Idiopathic hypogonadotropic hypogonadism: Diagnosis, pathogenesis, genetics, and treatment

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4 Scopus citations


Idiopathic hypogonadotropic hypogonadism (IHH) is a heterogeneous disorder displaying a range of phenotypic, genetic, and endocrinologic features. Current evidence suggests a deficiency in the hypothalamic release of pulsatile gonadatropin-releasing hormone (GnRH) in most patients. No genetic defects in the genes for GnRH, LHβ, or FSHβ have been described, although a deletion in the steroid sulfatase gene has been reported in X-linked Kallmann's syndrome with associated ichthyosis. Treatment with GnRH administered in a pulsatile fashion has been successful in inducing steroidogenesis and gametogenesis.

Original languageEnglish (US)
Pages (from-to)111-118
Number of pages8
JournalAdolescent and Pediatric Gynecology
Issue number3
StatePublished - Jan 1 1991

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Obstetrics and Gynecology


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