Table 1.
Age/sex | CGD type | Clinical disease | Manifestations of CGD present at transplant/pretransplant CRP (mg/L) and immunosuppression | Busulfan AUC min × microm/L | TNC (109) | Cd 34+ cells/kg (106) | CD3+ cells/kg (107) |
---|---|---|---|---|---|---|---|
14/M | XL (p91-phox) | Infection/protein-losing enteropathy, colitis | Protein-losing enteropathy and Nocardia pneumonia/2.90, prednisone 7.5 mg daily and mesalamine DR 400 mg every 8 h | 2461 | 31.5 | 7.98 | 16.89 |
26/F | P67 (NCF-2) | Infection | A. nidulans pneumonia and osteomyelitis/17.10, none | 3610 | 92.4 | 9.24 | 9.31 |
24/M | P47 (NCF-1) | Infection, colitis, and dermatitis | Colitis and dermatitis/5.00, prednisone 20 mg daily, and mesalamine DR, 1200 mg twice daily | 3828 | 50.3 | 9.84 | 21.5 |
16/M | XL (p91-phox) | Infection, colitis, cystitis, recurrent fever, and headaches | Colitis, bladder disease/12.60, prednisolone 5 mg twice daily | 3619 | 91.0 | 15.9 | 20.5 |
14/M | XL (p91-phox) | Infection and colitis | S. apiospermum pneumonia and pericarditis, Colitis/5.90, prednisone 5 mg daily, and sulfasalazine 500 mg daily | 3008 | 27.6 | 12 | 14.9 |
22/F | XL (p91-phox) carrier | Infection (Nocardia), colitis | Colitis and discoid lupus/5.40, azathioprine 150 mg daily, mesalamine 1.2 g every 12 h, prednisone 15 mg daily, and hydroxychloroquine 200 mg daily | 3349 | 105 | 10.5 | 14.1 |
19/M | XL (p91-phox) | Infection and colitis | Colitis/6.60, mesalamine DR 1200 mg twice daily, and prednisone 7.5 mg twice daily | 4250 | 55.2 | 9.85 | 12.32 |
CGD chronic granulomatous disease, CRP high-sensitivity comprehensive C-reactive protein, AUC area under the curve, TNC total nucleated cell dose, XL X-linked, DR delayed release: A. nidulans, Aspergillus nidulans; S. apiospermum, and Scedosporium apiospermum