Transient pseudohypoaldosteronism: a potentially severe condition affecting infants with urinary tract malformation

Secondary pseudohypoaldosteronism (S-PHA) is a life-threatening condition affecting young children with urinary tract malformation (UTM). The aim of the study was to highlight the diagnosis of S-PHA in children with UTM and propose appropriate management. The authors retrospectively reviewed cases o...

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Bibliographic Details
Published in:Journal of pediatric urology 2019-05, Vol.15 (3), p.265.e1-265.e7
Main Authors: Delforge, X., Kongolo, G., Cauliez, A., Braun, K., Haraux, E., Buisson, P.
Format: Article
Language:English
Subjects:
Hyperaldosteronism
Hyperkalemia
Hyponatremia
Life Sciences
Pseudohypoaldosteronism
Urinary tract infection
Urinary tract malformation
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Summary:Secondary pseudohypoaldosteronism (S-PHA) is a life-threatening condition affecting young children with urinary tract malformation (UTM). The aim of the study was to highlight the diagnosis of S-PHA in children with UTM and propose appropriate management. The authors retrospectively reviewed cases of S-PHA related to UTM observed at the institution and searched the PubMed® database to review the literature. A total of 116 cases of S-PHA associated with UTM, including the four cases from the institution, were reviewed. One hundred six cases (92.2%) were younger than 6 months, and 95 cases (81.9%) occurred in boys. Urinary tract infection was associated in 105 cases (90.5%). All types of UTM were observed. In the absence of urinary tract infection, S-PHA was related to bilateral UTM or solitary kidney. In 89 cases (76.5%), S-PHA resolved with medical treatment only. In cases of UTM requiring immediate surgery, electrolyte imbalance related to S-PHA also resolved after surgery. Children with associated urinary tract infection and bilateral UTM are at higher risk of developing S-PHA. The pathogenesis of S-PHA has not been fully elucidated. Renal tubular immaturity may be one of the factors involved, in view of the young age of the population being affected. A high rate of bilateral UTM (or UTM on solitary kidney) was observed (50.9%), suggesting an association with S-PHA. In the absence of urinary tract infection (UTI), S-PHA appeared to occur more frequently in the presence of bilateral UTM. Although the indication for early surgery remains unclear, it may have a role in the prevention of UTI and prevention of recurrence of S-PHA. Serum electrolytes should be checked in children with UTM before urological surgery, and/or presenting urinary tract infection, before the age of 6 months. The results of this study must be interpreted cautiously because of its retrospective nature and the fact that data were derived from various articles. Few articles on S-PHA related to UTM have been published in the literature. To the best of the authors' knowledge, the study constitutes the largest series published to date. S-PHA results in potentially severe electrolyte imbalance and affects children younger than 6 months with UTI and/or UTM. Electrolyte abnormalities related to S-PHA often resolve after administration of appropriate intravenous electrolyte solution and treatment of UTI and/or surgery.TableComparison of patients presenting S-PHA with and without UTI at diagnosi
ISSN:1477-5131
1873-4898
DOI:10.1016/j.jpurol.2019.03.002