Clinical management of critically ill patients with Cushing's disease due to ACTH-secreting pituitary macroadenomas: effectiveness of presurgical treatment with pasireotide
Clinical management of critically ill patients with Cushing's disease due to ACTH-secreting pituitary macroadenomas: effectiveness of presurgical treatment with pasireotide
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DOI：
10.1007/s12020-015-0601-2
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发表时间：
2016-06-01
期刊：
影响因子：
3.7
通讯作者：
Angileri, F.
中科院分区：
文献类型：
--
作者：
Cannavo, S.;Messina, E.;Angileri, F.
危重库欣病（CD）患者的管理极具挑战性。帕瑞肽适用于垂体手术不可行或无法治愈的CD患者的治疗，但尚无关于该药物用于危重患者术前治疗的数据。我们报告了术前帕瑞肽治疗CD患者的效果，这些患者皮质醇增多导致危及生命的低钾血症、水肿和心肺并发症，无法手术。两名患有ACTH分泌型垂体大腺瘤的危重患者在一线术前帕瑞肽治疗（600 μ g s.c. bid）。在治疗的前21天，帕瑞肽治疗诱导血浆ACTH，血清皮质醇和尿游离皮质醇水平的快速，部分下降，随之而来的血清钾浓度和动脉血气参数的正常化，在这两个病人。他们没有遇到无法控制的副作用，并在有效治疗4周后接受了内窥镜经蝶手术。术前MRI评价未显示垂体瘤缩小。在第一例患者中获得了CD的手术治愈，而在第二例患者中，减积允许皮质醇增多症的药物控制。我们认为，帕瑞肽可以诱导危重CD患者的临床和代谢状况的快速改善，在这些患者中，手术方法被认为是危险的，需要延迟。
The management of critically ill Cushing's disease (CD) patients is extremely challenging. Pasireotide is indicated for the treatment of CD patients when pituitary surgery is unfeasible or has not been curative, but no data are available about the use of this drug as pre-operative treatment in critically ill patients. We report the effects of presurgical pasireotide therapy in CD patients in whom hypercortisolism caused life-threatening hypokalemia, alkalosis, and cardio-respiratory complications precluding surgical approach. Clinical, biochemical, and radiological data of two critically ill patients with ACTH-secreting pituitary macroadenoma, before and during first-line presurgical pasireotide treatment (600 mu g s.c. bid). During the first 21 days of treatment, pasireotide therapy induced a rapid, partial decrease of plasma ACTH, serum cortisol, and urinary free cortisol levels, with the consequent normalization of serum potassium concentration and arterial blood gases parameters, in both the patients. They did not experience unmanageable side effects and underwent endoscopic transsphenoidal surgery after 4 weeks of effective treatment. Pre-operative MRI evaluation did not show pituitary tumor shrinkage. Surgical cure of CD was obtained in the first patient, while debulking allowed the pharmacological control of hypercortisolism in the second case. We suggest that pasireotide can induce a rapid improvement of clinical and metabolic conditions in critically ill CD patients in whom surgical approach is considered hazardous and need to be delayed.