FAMILIAL HYPERKALEMIA, HYPERTENSION, AND HYPORENINEMIA WITH NORMAL ALDOSTERONE LEVELS - TUBULAR DEFECT IN POTASSIUM HANDLING
FAMILIAL HYPERKALEMIA, HYPERTENSION, AND HYPORENINEMIA WITH NORMAL ALDOSTERONE LEVELS - TUBULAR DEFECT IN POTASSIUM HANDLING
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DOI：
10.1001/archinte.138.4.607
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发表时间：
1978-01-01
影响因子：
--
通讯作者：
KLEEMAN, CR
中科院分区：
文献类型：
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作者：
BRAUTBAR, N;LEVI, J;KLEEMAN, CR
1例52岁男性，伴有高血压、持续性高钾血症和高绿血症代谢性酸中毒；肾功能和肾上腺功能正常。该家族的其他四位成员也有同样的发现。患者血浆醛固酮（PA）水平在正常范围内，血浆肾素活性（PRA）未检出。通过增加内源性醛固酮水平来保存钠的能力，以及在使用外源性矿物皮质激素（醋酸氢化可的松）时不能增加钾的排泄，表明远端小管在钾处理方面存在缺陷。K+-Na+交换树脂对高钾血症的有效还原也纠正了酸中毒和高氯血症，提示高钾血症可能引起代谢性酸中毒。
A 52 yr old man had hypertension, persistent hyperkalemia and hyperchloremic metabolic acidosis; renal and adrenal functions were normal. Four other members of the family have the same findings. The patient''s plasma aldosterone (PA) level was within normal range, though plasma renin activity (PRA) was undetectable. The ability to conserve Na with increased endogenous aldosterone levels, and the inability to increase K excretion while exogenous mineralocorticoid (fludrocortisone acetate) was administered, indicated a distal tubular defect in K handling. Effective reduction of the hyperkalemia by K+-Na+ exchange resin also corrected the acidosis and the hyperchloremia, suggesting that hyperkalemia may cause metabolic acidosis.