Department of Medicine, Division of Nephrology, Jersey Shore University Medical Center, Hackensack-Meridian School of Medicine at Seton Hall University, Neptune, New Jersey, USA.
Department of Nephrology and Hypertension, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Glickman Urological and Kidney Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Saudi J Kidney Dis Transpl. 2020 Sep-Oct;31(5):1134-1139. doi: 10.4103/1319-2442.301183.
Gordon syndrome involves hyperkalemia, acidosis, and severe hypertension (HTN) with hypercalciuria, low renin and aldosterone levels. It is commonly observed in children and adolescents. Such patients respond successfully to sodium restriction and thiazide diuretics. In this article, we present three cases of metabolic acidosis, hyperkalemia, and renal unresponsiveness to aldosterone (MeHandRU Syndrome). All three patients did not have HTN or hypercalciuria and demonstrated normal renin and aldosterone levels. These patients did not respond to thiazide-type diuretic therapy and salt restriction. Two males (aged 55- and 62-year) and a female patient (aged 68-year) presented to the clinic with unexplained hyperkalemia (5.9 mEq/L, 5.9 mEq/L and 6.2 mEq/L, respectively). On physical examination, blood pressure (BP) was found to be normal (<140/90 mm Hg). Over the counter potassium supplement, nonsteroidal anti-inflammatory drugs, angiotensin converting enzyme inhibitors, angiotensin receptor blockers, potassium sparing diuretic use, as well as hyporeninemic hypoaldosteronism states such as diabetes mellitus were excluded. Plasma renin and aldosterone levels were normal. All three patients had low transtubular potassium gradient, despite high serum potassium levels. None of the patients reported a family history of hyperkalemia or kidney failure. All failed to demonstrate a response to hydrochlorothiazide and salt restriction. After careful consideration, strict low potassium diet (<2 g/day) was initiated in consultation with the dietician. Diuretic therapy was discontinued while BP remained within normal range (<140/90 mm Hg). At eight weeks, all three patients demonstrated normalization of potassium and correction of acidosis. At follow-up of six months, all patients are maintaining a normal potassium level. We suggest that potassium restriction can be successful in patients presenting with MeHandRU syndrome.
高戈登综合征表现为高钾血症、酸中毒和严重高血压（HTN）伴高钙尿症、低肾素和醛固酮水平。它在儿童和青少年中常见。此类患者对钠限制和噻嗪类利尿剂治疗反应良好。在本文中，我们报告了三例代谢性酸中毒、高钾血症和醛固酮肾无反应（MeHandRU 综合征）患者。所有患者均无 HTN 或高钙尿症，且肾素和醛固酮水平正常。这些患者对噻嗪类利尿剂治疗和盐限制无反应。两名男性（年龄分别为 55 岁和 62 岁）和一名女性患者（年龄为 68 岁）因不明原因高钾血症就诊（血钾分别为 5.9 mEq/L、5.9 mEq/L 和 6.2 mEq/L）。体格检查发现血压（BP）正常（<140/90 mm Hg）。排除了非甾体抗炎药、血管紧张素转换酶抑制剂、血管紧张素受体阻滞剂、保钾利尿剂的使用、以及糖尿病等低肾素低醛固酮状态引起的低钾血症。血浆肾素和醛固酮水平正常。尽管血清钾水平很高，但所有三名患者的跨小管钾梯度均较低。所有患者均未报告高钾血症或肾衰竭的家族史。所有患者均对氢氯噻嗪和盐限制无反应。经仔细考虑，与营养师协商后开始给予严格低钾饮食（<2 g/天）。在血压仍在正常范围内（<140/90 mm Hg）时，停止使用利尿剂。八周后，所有三名患者的血钾均恢复正常，酸中毒得到纠正。在六个月的随访中，所有患者均维持正常血钾水平。我们建议钾限制可成功治疗 MeHandRU 综合征患者。
