Neutrophil functions in Barth syndrome
. | NADPH oxidase activity . | . | . | Chemotaxis . | . | . | ||||
|---|---|---|---|---|---|---|---|---|---|---|
| Patient . | STZ . | PMA . | PAF + fMLP . | C5a . | IL-8 . | PAF . | ||||
| 1 | 0.52 (0.60) | NT | NT | 98 | 100 | 95 | ||||
| 2 | NT | NT | NT | 95 | NT | 68 | ||||
| 4 | 0.67 (0.66) | 2.21 (2.08) | 1.99 (1.92) | 96 | NT | 99 | ||||
| 5 | 0.98 (0.66) | 2.45 (1.98) | 2.15 (1.71) | 103 | NT | 77 | ||||
| 6 | 0.77 (0.62) | 2.14 (2.01) | 1.86 (1.90) | 115 | 114 | 97 | ||||
| 7 | 0.82 (0.71) | NT | NT | NT | NT | NT | ||||
. | NADPH oxidase activity . | . | . | Chemotaxis . | . | . | ||||
|---|---|---|---|---|---|---|---|---|---|---|
| Patient . | STZ . | PMA . | PAF + fMLP . | C5a . | IL-8 . | PAF . | ||||
| 1 | 0.52 (0.60) | NT | NT | 98 | 100 | 95 | ||||
| 2 | NT | NT | NT | 95 | NT | 68 | ||||
| 4 | 0.67 (0.66) | 2.21 (2.08) | 1.99 (1.92) | 96 | NT | 99 | ||||
| 5 | 0.98 (0.66) | 2.45 (1.98) | 2.15 (1.71) | 103 | NT | 77 | ||||
| 6 | 0.77 (0.62) | 2.14 (2.01) | 1.86 (1.90) | 115 | 114 | 97 | ||||
| 7 | 0.82 (0.71) | NT | NT | NT | NT | NT | ||||
Neutrophil functions were tested in freshly purified cells from 6 BTHS patients. NADPH-oxidase activity is expressed as maximal slope of H2O2 release in nmol H2O2/minute per 106 cells. The mean of 2 age-matched controls measured on the same day is given in parentheses beneath the corresponding patient value. Chemotaxis is expressed as percentage of mean maximal slope of 2 age-matched controls measured on the same day.
NT indicates not tested.