Effect of demographic characteristics, clinical phenotype, and fibrinogen supplementation modality on the total HRQoL score by univariate analysis
| Variable . | All patients N = 204 . | P . | Adult (aged ≥17 y) n = 119 . | P . | Children (aged 4-16 y) n = 85 . | P . |
|---|---|---|---|---|---|---|
| Sex (female) | +2.7 | .255 | +2.9 | .365 | +3.0 | .380 |
| Male vs female | ||||||
| Residence (Europe or United States) | +11 | <.001 | +8.3 | .049 | +9.7 | .080 |
| Asia and Africa vs Europe and United States | ||||||
| Past pregnancy (no)* | −2.7 | .520 | −2.7 | .520 | NA | NA |
| Yes vs no | ||||||
| Fetal loss (no)* | +1.8 | .675 | +1.8 | .675 | NA | NA |
| Yes vs no | ||||||
| ISTH BAT score (<6) | +5.2 | .107 | +7.6 | .090 | +3.2 | .465 |
| ≥6 vs <6 | ||||||
| Central nervous system bleeding (no) | +1.8 | .513 | +2.4 | .515 | +1.5 | .709 |
| Yes vs no | ||||||
| Frequency of bleeding (less than several times per month) | +2.8 | .250 | +2.2 | .511 | +2.1 | .540 |
| Several times per month vs less | ||||||
| Thrombotic phenotype (no) | +4 | .196 | +10 | .006 | +8.8 | .191 |
| Yes vs no | ||||||
| Ongoing antithrombotic treatment (no) | +10 | .040 | +10 | 0.40 | NA | NA |
| Yes vs no | ||||||
| Availability of fibrinogen concentrates or cryoprecipitates (no) | −10.2 | .018 | −1.9 | .781 | −14.5 | .006 |
| Yes vs no | ||||||
| Fibrinogen supplementation (on-demand) | +0.9 | .713 | +2.5 | .463 | +5.9 | .095 |
| On-demand vs on prophylaxis | ||||||
| Prophylaxis administration (at home) | +3.1 | .457 | +1.6 | .791 | +5.4 | .337 |
| At hospital vs at home | ||||||
| Frequency of infusion (≥1/week) | +0.4 | .924 | +5.4 | .381 | +6.5 | .248 |
| ≥1/wk vs <1/wk | ||||||
| Distance from treatment center (≥100 km) | +1.8 | .474 | +5.2 | .139 | +2.4 | .516 |
| <100 km vs ≥100 km | ||||||
| Bone cyst (no) | +3.8 | .231 | +2.1 | .664 | +2.6 | .508 |
| Yes vs no |
| Variable . | All patients N = 204 . | P . | Adult (aged ≥17 y) n = 119 . | P . | Children (aged 4-16 y) n = 85 . | P . |
|---|---|---|---|---|---|---|
| Sex (female) | +2.7 | .255 | +2.9 | .365 | +3.0 | .380 |
| Male vs female | ||||||
| Residence (Europe or United States) | +11 | <.001 | +8.3 | .049 | +9.7 | .080 |
| Asia and Africa vs Europe and United States | ||||||
| Past pregnancy (no)* | −2.7 | .520 | −2.7 | .520 | NA | NA |
| Yes vs no | ||||||
| Fetal loss (no)* | +1.8 | .675 | +1.8 | .675 | NA | NA |
| Yes vs no | ||||||
| ISTH BAT score (<6) | +5.2 | .107 | +7.6 | .090 | +3.2 | .465 |
| ≥6 vs <6 | ||||||
| Central nervous system bleeding (no) | +1.8 | .513 | +2.4 | .515 | +1.5 | .709 |
| Yes vs no | ||||||
| Frequency of bleeding (less than several times per month) | +2.8 | .250 | +2.2 | .511 | +2.1 | .540 |
| Several times per month vs less | ||||||
| Thrombotic phenotype (no) | +4 | .196 | +10 | .006 | +8.8 | .191 |
| Yes vs no | ||||||
| Ongoing antithrombotic treatment (no) | +10 | .040 | +10 | 0.40 | NA | NA |
| Yes vs no | ||||||
| Availability of fibrinogen concentrates or cryoprecipitates (no) | −10.2 | .018 | −1.9 | .781 | −14.5 | .006 |
| Yes vs no | ||||||
| Fibrinogen supplementation (on-demand) | +0.9 | .713 | +2.5 | .463 | +5.9 | .095 |
| On-demand vs on prophylaxis | ||||||
| Prophylaxis administration (at home) | +3.1 | .457 | +1.6 | .791 | +5.4 | .337 |
| At hospital vs at home | ||||||
| Frequency of infusion (≥1/week) | +0.4 | .924 | +5.4 | .381 | +6.5 | .248 |
| ≥1/wk vs <1/wk | ||||||
| Distance from treatment center (≥100 km) | +1.8 | .474 | +5.2 | .139 | +2.4 | .516 |
| <100 km vs ≥100 km | ||||||
| Bone cyst (no) | +3.8 | .231 | +2.1 | .664 | +2.6 | .508 |
| Yes vs no |
Data are presented as difference of HRQoL total score, with + values implying higher impairments in HRQoL. Reference is indicated in parentheses. NA, not applicable.
Adult women only.