Toggle Main Menu Toggle Search

Open Access padlockePrints

Rarer Syndromes Characterized by Hypogonadotropic Hypogonadism

Lookup NU author(s): Professor Timothy Cheetham


Full text for this publication is not currently held within this repository. Alternative links are provided below where available.


Hypogonadotropic hypogonadism (HH) secondary to hypothalamic gonadotropin-releasing hormone deficiency is a notable feature of a number of rare syndromes, where unlike idiopathic (isolated) HH, other endocrinopathies may also be apparent. The presence of a particular spectrum of clinical features in addition to HH may suggest a particular underlying diagnosis. Placing the diagnosis of HH into that context will then have important implications in terms of management and predicting long-term functional outcome. In some instances, establishing the genetic basis of a particular syndrome or disorder has advanced the understanding of normal hypothalamo-pituitary-gonadal function (e.g. LEP deficiency, DAX-1 and CHARGE syndrome) whilst in other disorders much has still to be learnt (e.g. Bardet-Biedl and Prader-Willi syndrome). In this chapter the above syndromes, where HH is a feature in most or all affected individuals, will be discussed. Recent advances in our understanding of the pathophysiology of the HH will be highlighted and management options presented. Longer term therapy with sex steroid replacement is becoming even more important if improvements in life expectancy are to be matched by improvements in quality of life. Copyright (C) 2010 S. Karger AG, Basel

Publication metadata

Author(s): Aminzadeh M, Kim HG, Layman LC, Cheetham TD

Editor(s): Quinton, R

Series Editor(s): Grossman, AB

Publication type: Book Chapter

Publication status: Published

Book Title: Kallmann Syndrome and Hypogonadotropic Hypogonadism

Year: 2010

Volume: 39

Pages: 154-167

Print publication date: 01/01/2010

Series Title: Frontiers of Hormone Research

Publisher: S. Karger AG

Place Published: Switzerland


DOI: 10.1159/000312701

Library holdings: Search Newcastle University Library for this item

ISBN: 9783805586177