Transient pseudohypoaldosteronism: a potentially severe condition affecting infants with urinary tract malformation.
Hyperaldosteronism
Hyperkalemia
Hyponatremia
Pseudohypoaldosteronism
Urinary tract infection
Urinary tract malformation
Journal
Journal of pediatric urology
ISSN: 1873-4898
Titre abrégé: J Pediatr Urol
Pays: England
ID NLM: 101233150
Informations de publication
Date de publication:
May 2019
May 2019
Historique:
received:
03
12
2018
accepted:
01
03
2019
pubmed:
10
4
2019
medline:
5
6
2020
entrez:
10
4
2019
Statut:
ppublish
Résumé
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.
Sections du résumé
BACKGROUND
BACKGROUND
Secondary pseudohypoaldosteronism (S-PHA) is a life-threatening condition affecting young children with urinary tract malformation (UTM).
OBJECTIVE
OBJECTIVE
The aim of the study was to highlight the diagnosis of S-PHA in children with UTM and propose appropriate management.
STUDY DESIGN
METHODS
The authors retrospectively reviewed cases of S-PHA related to UTM observed at the institution and searched the PubMed® database to review the literature.
RESULTS
RESULTS
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.
DISCUSSION
CONCLUSIONS
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.
CONCLUSIONS
CONCLUSIONS
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.
Identifiants
pubmed: 30962012
pii: S1477-5131(19)30061-0
doi: 10.1016/j.jpurol.2019.03.002
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
265.e1-265.e7Informations de copyright
Copyright © 2019 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.