Nandagopal Radha, Vaidyanathan Priya, Kaplowitz Paul
Inter-Institute Pediatric Endocrinology Training Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.
Int J Pediatr Endocrinol. 2009;2009:195728. doi: 10.1155/2009/195728. Epub 2009 May 21.
Hyponatremia with hyperkalemia in infancy is an uncommon but life-threatening occurrence. In the first weeks of life, this scenario is often associated with aldosterone deficiency due to salt-wasting congenital adrenal hyperplasia. However, alternative diagnoses involving inadequate mineralocorticoid secretion or action must be considered, particularly for infants one month of age or older. We report four infants who presented with profound hyponatremia accompanied by urinary tract infection, ultimately leading to the diagnosis of transient pseudohypoaldosteronism. Our cases provide support for the idea that the renal tubular resistance to aldosterone is due to urinary tract infection itself rather than to underlying urinary tract anomalies typically found in these infants. Awareness of this condition is important so that serum aldosterone, urine sodium, and urine cultures may be obtained immediately in any infant presenting with hyponatremia and hyperkalemia in whom a diagnosis of congenital adrenal hyperplasia was not found. Adequate replacement with intravenous saline and antibiotic therapy is sufficient to correct sodium levels over 24-48 hours.
婴儿期低钠血症伴高钾血症虽不常见但危及生命。在出生后的头几周,这种情况常与盐耗型先天性肾上腺增生导致的醛固酮缺乏有关。然而,必须考虑涉及盐皮质激素分泌或作用不足的其他诊断,尤其是对于1个月及以上的婴儿。我们报告了4例患有严重低钠血症并伴有尿路感染的婴儿,最终诊断为暂时性假性醛固酮减少症。我们的病例支持这样一种观点,即肾小管对醛固酮的抵抗是由尿路感染本身引起的,而非通常在这些婴儿中发现的潜在尿路异常。认识到这种情况很重要,以便在任何出现低钠血症和高钾血症且未发现先天性肾上腺增生诊断的婴儿中,能够立即检测血清醛固酮、尿钠和进行尿培养。静脉输注生理盐水和抗生素治疗进行充分替代足以在24至48小时内纠正钠水平。