Clinical characteristics of the participants with HA
| Total . | Historic INH ≥ 5 BU/mL∗ . | Historic INH < 5 BU/mL . | Recent INH ≥ 5 BU/mL . | Recent INH < 5 BU/mL . | Current INH ≥ 0.6 BU/mL . | Recent titer unknown . | |
|---|---|---|---|---|---|---|---|
| Total | 394 | 66 | 58 | 45 | 39 | 28 | 71 |
| Black | 188 | 36 | 31 | 36 | 31 | 16 | 31 |
| White | 206 | 30 | 26 | 9 | 8 | 12 | 40 |
| Severe HA, Black | 162 | 34 | 27 | 34 | 26 | 14 | 27 |
| Severe HA, White | 185 | 27 | 22 | 8 | 7 | 11 | 39 |
| Mild + moderate HA, Black | 26 | 2 | 5 | 2 | 5 | 2 | 3 |
| Mild + moderate HA, White | 21 | 3 | 4 | 1 | 1 | 1 | 1 |
| Haplotype, race | |||||||
| H1, Black | 60 | 12 | 10 | 4 | 1 | 4 | 10 |
| H2, Black | 90 | 15 | 15 | 6 | 5 | 7 | 16 |
| H3, Black | 28 | 4 | 4 | 0 | 1 | 1 | 2 |
| H4, Black | 3 | 2 | 1 | 1 | 0 | 1 | 0 |
| H5, Black | 2 | 2 | 0 | 0 | 0 | 0 | 1 |
| Haplotype unknown, Black | 4 | 1 | 1 | 1 | 0 | 1 | 2 |
| H1, White | 156 | 21 | 20 | 7 | 4 | 10 | 30 |
| H2, White | 43 | 7 | 6 | 2 | 2 | 0 | 10 |
| H3, White | 1 | 0 | 0 | 0 | 0 | 0 | 0 |
| H4, White | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| H5, White | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Haplotype unknown, White | 6 | 2 | 0 | 0 | 2 | 2 | 0 |
| Mutation class, race | |||||||
| Intron-22 inversion, Black | 57 | 13 | 13 | 5 | 2 | 7 | 11 |
| Intron-1 inversion, Black | 6 | 4 | 0 | 0 | 0 | 0 | 2 |
| Mutation unknown, Black | 26 | 5 | 3 | 1 | 2 | 4 | 6 |
| Intron-22 inversion, White | 87 | 12 | 11 | 4 | 11 | 7 | 20 |
| Intron-1 inversion White | 5 | 1 | 0 | 0 | 0 | 0 | 2 |
| Mutation unknown White | 12 | 3 | 2 | 3 | 0 | 4 | 3 |
| Age (y) at enrollment, race | |||||||
| 2-3, Black | 10 | 3 | 1 | 3 | 0 | 2 | 1 |
| 2-3, White | 5 | 2 | 1 | 2 | 0 | 2 | 2 |
| 4-17, Black | 74 | 10 | 11 | 10 | 11 | 3 | 12 |
| 4-17, White | 73 | 12 | 12 | 12 | 12 | 4 | 15 |
| ≥18, Black | 104 | 23 | 19 | 9 | 5 | 11 | 17 |
| ≥18, White | 128 | 16 | 13 | 4 | 5 | 6 | 23 |
| Total . | Historic INH ≥ 5 BU/mL∗ . | Historic INH < 5 BU/mL . | Recent INH ≥ 5 BU/mL . | Recent INH < 5 BU/mL . | Current INH ≥ 0.6 BU/mL . | Recent titer unknown . | |
|---|---|---|---|---|---|---|---|
| Total | 394 | 66 | 58 | 45 | 39 | 28 | 71 |
| Black | 188 | 36 | 31 | 36 | 31 | 16 | 31 |
| White | 206 | 30 | 26 | 9 | 8 | 12 | 40 |
| Severe HA, Black | 162 | 34 | 27 | 34 | 26 | 14 | 27 |
| Severe HA, White | 185 | 27 | 22 | 8 | 7 | 11 | 39 |
| Mild + moderate HA, Black | 26 | 2 | 5 | 2 | 5 | 2 | 3 |
| Mild + moderate HA, White | 21 | 3 | 4 | 1 | 1 | 1 | 1 |
| Haplotype, race | |||||||
| H1, Black | 60 | 12 | 10 | 4 | 1 | 4 | 10 |
| H2, Black | 90 | 15 | 15 | 6 | 5 | 7 | 16 |
| H3, Black | 28 | 4 | 4 | 0 | 1 | 1 | 2 |
| H4, Black | 3 | 2 | 1 | 1 | 0 | 1 | 0 |
| H5, Black | 2 | 2 | 0 | 0 | 0 | 0 | 1 |
| Haplotype unknown, Black | 4 | 1 | 1 | 1 | 0 | 1 | 2 |
| H1, White | 156 | 21 | 20 | 7 | 4 | 10 | 30 |
| H2, White | 43 | 7 | 6 | 2 | 2 | 0 | 10 |
| H3, White | 1 | 0 | 0 | 0 | 0 | 0 | 0 |
| H4, White | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| H5, White | 0 | 0 | 0 | 0 | 0 | 0 | 0 |
| Haplotype unknown, White | 6 | 2 | 0 | 0 | 2 | 2 | 0 |
| Mutation class, race | |||||||
| Intron-22 inversion, Black | 57 | 13 | 13 | 5 | 2 | 7 | 11 |
| Intron-1 inversion, Black | 6 | 4 | 0 | 0 | 0 | 0 | 2 |
| Mutation unknown, Black | 26 | 5 | 3 | 1 | 2 | 4 | 6 |
| Intron-22 inversion, White | 87 | 12 | 11 | 4 | 11 | 7 | 20 |
| Intron-1 inversion White | 5 | 1 | 0 | 0 | 0 | 0 | 2 |
| Mutation unknown White | 12 | 3 | 2 | 3 | 0 | 4 | 3 |
| Age (y) at enrollment, race | |||||||
| 2-3, Black | 10 | 3 | 1 | 3 | 0 | 2 | 1 |
| 2-3, White | 5 | 2 | 1 | 2 | 0 | 2 | 2 |
| 4-17, Black | 74 | 10 | 11 | 10 | 11 | 3 | 12 |
| 4-17, White | 73 | 12 | 12 | 12 | 12 | 4 | 15 |
| ≥18, Black | 104 | 23 | 19 | 9 | 5 | 11 | 17 |
| ≥18, White | 128 | 16 | 13 | 4 | 5 | 6 | 23 |
INH, inhibitor titer ≥0.6 BU/mL. “Recent” titers include those measured by CNBA assays performed as part of this study. In cases in which plasma volumes were not available to perform a CNBA assay, recent titers are from clinical records of assays performed within 2 years of enrollment in this study. In cases in which the most recent clinical titer was ≥5 BU/mL but the CNBA assay results showed a titer <5 BU/mL, the participant was classified as having a recent inhibitor titer of ≥5 BU/mL. Note that the “current inhibitor” designation refers only to the 28 patients whose plasma samples collected for this study showed titers ≥0.6 CNBU/mL.