Weidmann P
Schweiz Med Wochenschr. 1982 Dec 4;112(49):1764-74.
Selective hypoaldosteronism is defined as diminished production of aldosterone, and sometimes also of 18-hydroxycorticosterone, with otherwise intact adrenal function. A decrease in the secretion of potassium and H+-ions and in the reabsorption of sodium in the distal nephron may result and lead to hyperkalemia, hyperchloremic acidosis, and impaired renal sodium conservation. The form of hypoaldosteronism which occurs in the adult is characterized by the following additional features: the aldosterone deficiency is due in the majority of cases to a decrease in enzymatically active plasma renin ("hyporeninemic hypoaldosteronism"), while various endogenous mechanisms as well as certain drugs (prostaglandin inhibitors, beta-blockers) may contribute. Other disturbances of the renin-angiotensin system (e.g. during treatment with converting-enzyme inhibitors) may rarely be responsible. Abnormalities in adrenal cortical synthesis may sometimes coexist, but proof that adrenal enzymatic defects play a primary pathogenic role in selective hyperaldosteronism in the adult is lacking. Such patients are frequently older (greater than 50 years), and often have diabetes mellitus and/or nephropathy (diabetic, interstitial, or hydronephrosis). Hyperkalemia and acidosis tend particularly to develop in association with mild to moderate impairment of renal function. The differential diagnosis should include other causes of impaired renal potassium secretion (Addison's disease, renal resistance to mineralocorticoids, potassium-sparing diuretics). Moreover, possible extrarenal factors contributing to hyperkalemia (oral potassium intake and redistribution of intracellular/extracellular space, particularly with associated insulin deficiency) should also be considered. For treatment, dietary potassium restriction is recommended as a general step. Replacement with the mineralocorticoid fludrocortisone acetate usually reverses the hyperkalemia and acidosis, but may sometimes induce sodium retention and hypertension. Loop diuretics, potassium-exchanging preparations and/or bicarbonate may also be useful as alternatives or additives.
选择性醛固酮减少症的定义是醛固酮分泌减少，有时18-羟皮质酮分泌也减少，而肾上腺功能的其他方面保持正常。远端肾单位钾离子和氢离子分泌减少以及钠离子重吸收减少，可能导致高钾血症、高氯性酸中毒以及肾钠潴留受损。成人发生的醛固酮减少症具有以下其他特征：醛固酮缺乏在大多数情况下是由于具有酶活性的血浆肾素减少（“低肾素性醛固酮减少症”），而各种内源性机制以及某些药物（前列腺素抑制剂、β受体阻滞剂）可能起作用。肾素-血管紧张素系统的其他紊乱（例如在使用转换酶抑制剂治疗期间）很少会导致该病。肾上腺皮质合成异常有时可能并存，但缺乏证据表明肾上腺酶缺陷在成人选择性醛固酮减少症中起主要致病作用。这类患者通常年龄较大（大于50岁），且常患有糖尿病和/或肾病（糖尿病性、间质性或肾积水性）。高钾血症和酸中毒尤其容易在肾功能轻度至中度受损时发生。鉴别诊断应包括其他导致肾钾分泌受损的原因（艾迪生病、对盐皮质激素的肾抵抗、保钾利尿剂）。此外，还应考虑导致高钾血症的可能肾外因素（口服钾摄入以及细胞内/细胞外空间的重新分布，特别是伴有胰岛素缺乏时）。对于治疗，一般建议限制饮食中的钾摄入。用盐皮质激素醋酸氟氢可的松替代通常可纠正高钾血症和酸中毒，但有时可能会导致钠潴留和高血压。袢利尿剂、钾交换制剂和/或碳酸氢盐也可用作替代或辅助治疗。
