von Mühlendahl K E, Sippell W G
Kinderhospital Osnabrück.
Monatsschr Kinderheilkd. 1989 Jun;137(6):341-4.
A ten year old girl with congenital adrenal hyperplasia was bilaterally adrenalectomized because even high doses of glucocorticosteroids and mineralocorticosteroids did not sufficiently suppress androgen hypersecretion. Thus, her bone age had advanced over chronological age by three years. The difficulties and started after the institution of phenytoin and clonazepam therapy for epilepsy at the age of three years. The surgical removal of the adrenal glands in such patients appears to be a logical therapeutical approach in such situations, since the organs principally are useless in patients with congenital adrenal hyperplasia, lacking the ability to react with adequate cortisol secretion.
一名患有先天性肾上腺皮质增生症的10岁女孩接受了双侧肾上腺切除术,因为即使大剂量使用糖皮质激素和盐皮质激素也无法充分抑制雄激素分泌过多。因此,她的骨龄比实际年龄超前了三年。困难始于她三岁时因癫痫开始接受苯妥英钠和氯硝西泮治疗之后。对于这类患者,手术切除肾上腺在这种情况下似乎是一种合理的治疗方法,因为对于先天性肾上腺皮质增生症患者来说,这些器官基本上没有用处,缺乏分泌足够皮质醇的反应能力。