Table 1.
Patient demographics and baseline characteristics, interventional radiology & open surgery compared to high-dose corticosteroid management
| IR & OS | CM | |
|---|---|---|
| Number of patients | 26 | 8 |
| Male/Female | 22/4 | 8/0 |
| Caucasian | 21 (81%) | 5 (63%) |
| African American | 4 (15%) | 1 (13%) |
| Asian | 1 (4%) | 1 (13%) |
| Other | 0 (0%) | 1 (13%) |
| Median age at CGD diagnosis (years) | 2 (0–22) | 2 (1 month-31 years) |
| NAPDH oxidase genotype | gp91phox: 16 (61%) | gp91phox: 8 (100%) |
| p47phox: 7 (27%) | ||
| p22phox: 2 (8%) | ||
| p67phox: 1 (4%) | ||
| Residual ROI (superoxide nmol/hr) (n=24, n=7)a | <2.3: 9 (38%)≥2.3: 15 (62%) | <2.3: 7 (100%) |
| ≥2.3: 0 (0%) | ||
| Quartilesb | Q1: 4 (16%) | Q1: 2 (28.5%) |
| Q2: 9 (38%) | ||
| Q3: 3 (13%) | ||
| Q4: 8 (33%) | ||
| Median overall follow-up (years) | 15.5 (8.5–32.9) | 4.8 (2.9–5.8) |
| Number alive at final analysis | 18 (69)% | 6 (75%) |
| 90-day mortality rate | 0% | 0% |
| Mean number of liver abscesses | 2.5 (1–13) | 4.9 (2–14) |
| Mean size of largest liver abscess (cm) | 3.5 (1–8) | 4.7 (3–8) |
| Mean ESR prior to intervention (mm/hr)c | 69 (10–145) | 73.6 (34–140) |
| Median age at first liver procedure at NIH (years) | 18.14 (2.8–32.7) | |
| Number of total liver procedures per patient | 3.7 (1–13) | |
| Median age at high-dose steroid treatment (years) | 19 (0.5–46) | |
| Median dose of high-dose steroid treatment (mg/kg/day) | 1 (0.6–1.8) | |
| Median duration of steroid taper (months) | 5 (1–7) |
Data are presented as No. (%) unless otherwise indicated.
Abbreviations: CGD, chronic granulomatous disease; CM, corticosteroid management; ESR, erythrocyte sedimentation rate; IR, interventional radiology; NADPH, nicotinamide adenine dinucleotide phosphate; NIH, National Institutes of Health; OS, open surgery; ROI, reactive oxygen intermediate.
an = 2 and n = 1 without data , refers to the number of represented patients from the IR and OS cohorts, respectively.
bAs defined per reference [2], ranges of superoxide production for Q1, Q2, Q3, and Q4 are0.26–0.94, 0.95–1.67, 1.70–2.71, and 2.72–60.5, respectively.
cMeasured at time of admission.