Kemink L, Pieters G, Hermus A, Smals A, Kloppenborg P
Department of Medicine, St. Radboud University Hospital, Nijmegen, The Netherlands.
J Clin Endocrinol Metab. 1994 Sep;79(3):887-9. doi: 10.1210/jcem.79.3.8077377.
Reportedly between 8-38% of patients who receive bilateral adrenalectomy for treatment of Cushing's disease will develop Nelson's syndrome. We investigated which factors may predict the development of the syndrome. Eight of 48 patients, bilaterally adrenalectomized for pituitary-dependent Cushing's syndrome 1-30 yr previously, developed Nelson's syndrome 1.5-13 yr (6.6 +/- 4.3 yr) after adrenalectomy. The mean age at adrenalectomy in the group of patients who developed Nelson's syndrome was significantly lower than that in the group without the syndrome (mean +/- SD, 26.0 +/- 6.0 and 35.6 +/- 11.7 yr, respectively; P < 0.02). In the patients adrenalectomized before the age of 35 yr, 8 of 27 (30%) developed Nelson's syndrome, whereas in the patients older than 35 yr, no one did (P < 0.02). No statistically significant differences between the two groups were found in sex ratio, duration of disease before adrenalectomy, or duration of follow-up thereafter. There were no statistically significant differences between the two groups in mean plasma cortisol and ACTH levels before adrenalectomy, cortisol suppressibility after the administration of 8 and 16 mg dexamethasone, or cortisol responses to CRH, TRH, and LH-releasing hormone before adrenalectomy. We conclude that age at the time of adrenalectomy is an important predictive factor for the development of Nelson's syndrome.
据报道,因库欣病接受双侧肾上腺切除术的患者中,有8% - 38%会发生纳尔逊综合征。我们研究了哪些因素可能预测该综合征的发生。48例因垂体依赖性库欣综合征在1 - 30年前接受双侧肾上腺切除术的患者中,有8例在肾上腺切除术后1.5 - 13年(平均6.6±4.3年)发生了纳尔逊综合征。发生纳尔逊综合征的患者组肾上腺切除时的平均年龄显著低于未发生该综合征的患者组(分别为平均±标准差,26.0±6.0岁和35.6±11.7岁;P<0.02)。在35岁之前接受肾上腺切除术的患者中,27例中有8例(30%)发生了纳尔逊综合征,而在35岁以上的患者中,无人发生(P<0.02)。两组在性别比例、肾上腺切除术前疾病持续时间或此后的随访持续时间方面未发现统计学上的显著差异。两组在肾上腺切除术前的平均血浆皮质醇和促肾上腺皮质激素水平、给予8毫克和16毫克地塞米松后的皮质醇抑制率或肾上腺切除术前对促肾上腺皮质激素释放激素、促甲状腺激素和促黄体生成素释放激素的皮质醇反应方面均未发现统计学上的显著差异。我们得出结论,肾上腺切除时的年龄是纳尔逊综合征发生的一个重要预测因素。