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妊娠期间继发于肾上腺腺瘤的库欣综合征:甲吡酮治疗与腹腔镜肾上腺切除术

Cushing's syndrome during pregnancy secondary to adrenal adenoma: metyrapone treatment and laparoscopic adrenalectomy.

作者信息

Blanco C, Maqueda E, Rubio J A, Rodriguez A

机构信息

Department of Endocrinology, Hospital Principe de Asturias, University of Alcalá, Spain.

出版信息

J Endocrinol Invest. 2006 Feb;29(2):164-7. doi: 10.1007/BF03344091.

DOI:10.1007/BF03344091
PMID:16610244
Abstract

Cushing's syndrome during pregnancy is a rare and difficult to diagnose disorder. We describe the case of a 30-yr-old woman presenting with symptoms and signs of mild hypercortisolism, in which ACTH-independent Cushing's syndrome was diagnosed. Urinary cortisol excretion was elevated and circadian rhythm of cortisol was absent. ACTH levels were low. In addition, plasma cortisol failed to suppress after a high dexamethasone dose. An abdominal computed tomography scan confirmed a left adrenal mass. While diagnosis work-up was still in progress, the patient became pregnant and wanted to carry her pregnancy to full-term. Hypercortisolism was successfully controlled with metyrapone, which was started at 8 weeks of gestation. At 16 weeks of gestation, a laparoscopic left adrenalectomy was performed. Pathologic examination of the gland showed a benign adrenocortical adenoma. The patient developed secondary adrenal insufficiency and was discharged on 20 mg hydrocortisone daily dose. At 30 weeks of gestation, the patient had a pre-term rupture of membranes and underwent spontaneous vaginal delivery. The newborn was a normal virilized male who weighed 1280 g. No apparent metyrapone-induced teratogenic effects were observed and there was no clinical or biochemical suppression of adrenocortical function. In conclusion, in adrenal Cushing's syndrome during pregnancy, medical treatment with metyrapone as soon as the diagnosis is made, in combination with laparoscopic surgery during the second trimester, are useful in preventing complications secondary to hypercortisolism and safe both for the mother and infant.

摘要

妊娠期库欣综合征是一种罕见且难以诊断的疾病。我们描述了一例30岁女性患者,其表现出轻度皮质醇增多症的症状和体征,最终被诊断为促肾上腺皮质激素(ACTH)非依赖性库欣综合征。尿皮质醇排泄升高,且皮质醇昼夜节律消失。ACTH水平较低。此外,高剂量地塞米松后血浆皮质醇未能被抑制。腹部计算机断层扫描证实左肾上腺有肿块。在诊断检查仍在进行时,患者怀孕并希望足月分娩。妊娠8周时开始用甲吡酮成功控制了皮质醇增多症。妊娠16周时,进行了腹腔镜左肾上腺切除术。腺体的病理检查显示为良性肾上腺皮质腺瘤。患者出现继发性肾上腺功能不全,出院时每日服用20mg氢化可的松。妊娠30周时,患者胎膜早破,随后经阴道自然分娩。新生儿为正常男性,体重1280g,有男性化表现。未观察到明显的甲吡酮致畸作用,且肾上腺皮质功能无临床或生化抑制。总之,对于妊娠期肾上腺性库欣综合征,一旦确诊即使用甲吡酮进行药物治疗,并在孕中期联合腹腔镜手术,有助于预防皮质醇增多症继发的并发症,且对母婴均安全。

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ACTH-independent Cushing's syndrome in pregnancy with spontaneous resolution after delivery: control of the hypercortisolism with metyrapone.妊娠期间的促肾上腺皮质激素(ACTH)非依赖性库欣综合征,产后自发缓解:用甲吡酮控制高皮质醇血症
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