新生儿继发性假性醛固酮增多症1例,原因并非尿路问题。

IF 1.5 4区 医学 Q4 ENDOCRINOLOGY & METABOLISM
Ecem İpek Altınok, Yavuz Özer
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引用次数: 0

摘要

在本报告中,我们报告了一例被诊断为继发性PHA的女婴,她表现出体重减轻、低钠血症、高钾血症和代谢性酸中毒,但没有UTA或UTI的存在。该患者是一名妊娠35周出生的女婴,她出现电解质异常,并被诊断为继发性假性醛固酮减少症(PHA)。最初处理为新生儿短暂性呼吸急促,她出现呼吸窘迫,需要机械通气。随后,尽管进行了适当的补液治疗,她仍表现出持续的低钠血症、高钾血症和代谢性酸中毒,这促使人们考虑肾上腺功能不全和先天性肾上腺增生(CAH)。氢化可的松和氟化可的松的治疗最初是经验性的,直到激素分析排除了CAH。进一步的评估排除了泌尿道异常和感染作为潜在原因,暗示继发性PHA。婴儿对生理盐水和电解质替代治疗反应良好,电解质水平正常化,临床改善。随访评估显示电解质失衡得到解决,患者27天后出院,无进一步并发症。继发性PHA以肾小管对醛固酮的抵抗为特征,通常在婴儿期出现严重的电解质紊乱。它可以独立于尿路异常或感染而发生,强调了在出现低钠血症、高钾血症和代谢性酸中毒的新生儿和婴儿中考虑这种诊断的重要性,这些对常规治疗无效。早期识别和适当管理,包括必要时的体液电解质纠正和激素替代,对于预防这一脆弱人群中与盐耗综合征相关的危及生命的并发症至关重要。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Case of Secondary Pseudohypoaldosteronism in a Neonate not Due to Urinary Tract Issues.

In this report, we present a case of a female infant diagnosed with secondary PHA who exhibited weight loss, hyponatremia, hyperkalemia, and metabolic acidosis without the presence of UTA or UTI. The patient was a female infant born at 35 weeks gestation who developed electrolyte abnormalities and was diagnosed with secondary pseudohypoaldosteronism (PHA). Initially managed for transient tachypnea of the newborn, she developed respiratory distress requiring mechanical ventilation. Subsequently, she exhibited persistent hyponatremia, hyperkalemia, and metabolic acidosis despite adequate fluid therapy, prompting consideration of adrenal insufficiency and congenital adrenal hyperplasia (CAH). Treatment with hydrocortisone and fludrocortisone was initiated empirically until hormonal analyses excluded CAH. Further evaluation excluded urinary tract anomalies and infections as underlying causes, implicating secondary PHA. The infant responded well to saline and electrolyte replacement therapy, with normalization of electrolyte levels and clinical improvement. Follow-up assessments demonstrated resolution of electrolyte imbalances, and the patient was discharged after 27 days without further complications. Secondary PHA, characterized by renal tubular resistance to aldosterone, typically presents with severe electrolyte disturbances in infancy. It can occur independently of urinary tract abnormalities or infections, highlighting the importance of considering this diagnosis in neonates and infants presenting with hyponatremia, hyperkalemia, and metabolic acidosis that do not respond to conventional therapies. Early recognition and appropriate management, including fluid-electrolyte correction and hormone replacement if indicated, are crucial to prevent life-threatening complications associated with salt-wasting syndromes in this vulnerable population.

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来源期刊
Journal of Clinical Research in Pediatric Endocrinology
Journal of Clinical Research in Pediatric Endocrinology ENDOCRINOLOGY & METABOLISM-PEDIATRICS
CiteScore
3.60
自引率
5.30%
发文量
73
审稿时长
20 weeks
期刊介绍: The Journal of Clinical Research in Pediatric Endocrinology (JCRPE) publishes original research articles, reviews, short communications, letters, case reports and other special features related to the field of pediatric endocrinology. JCRPE is published in English by the Turkish Pediatric Endocrinology and Diabetes Society quarterly (March, June, September, December). The target audience is physicians, researchers and other healthcare professionals in all areas of pediatric endocrinology.
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