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

Summary Background Secondary pseudohypoaldosteronism (S-PHA) is a life-threatening condition affecting young children with urinary tract malformation (UTM). Objective The aim of the study was to highlight the diagnosis of S-PHA in children with UTM and propose appropriate management. Study design 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 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 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 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. Table . Comparison of patients presenting S-PHA with and without UTI at diagnosis. UTM No UTI % UTI % P UPJO 2 18.2 12 11.4 0.62 OMU 3 27.3 15 14.3 0.37 PUV 3 27.3 13 12.4 0.18 VUR 2 18.2 53 50.5 0.06 Other 1 9.1 1 0.9 0.18 Duplex O/R 0 0 11 10.5 0.6 Bilateral 8 72.7 51 48.6 0.2 Total 11 9.5 105 90.5 ‘Other’ includes one case of refluxing obstructive megaureter (ROMU) and one case of complex genital malformation. UTM, Urinary Tract Malformation; UPJO, Ureteropelvic Junction Obstruction; OMU, Obstructive Megaureter; PUV, posterior urethral valve; S-PHA, secondary pseudohypoaldosteronism; UTI, urinary tract infection; VUR, vesicoureteral reflux.