Castinetti Frederic
Department of Endocrinology, Aix Marseille University, Assistance Publique-Hopitaux de Marseille, INSERM, Marseille Medical Genetics, Marmara Institute, French Reference Center for Rare Pituitary Diseases, Endo-European Reference Network and EURACAN European Expert Center on Rare Pituitary Tumors, La Conception Hospital, Marseille, France.
J Neuroendocrinol. 2022 Aug;34(8):e13120. doi: 10.1111/jne.13120. Epub 2022 Mar 29.
Transsphenoidal surgery is the first-line treatment of Cushing's disease. However, medical treatment can be given in a high proportion of patients with this rare condition. This is especially the case in severe hypercortisolism for which medical treatment will be given for a short period of time to prepare the patient for surgery, or on a long-term basis after failed transsphenoidal surgery, or when waiting for the maximal beneficial effect of radiation techniques. These different situations all require that hypercortisolism be perfectly controlled. Severe hypercortisolism is frequently treated by a block and replace approach during which a substitutive treatment is given after the induction of adrenal insufficiency. Mild hypercortisolism, the most frequent situation, is usually treated with a titration approach with a progressive dose increase. This approach requires certainty of eucortisolism because patients may be treated for a prolonged period. This review details the main situations during which medical treatment should be considered, as well as the way in which to monitor them to avoid the long-term consequences of mild hypo- or hypercortisolism.
经蝶窦手术是库欣病的一线治疗方法。然而，对于这种罕见疾病的大部分患者而言，可以进行药物治疗。在严重皮质醇增多症中尤其如此，对于此类病症，药物治疗可在短期内用于使患者为手术做准备，或在经蝶窦手术失败后长期使用，或在等待放射技术产生最大有益效果时使用。这些不同情况均要求完美控制皮质醇增多症。严重皮质醇增多症通常采用阻断并替代疗法进行治疗，即在诱发肾上腺功能不全后给予替代治疗。轻度皮质醇增多症是最常见的情况，通常采用逐步增加剂量的滴定法进行治疗。这种方法需要确保皮质醇正常，因为患者可能需要接受长期治疗。本综述详细介绍了应进行药物治疗的主要情况，以及监测这些情况的方法，以避免轻度皮质醇过少或过多带来的长期后果。
