Treatment of idiopathic precocious puberty.
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Advances in understanding of normal puberty have clarified many of the mechanisms underlying precocity and led in several instances to better therapy. The essential diagnostic task is to determine if the normal process has been triggered prematurely or if an abnormal process (usually inappropriate sex steroid secretion) has circumvented the normal physiology.
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An almost 3-year-old boy was evaluated for pubic hair and unilateral testicular enlargement 1 week after resection of an astrocytoma of the posterior fossa. Rather than the expected diagnosis of central precocity due to increased intracranial pressure, a diagnosis of congenital adrenal hyperplasia (21-hydroxylase deficiency) with testicular adrenal rest cells was made. The differential diagnosis, laboratory evaluation, and currently accepted medical management of congenital adrenal hyperplasia are described.