Abstract：
BACKGROUND: Secondary pseudohypoaldosteronism (S-PHA) is a rare condition resulting from renal tubular resistance to aldosterone in children with urinary tract infection (UTI) and/or nephrourological malformations. It is characterized by nonspecific symptoms but with the potential for life-threatening complications. We aim to evaluate the clinical manifestations, diagnostic approach, and therapeutic interventions in children with S-PHA, along with a review of recent publications.
METHODS: A retrospective observational descriptive study was conducted on S-PHA cases diagnosed over the last 15 years at a tertiary pediatric nephrology unit. The literature for the last 10 years was reviewed.
RESULTS: Twelve patients (10 males, 6 days to 6 months) were identified. Weight loss was the main reason for consultation (50%). Ninety-two percent of patients had an underlying nephrourological pathology and 62% concomitant confirmed UTI. Seven out of 12 children were admitted to the PICU. A subsequent extrapontine myelinolysis was observed in one patient as neurological sequelae. Twenty-one articles related to S-PHA have been identified on PubMed and Embase.
CONCLUSIONS: S-PHA should be considered in infants under 6 months of age with UTI and/or CAKUT. Obstructive anomalies and vesicoureteral reflux can be found, affecting both unilateral and bilateral systems. Early medical and surgical interventions are crucial and require close monitoring to avoid iatrogenic complications.
METHODS: A retrospective observational descriptive study was conducted on S-PHA cases diagnosed over the last 15 years at a tertiary pediatric nephrology unit. The literature for the last 10 years was reviewed.
RESULTS: Twelve patients (10 males, 6 days to 6 months) were identified. Weight loss was the main reason for consultation (50%). Ninety-two percent of patients had an underlying nephrourological pathology and 62% concomitant confirmed UTI. Seven out of 12 children were admitted to the PICU. A subsequent extrapontine myelinolysis was observed in one patient as neurological sequelae. Twenty-one articles related to S-PHA have been identified on PubMed and Embase.
CONCLUSIONS: S-PHA should be considered in infants under 6 months of age with UTI and/or CAKUT. Obstructive anomalies and vesicoureteral reflux can be found, affecting both unilateral and bilateral systems. Early medical and surgical interventions are crucial and require close monitoring to avoid iatrogenic complications.
摘要：
背景：继发性假性醛固酮增多症（S-PHA）是尿路感染（UTI）和/或肾脏畸形患儿因肾小管对醛固酮抵抗而引起的罕见疾病。它的特征是非特异性症状，但有可能危及生命的并发症。我们的目的是评估临床表现，诊断方法，以及对S-PHA儿童的治疗干预，以及对最近出版物的评论。
方法：一项回顾性观察性描述性研究是在一个三级儿科肾病病房对过去15年确诊的S-PHA病例进行的。回顾了过去10年的文献。
结果：12名患者（10名男性，6天至6个月)。体重减轻是咨询的主要原因（50％）。92%的患者有潜在的肾病学病理，62%的患者同时有明确的UTI。12名儿童中有7名被送入PICU。在一名患者中观察到随后的脑桥外髓鞘溶解作为神经系统后遗症。在PubMed和Embase上鉴定了21篇与S-PHA相关的文章。
结论：6月龄以下的UTI和/或CAKUT婴儿应考虑使用S-PHA。可发现梗阻性异常和膀胱输尿管反流，影响单边和双边系统。早期医疗和手术干预至关重要，需要密切监测以避免医源性并发症。
方法：一项回顾性观察性描述性研究是在一个三级儿科肾病病房对过去15年确诊的S-PHA病例进行的。回顾了过去10年的文献。
结果：12名患者（10名男性，6天至6个月)。体重减轻是咨询的主要原因（50％）。92%的患者有潜在的肾病学病理，62%的患者同时有明确的UTI。12名儿童中有7名被送入PICU。在一名患者中观察到随后的脑桥外髓鞘溶解作为神经系统后遗症。在PubMed和Embase上鉴定了21篇与S-PHA相关的文章。
结论：6月龄以下的UTI和/或CAKUT婴儿应考虑使用S-PHA。可发现梗阻性异常和膀胱输尿管反流，影响单边和双边系统。早期医疗和手术干预至关重要，需要密切监测以避免医源性并发症。
