Division of Nephrology, Department of Internal Medicine, National Taiwan University Hospital, National Taiwan University College of Medicine, Taiwan.
J Renin Angiotensin Aldosterone Syst. 2013 Jun;14(2):167-73. doi: 10.1177/1470320312452767. Epub 2012 Jul 12.
Hypertension is a frequent early manifestation of autosomal dominant polycystic kidney disease (ADPKD). Several mechanisms can cause hypertension in ADPKD patients, although, primary aldosteronism (PA) as a possible manifestation of hypertension in ADPKD is extremely rare. We retrospectively reviewed the Taiwan Primary Aldosteronism Investigation (TAIPAI) database, which listed a total of 346 patients diagnosed with PA. Of these 346 patients, only three cases of concurrent PA and ADPKD were identified. These patients presented with hypertensive crisis and hypokalemia, and subsequent testing revealed aldosterone-producing adenomas (APAs) that were removed by laparoscopic adrenalectomy. Postoperatively, aldosterone-renin ratios (ARRs) and potassium levels normalized, and blood pressure improved. The diagnosis of PA in ADPKD is extremely challenging because multiple renal cysts can obscure the identification of adrenal adenomas, and ADPKD is associated with hypertension in almost all cases.(1) Because of frequent delays in the diagnosis of PA in ADPKD patients, future prospective studies to screen PA in hypertensive ADPKD patients may be necessary to evaluate the exact prevalence of coexistence of PA and ADPKD.
高血压是常染色体显性多囊肾病（ADPKD）的早期表现之一。尽管醛固酮增多症（PA）作为 ADPKD 患者高血压的一种表现形式极为罕见，但几种机制可导致 ADPKD 患者发生高血压。我们回顾性分析了台湾原发性醛固酮增多症调查（TAIPAI）数据库，该数据库共列出了 346 例确诊为 PA 的患者。在这 346 例患者中，仅发现 3 例并发 PA 和 ADPKD。这些患者表现为高血压危象和低钾血症，随后的检查发现产生醛固酮的腺瘤（APAs），通过腹腔镜肾上腺切除术切除。术后，醛固酮肾素比值（ARR）和血钾水平恢复正常，血压得到改善。ADPKD 中 PA 的诊断极具挑战性，因为多个肾囊肿可能会掩盖肾上腺腺瘤的识别，而且 ADPKD 几乎在所有情况下都与高血压相关。(1)由于 ADPKD 患者中 PA 的诊断经常延迟，未来可能需要对高血压性 ADPKD 患者进行 PA 筛查的前瞻性研究，以评估 PA 和 ADPKD 共存的确切患病率。
