摘要
库欣综合征是一种因内源性皮质醇产生过多而具有多种临床表现的疾病,手术为首选治疗。但部分患者因病情疑难无法明确病因而进行手术,或者术后治疗效果不好,持续的高皮质醇血症会带来各种严重并发症。在等待治疗和治疗起效期间,药物治疗可以暂时抑制过多的皮质醇产生和缓解临床表现。在抑制促肾上腺皮质激素分泌的药物中,临床试验已验证了生长抑素受体激动剂帕瑞肽的疗效,而多巴胺受体激动剂卡麦角林也显示了潜在的疗效。在抑制皮质醇分泌的药物中,单用和与其他治疗联合使用,酮康唑、氟康唑、氨基导眠能、甲吡酮、米托坦和依托咪酯均能有效抑制皮质醇分泌。第三类药物为糖皮质激素受体拮抗剂,米非司酮被批准用于未缓解或复发的不适于手术的库欣综合征。本文对目前用于库欣综合征的治疗药物的临床使用和进展进行了全面的综述。在临床工作中,需要综合考虑患者的具体情况来选择不同的药物治疗。
Cushing's syndrome is a heterogeneous complex endocrine disease with overproduction of endogenous cortisol.The operation is the first choice for Cushing's syndrome.However,it is possible that the cause is difficult to identify and the disease is persistent after surgery in some patients.It has potential serious complications if untreated or inadequately treated.Medical treatment can temporarily suppress excessive cortisol production and ameliorate its clinical manifestations while ultimate therapy becomes effective.We describe pharmacological approaches to the treatment of Cushing's syndrome.For the drugs that suppress the secretion of adrenocorticotropic hormone,somatostatin analogs such as pasireotide has been proved effective in clinical trials.Dopamine receptor agonist such as cabergoline has also been tested to prove the potential therapeutic benefit.For the drugs that suppress cortisol secretion,ketoconazole,fluconazole,aminoglutethimide,metyrapone,mitotane and etomidate are playing a potential role either alone or in combination.A third category of drugs is glucocorticoid receptor antagonists.Mifepristone has been approved for the patients with persistent or recurrent Cushing's syndrome who was not amenable to surgery.The review provides a complete survey of the current drugs used and new advances in the medical treatment of Cushing's syndrome.In Conclusion,the most appropriate medical management should be given for the patient depending on the different situations.
出处
《中国实用内科杂志》
CAS
CSCD
北大核心
2013年第7期504-508,共5页
Chinese Journal of Practical Internal Medicine
