Warinner S A, Zimmerman D, Thompson G B, Grant C S
Department of Surgery, Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905, USA.
World J Surg. 2000 Nov;24(11):1347-52. doi: 10.1007/s002680010223.
Medical management of congenital adrenal hyperplasia (CAH) patients has led to suboptimal results in most cases. High glucocorticoid doses, often needed to suppress adrenal androgen production, may lead to signs of Cushing syndrome. Incompletely suppressed androgen levels commonly lead to premature closure of growth centers, acne, virilization, precocious puberty, irregular or absent menses, and decreased fertility in female CAH patients. A newly proposed therapy for CAH patients is bilateral adrenalectomy. Three Caucasian female patients with 21-hydroxylase deficiency were treated with bilateral adrenalectomy. Two of the three procedures were accomplished laparoscopically. In each patient, medical management alone was unsuccessful. Two patients had salt-losing 21-hydroxylase deficiency. The third patient had uncontrolled hyperandrogenism complicated by obesity and glucose intolerance. All patients had low height percentiles with respect to their normalized percentiles for weight. Bone age was advanced in one patient. Androgen and renin levels were well controlled in two patients, whereas the third patient had persistent hyperandrogenism. Bilateral adrenalectomy was performed at the ages of 14, 19, and 30 years with follow-up, to date, of 25 months, 10 months, and 26 months, respectively. Postoperatively, all patients were free from hyperandrogenism. One patient experienced one episode of urosepsis precipitating an addisonian crisis. Bilateral adrenalectomy may successfully address the problems of increasing steroid requirements and hyperandrogenism in patients with severe CAH. The ability to perform this operation laparoscopically coupled with the overall metabolic benefits make bilateral adrenalectomy a reasonable alternative to lifelong androgen suppression in select patients.
先天性肾上腺皮质增生症（CAH）患者的药物治疗在大多数情况下效果欠佳。为抑制肾上腺雄激素分泌，通常需要高剂量的糖皮质激素，这可能会导致库欣综合征的症状。雄激素水平未被完全抑制通常会导致生长中心过早闭合、痤疮、男性化、性早熟、月经不规律或闭经，以及女性CAH患者生育能力下降。一种新提出的治疗CAH患者的方法是双侧肾上腺切除术。三名患有21 - 羟化酶缺乏症的白人女性患者接受了双侧肾上腺切除术。其中三例手术中有两例是通过腹腔镜完成的。在每位患者中，仅药物治疗均未成功。两名患者患有失盐型21 - 羟化酶缺乏症。第三名患者存在无法控制的高雄激素血症，并伴有肥胖和葡萄糖不耐受。所有患者的身高百分位数相对于其体重标准化百分位数均较低。一名患者的骨龄提前。两名患者的雄激素和肾素水平得到良好控制，而第三名患者持续存在高雄激素血症。双侧肾上腺切除术分别在14岁、19岁和30岁时进行，迄今为止的随访时间分别为25个月、10个月和26个月。术后，所有患者均无高雄激素血症。一名患者经历了一次泌尿道感染引发的艾迪生病危象。双侧肾上腺切除术可能成功解决重症CAH患者中类固醇需求增加和高雄激素血症的问题。腹腔镜下进行该手术的能力以及总体代谢益处，使得双侧肾上腺切除术成为某些患者终身抑制雄激素的合理替代方案。
