Although many diseases can present with hypo- natremia, hyperkalemia, and metabolic acidosis ininfancy, congenital adrenal hyperplasia (CAH) remainsthe most common cause. The other less incriminatedcauses are pseudohypoaldosteronism (PHA), transientmineralocorticoid deficiency of infancy, and cortisoldeficiency following adrenal apoplexy.1 PHA has beenclassified into 2 types. PHA type 1 can be either primary or secondary. Primary PHA 1 manifests as eitheran autosomal recessive generalized form (mutation inepithelial sodium channels found in distal nephron,distal colon, salivary glands, and so forth) or an autosomal dominant form (mutation in mineralocorticoidreceptor) with only the salt-losing syndrome. PHAtype 2 (Gordon syndrome) presents with hyperkalemiaand hypertension.
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