肾上腺切除在库欣病治疗中的作用

Effects of adrenalectomy on the treatment of Cushing disease

目的探讨肾上腺切除手术在库欣病治疗中的作用。方法总结1980年1月至2005年12月收治的15例库欣病行肾上腺切除术患者临床资料，评价手术适应证、并发症和肾上腺切除手术前后患者高皮质醇血症和激素的变化。结果15例患者均首先经蝶窦垂体手术，因术后不缓解或复发再次经蝶手术4例，垂体放疗4例。从首次经蝶手术到肾上腺切除的平均时间25．7个月（5～84个月）。双侧肾上腺切除前、后的血皮质醇的中位数分别是1156．4nmol／L和99．4nmol／L，24h尿游离皮质醇的中位数分别是315．0和5．4μg，术后均需要皮质激素替代治疗。术后随访9～120个月（平均47个月），出现Nelson综合征5例（33．3％），10例未出现Nelson综合征。结论肾上腺切除对缓解库欣病所致的高皮质醇血症是一种有效的对症治疗方法，但易出现Nelson综合征，术后需要长期激素替代治疗和定期随访。

Objective To discuss the effects of adrenalectomy （ADX） on the treatment of Cushing＇s disease（CD）. Methods Clinical data of 15 cases of CD between January 1980 and December 2005 were analyzed to evaluate operative indications, complications and the changes of hypercortisolism and hormone levels pre- and post- adrenalectomy. Results All the patients involved underwent transsphenoidal pituitary surgery previously. Repeated transsphenoidal surgery was performed in 4 cases. Pituitary radiotherapy was done in 4 cases. The average time from original transsphenoidal operation to ADX was 25.7 months. Pre- and post- adrenalectomy serum cortisol median level were 1156. 4 nmol/L and 99.4 nmol/L, the 24 h urinary-free cortisol median level were 315.0 and 5.4 μg, respectively. Hormone replacement therapy was needed in all cases. Average follow-up period was 47 months （9-120 months）. Nelson syndrome （NS） appeared in 5 cases （33.3%）, while 10 cases showed no NS. Conclusions ADX is an effective and symptomatic treatment to relieve hypercortisolism caused by CD but with the risk of NS . Longtime hormone replacement therapy and follow up are needed after ADX.