Nishikawa Tetsuo, Saito Jun, Omura Masao
Department of Medicine, Yokohama Rosai Hospital.
Nihon Rinsho. 2008 Jan;66(1):186-91.
Systemic cortisol plays an important role in the metabolism of glucose, lipids and proteins, as well as in the regulation of electrolyte balance. It is well known that the development of the microvascular disease of various organs such as the heart and kidney, in patients with diabetes mellitus, hyperlipidemia and hypertension of which disorders are frequently associated with Cushing's syndrome. Thus, we should treat Cushing's syndrome as soon as possible, since many complications, including cardiovascular diseases and infections, will soon occur when the definite diagnosis is delayed. Adrenalectomy is essential for treatment for Cushing's syndrome even in the patients with pituitary or ectopic ACTH-producing tumor. Some case can not be treated with surgical procedures because of worsened conditions with several complications of infection and diabetes. Then we choose medical treatment. Medical adrenalectomy is achieved by using with mitotane which is usually used for adrenocortical cancer. We commonly treat the patients with Cushing's syndrome due to adrenal tumor and pituitary or ectopic ACTH producing tumor by using metyrapone which mainly inhibits 11-hydroxylase. Metyrapone is also recommended to treat the patients who are not well differentiated Cushing's disease from ectopic ACTH syndrome. We rarely use trilostane which is an inhibitor against 3beta-hydroxysteroid dehydrogenase (3beta-HSD). Replacement therapy with hydrocortisone should be considered if adrenal failure will occur during treatment with those drugs.
全身皮质醇在葡萄糖、脂质和蛋白质代谢以及电解质平衡调节中发挥重要作用。众所周知,在糖尿病、高脂血症和高血压患者中,心脏和肾脏等各种器官的微血管疾病的发生,而这些疾病常与库欣综合征相关。因此,我们应尽快治疗库欣综合征,因为如果延迟明确诊断,很快就会出现包括心血管疾病和感染在内的许多并发症。即使对于患有垂体或异位促肾上腺皮质激素(ACTH)分泌肿瘤的患者,肾上腺切除术对于库欣综合征的治疗也是必不可少的。由于感染和糖尿病等多种并发症导致病情恶化,一些病例无法通过手术治疗。然后我们选择药物治疗。药物性肾上腺切除术是通过使用通常用于肾上腺皮质癌的米托坦来实现的。我们通常使用主要抑制11-β-羟化酶的美替拉酮来治疗因肾上腺肿瘤、垂体或异位ACTH分泌肿瘤引起的库欣综合征患者。美替拉酮也被推荐用于治疗难以区分库欣病和异位ACTH综合征的患者。我们很少使用作为3β-羟基类固醇脱氢酶(3β-HSD)抑制剂的曲洛司坦。如果在使用这些药物治疗期间发生肾上腺功能衰竭,应考虑使用氢化可的松进行替代治疗。