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醛固酮瘤和其他可通过手术矫正的原发性醛固酮增多症。

Aldosterone-producing adenoma and other surgically correctable forms of primary aldosteronism.

机构信息

Université Paris Descartes, Hôpitaux de Paris, France.

出版信息

Orphanet J Rare Dis. 2010 May 19;5:9. doi: 10.1186/1750-1172-5-9.


DOI:10.1186/1750-1172-5-9
PMID:20482833
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2889888/
Abstract

Surgically correctable forms of primary aldosteronism are characterized by unilateral aldosterone hypersecretion and renin suppression, associated with varying degrees of hypertension and hypokalemia. Unilateral aldosterone hypersecretion is caused by an aldosterone-producing adenoma (also known as Conn's adenoma and aldosteronoma), primary unilateral adrenal hyperplasia and rare cases of aldosterone-producing adrenocortical carcinoma. In these forms, unilateral adrenalectomy can cure aldosterone excess and hypokalemia, but not necessarily hypertension. The prevalence of primary aldosteronism in the general population is not known. Its prevalence in referred hypertensive populations is estimated to be between 6 and 13%, of which 1.5 to 5% have an aldosterone-producing adenoma or primary unilateral adrenal hyperplasia. Taking into account referral biases, the prevalence of surgically correctable primary aldosteronism is probably less than 1.5% in the hypertensive population and less than 0.3% in the general adult population. Surgically correctable primary aldosteronism is sought in patients with hypokalemic, severe or resistant forms of hypertension. Recent recommendations suggest screening for primary aldosteronism using the aldosterone to renin ratio. Patients with a raised ratio then undergo confirmatory suppression tests. The differential diagnosis of hypokalemic hypertension with low renin includes mineralocorticoid excess, with the mineralocorticoid being cortisol or 11-deoxycorticosterone, apparent mineralocorticoid excess, pseudo-hypermineralocorticoidism in Liddle syndrome or exposure to glycyrrhizic acid. Once the diagnosis is confirmed, adrenal computed tomography is performed for all patients. If surgery is considered, taking into consideration the clinical context and the desire of the patient, adrenal vein sampling is performed to detect whether or not aldosterone hypersecretion is unilateral. Laparoscopic surgery for unilateral aldosterone hypersecretion is associated with a morbidity of about 8%, with most complications being minor. It generally results in the normalization of aldosterone secretion and kalemia, and in a large decrease in blood pressure, but normotension without treatment is only achieved in half of all cases. Normotension following adrenalectomy is more frequent in young patients with recent hypertension than in patients with long-standing hypertension or a family history of hypertension.

摘要

可通过手术矫正的原发性醛固酮增多症的特点是单侧醛固酮分泌过多和肾素抑制,伴有不同程度的高血压和低钾血症。单侧醛固酮分泌过多是由醛固酮分泌腺瘤(也称为Conn 腺瘤和醛固酮瘤)、单侧肾上腺增生和罕见的醛固酮分泌肾上腺皮质癌引起的。在这些形式中,单侧肾上腺切除术可以治愈醛固酮过多和低钾血症,但不一定能治愈高血压。原发性醛固酮增多症在普通人群中的患病率尚不清楚。在转诊的高血压人群中,其患病率估计在 6%至 13%之间,其中 1.5%至 5%患有醛固酮分泌腺瘤或单侧肾上腺增生。考虑到转诊偏倚,手术可矫正的原发性醛固酮增多症在高血压人群中的患病率可能低于 1.5%,在普通成年人群中的患病率可能低于 0.3%。在低钾血症、严重或难治性高血压患者中寻找可通过手术矫正的原发性醛固酮增多症。最近的建议建议使用醛固酮与肾素比值筛查原发性醛固酮增多症。然后对比值升高的患者进行确认抑制试验。低钾血症伴低肾素性高血压的鉴别诊断包括盐皮质激素过多症,其盐皮质激素为皮质醇或 11-脱氧皮质酮、假性盐皮质激素过多症、Liddle 综合征中的假性盐皮质激素过多症或甘草酸暴露。一旦确诊,对所有患者进行肾上腺计算机断层扫描。如果考虑手术,考虑到临床情况和患者的意愿,进行肾上腺静脉采样以检测醛固酮是否过度分泌是单侧的。单侧醛固酮过度分泌的腹腔镜手术发病率约为 8%,大多数并发症较轻。它通常导致醛固酮分泌和钾血症正常化,并使血压大幅下降,但所有病例中只有一半未经治疗即可实现正常血压。在近期高血压的年轻患者中,肾上腺切除术后的正常血压比长期高血压或高血压家族史的患者更常见。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/018bc2291368/1750-1172-5-9-5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/1383941091b5/1750-1172-5-9-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/bef5b5d69295/1750-1172-5-9-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/50b817a36d14/1750-1172-5-9-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/2242d44f42f2/1750-1172-5-9-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/018bc2291368/1750-1172-5-9-5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/1383941091b5/1750-1172-5-9-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/bef5b5d69295/1750-1172-5-9-2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/50b817a36d14/1750-1172-5-9-3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/2242d44f42f2/1750-1172-5-9-4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/78c8/2889888/018bc2291368/1750-1172-5-9-5.jpg

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本文引用的文献

[1]
Within-patient reproducibility of the aldosterone: renin ratio in primary aldosteronism.

Hypertension. 2009-11-23

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