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2016 ; 29
(ä): 230-9
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English Wikipedia
Treatment of Peripheral Precocious Puberty
#MMPMID26680582
Schoelwer M
; Eugster EA
Endocr Dev
2016[]; 29
(ä): 230-9
PMID26680582
show ga
There are many etiologies of peripheral precocious puberty (PPP) with diverse
manifestations resulting from exposure to androgens, estrogens, or both. The
clinical presentation depends on the underlying process and may be acute or
gradual. The primary goals of therapy are to halt pubertal development and
restore sex steroids to prepubertal values. Attenuation of linear growth velocity
and rate of skeletal maturation in order to maximize height potential are
additional considerations for many patients. McCune-Albright syndrome (MAS) and
familial male-limited precocious puberty (FMPP) represent rare causes of PPP that
arise from activating mutations in GNAS1 and the LH receptor gene, respectively.
Several different therapeutic approaches have been investigated for both
conditions with variable success. Experience to date suggests that the ideal
therapy for precocious puberty secondary to MAS in girls remains elusive. In
contrast, while the number of treated patients remains small, several successful
therapeutic options for FMPP are available.