Department of Endocrinology and Metabolism, Chongqing University Fuling Hospital, No. 2 Gaosuntang Road, Fuling, China.
Department of General Practice, Chongqing University Fuling Hospital, Fuling, China.
J Med Case Rep. 2024 Oct 6;18(1):463. doi: 10.1186/s13256-024-04798-4.
Primary aldosteronism is characterized by high plasma aldosterone and low renin. The plasma aldosterone-to-renin ratio is recommended for screening. Severe hydronephrosis leads to renal parenchymal ischemia, resulting in increased renin secretion. Since nonsuppression of renin may cause a negative result in the aldosterone-to-renin ratio test, severe hydronephrosis and primary aldosteronism occurring simultaneously in a patient are challenging to diagnose.
A 54-year-old Chinese man of Han ethnicity was diagnosed with hypertension and severe hypokalemia (minimum 1.57 mmol/L) 13 years prior, and was also diagnosed with severe hydronephrosis due to congenital ureteral stenosis on the left side. His clinical features suggested primary aldosteronism, but the aldosterone-to-renin ratio result of the patient was negative every time he underwent the primary aldosteronism screening test. No further treatment for primary aldosteronism was performed, which led the patient to suffer from severe hypokalemia, such that he was taking 12-15 g/day potassium chloride orally to keep his blood potassium between 3.0 and 3.5 mmol/L (reference value, 3.5-5.5 mmol/L) for 13 years, and the patient needed to be hospitalized in the intensive care unit for rescue several times. At admission, although the aldosterone-to-renin ratio result of the patient was negative, we still did the saline stress test and captopril inhibition test, and the results showed that the plasma aldosterone level was not lower after the test than before the test. Adrenal enhanced computed tomography suggested an adenoma in the left adrenal gland, and the results of adrenal vein sampling suggested that the left side was the dominant side. Therefore, laparoscopic total resection of the left adrenal gland was performed, and 2 weeks later, the patient developed short-term renal function impairment and hyperkalemia, but his renal function and blood potassium returned to normal after treatment that included fluid rehydration. The patient's biochemical test results and clinical symptoms were completely normal after 1 year.
We suggest that for patients with a high suspicion of primary aldosteronism in the clinic, comprehensive analysis must be performed in combination with clinical characteristic assessments, such as severe hydronephrosis, if renin is within the normal range or if the aldosterone-to-renin ratio result is negative at screening and diagnostic tests, and adrenal vein sampling should be performed if necessary. It can help avoid misdiagnoses and contribute to the treatment of patients with severe hydronephrosis and primary aldosteronism.
原发性醛固酮增多症的特征是血浆醛固酮升高和肾素降低。推荐使用血浆醛固酮与肾素比值进行筛查。严重的肾积水会导致肾实质缺血，导致肾素分泌增加。由于肾素不抑制可能导致醛固酮与肾素比值试验的结果为阴性，因此同时患有严重肾积水和原发性醛固酮增多症的患者的诊断具有挑战性。
一名 54 岁的汉族男性，13 年前被诊断为高血压和严重低钾血症（最低 1.57mmol/L），并被诊断为左侧先天性输尿管狭窄引起的严重肾积水。他的临床特征提示为原发性醛固酮增多症，但每次进行原发性醛固酮增多症筛查试验时，患者的醛固酮与肾素比值结果均为阴性。由于没有进一步治疗原发性醛固酮增多症，导致患者出现严重低钾血症，他每天需要口服 12-15g 氯化钾，以将血钾维持在 3.0-3.5mmol/L（参考值 3.5-5.5mmol/L）之间，持续 13 年，并且患者需要多次住院重症监护室进行抢救。入院时，尽管患者的醛固酮与肾素比值结果为阴性，但我们仍进行了盐水应激试验和卡托普利抑制试验，结果表明试验后血浆醛固酮水平并未低于试验前。肾上腺增强 CT 提示左侧肾上腺有腺瘤，肾上腺静脉取样结果提示左侧为优势侧。因此，行腹腔镜左肾上腺全切术，术后 2 周，患者出现短期肾功能损害和高钾血症，但经补液等治疗后，患者的肾功能和血钾恢复正常。术后 1 年，患者的生化检查结果和临床症状完全正常。
我们建议，对于临床上高度怀疑原发性醛固酮增多症的患者，如果肾素在正常范围内，或筛查和诊断性检查时醛固酮与肾素比值结果为阴性，且存在严重肾积水等临床特征，应进行综合分析，必要时进行肾上腺静脉取样。这有助于避免误诊，有助于治疗严重肾积水和原发性醛固酮增多症患者。
