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Kallman’s Syndrome and Hypopituitarism

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Fundamentals of Male Infertility
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Abstract

Idiopathic congenital hypogonadotropic hypogonadism (CHH) is a rare reproductive disorder that is primarily caused by a gonadotropin-releasing hormone (GnRH) deficiency but with significant genetic heterogeneity. Clinically, this disorder is characterized by abnormally low plasma levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) in conjunction with very low concentrations of circulating azoospermia and testosterone, and of course azoospermia. In approximately 50% of cases, CHH patients also suffer from a reduced or deficient sense of smell (hyposmia or anosmia, respectively), which is then termed as Kallmann syndrome (KS) [1–3].

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References

  1. Kallmann FJ, Schoenfeld WA, Barrera SE (1944) The genetic aspects of primary eunuchoidism. Am J Ment Defic 158:203–236

    Google Scholar 

  2. Kim SH (2015) Congenital hypogonadotropic hypogonadism and Kallmann syndrome: past, present, and future. Endocrinol Metab (Seoul) 30:456–466

    Article  Google Scholar 

  3. De Morsier G, Gauthier G (1963) Olfacto-genital dysplasia. Pathol Biol (Paris) 11:1267–1272

    Google Scholar 

  4. Quaynor SD, Kim HG, Cappello EM et al (2011) The prevalence of digenic mutations in patients with normosmic hypogonadotropic hypogonadism and Kallmann syndrome. Fertil Steril 96:1424–30.e6

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  5. Sherins RJ (2000) The future of andrology in the next millennium. J Androl 21:166–167

    PubMed  CAS  Google Scholar 

  6. Xu N, Kim HG, Bhagavath B et al (2011) Nasal embryonic LHRH factor (NELF) mutations in patients with normosmic hypogonadotropic hypogonadism and Kallmann syndrome. Fertil Steril 95:1613–20.e1–7

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Park JK, Ozata M, Chorich LP et al (2004) Analysis of the PROP1 gene in a large cohort of patients with idiopathic hypogonadotropic hypogonadism. Clin Endocrinol (Oxf) 60:147–149

    Article  CAS  Google Scholar 

  8. Gromoll J, Eiholzer U, Nieschlag E, Simoni M (2000) Male hypogonadism caused by homozygous deletion of exon 10 of the luteinizing hormone (LH) receptor: differential action of human chorionic gonadotropin and LH. J Clin Endocrinol Metab 85(6):2281

    Article  CAS  PubMed  Google Scholar 

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Silber, S. (2018). Kallman’s Syndrome and Hypopituitarism. In: Fundamentals of Male Infertility. Springer, Cham. https://doi.org/10.1007/978-3-319-76523-5_16

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  • DOI: https://doi.org/10.1007/978-3-319-76523-5_16

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-76522-8

  • Online ISBN: 978-3-319-76523-5

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