Familial male-limited precocious puberty
| Familial male-limited precocious puberty | |
|---|---|
| Classification and external resources | |
| Specialty | Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value). |
| OMIM | 176410 |
| Patient UK | Familial male-limited precocious puberty |
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
- Hypergonadism, hyperandrogenism, and precocious puberty
- Leydig cell hypoplasia (or LH insensitivity)
- Follicle-stimulating hormone insensitivity
- Gonadotropin-releasing hormone insensitivity
- Inborn errors of steroid metabolism
References
External links
- Testotoxicosis at NIH's Office of Rare Diseases
<templatestyles src="Asbox/styles.css"></templatestyles>
 |
This genetic disorder article is a stub. You can help Wikipedia by expanding it. |
