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Premature adrenarche is the most common cause of the early appearance of pubic hair ("premature pubarche") in childhood. In a large proportion of children it seems to be a variation of normal development requiring no treatment. However, there are three clinical issues related to premature adrenarche.
Congenital adrenal hyperplasia due to 3β-hydroxysteroid dehydrogenase deficiency is an uncommon form of congenital adrenal hyperplasia (CAH) resulting from a mutation in the gene for one of the key enzymes in cortisol synthesis by the adrenal gland, 3β-hydroxysteroid dehydrogenase (3β-HSD) type II ().
Late onset congenital adrenal hyperplasia (LOCAH), also known as nonclassic congenital adrenal hyperplasia (NCCAH or NCAH), is a milder form of congenital adrenal hyperplasia (CAH), [1] a group of autosomal recessive disorders characterized by impaired cortisol synthesis that leads to variable degrees of postnatal androgen excess.
Gonadarche (/ ˌ ɡ oʊ n ə ˈ d ɑːr k i /) refers to the earliest gonadal (reproductive gland) changes of puberty. [1] In response to pituitary gonadotropins, the ovaries in females and the testes in males begin to grow and increase the production of the sex steroids, especially estradiol and testosterone. [2]
However, premature pubarche may also arise independently of adrenarche. Premature pubarche is a subset of precocious puberty which divide into 1) true precocious puberty that includes complete and central precocious puberty and 2) incomplete puberty which has 3 subsets: premature thelacrche, premature pubarche and isolated menarche. [13]
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Adrenarche is independent of ACTH or gonadotropins and correlates with a progressive thickening of the zona reticularis layer of the cortex. Functionally, adrenarche provides a source of androgens for the development of axillary and pubic hair before the beginning of puberty.
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