Nakada T, Koike H, Akiya T, Katayama T, Kawamata S, Takaya K, Shigematsu H
J Urol. 1987 Apr;137(4):636-40. doi: 10.1016/s0022-5347(17)44161-9.
Liddle's syndrome was diagnosed in a 72-year-old man who presented clinically with hypertension and muscle weakness. This disorder has been characterized by hyporeninemic hypoaldosteronism, hypertension, hypokalemia and enhanced erythrocyte sodium influx. Administration of spironolactone failed to correct the hypertension and electrolyte abnormality, which subsequently improved with triamterene therapy and a low salt diet. However, suppression of the renin-angiotensin-aldosterone system remained unchanged after this treatment. In addition, an atrophic juxtaglomerular apparatus and hypertensive lesions in the arterioles were confirmed by kidney biopsy after triamterene therapy. Therefore, a process of intrinsic hyperactive distal sodium reabsorption, probably affected by aldosterone-independent sodium transport into erythrocytes, appears to be important in the pathogenesis of this syndrome. Triamterene therapy, which usually is performed in patients with this disease, might not be the ultimate therapy in the future even if electrolyte abnormalities were to be improved temporarily.
一名72岁男性被诊断为利德尔综合征，其临床表现为高血压和肌肉无力。该疾病的特征为低肾素性低醛固酮血症、高血压、低钾血症以及红细胞钠内流增加。给予螺内酯未能纠正高血压和电解质异常，而氨苯蝶啶治疗及低盐饮食后情况有所改善。然而，治疗后肾素 - 血管紧张素 - 醛固酮系统的抑制状态未改变。此外，氨苯蝶啶治疗后肾活检证实肾小球旁器萎缩和小动脉高血压性病变。因此，一种内在的远端钠重吸收亢进过程，可能受醛固酮非依赖性钠转运入红细胞的影响，似乎在该综合征的发病机制中起重要作用。即使能暂时改善电解质异常，通常用于该病患者的氨苯蝶啶治疗可能并非未来的最终治疗方法。
