ORAL AND MAXILLOFACIAL MANIFESTATIONS OF LARON SYNDROME

Shin, Cha-Uk; Kim, Young-Jae; Kim, Young-Jae; Jang, Ki-Taek; Lee, Sang-Hoon; Hahn, Se-Hyun; Kim, Chong-Chul

Journal of the Korean Academy of Pediatric Dentistry 2009;36(1):139-144.
Published online February 28, 2009.

ORAL AND MAXILLOFACIAL MANIFESTATIONS OF LARON SYNDROME

Cha-Uk Shin, Young-Jae Kim, Young-Jae Kim, Ki-Taek Jang, Sang-Hoon Lee, Se-Hyun Hahn, Chong-Chul Kim

Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University

라론 증후군의 구강 악안면 증상

신차욱, 김영재, 김정욱, 장기택, 이상훈, 한세현, 김종철

서울대학교 치과대학 소아치과학교실

Correspondence: Chong-Chul Kim, Tel: 02-2072-3395, Email: kimcc@snu.ac.kr

Received: 6 October 2008 • Accepted: 10 December 2008

Abstract

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.

Key Words: Growth hormone, Growth failure, Microdontia, Bulbous crown, Spiculate root