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Laron Syndrome (Primary Growth Hormone Resistance or Insensitivity): The Personal Experience 1958–2003

Laron Syndrome (Primary Growth Hormone Resistance or Insensitivity): The Personal Experience... AbstractClinical and laboratory investigations starting in 1958 of a group of dwarfed children resembling isolated GH deficiency but who had very high serum levels of GH led to the description of the syndrome of primary GH resistance or insensitivity (Laron syndrome) and subsequently to the discovery of its molecular defects residing in the GH receptor and leading to an inability of IGF-I generation. With the biosynthesis of IGF-I in 1986, therapeutic trials started. Continuously more and more patients are being diagnosed in many parts of the world with a variety of molecular defects. This syndrome proved to be a unique model that enables the study of the consequences of GH receptor defects, the physiopathology of GH-IGF-I disruption, and comparison of the GH-independent IGF-I effects. This review presents the personal experience gained from the study follow-up and treatment of the 60 patients followed up for many years in the Israeli cohort. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Clinical Endocrinology and Metabolism Oxford University Press

Laron Syndrome (Primary Growth Hormone Resistance or Insensitivity): The Personal Experience 1958–2003

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References (111)

Publisher
Oxford University Press
Copyright
Copyright © 2004 by The Endocrine Society
ISSN
0021-972X
eISSN
1945-7197
DOI
10.1210/jc.2003-031033
pmid
15001582
Publisher site
See Article on Publisher Site

Abstract

AbstractClinical and laboratory investigations starting in 1958 of a group of dwarfed children resembling isolated GH deficiency but who had very high serum levels of GH led to the description of the syndrome of primary GH resistance or insensitivity (Laron syndrome) and subsequently to the discovery of its molecular defects residing in the GH receptor and leading to an inability of IGF-I generation. With the biosynthesis of IGF-I in 1986, therapeutic trials started. Continuously more and more patients are being diagnosed in many parts of the world with a variety of molecular defects. This syndrome proved to be a unique model that enables the study of the consequences of GH receptor defects, the physiopathology of GH-IGF-I disruption, and comparison of the GH-independent IGF-I effects. This review presents the personal experience gained from the study follow-up and treatment of the 60 patients followed up for many years in the Israeli cohort.

Journal

Journal of Clinical Endocrinology and MetabolismOxford University Press

Published: Mar 1, 2004

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