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妊娠合并肾上腺皮质腺瘤所致库欣综合征:病例系列及综述。

Cushing's Syndrome in Pregnancy Secondary to Adrenocortical Adenoma: A Case Series and Review.

机构信息

Department of Obstetrics, The Affiliated Hospital of Qingdao University, Qingdao, China.

Department of Pathology, The Affiliated Hospital of Qingdao University, Qingdao, China.

出版信息

Endocrinol Diabetes Metab. 2024 Mar;7(2):e00474. doi: 10.1002/edm2.474.

Abstract

PURPOSE

To present a case series of Cushing's syndrome (CS) during pregnancy caused by adrenocortical adenomas, highlighting clinical features, hormonal assessments and outcomes.

METHODS

We describe five pregnant women with CS, detailing clinical presentations and laboratory findings.

RESULTS

Common clinical features included a full moon face, buffalo back and severe hypertension. Elevated blood cortisol levels with circadian rhythm disruption and suppressed adrenocorticotrophic hormone (ACTH) levels were observed. Imaging revealed unilateral adrenal tumours. Two cases underwent laparoscopic adrenalectomies during the second trimester, while three had postpartum surgery. All required hormone replacement therapy, with postoperative pathological confirmation of adrenocortical adenomas.

CONCLUSION

Diagnosis of CS during pregnancy is challenging due to overlapping features with normal pregnancy: elevated blood cortisol levels and abnormal diurnal rhythm of blood cortisol, suppressed aid diagnosis. Treatment should be individualised due to a lack of explicit optimum therapeutic approaches. Laparoscopic adrenalectomy may be an optimal choice, along with multidisciplinary management including hormone replacement therapy.

摘要

目的

介绍 5 例由肾上腺腺瘤引起的妊娠合并库欣综合征(CS)病例,重点介绍临床特征、激素评估和结局。

方法

详细描述了 5 例妊娠合并 CS 患者的临床表现和实验室检查结果。

结果

常见的临床特征包括满月脸、水牛背和严重高血压。观察到血皮质醇水平升高,伴有昼夜节律紊乱和促肾上腺皮质激素(ACTH)水平抑制。影像学显示单侧肾上腺肿瘤。2 例在孕中期行腹腔镜肾上腺切除术,3 例产后手术。所有患者均需激素替代治疗,术后病理证实为肾上腺皮质腺瘤。

结论

由于与正常妊娠重叠的特征,妊娠合并 CS 的诊断具有挑战性:血皮质醇水平升高,血皮质醇昼夜节律异常,ACTH 抑制。由于缺乏明确的最佳治疗方法,治疗应个体化。腹腔镜肾上腺切除术可能是一种理想的选择,同时还需要包括激素替代治疗在内的多学科管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/99a1/10933386/c639645c810c/EDM2-7-e00474-g003.jpg

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