| 词条 | Familial male-limited precocious puberty |
| 释义 |
| name = Familial male-limited precocious puberty | synonyms = | image = Autosomal dominant - en.svg | caption = Male-limited precocious puberty has an autosomal dominant pattern of inheritance. However, only males are affected; females with the mutant gene are not affected. | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }} Familial male-limited precocious puberty, often abbreviated as FMPP, also known as familial sexual precocity or gonadotropin-independent testotoxicosis,[1] is a form of gonadotropin-independent precocious puberty in which boys experience early onset and progression of puberty.[2] Signs of puberty can develop as early as an age of 1 year. The spinal length in boys may be short due to a rapid advance in epiphyseal maturation. It is an autosomal dominant[1] condition with a mutation of the luteinizing hormone (LH) receptor. Treatment is with drugs that suppress gonadal steroidogenesis, such as cyproterone acetate, ketoconazole, spironolactone, and testolactone.[3] Alternatively, the combination of the androgen receptor antagonist bicalutamide and the aromatase inhibitor anastrozole may be used.[4] See also
References1. ^1 {{OMIM|176410}} 2. ^{{cite journal |vauthors=Traggiai C, Stanhope R | title=Disorders of pubertal development | journal=Best Pract Res Clin Obstet Gynaecol | year=2003 | pages=41–56 | volume=17 | issue=1 | pmid=12758225 | doi=10.1053/ybeog.2003.0360}} 3. ^{{cite journal |vauthors=Reiter EO, Norjavaara E | title=Testotoxicosis: current viewpoint | journal=Pediatr Endocrinol Rev | year=2005 | pages=77–86 | volume=3 | issue=2 | pmid=16361981}} 4. ^{{cite journal | vauthors = Kreher NC, Pescovitz OH, Delameter P, Tiulpakov A, Hochberg Z | title = Treatment of familial male-limited precocious puberty with bicalutamide and anastrozole | journal = The Journal of Pediatrics | volume = 149 | issue = 3 | pages = 416–20 | date = Sep 2006 | pmid = 16939760 | doi = 10.1016/j.jpeds.2006.04.027 }} 5. ^{{cite web |last1=Burleigh |first1=Patrick |title=A 4-Year-Old Trapped in a Teenager’s Body |url=https://www.thecut.com/2019/01/precocious-puberty-patrick-burleigh.html#_ga=2.29705599.1186823492.1548776260-1747401009.1541692780 |website=thecut.com |publisher=New York Magazine |accessdate=6 February 2019}} External links{{Medical resources| DiseasesDB = | ICD10 = E30.1 | ICD9 = | ICDO = | OMIM = 176410 | MedlinePlus = | eMedicineSubj = | eMedicineTopic = | MeshID = C536961 | Orphanet = 3000 }}
5 : Autosomal dominant disorders|Congenital disorders|Gonadotropin-releasing hormone and gonadotropins|Rare diseases|Cell surface receptor deficiencies |
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