Jansen M, Wit J M, van den Brande J L
Acta Paediatr Scand. 1981 Mar;70(2):229-33. doi: 10.1111/j.1651-2227.1981.tb05547.x.
The results of reintroduction of mineralocorticoid substitution therapy (9-alpha-fluorohydrocortisone) in five children with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency are described. All children had shown a salt-losing syndrome in infancy, but were off mineralocorticoids during several years; at the start of this study they all had elevated plasma renin activity (PRA). Four of them had increased and fluctuating pregnanetriol excretion during the year preceding reintroduction of mineralocorticoids, indicating poor control despite substantial substitution with hydrocortisone: 26 +/- 1.9 mg/m2/day (mean +/- S.E.M.). Reintroduction of mineralocorticoid therapy at ages 5.0-9.4 years resulted in a marked improvement of control, significant reduction in hydrocortisone requirements (to 17.6 +/- 1.4 mg/m2/day) and improvement in linear growth. The data suggest that, in all children with CAH and elevated PRA, continuation throughout childhood of mineralocorticoid therapy in addition to glucocorticoid therapy is necessary for optimal control and linear growth.
本文描述了对5名因21-羟化酶缺乏导致先天性肾上腺皮质增生症（CAH）的儿童重新引入盐皮质激素替代疗法（9-α-氟氢可的松）的结果。所有儿童在婴儿期均表现出失盐综合征，但在数年中未使用盐皮质激素；在本研究开始时，他们的血浆肾素活性（PRA）均升高。其中4名儿童在重新引入盐皮质激素之前的一年中孕三醇排泄增加且波动，这表明尽管使用了大量氢化可的松替代治疗，但病情控制不佳：26±1.9mg/m²/天（平均值±标准误）。在5.0至9.4岁重新引入盐皮质激素治疗后，病情控制得到显著改善，氢化可的松需求量显著降低（降至17.6±1.4mg/m²/天），线性生长也有所改善。数据表明，对于所有CAH且PRA升高的儿童，除糖皮质激素治疗外，整个儿童期持续进行盐皮质激素治疗对于最佳病情控制和线性生长是必要的。
