A 17-year-old female presented with diffuse muscle weakness secondary to severe hypokalemia, metabolic alkalosis, and hypertension. Additional findings included delayed puberty with primary amenorrhea. Laboratory evaluation led to a diagnosis of 17 alpha-hydroxylase/17,20-lyase (P450c17) deficiency, a form of congenital adrenal hyperplasia (CAH). Her symptoms and metabolic derangements improved with glucocorticoid replacement to suppress ACTH production and mineralocorticoid excess, although she continues to require antihypertensive therapy. Estrogen replacement was initiated due to sex hormone insufficiency. This rare disorder should be considered when evaluating patients with pubertal delay and hypertension, particularly if there is associated hypokalemia.
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