라론 증후군의 구강 악안면 증상
ORAL AND MAXILLOFACIAL MANIFESTATIONS OF LARON SYNDROME
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Growth hormone insensitivity(GHI) 증후군은 Laron에 의해 처음 보고된 질환으로 성장 호르몬 결핍증과 유사하게 악 악면 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 증후군 을 보이는 경우를 라론 증후군으로 분류하고 있으며, 세계적으로 약 200여 증례가 보고되고 있으나, 치의학적인 보고는 극히 드물다. 본 증례는 라론 증후군을 보이는 두 증례를 관찰한 바 작은 두개저와 상하악골, 왜소치, 구근상 치관, 침상 치근 등의 치아 형태 이상 등의 다양한 구강 악안면 이상을 보였기에 이를 보고하는 바이다.
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.
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