Table 1.
Cohort Description of Sexually Active Male Patients With 21-Hydroxylase Deficiency (N = 91)
| Parameter | Cohort Results |
|---|---|
| Median age (IQR; range) | 30 y (23–41; 16–68) |
| Height, mean (95% CI), n = 90 | 170.8 cm (169.3–172.4) |
| BMI, median (IQR), n = 90 | 25.6 kg/m2 (22.6–29.9) |
| Severity of the disease, n = 91 | |
| Clinical classification | |
| Classic: SW | 53 (58.2%) |
| Classic: SV | 32 (35.2%) |
| Nonclassic | 5 (5.5%) |
| No clinical classification | 1 (1.1%) |
| Genetic classification | |
| 0 | 18 (19.8%) |
| A | 25 (27.5%) |
| B | 26 (28.6%) |
| C | 3 (3.3%) |
| No mutation reported or not classified | 19 (20.8%) |
| Medication, n = 91 | |
| Hydrocortisone | 53 (58.2%) |
| Prednisone or prednisolonea | 23 (25.3%) |
| Dexamethasone or hydrocortisone and dexamethasoneb | 10 (11.0%) |
| Fludrocortisone in addition to any of the glucocorticoid combinations here | 62 (68.1%) |
| Fludrocortisone alone | 2 (2.2%) |
| No medication reported | 3 (3.3%) |
| Education, n = 90 | |
| High | 24 (26.7%) |
| Intermediate | 49 (54.4%) |
| Low | 8 (8.9%) |
| Other | 9 (10.0%) |
| Total testosterone concentrations, n = 85 | |
| Above reference range | 4 (5.3%) |
| Within reference range | 58 (77.3%) |
| Below reference range | 13 (17.3%) |
| Subjective health status, n = 91 | |
| Bad | 5 (5.5%) |
| Fair | 16 (17.6%) |
| Good | 47 (51.6%) |
| Very good | 23 (25.3%) |
| HADS-anxiety, n = 91 | |
| Normal | 68 (74.7%) |
| Borderline abnormal | 7 (7.7%) |
| Abnormal | 16 (17.6%) |
| HADS-depression, n = 91 | |
| Normal | 84 (92.3%) |
| Borderline abnormal | 2 (2.2%) |
| Abnormal | 5 (5.5%) |
Abbreviation: BMI, body mass index.
a
Eleven patients were on prednisolone, 11 on prednisone, and 1 on prednisone retard.
b
Dexamethasone was used combined with hydrocortisone in 3 patients, whereas 7 patients were on dexamethasone.