SciELO - Scientific Electronic Library Online

 
vol.37 issue4Probiotics: clinical perspectivesGiant nasal vascular leiomyoma: Transnasal endoscopic management. Report of a case and review of the literature author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand

Journal

Article

Indicators

Related links

  • On index processCited by Google
  • Have no similar articlesSimilars in SciELO
  • On index processSimilars in Google

Share


Colombia Médica

On-line version ISSN 1657-9534

Abstract

JUBIZ, William  and  CRUZ, Eduardo Antonio. Kallmann’s syndrome: A propos of a case. Colomb. Med. [online]. 2006, vol.37, n.4, pp.315-318. ISSN 1657-9534.

Kallmann’s syndrome is a type of hypogonadotropic hypogonadism which affects males and females and is characterized by eunuchoidal habitus, lack of sexual development, and anosmia, caused by a defective development of the olfactory bulbs. Cleft palate, deafness, seizures, short fourth metacarpal bones, cardiac abnormalities and gynecomastia may also occur. The mode of transmission can be autosomal dominant, autosomal recessive or X-linked. The latter is caused by mutations or deletions of the KAL gene which encodes the synthesis of anosmin-1, a protein associated with cellular adherence and antiprotease activity. The concentrations of testosterone, follicle stimulating hormone (FSH) and luteinizing hormone (LH) in serum is decreased, but they respond to the administration of the gonadotropin releasing hormone (GnRH). Infertility is treated with a combination of human chorionic gonadotropin (hCG) and human menopausal gonadotropins (hMG). Androgen deficiency is corrected with testosterone in the form of parenteral enanthate (Testoviron depot®) or undecanoate (Nebido®), patches (Androderm®, Testoderm®) or gels (Androgel®, Testim®). Sriant SR® is absorbed through the oral mucosa and it appears to be effective and convenient. An 18 year-old male who consulted for sexual retardation is presented. He could not smell. Testes and penis were small and he had an eunuchoidal habitus. Serum testosterone, follicle stimulating (FSH) and luteinizing (LH) hormones were decreased with a subnormal response to gonadotropin-releasing hormone (GnRH). He responded to testosterone therapy with the development of axillary and pubic hair, increased penis size, and deepening of the voice.

Keywords : Kallmann’s syndrome; Testosterone; Gonadotropins.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )

 

Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License