Familial male-limited precocious puberty
Last updated 18 Sept 2024
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Familial male-limited precocious puberty is a condition that causes early sexual maturity in males; females are not affected. Boys with this disorder begin exhibiting the signs of puberty in early childhood, between the ages of 2 and 5. Signs of male puberty include a deepening voice, acne, increased body hair, underarm odor, growth of the penis and testes, and spontaneous erections. Changes in behaviour, such as increased aggression and early interest in sex, may also occur. Without treatment, affected boys grow quickly at first, but they stop growing earlier than usual. As a result, they tend to be shorter in adulthood compared with other members of their family.
Description
Familial male-limited precocious puberty is a condition that causes early sexual maturity in males; females are not affected. Boys with this disorder begin exhibiting the signs of puberty in early childhood, between the ages of 2 and 5. Signs of male puberty include a deepening voice, acne, increased body hair, underarm odor, growth of the penis and testes, and spontaneous erections. Changes in behaviour, such as increased aggression and early interest in sex, may also occur. Without treatment, affected boys grow quickly at first, but they stop growing earlier than usual. As a result, they tend to be shorter in adulthood compared with other members of their family.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal dominant. More than one pattern can apply in different cases.
Related genes
Other names
Familial gonadotrophin-independent sexual precocity
GIPP
Gonadotrophin-independent precocious puberty
Precocious pseudopuberty
Pubertas praecox
Testotoxicosis
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Further reading and references
- Familial male-limited precocious puberty; MedlinePlus, National Library of Medicine.
- Brito VN, Latronico AC, Arnhold IJ, Mendonca BB. Update on the etiology, diagnosis and therapeutic management of sexual precocity. Arq Bras Endocrinol Metabol. 2008 Feb;52(1):18-31. doi: 10.1590/s0004-27302008000100005.
- Chan WY. Disorders of sexual development caused by luteinizing hormone receptor mutations. Beijing Da Xue Xue Bao Yi Xue Ban. 2005 Feb 18;37(1):32-8.
- Soriano-Guillen L, Mitchell V, Carel JC, Barbet P, Roger M, Lahlou N. Activating mutations in the luteinizing hormone receptor gene: a human model of non-follicle-stimulating hormone-dependent inhibin production and germ cell maturation. J Clin Endocrinol Metab. 2006 Aug;91(8):3041-7. doi: 10.1210/jc.2005-2564. Epub 2006 May 9.
- Themmen AP, Verhoef-Post M. LH receptor defects. Semin Reprod Med. 2002 Aug;20(3):199-204. doi: 10.1055/s-2002-35384.
- Traggiai C, Stanhope R. Disorders of pubertal development. Best Pract Res Clin Obstet Gynaecol. 2003 Feb;17(1):41-56. doi: 10.1053/ybeog.2003.0360.
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