ESPE Abstracts (2021) 94 P2-51

Adrenals and HPA Axis

A Rare Cause of Hyponatremia in a Child with Hirschsprung Disease Operated with an Ileostomy

Abeer Alassaf


Amman, Amman, Jordan

Background and Objectives: Pseudohypoaldosteronism (PHA) is a rare condition characterized by hyponatremia, hyperkalemia, metabolic acidosis and high plasma aldosterone level. Pseudohypoaldosteronism secondary to gastrointestinal losses is rare in the pediatric population. We present a case with secondary pseudohypoaldosteronism due to excessive gastrointestinal losses through ileostomy.

Methods: A 1.5 year-old-male child, who was diagnosed with Hirschsprung disease at the age of 2 months with ileostomy creation, presented 2 weeks postoperatively with picture of hypovolemic shock. There was history of gastrointestinal fluid losses through ileostomy of 5 days duration. Laboratory tests showed picture of pseudohypoaldosteronism. Hormonal work up was sent and patient was started on hydrocortisone and intravenous fluid, in addition to management of hyperkalemia.

Results: Hormonal work up showed picture of pseudohypoaldosteronism. Hydrocortisone was tapered and discontinued. Excessive losses of fluids and sodium, leads to activation of renin-angiotensin system. Subsequently, aldosterone can no longer cause potassium excretion in presence of excess sodium loss; leading to hyperkalemia. At the age of 14 months, Duhamel pull-through operation was done with closure of ileostomy, with maintenance of normal sodium and aldosterone levels.

Conclusion: Pseudohypoaldosteronism should be included in the differential diagnosis when evaluating hyponatremia especially in those with excessive fluid losses. Due to the rarity of this condition, there is a high possibility of delayed diagnosis; which would lead to increased morbidity and mortality.

References: 1. Wataneble T. (2003) Reversible secondary pseudohypoaldosteronism. Pediatr Nephrol 18(5): 486. 2. Sugawara M, Lebron BA, Calabria R. (1989) Pseudohypoaldosteronism following resection of ileum and colon. Nephro.51(4): 567-8.

Volume 94

59th Annual ESPE (ESPE 2021 Online)

22 Sep 2021 - 26 Sep 2021

European Society for Paediatric Endocrinology 

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