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KMID : 0358920090360010139
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Journal of the Korean Academy of Pedodontics
2009 Volume.36 No. 1 p.139 ~ p.144
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ORAL AND MAXILLOFACIAL MANIFESTATIONS OF LARON SYNDROME
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Shin Cha-Uk
Kim Young-Jae
Kim Jung-Wook
Jang Ki-Taeg
Lee Sang-Hoon
Hahn Se-Hyun
Kim Chong-Chul
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Abstract
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Laron syndrome was first described by Dr. Laron. Administration of exogenous growth hormone failed to stimulate insulin-like growth factor-I(IGF-I) production which was related to postnatal growth, because these patients lacked receptors in the liver for this hormone. The diagnosis of this syndrome is based on the typical features of GH resistance such as normal or elevated serum GH, low serum IGF-I, and impaired IGF-I response to hGH. Laron syndrome patients showed characteristically severe postnatal growth failure and markedly reduced adult height. This report describes the oral and maxillofacial manifestations of children associated with Laron syndrome. Children with Laron syndrome have several dental and skeletal irregularities. Relatively little is known of the direct effect of Laron syndrome on dental development. Further research should be needed.
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KEYWORD
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Growth hormone, Growth failure, Microdontia, Bulbous crown, Spiculate root
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