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因双侧分泌醛固酮的肾上腺皮质微腺瘤导致的难以控制的高血压,同时伴有一个分泌皮质醇的肾上腺大腺瘤。

Difficult-to-control hypertension due to bilateral aldosterone-producing adrenocortical microadenomas associated with a cortisol-producing adrenal macroadenoma.

机构信息

Division of Nephrology, Endocrinology, and Vascular Medicine, Department of Medicine, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.

出版信息

J Hum Hypertens. 2011 Feb;25(2):114-21. doi: 10.1038/jhh.2010.35. Epub 2010 May 13.

Abstract

The patient was a 54-year-old woman who developed a right adrenal tumour, Cushingoid features, elevated levels of cortisol that were not suppressed by 1 nor 8 mg of dexamethasone, and suppression of adrenocorticotropin (ACTH) during treatment for severe hypertension. Computed tomography (CT) revealed a right adrenal tumour and an atrophic left adrenal gland. In addition, elevated plasma aldosterone concentration (PAC) and suppressed plasma renin activity (PRA) with an aldosterone-to-renin ratio of 128 (ng per 100 ml per ng ml⁻¹ h⁻¹) suggested aldosterone excess. Urinary excretion of aldosterone was relatively high, and the captopril and rapid ACTH tests resulted in no response of PRA and exaggerated increase in PAC, respectively. ACTH-loaded adrenal venous sampling showed bilateral excess of aldosterone with right predominance of cortisol. Right laparoscopic partial adrenalectomy (ADX) and immunohistochemical analysis showed both a cortisol-producing adenoma and an aldosterone-producing microadenoma (microAPA) within the attached adrenal, which had not been detected by CT preoperatively. After the right partial ADX, her blood pressure, aldosterone level and suppressed PRA remained unchanged. Subsequently, laparoscopic total left ADX was performed. Two microAPAs with paradoxical hyperplasia were revealed within the apparently atrophic left adrenal gland. Soon after the second surgery, her blood pressure normalized without requiring any anti-hypertensive medication.

摘要

患者为 54 岁女性,因右侧肾上腺肿瘤、库欣样特征、皮质醇水平升高(1mg 和 8mg 地塞米松均不能抑制)和促肾上腺皮质激素(ACTH)抑制而就诊,同时患者还患有严重高血压。计算机断层扫描(CT)显示右侧肾上腺肿瘤和左侧肾上腺萎缩。此外,血浆醛固酮浓度(PAC)升高,血浆肾素活性(PRA)受抑制,醛固酮与肾素比值为 128(ng/100ml/ng/ml/h),提示醛固酮过多。尿醛固酮排泄相对较高,卡托普利和快速 ACTH 试验分别导致 PRA 无反应和 PAC 明显增加。ACTH 负荷肾上腺静脉采样显示双侧醛固酮过多,右侧皮质醇优势。右侧腹腔镜部分肾上腺切除术(ADX)和免疫组织化学分析显示,附着肾上腺内既有皮质醇产生腺瘤,也有醛固酮产生微腺瘤(微 APA),术前 CT 未检测到。右侧部分 ADX 后,其血压、醛固酮水平和抑制后的 PRA 保持不变。随后,进行了腹腔镜全左 ADX。在明显萎缩的左侧肾上腺内发现了两个具有反常增生的微 APA。第二次手术后不久,她的血压恢复正常,无需任何降压药物。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c1b3/3023071/c94eba861ca2/jhh201035f1.jpg

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