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Pseudohypoaldosteronism is a rare condition resulting from renal tubular unresponsiveness to aldosterone. It generally presents in the neonatal period with salt wasting, hyperkalaemia and hypertension.

It may inherited in autosomal dominant and recessive forms.

This condition should be considered as a differential in the diagnosis of congenital adrenal hyperplasia, especially in boys where the additional clue of ambiguous genitalia for that diagnosis is not available.

Last reviewed 01/2018