval 95%.
5
IP [%]: Absolute inhibitor prevalence, (CI95): Confidence interval 95%. Null hypothesis is 3.05%, which represents the global HB inhibitor prevalence in Argentina.
6
P values: Fisher exact test P value
*
P<0.05 significant
**
P<0.001 highly significant.