Precocious puberty

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Classification according to ICD-10
E22.8 Other overactive pituitary gland
- Centrally triggered precocious puberty
E25 Adrenogenital disorders
- Heterosexual pseudopubertas praecox feminina
- Isosexual pseudopubertas praecox masculina
E30.1 Premature puberty ( pubertas praecox )
ICD-10 online (WHO version 2019)

As precocious puberty (from Latin puberty "maturity" and praecox "prematurely") is a rare disease with development of secondary sexual characteristics referred to in males before the ninth and in females before the age of eight. If left untreated, this premature sexual development is associated with short stature, caused by the premature closure of the growth plates of the bones. Girls are 5 times more likely to be affected than boys. Delayed development is called puberty tarda .

Forms and causes

There are several forms:

  • The cerebrally caused real puberty praecox ( pubertas praecox vera ) comes about through an overproduction of regulating hormones of the sex hormone secretion. The reasons for this often cannot be clearly identified, especially in girls. Occasionally, pathological processes (for example tumors of the pituitary gland or hypothalamus ) or reactive or ectopic hormone production (for example in hypothyroidism ) lead to precocious puberty.
  • The genetically -induced precocious puberty occurs in various syndromes on, such as in familial gonadotropin-independent male-limited precocious puberty (FMPP, English familial male-limited precocious puberty ), by a mutation of the LHCGR gene on the chromosome 2 is caused . In addition to the genes for kisspeptin-1 (KISS1) and its receptor, the MKRN3 gene has now also been identified as the cause of the early onset of puberty.
  • As pseudopuberty praecox is called the overproduction of sex hormones without a detectable increase in the regulation of hormone gonadotropin , for example in congenital adrenal hyperplasia .
  • The clinical picture of precocious puberty can also be triggered by primary hepatocellular carcinoma , hence the outdated term hepatogenital syndrome .

clinic

The development of the organism in precocious puberty takes place in the normal sequence ( thelarche , pubarche , growth spurt , menarche ), but too early. Since the skeleton also matures prematurely, the epiphyseal plates close prematurely with short stature as a result.

Diagnosis

To confirm the findings after a clinical examination and family history, the skeletal age determination is the first option.

Sonographs are performed to search for rare hormone-forming tumors .

In addition, estrogens , prolactin and gonadotropins ( FSH , LH ) are determined in the blood.

therapy

Tumors require surgical or radiotherapy or chemotherapy treatment. In addition, the chronic (i.e. non- pulsatile ) administration of GnRH analogues allows the pituitary gland to function at rest, which leads to a immobilization of the endocrine gonadal function through reduced gonadotropin secretion .

literature

  • Lois Jovanovic, Genell J. Subak-Sharpe: Hormones. The medical manual for women. (Original edition: Hormones. The Woman's Answerbook. Atheneum, New York 1987) From the American by Margaret Auer, Kabel, Hamburg 1989, ISBN 3-8225-0100-X , pp. 79 ff. And 385.
  • Ludwig Weissbecker: Diseases of the pituitary-diencephalon system. In: Ludwig Heilmeyer (ed.): Textbook of internal medicine. Springer-Verlag, Berlin / Göttingen / Heidelberg 1955; 2nd edition, ibid. 1961, pp. 1008-1013, here: p. 1013 ( Pubertas praecox ).

Individual evidence

  1. ↑ National Center for Biotechnology: Genome Data Viewer (GDV ): LHCGR luteinizing hormone / choriogonadotropin receptor / Homo sapiens (human), details on the LHCGR gene (English)
  2. ↑ Patrick Burleigh. A 4-Year-Old Trapped in a Teenager's Body “I was all of the things people are when they're 14 or 15” - except a decade younger. On: thecut.com on January 16, 2019; accessed on September 10, 2019.
  3. ↑ Ana Paula Abreu, Andrew Dauber, Delanie B. Macedo, Sekoni D. Noel, Vinicius N. Brito, John C. Gill, Priscilla Cukier, Iain R. Thompson, Victor M. Navarro, Priscila C. Gagliardi, T nia Rodrigues, Cristiane Kochi, Carlos Alberto Longui, Dominique Beckers, Francis de Zegher, Luciana R. Montenegro, Berenice B. Mendonca, Rona S. Carroll, Joel N. Hirschhorn, Ana Claudia Latronico, Ursula B. Kaiser: Central Precocious Puberty Caused by Mutations in the Imprinted Gene. In: New England Journal of Medicine. 2013, pp. 2467-2475, doi: 10.1056 / NEJMoa1302160 .
  4. ↑ W. Kosenow, G. Feil, H. von Törne, JR Bierich, M. Apostolakis: Sexual precociousness through primary liver carcinoma: "Hepatogenital syndrome". In: Monthly for Pediatrics. Volume 115, Number 1, January 1967, pp. 37-46, ISSN  0026-9298 . PMID 4291692 .
  5. ^ B. Leiber: The clinical syndromes. Syndromes, sequences and symptom complexes. Edited by G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger, 7th edition. Urban & Schwarzenberg 1990, ISBN 3-541-01727-9 .

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