Nakayama T, Soma M, Kubo A, Matsuoka M, Abe Y, Ito M, Watanabe M, Watanabe Y, Izumi Y, Yasugi T
Second Department of Internal Medicine, Nihon University School of Medicine, Tokyo, Japan.
Nihon Naibunpi Gakkai Zasshi. 1992 Oct 20;68(10):1130-49. doi: 10.1507/endocrine1927.68.10_1130.
Pregnancy in cases of Cushing's syndrome is rare. A pregnant patient with Cushing's syndrome due to an adrenal adenoma who was diagnosed in the third trimester is described. She underwent conservative treatment for Cushing's syndrome and delivered a normal infant by Caesarean section. Currently, 121 pregnancies in 97 patients have been reported, but a principle for the treatment of the mother and fetus has not yet been established. We reviewed pregnancy in Cushing's syndrome based on the world literature and evaluated the choice of treatment to take. In the first trimester of pregnancy, therapeutic abortion or surgical treatment, such as adrenalectomy or resection of the pituitary tumor in Cushing's syndrome, is recommended for patients with severe hypercorticism (plasma cortisol > or = 30 micrograms/dl, urinary 17-OHCS > or = 15 mg/day, urinary free cortisol > or = 1000 micrograms/day), while conservative treatment is recommended for patients with mild hypercorticism (plasma cortisol < 30 micrograms/dl, urinary 17-OHCS < 15 mg/day, urinary free cortisol < 1000 micrograms/day). In the second trimester of pregnancy, surgical treatment is recommended for patients with severe hypercorticism, while conservative treatment is recommended for patients with mild hypercorticism. In the third trimester of pregnancy, Caesarean section is recommended for most cases. Drug treatments such as with metyrapone should be limited to patients showing severe hypercorticism or a maternal high risk who have contraindications to surgical treatment.
库欣综合征患者怀孕的情况较为罕见。本文描述了一名在孕晚期被诊断为肾上腺腺瘤所致库欣综合征的孕妇。她接受了库欣综合征的保守治疗，并通过剖宫产分娩了一名正常婴儿。目前，已报道97例患者中有121次妊娠，但尚未确立针对母亲和胎儿的治疗原则。我们基于世界文献对库欣综合征患者的妊娠情况进行了回顾，并评估了应采取的治疗选择。在妊娠早期，对于严重皮质醇增多症患者（血浆皮质醇≥30微克/分升、尿17-羟皮质类固醇≥15毫克/天、尿游离皮质醇≥1000微克/天），建议进行治疗性流产或手术治疗，如肾上腺切除术或库欣综合征患者的垂体瘤切除术，而对于轻度皮质醇增多症患者（血浆皮质醇<30微克/分升、尿17-羟皮质类固醇<15毫克/天、尿游离皮质醇<1000微克/天），建议进行保守治疗。在妊娠中期，对于严重皮质醇增多症患者建议进行手术治疗，而对于轻度皮质醇增多症患者建议进行保守治疗。在妊娠晚期，大多数情况下建议进行剖宫产。诸如甲吡酮等药物治疗应仅限于出现严重皮质醇增多症或有手术治疗禁忌证的高危孕妇。
