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中华腔镜泌尿外科杂志(电子版) ›› 2022, Vol. 16 ›› Issue (06) : 563 -568. doi: 10.3877/cma.j.issn.1674-3253.2022.06.016

病例研究

妊娠合并库欣综合征二例报告并文献复习
王毅1, 杨超1, 江羽1, 魏冉1, 方露1, 于德新1,()   
  1. 1. 230601 合肥,安徽医科大学第二附属医院泌尿外科
  • 收稿日期:2021-03-23 出版日期:2022-12-01
  • 通信作者: 于德新

Pregnancy complicated with Cushing syndrome: a report of 2 cases and literature review

Yi Wang1, Chao Yang1, Yu Jiang1, Ran Wei1, Lu Fang1, Dexin Yu1,()   

  1. 1. Department of Urology, the Second Affiliated Hospital of Anhui Medical University, Hefei 230601, China
  • Received:2021-03-23 Published:2022-12-01
  • Corresponding author: Dexin Yu
引用本文:

王毅, 杨超, 江羽, 魏冉, 方露, 于德新. 妊娠合并库欣综合征二例报告并文献复习[J/OL]. 中华腔镜泌尿外科杂志(电子版), 2022, 16(06): 563-568.

Yi Wang, Chao Yang, Yu Jiang, Ran Wei, Lu Fang, Dexin Yu. Pregnancy complicated with Cushing syndrome: a report of 2 cases and literature review[J/OL]. Chinese Journal of Endourology(Electronic Edition), 2022, 16(06): 563-568.

目的

探讨罕见的妊娠合并库欣综合征病例的病因、临床表现、诊断和治疗。

方法

回顾性分析2例我院收治的妊娠合并库欣综合征病例的临床资料,复习相关文献并予以讨论。例1:患者29岁,因"妊娠25周,四肢皮肤瘀点、瘀斑2个月,左小腿外伤半天"入住内分泌科。腹部超声检查发现左侧肾上腺区35 mm×29 mm低回声肿块。进一步完善各项检查后患者诊断为妊娠合并库欣综合征,并存在严重精神障碍。患者及家属要求手术治疗后终止妊娠。纠正低钾血症后转入泌尿外科行腹腔镜下左侧肾上腺肿瘤切除术,术中保留部分正常腺体。例2:患者28岁,因"妊娠22周,发现血糖升高1个月"入住我院内分泌科。腹部MRI检查发现左侧肾上腺区45 mm×59 mm占位。经进一步检查患者诊断为妊娠合并库欣综合征,并存在肝功能异常,考虑与患者皮质醇异常增高相关。患者及家属要求手术治疗后终止妊娠,遂转入泌尿外科行腹腔镜下左侧肾上腺肿瘤切除术。

结果

2例手术均顺利完成。2例术后病理回报均为肾上腺皮质腺瘤。例1术后监测皮质醇水平低于正常,予小剂量糖皮质激素补充治疗。2例患者均术后2周引产终止妊娠。例1术后随访12个月,该患者术后小剂量糖皮质激素补充3个月后检查皮质醇恢复正常水平予停药,其后随访皮质醇均正常;患者精神障碍症状经过药物对症治疗后逐渐好转并恢复正常。例2术后已随访6个月,皮质醇及转氨酶术后均恢复正常。2例患者电解质及其他相关激素等随访检查均正常。

结论

妊娠合并库欣综合征临床罕见,对孕妇及胎儿危害较大,需及时诊断。治疗时应综合评估孕妇及胎儿情况,选择合适的个体化治疗方案。

Objective

To explore the etiology, clinical manifestations, diagnosis and treatment of rare cases of pregnancy complicated with Cushing syndrome.

Methods

The clinical data of 2 cases of pregnancy complicated with Cushing syndrome in our hospital were retrospectively analyzed, and the relevant literatures were reviewed and discussed. Case 1, a 29-year-old patient, was admitted to the Department of Endocrinology due to "25 weeks of gestation, skin silt and ecchyma on the limbs for 2 months, and left leg trauma for half a day". Abdominal ultrasound revealed a 35 mm×29 mm hypoechoic mass in the left adrenal region. After examination, she was diagnosed as pregnant with cushing syndrome and had severe mental disorder. The patient and her family members asked to terminate the pregnancy. After correction of hypokalemia, the patient was transferred to Urology Department for laparoscopic resection of the left adrenal gland tumor, with some normal glands preserved during the operation. Case 2, a 28-year-old patient, was admitted to the Department of Endocrinology of our hospital due to "22 weeks of gestation and blood glucose elevation for 1 month". Abdominal MRI examination revealed a 45 mm×59 mm mass in the left adrenal gland. Upon examination, the patient was diagnosed as pregnant with Cushing syndrome, and abnormal liver function. The abnormal liver function was considered to be related to her abnormal increase of cortisol. The patient and her family members required the termination of pregnancy after adrenal surgery, so the patient was transferred to the urology department for laparoscopic resection of the left adrenal gland tumor.

Results

Both operations were completed successfully. Postoperative pathological findings of both cases were adrenal cortical adenoma. Case 1 was treated with low dose Glucocorticoid supplementation because the postoperative monitoring cortisol level was lower than normal. The pregnancy was terminated 2 weeks after operation in both patients. Case 1 was followed up for 12 months. In this case, cortisol returned to normal 3 months after low-dose Glucocorticoid supplementation, and hormone supplementation was stopped. Her cortisol was normal in subsequent follow-up visits. The patient's mental disorder symptoms after symptomatic drug treatment gradually improved and gradually returned to normal. Case 2 had been followed up for 6 months, and the cortisol and aminotransferase of the patient returned to normal after the operation. Follow-up examinations of electrolyte and other related hormones were normal in both patients.

Conclusion

Pregnancy with Cushing syndrome is rare in clinic, which is harmful to pregnant women and fetus, and should be diagnosed timely. The condition of the pregnant woman and the fetus should be evaluated comprehensively, and the appropriate individualized treatment plan should be selected.

图1 两例妊娠合并库欣综合征患者术前CT检查注:1a为病例1 CT平扫见左侧肾上腺直径约33 mm软组织密度灶,CT值33 Hu;1b为病例2 CT平扫见左肾前缘、近左侧肾上腺外侧支见64 mm×47 mm×54 mm团块状软组织密度影,其内密度欠均匀,1c为病例2 CT增强见肿瘤增强后轻、中度渐进强化
图2 两例妊娠合并库欣综合征患者术后病理注:2a为病例1(HE染色×40),2b为病例2(HE染色×100)
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