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Case ReportOpen Access
Pseudohypoaldosteronism : Mineralocorticoid Unresponsiveness Syndrome
Jaruratanasirikul S 
,
Janjindamai W
,
Janjindamai W We 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.
Key word
: Hyponatremia, Mineralocorticoids, Mineralocorticoid Resistance, Mineralocorticoid
Unresponsiveness, Pseudohypoaldosteronism
JARURATANASIRIKUL
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.
Key word
: Hyponatremia, Mineralocorticoids, Mineralocorticoid Resistance, Mineralocorticoid
Unresponsiveness, Pseudohypoaldosteronism
JARURATANASIRIKUL
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