TABLE 2.
Levels of oxysterols, cholesterol precursors, and cholesterol-7,8-epoxide in the circulation of CTX patients 1 and 2 after treatment with chenodeoxycholic acid
| Patient 1 | Patient 2 | ||||
| Steroid | Before Treatment | Treatment for 9 Months | Treatment for 21 Monthsa | Before Treatment | Treatment for 6 Months |
| 7α-Hydroxycholesterol (ng/ml) | 3.4 | 0.4 | 0.09 | 2.2 | 0.04 |
| 7β-Hydroxycholesterol (μg/ml) | 1.0 | 0.3 | 0.05 | 0.3 | 0.05 |
| 7-Ketocholesterol (μg/ml) | 2.1 | 0.3 | 0.09 | 0.6 | 0.1 |
| 7-Dehydrocholesterol (μg/ml) | 55 | 1.5 | 0.3 | 1.2 | 0.5 |
| Lathosterol (μg/ml) | 12 | 1.3 | 1.1 | 3.2 | 0.6 |
| Cholesterol-7,8-epoxide (μg/ml) | 1.8 | 0.1 | <0.05 | 0.1 | 0.07 |
Values were determined by GC-MS and are for the sum of esterified and nonesterified sterols. Normal levels of 7α-hydroxycholesterol, <0.12 μg/ml; 7β-hydroxycholesterol, <0.02 μg/ml; 7-ketocholesterol, <0.03 μg/ml; 7-dehydrocholesterol, < 0.2 μg/ml; lathosterol, <4 μg/ml; 7,8 epoxide, <0.02 μg/ml.
a
During the last 12 months the dose of chenodeoxycholic acid was increased from 250 mg (×3) to 500 mg (×3).