Department of Medicine.
Int J Gen Med. 2009 Dec 29;2:209-17. doi: 10.2147/ijgm.s7545.
Cushing's disease, due to pituitary adrenocorticotropic hormone (ACTH) hypersecretion, is the most common etiology of spontaneous excess cortisol production. The majority of pituitary tumors causing Cushing's disease measure <1 cm and the excess morbidity associated with these tumors is mostly due to the effects of elevated, nonsuppressible, ACTH levels leading to adrenal steroid hypersecretion. Elevated circulating cortisol levels lead to abnormal fat deposition, hypertension, diabetes, coronary artery disease, osteoporosis, muscle weakness and psychological disturbances. At experienced centers, initial surgical remission rate via transnasal, transphenoidal resection approaches 80% for tumors less than 1 cm, but may be as low as 30% for larger lesions and long-term recurrence in all groups approaches 25%. Residual disease may be managed with more radical surgery, pituitary-directed radiation, bilateral adrenalectomy, or medical therapy. This paper addresses current and novel therapies in various stages of development for Cushing's disease.
库欣病是由于垂体促肾上腺皮质激素(ACTH)分泌过度引起的,是自发性皮质醇过度产生的最常见病因。大多数导致库欣病的垂体肿瘤小于 1 厘米,这些肿瘤引起的发病率增加主要是由于升高的、不可抑制的、ACTH 水平导致肾上腺类固醇过度分泌所致。循环中皮质醇水平升高会导致异常脂肪沉积、高血压、糖尿病、冠状动脉疾病、骨质疏松症、肌肉无力和心理障碍。在有经验的中心,经鼻蝶入路的初次手术缓解率对于小于 1 厘米的肿瘤为 80%,但对于较大的病变可能低至 30%,并且所有组别的长期复发率接近 25%。残余疾病可以通过更激进的手术、垂体定向放疗、双侧肾上腺切除术或药物治疗来管理。本文介绍了库欣病各阶段的现有和新疗法。