Gonadotropin-releasing hormone deficiency in the human (idiopathic hypogonadotropic hypogonadism and Kallmann's syndrome): pathophysiological and genetic considerations.

Abstract

I. Introduction II. Ontogeny of Normal GnRH Secretion A. Fetal life B. Neonatal and childhood periods C. Puberty D. Adulthood III. Historical Perspective IV. Clinical Presentation of GnRH Deficiency A. Congenital GnRH deficiency B. Adult-onset GnRH deficiency C. Variant or partial forms of GnRH deficiency D. Associated anomalies in KS E. Differential diagnosis V. Approaches to Studying GnRH Secretion A. Animal and in vitro studies B. Human studies VI. Patterns of GnRH Secretion in Men with GnRH Deficiency VII. Patterns of GnRH Secretion in Women with GnRH Deficiency VIII. Genetic Studies IX. X-Linked Genes Controlling GnRH Secretion A. Kallmann’s syndrome gene B. Adrenal hypoplasia congenita (AHC) gene product X. Autosomal Genes Controlling GnRH Secretion A. The genetics of autosomal inheritance B. GnRH gene C. GnRH receptor gene D. Other autosomal genes XI. Conclusion