Case Report: Newborns With Pseudohypoaldosteronism Secondary to Excessive Gastrointestinal Losses Through High Output Stoma

Chia-Yu Ou,Yen-Ju Chen,Mei-Fan Chen,Geng-Bai Lin,Shu-Ti Chia

doi:10.3389/fped.2021.773246

Chia-Yu Ou, Yen-Ju Chen + Show 3 more

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https://doi.org/10.3389/fped.2021.773246

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Abstract

Life-threatening electrolyte imbalance is not uncommon in preemies. Differential diagnosis is important for immediate treatment. The syndrome of pseudohypoaldosteronism (PHA) is characterized by increased aldosterone secretion associated with clinical signs of hypoaldosteronism reflecting mineralocorticoid resistance. There are type I, type II, and secondary type of PHA. Most secondary PHA reported in the pediatric population result from urinary infection and obstructive uropathy and extremely rarely from gastrointestinal fluid loss. Seven preemies accepted jejunostomy or ileostomy, and they suffered from high output stoma. Electrolyte imbalance with bodyweight loss or cardiac event was noted. We found a high level of aldosterone and renin and diagnosed them with secondary PHA due to excessive gastrointestinal losses. After stomal reversal, aldosterone and renin level became normalized, and electrolyte was corrected. This study reports the finding of secondary pseudohyperaldosteronism (hyponatremia, hyperkalemia, and metabolic acidosis) in a series of cases with intestinal resection and ostomy of different causes. Early stomal reversal was recommended.

Highlights

The syndrome of pseudohypoaldosteronism (PHA) is characterized by increased aldosterone secretion associated with clinical signs of hypoaldosteronism reflecting mineralocorticoid resistance [1]
Most secondary PHA reported in the infant population resulted from urinary tract infection, obstructive uropathy, and extremely rare gastrointestinal fluid loss
A few adult cases of secondary PHA caused by ileum resection have been reported in the literature, but none of the newborns have reported

Summary

INTRODUCTION

The syndrome of pseudohypoaldosteronism (PHA) is characterized by increased aldosterone secretion associated with clinical signs of hypoaldosteronism reflecting mineralocorticoid resistance [1]. Instead of high output stoma-related hypokalemia, we suspected PHA and checked the aldosterone level. After this case, we routinely checked serum aldosterone levels in all patients with high output syndrome. We found that all patients with high output stoma had increased aldosterone levels (Table 2). These patients were asymptomatic (cases 2 and 3), manifested dehydration with bodyweight loss or oliguria (cases 1, 5, and 6), or experienced cardiac events (cases 4 and 7) at the time we diagnosed them as PHA. Electrolyte imbalance and high aldosterone level were corrected to normal range after the salvage procedure. PHA1 and PHA2 result from mutations for epithelial sodium channel (ENaC) subunits and renal outer medullary potassium (ROMK) channel, respectively [5, 7, 8]

F Necrotizing enterocolitis

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