Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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ORAL AND MAXILLOFACIAL MANIFESTATIONS OF LARON SYNDROME

라론 증후군의 구강 악안면 증상

  • Shin, Cha-Uk (Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University) ;
  • Kim, Young-Jae (Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University) ;
  • Kim, Jung-Wook (Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University) ;
  • Jang, Ki-Taek (Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University) ;
  • Lee, Sang-Hoon (Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University) ;
  • Hahn, Se-Hyun (Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University) ;
  • Kim, Chong-Chul (Department of Pediatric Dentistry, College of Dentistry and Dental Research Institute, Seoul National University)
  • 신차욱 (서울대학교 치과대학 소아치과학교실) ;
  • 김영재 (서울대학교 치과대학 소아치과학교실) ;
  • 김정욱 (서울대학교 치과대학 소아치과학교실) ;
  • 장기택 (서울대학교 치과대학 소아치과학교실) ;
  • 이상훈 (서울대학교 치과대학 소아치과학교실) ;
  • 한세현 (서울대학교 치과대학 소아치과학교실) ;
  • 김종철 (서울대학교 치과대학 소아치과학교실)
  • Published : 2009.02.27
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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.

Growth hormone insensitivity(GHI) 증후군은 Laron에 의해 처음 보고된 질환으로 성장 호르몬 결핍증과 유사하게 악악면 양상과 심각한 성장 지연을 보이지만, 이와는 달리 혈중 성장호르몬은 정상이거나 증가되어 있고 혈중 Insulin like growth factor-I(IGF-I)과 성장 호르몬 결합 단백질은 감소되어 있는 특징을 보인다. 이 중에서 원발성으로 GHI 증후군을 보이는 경우를 라론 증후군으로 분류하고 있으며, 세계적으로 약 200여 증례가 보고되고 있으나, 치의학적인 보고는 극히드물다. 본 증례는 라론 증후군을 보이는 두 증례를 관찰한 바 작은 두개저와 상하악골, 왜소치, 구근상 치관, 침상 치근 등의 치아형태 이상 등의 다양한 구강 악안면 이상을 보였기에 이를 보고하는 바이다.

Keywords

References

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