Morimoto R, Satani N, Iwakura Y, Ono Y, Kudo M, Nezu M, Omata K, Tezuka Y, Seiji K, Ota H, Kawasaki Y, Ishidoya S, Nakamura Y, Arai Y, Takase K, Sasano H, Ito S, Satoh F
Division of Nephrology, Endocrinology and Vascular Medicine, Department of Medicine, Tohoku University Hospital, Sendai, Japan.
Department of Diagnostic Radiology, Tohoku University Hospital, Sendai, Japan.
J Hum Hypertens. 2016 Jun;30(6):379-85. doi: 10.1038/jhh.2015.100. Epub 2015 Nov 5.
Primary aldosteronism due to unilateral aldosterone-producing adenoma (APA) is a surgically curable form of hypertension. Bilateral APA can also be surgically curable in theory but few successful cases can be found in the literature. It has been reported that even using successful adrenal venous sampling (AVS) via bilateral adrenal central veins, it is extremely difficult to differentiate bilateral APA from bilateral idiopathic hyperaldosteronism (IHA) harbouring computed tomography (CT)-detectable bilateral adrenocortical nodules. We report a case of bilateral APA diagnosed by segmental AVS (S-AVS) and blood sampling via intra-adrenal first-degree tributary veins to localize the sites of intra-adrenal hormone production. A 36-year-old man with marked long-standing hypertension was referred to us with a clinical diagnosis of bilateral APA. He had typical clinical and laboratory profiles of marked hypertension, hypokalaemia, elevated plasma aldosterone concentration (PAC) of 45.1 ng dl(-1) and aldosterone renin activity ratio of 90.2 (ng dl(-1) per ng ml(-1 )h(-1)), which was still high after 50 mg-captopril loading. CT revealed bilateral adrenocortical tumours of 10 and 12 mm in diameter on the right and left sides, respectively. S-AVS confirmed excess aldosterone secretion from a tumour segment vein and suppressed secretion from a non-tumour segment vein bilaterally, leading to the diagnosis of bilateral APA. The patient underwent simultaneous bilateral sparing adrenalectomy. Histopathological analysis of the resected adrenals together with decreased blood pressure and PAC of 5.2 ng dl(-1) confirmed the removal of bilateral APA. S-AVS was reliable to differentiate bilateral APA from IHA by direct evaluation of intra-adrenal hormone production.
由单侧醛固酮分泌腺瘤（APA）引起的原发性醛固酮增多症是一种可通过手术治愈的高血压形式。双侧APA理论上也可通过手术治愈，但文献中鲜有成功案例。据报道，即使通过双侧肾上腺中央静脉成功进行肾上腺静脉采样（AVS），要将双侧APA与伴有计算机断层扫描（CT）可检测到的双侧肾上腺皮质结节的双侧特发性醛固酮增多症（IHA）区分开来也极其困难。我们报告一例通过节段性AVS（S-AVS）和经肾上腺一级分支静脉采血来定位肾上腺内激素产生部位而诊断为双侧APA的病例。一名患有显著长期高血压的36岁男性因临床诊断为双侧APA转诊至我们这里。他具有典型的临床和实验室特征，即显著高血压、低钾血症、血浆醛固酮浓度（PAC）升高至45.1 ng dl⁻¹以及醛固酮肾素活性比值为90.2（ng dl⁻¹ per ng ml⁻¹ h⁻¹），在服用50 mg卡托普利后该比值仍很高。CT显示右侧和左侧分别有直径为10和12 mm的双侧肾上腺皮质肿瘤。S-AVS证实双侧肿瘤节段静脉有过量醛固酮分泌，非肿瘤节段静脉分泌受抑制，从而诊断为双侧APA。该患者接受了同期双侧保留肾上腺切除术。切除肾上腺的组织病理学分析以及血压下降和PAC降至5.2 ng dl⁻¹证实切除了双侧APA。S-AVS通过直接评估肾上腺内激素产生情况，对于区分双侧APA和IHA是可靠的。
