Theintz G E, Sizonenko P C
Arch Fr Pediatr. 1985 Mar;42(3):211-7.
Twenty patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency have been followed during a mean period of 68.4 months (from 19 to 120 months). Plasma 17 alpha-hydroxyprogesterone, delta 4-androstenedione and testosterone have been measured at regular intervals and correlated to growth and bone maturation. Satisfactory growth was obtained through repeated changes in the daily dose of oral hydrocortisone which varied from 58.7 +/- 37 mg/m2/day in infants to respectively 19.3 +/- 7.0 and 25.3 +/- 7.2 mg/m2/day in prepuberty and puberty. Fludrocortisone was added in 15 cases because of evidence of salt loss. delta 4-androstenedione and 17 alpha-hydroxyprogesterone are the best markers of adequate suppression of the pituitary-adrenal axis in both sexes. Testosterone can be used in girls and prepubertal boys. In some cases with low levels of androgens suggesting oversuppression, a reduced velocity of bone maturation was observed, particularly in young subjects.
对20例因21 -羟化酶缺乏导致先天性肾上腺皮质增生症的患者进行了平均68.4个月（19至120个月）的随访。定期测定血浆17α -羟孕酮、δ4 -雄烯二酮和睾酮水平，并将其与生长和骨成熟情况进行关联分析。通过反复调整口服氢化可的松的日剂量实现了令人满意的生长，婴儿期日剂量为58.7±37mg/m²/天，青春期前和青春期分别为19.3±7.0mg/m²/天和25.3±7.2mg/m²/天。15例因有失盐证据而加用了氟氢可的松。δ4 -雄烯二酮和17α -羟孕酮是两性中垂体 -肾上腺轴得到充分抑制的最佳标志物。睾酮可用于女孩和青春期前男孩。在一些雄激素水平较低提示抑制过度的病例中，观察到骨成熟速度减慢，尤其是在年轻患者中。
