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Secondary Pseudohypoaldosteronism Associated With Mild Hydronephrosis in a Newborn

机译:新生儿中患有轻度肾复子病症的二次伪型蛋白酶体

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摘要

Neonatal hyponatremia with hyperkalemia is a rare but potentially life-threatening occurrence. Aldosterone deficiency secondary to congenital adrenal hyperplasia (CAH) is often suspected in these cases, although it is not easy to accurately diagnose it initially. We report the case of?a 12-day-old female infant presenting with poor sucking, hyperkalemia, and hyponatremia. Plasma renin activity (PRA) and aldosterone levels were markedly elevated, and mild hydronephrosis [Society for Fetal Urology?(SFU) grade 1] was noted. We then suspected secondary pseudohypoaldosteronism (S-PHA); however, her serum potassium level remained elevated despite sodium infusion. Because we could not rule out a diagnosis of adrenal insufficiency caused by CAH, we cautiously initiated hydrocortisone. After reviewing the results of a mass screening test and a urine steroid profile analysis, adrenal diseases were ruled out and we diagnosed the patient with S-PHA. This report aims to illustrate that mild hydronephrosis can cause S-PHA by inducing renal tubular resistance to aldosterone. Because the symptoms of S-PHA are similar to those of CAH, we recognize that further studies are needed to clarify their differences.
机译:具有高钾血症的新生儿低钠血症是一种罕见但潜在的危及生命的发生。在这些病例中通常怀疑先天性肾上腺增生(CAH)的醛固酮缺乏症,尽管最初不容易准确地诊断。我们举报了一个12天老的女性婴儿,患有糟糕的吸吮,高钾血症和低钠血症。血浆肾素活性(PRA)和醛固酮水平明显升高,并注意到患有轻微的肾多(胎儿泌尿外科(SFU)第1级]。然后涉嫌涉嫌次蛋白剂(S-PHA);然而,尽管钠输注,她的血清钾水平仍然升高。因为我们不能排除CAH引起的肾上腺功能不全的诊断,我们谨慎启动氢化鞘。在审查质量筛选试验的结果和尿液类固醇分析分析后,排除了肾上腺疾病,并诊断了S-PHA的患者。该报告旨在说明通过诱导肾小管酮抗醛固酮的肾小管抗性,肾内肾小粒可引起S-PHA。由于S-PHA的症状类似于CAH的症状,因此我们认识到需要进一步的研究来澄清它们的差异。

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