Demographics of CT patients and HCT-treated patients
| Characteristic . | Total N = 391 . | Retrospective CT patients∗ n = 151 . | Retrospective or prospective HCT† n = 240 . | P value . |
|---|---|---|---|---|
| Age, median y (range)‡ | 9.45 (0.32-29.85) | 15.7 (0.3-29.9) | 5 (0.3-28.1) | <.001 |
| Sex,n (%) | .261 | |||
| Male | 336 (85.9) | 126 (83.4) | 210 (87.5) | |
| Female | 55 (14.1) | 25 (16.6) | 30 (12.5) | |
| Race, n (%) | <.001 | |||
| American Indian/Alaska native | 1 (0.4) | 1 (0.6) | 0 | |
| Asian | 20 (5.1) | 8 (5.3) | 12 (5) | |
| Black/African American | 47 (12) | 33 (21.9) | 14 (5.8) | |
| Caucasian | 275 (70.3) | 87 (57.6) | 188 (78.4) | |
| Unknown | 48 (12.2) | 22 (14.6) | 26 (10.8) | |
| Ethnicity n (%) | .784 | |||
| Hispanic/Latino/Spanish | 58 (14.8) | 22 (14.6) | 36 (15) | |
| Not Hispanic/Latino/Spanish | 291 (74.5) | 116 (76.8) | 175 (72.9) | |
| Unknown | 42 (10.7) | 13 (8.6) | 29 (12.1) | |
| Genotype (%) | .035 | |||
| CYBB (gp91phox) | 270 (69) | 94 (62.3) | 176 (73.3) | |
| CYBA (p22phox) | 20 (5.2) | 9 (6) | 11 (4.6) | |
| NCF1 (p47phox) | 59 (15.1) | 34 (22.5) | 25 (10.4) | |
| NCF2 (p67phox) | 17 (4.3) | 5 (3.3) | 12 (5) | |
| NCF4 (p40phox) | 2 (0.5) | 0 | 2 (0.8) | |
| Unknown | 23 (5.9) | 9 (6) | 14 (5.9) | |
| Oxidase status (%) | .046 | |||
| Null | 231 (59.1) | 85 (56.3) | 146 (60.8) | |
| Positive | 110 (28.1) | 52 (34.4) | 58 (24.2) | |
| Unknown | 50 (12.8) | 14 (9.3) | 36 (15) | |
| Birth y n (%) | <.001 | |||
| 1988-1993 | 68 (17.4) | 40 (26.5) | 28 (11.7) | |
| 1994-1999 | 73 (18.7) | 32 (21.2) | 41 (17.1) | |
| 2000-2005 | 99 (25.3) | 38 (25.2) | 61 (25.4) | |
| 2006-2011 | 89 (22.8) | 31 (20.5) | 58 (24.2) | |
| 2012-2017 | 62 (15.9) | 10 (6.6) | 52 (21.7) |
| Characteristic . | Total N = 391 . | Retrospective CT patients∗ n = 151 . | Retrospective or prospective HCT† n = 240 . | P value . |
|---|---|---|---|---|
| Age, median y (range)‡ | 9.45 (0.32-29.85) | 15.7 (0.3-29.9) | 5 (0.3-28.1) | <.001 |
| Sex,n (%) | .261 | |||
| Male | 336 (85.9) | 126 (83.4) | 210 (87.5) | |
| Female | 55 (14.1) | 25 (16.6) | 30 (12.5) | |
| Race, n (%) | <.001 | |||
| American Indian/Alaska native | 1 (0.4) | 1 (0.6) | 0 | |
| Asian | 20 (5.1) | 8 (5.3) | 12 (5) | |
| Black/African American | 47 (12) | 33 (21.9) | 14 (5.8) | |
| Caucasian | 275 (70.3) | 87 (57.6) | 188 (78.4) | |
| Unknown | 48 (12.2) | 22 (14.6) | 26 (10.8) | |
| Ethnicity n (%) | .784 | |||
| Hispanic/Latino/Spanish | 58 (14.8) | 22 (14.6) | 36 (15) | |
| Not Hispanic/Latino/Spanish | 291 (74.5) | 116 (76.8) | 175 (72.9) | |
| Unknown | 42 (10.7) | 13 (8.6) | 29 (12.1) | |
| Genotype (%) | .035 | |||
| CYBB (gp91phox) | 270 (69) | 94 (62.3) | 176 (73.3) | |
| CYBA (p22phox) | 20 (5.2) | 9 (6) | 11 (4.6) | |
| NCF1 (p47phox) | 59 (15.1) | 34 (22.5) | 25 (10.4) | |
| NCF2 (p67phox) | 17 (4.3) | 5 (3.3) | 12 (5) | |
| NCF4 (p40phox) | 2 (0.5) | 0 | 2 (0.8) | |
| Unknown | 23 (5.9) | 9 (6) | 14 (5.9) | |
| Oxidase status (%) | .046 | |||
| Null | 231 (59.1) | 85 (56.3) | 146 (60.8) | |
| Positive | 110 (28.1) | 52 (34.4) | 58 (24.2) | |
| Unknown | 50 (12.8) | 14 (9.3) | 36 (15) | |
| Birth y n (%) | <.001 | |||
| 1988-1993 | 68 (17.4) | 40 (26.5) | 28 (11.7) | |
| 1994-1999 | 73 (18.7) | 32 (21.2) | 41 (17.1) | |
| 2000-2005 | 99 (25.3) | 38 (25.2) | 61 (25.4) | |
| 2006-2011 | 89 (22.8) | 31 (20.5) | 58 (24.2) | |
| 2012-2017 | 62 (15.9) | 10 (6.6) | 52 (21.7) |
Two patients included in the retrospective conventional arm ultimately underwent HCT. They were not included in the HCT-treated cohort.
There were no retrospective HCT-treated patients followed up prospectively.
Age for CT patients was at the time of enrollment. Age for HCT-treated patients was at the time of HCT.