Gejyo F, Ishida K, Arakawa M
2nd Department of Internal Medicine, Niigata University School of Medicine, Japan.
Am J Nephrol. 1994;14(3):236-8. doi: 10.1159/000168725.
We report the case of a 42-year-old woman with autosomal dominant polycystic kidney disease complicated by primary aldosteronism. She had a history of hypertension for 12 years and was found to have hypokalemia and polycystic renal and hepatic disease. Endocrinological tests revealed hyporeninemia and hyperaldosteronemia. Adrenal scintigraphy and venography demonstrated a left adrenal adenoma. Blood sampled from the adrenal veins confirmed hyperaldosteronemia originating from the left adrenal gland. Left adrenalectomy was performed. After surgery, plasma renin activity, plasma aldosterone titer, and serum potassium level normalized. The mechanism for the development of primary aldosteronism with autosomal dominant polycystic kidney disease may be related to the activation of the renin-angiotensin system. Four years after left adrenalectomy, hepatic but not renal cysts showed a remarkable increase; the improvement in hypokalemia may have delayed the progression of kidney cysts.
我们报告一例42岁患有常染色体显性遗传性多囊肾病并伴有原发性醛固酮增多症的女性病例。她有12年高血压病史，被发现患有低钾血症以及多囊肾和多囊肝疾病。内分泌检查显示肾素水平降低和醛固酮水平升高。肾上腺闪烁扫描和静脉造影显示左侧肾上腺有一个腺瘤。从肾上腺静脉采集的血液证实醛固酮增多症源于左侧肾上腺。于是进行了左侧肾上腺切除术。术后，血浆肾素活性、血浆醛固酮水平和血清钾水平恢复正常。常染色体显性遗传性多囊肾病伴原发性醛固酮增多症的发病机制可能与肾素 - 血管紧张素系统的激活有关。左侧肾上腺切除术后四年，肝脏囊肿而非肾脏囊肿显著增大；低钾血症的改善可能延缓了肾囊肿的进展。
