Pseudohypoaldosteronism : Mineralocorticoid Unresponsiveness Syndrome

SOMCHIT JARURATANASIRIKUL, M.D.*, WARICHA JANJINDAMAI, M.D.*

Affiliation : * Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla 90 II 0, Thailand.

AbstractWe described a 10 day old boy who presented with hyponatremia, hyperkalemia, and metabolic acidosis. Therapeutic treatment with exogenous glucocorticoid and mineralocorticoid for 8 months failed to correct the electrolyte abnormalities. The elevated serum cortisol up to 44.34 Jlg/dl along with the absence of skin hyperpigmentation excluded defects in the glucocor- ticoid pathway. Pseudohypoaldosteronism was diagnosed on the basis of hyponatremia, severe urinary salt loss despite the markedly elevated serum aldosterone up to 6,500 pg/ml (normal range 50-800 pg/ml). The patient responded very well to oral salt supplementation and cation exchange resin therapy shown by normal physical growth and normal levels of serum electrolytes.

Keywords : Hyponatremia, Mineralocorticoids, Mineralocorticoid Resistance, Mineralocorticoid Unresponsiveness, Pseudohypoaldosteronism

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