Torchinsky Michael Y, Deputy Stephen, Rambeau Fred, Chalew Stuart A
Department of Endocrinology, (SD) at the Children's Hospital of New Orleans and Louisiana State University Health Sciences Center in New Orleans, New Orleans, LA 70118, USA.
Horm Res. 2004;62(4):187-90. doi: 10.1159/000081067. Epub 2004 Sep 24.
Idiopathic adipsic hypernatremia (AH) is a rare disorder associated with hypokalemia and alkalosis. Hypokalemic alkalosis has been presumed to be secondary to hyperaldosteronism. We evaluated plasma renin activity, serum aldosterone, serum and urine electrolytes in a 17-year-old patient with AH on several occasions. Despite evidence of mild dehydration, serum Na >160 and K <3.2, aldosterone levels were suppressed and plasma renin activity was not elevated. Urine Na and K were not conserved. We also examined electrolyte and hormone levels in previously reported cases of AH. Aldosterone levels were not increased in any of the cases when measured. Renin secretion was increased in 2 patients. Among the compiled cases serum K was inversely correlated with serum Na (r = -0.73, p < 0.002, n = 15). Hypokalemia and alkalosis occurring in AH are not associated with secondary hyperaldosteronism. Patients with AH may have chronic renal losses of potassium leading to hypokalemia and alkalosis.
特发性无渴感高钠血症(AH)是一种与低钾血症和碱中毒相关的罕见疾病。低钾性碱中毒被认为继发于醛固酮增多症。我们多次评估了一名17岁AH患者的血浆肾素活性、血清醛固酮、血清和尿液电解质。尽管有轻度脱水的证据,血清钠>160且钾<3.2,但醛固酮水平受到抑制,血浆肾素活性未升高。尿钠和钾未得到保留。我们还检查了先前报道的AH病例的电解质和激素水平。测量时,所有病例的醛固酮水平均未升高。2例患者的肾素分泌增加。在汇总病例中,血清钾与血清钠呈负相关(r = -0.73,p < 0.002,n = 15)。AH中发生的低钾血症和碱中毒与继发性醛固酮增多症无关。AH患者可能存在慢性肾脏钾丢失,导致低钾血症和碱中毒。