Table 1.
Hormonal assessment in the patient at 1–2 months and at 9 yr
| 1–2 months | 9 yr | |||
|---|---|---|---|---|
| 17OHP (nmol/liter) | ||||
| At baseline | 9.38 | (1.8–7.5)a | 37.9 | (<6) |
| 60 min after ACTH 250 μg/m2 iv | — | 47.2 | ||
| Cortisol (nmol/liter) | ||||
| At baseline | — | 391 | (150–450) | |
| 60 min after ACTH 250 μg/m2 iv | — | 494 | (>550) | |
| DHEAS (μmol/liter) | 0.23 | (f: 0.04–1.32)b (m: 0.04–1.96) | 2.49 | (f: 0.23–2.37) (m: 0.42–2.13) |
| Androstenedione (nmol/liter) | ||||
| At baseline | 0.41 | (f: 0.7–1.9) (m: 1.3–4.25) | 0.73 | (f: 0.3–1.2) (m: 0.2–2.8) |
| 4 days after hCG 2000 IU/m2 | 2.15 | — | ||
| Testosterone (nmol/liter) | ||||
| At baseline | <0.20 | (f: 0.17–0.40) (m: 1.4–8.2) | <0.17 | (f: 0.17–0.30) (m: 0.15–0.65) |
| 4 days after hCG 2000 IU/m2 | 2.15 | — | ||
| ACTH (pg/ml) | — | 50.9 | (10–60) | |
| LH (U/liter) | ||||
| At baseline | 0.4 | (0.1–4) | — | |
| 60 min after LHRH 75 μg/m2 iv | 3.7 | (2–5 fold of baseline) | — | |
| FSH (U/liter) | ||||
| At baseline | 2.0 | (0.1–4) | — | |
| 60 min after LHRH 75 μg/m2 iv | 6.7 | (2–3 fold of baseline) | — | |
Bilateral gonadectomy had been carried out at the age of 4 yr. —, Not measured.
a
Age-specific normal reference range.
b
Age-specific reference ranges for androgens are listed for both girls (f) and boys (m).