Hypogenitalism

Classification according to ICD-10
Q50-	Congenital malformations of the ovaries, the uterine tubes and the ligaments. lata uteri
Q51-	Congenital malformations of the uterus and the cervix uteri
Q52-	Other congenital malformations of the female genital organs
Q55-	Other congenital malformations of the male genital organs
ICD-10 online (WHO version 2019)

As hypogenitalism ( give a wiki. Ὑπό Hypo = under ') refers to the underdevelopment of the genital organs, Latin Genitalhypoplasie , (infantile development of the genitalia), including mostly the secondary sexual characteristics are expected. The cause of hypogenitalism is mostly of a hormonal nature, for example as a result of an underfunction of the gonads (hypogonadism). The opposite of hypogenitalism is excessive sex development ( hypergenitalism ).

Clinical picture

In men, hypogenitalism manifests itself through a small child's penis (see also micropenis ) and a small, smooth scrotum . The prostate cannot be felt, or it can only be felt as a hazelnut-sized lump. In women, in the case of hypogenitalism, the development of the uterus and fallopian tubes is disturbed. In both sexes, the secondary sexual characteristics are incomplete.

Underdeveloped sexual organ in a man

Hypogenitalism as a symptom / in syndromes

In many cases, hypogenitalism is a symptom of various diseases and syndromes . These include:

• Adipose gigantism
• Acrocallosal Syndrome
• Alström syndrome
• congenital anorchy
• Biemond syndrome type II
• Börjeson-Forssman-Lehmann syndrome
• Carpenter syndrome
• CHARGE syndrome
• DeSanctis-Cacchione syndrome
• Down syndrome (trisomy 21)
• Dyskeratosis congenita
• idiopathic eunuchoidism
• Fanconi anemia
• Fröhlich syndrome ( dystrophia adiposogenitalis )
• Hairless woman syndrome (testicular feminization)
• Harrod syndrome
• Hand-Schüller-Christian syndrome
• Hermaphroditism
• Kallmann syndrome
• Klinefelter Syndrome
• Laurence-Moon-Biedl-Bardet syndrome
• LEOPARD syndrome
• Leschke syndrome
• MacDermot-Winter Syndrome
• Martsolf syndrome
• MEHMO syndrome
• Pituitary dwarfism
• Prader-Willi Syndrome
• Progeria-Hutchinson
• adrenogenital pseudohermaphroditism feminus
• Pseudohermaphroditism feminine, not adrenal
• Pseudohermaphroditism masculinus
• Richards-Rundle Syndrome
• Robinow syndrome
• Ruvalcaba syndrome
• Turner Syndrome
• Ullna Mamma Syndrome (Schinzel Syndrome)
• Van Bogaert-Scherer-Epstein Syndrome (cerebrotendinous xanthomatosis)
• Warburg micro syndrome
• Williams-Beuren Syndrome
• XX man

therapy

Hypogenitalism can be treated with hormone therapy . In male patients, for example, testosterone is administered in the form of injections or as a patch ( testosterone patch ). In female patients, depending on age and degree of hypogenitalism, ethinylestradiol , estradiol or chlormadinone are usually given. In the case of Klinefelter syndrome, for example, the administration of testosterone improves the quality of life of the patient and is effective against anemia , osteoporosis , muscle weakness and impotence .

Individual evidence

1. ↑ ^{a b} H. Vogl: Differential diagnosis of the medical-clinical symptoms. UTB-Verlag, 1994, ISBN 3-8252-8066-7 restricted preview in the Google book search
2. ↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
3. ^ SA Temtamy, NA Meguid: Hypogenitalism in the acrocallosal syndrome. In: Am J Med Genet , 32, 1989, pp. 301-305. PMID 2658583
4. ↑ Biemond Syndrome II.  In: Online Mendelian Inheritance in Man . (English), accessed February 19, 2010.
5. ↑ E. Tarhan et al. a .: The carpenter syndrome phenotype In: Int J Pediatr Otorhinolaryngol , 68, 2004, pp. 353-357. PMID 15129947
6. ↑ Charge Syndrome.  In: Online Mendelian Inheritance in Man . (English); Retrieved February 19, 2010.
7. ↑ A. Schinzel: Down Syndrome - Trisomy 21. Institute for Medical Genetics of the University of Zurich. 1/2007
8. ↑ Harrod Syndrome.  In: Online Mendelian Inheritance in Man . (English); Retrieved February 19, 2010.
9. ↑ Orpha.net: Prominent Glabella - Microcephaly - Hypogenitalism. Retrieved February 19, 2010
10. ↑ Martsolf Syndrome.  In: Online Mendelian Inheritance in Man . (English), accessed February 19, 2010.
11. ↑ Orpha.net: MEHMO syndrome. Retrieved February 19, 2010.
12. ↑ Robinow Syndrome, autosomal recessive.  In: Online Mendelian Inheritance in Man . (English); Retrieved February 19, 2010.
13. ↑ Ulnar-Mammary Syndrome; UMS.  In: Online Mendelian Inheritance in Man . (English), accessed February 19, 2010.
14. ^ Warburg Micro Syndrome; WARBM.  In: Online Mendelian Inheritance in Man . (English); Retrieved February 19, 2010.
15. ↑ E. Weimann and G. Horneff: Endocrinological and immunological disease pictures in pediatrics. Schattauer Verlag, 2002, ISBN 3-7945-2105-6 , pp. 44-49. limited preview in Google Book search
16. ↑ M. Zitzmann et al. a .: X-chromosome inactivation patterns and androgen receptor functionality influence phenotype and social characteristics as well as pharmacogenetics of testosterone therapy in Klinefelter patients. In: J Clin Endocrinol Metab , 89, 2004, pp. 6208-6217. PMID 15579779
17. ↑ A. Kübler et al. a .: The influence of testosterone substitution on bone mineral density in patients with Klinefelter's syndrome. In: Exp Clin Endocrinol , 100, pp. 1992, pp. 129-132. PMID 1305064
18. ↑ J. Nielsen et al. a .: Follow-up of 30 Klinefelter males treated with testosterone. In: Clin Genet 33, 1988, pp. 262-269. PMID 3359683
19. ↑ A. Bade: The Klinefelter Syndrome: Consideration in medical practice and literature. Dissertation, Westfälische Wilhelms-Universität Münster, 2007

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !