소두증 골형성이상 원발성 왜소증 제 II 형의 치과적 특성 = Dental Characteristics of Microcephalic Osteodysplastic Primordial Dwarfism Type II

국문 초록 (Abstract)

소두증 골형성이상 원발성 왜소증 제 II 형(MOPD II)은 원발성 왜소증의 일종으로 pericentrin 유전자의 돌연변이에 의해 유발되며 상염색체 열성으로 유전된다. 이번 연구는 MOPD II의 임상적 및 방사선학적 소견과 더불어 치아의 물리화학적 특성 및 미세구조에 대한 분석을 목적으로 하였다. MOPD II 환자의 발치된 하악 제2대구치를 대상으로 하였으며 미세 전산화 단층 촬영, 주사형 전자 현미경과 에너지 분산형 분광분석법 및 비커스 미세경도 분석을 이용하여 분석하였다. MOPD II 치아는 정상 치아와 비교 시 치근 및 치수의 이형성을 보였으며 크기가 작았다. 치아의 무기질 밀도는 법랑질에서는 비슷한 값을 보였으며 상아질에서는 치근 1/2 및 치근단 부위에서 MOPD II 치아가 정상 치아보다 작게 나타났다. 미세경도의 비교 시 교두 법랑질과 치근 1/2 및 치근단 부위의 상아질에서 MOPD II 치아의 미세경도가 정상 치아보다 작게 나타났다. 이 연구에서는 MOPD II의 치과적인 특징 및 치아의 물리화학적 특성에 대하여 분석함으로써, 질환의 구강 내 소견에 대한 이해도를 높이고 치과 치료 시 주의점을 확인하여 적절한 치료를 하는데 도움이 되고자 하였다.

다국어 초록 (Multilingual Abstract)

Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) is an autosomal recessive inherited disorder form of primordial dwarfism, caused by mutations in the pericentrin gene. The purpose of the study was to examine the clinical and radiological features, physicochemical properties and microstructures of the tooth affected with MOPD II. The mandibular 2nd molar was collected from the MOPD II patient. Micro-computerized tomography, scanning electron microscopy, energy dispersive spectrometry and Vickers microhardness analysis were performed on the MOPD II and the normal control. The morphology of the MOPD II tooth appeared to have malformed pulp and root and showed a small size. The mineral density measurement showed that the MOPD II tooth had similar scores in the enamel, but lower scores in the root 1/2 and apical dentin compared to the normal control. The microhardness values were smaller in the cusp enamel, root 1/2 dentin and apical dentin of the MOPD II compared to the normal control. In this study, the dental characteristics and the physicochemical properties of a tooth affected with MOPD II were analyzed to improve understanding of the oral manifestations of the disease and to assist in proper dental treatment by identifying precautions.

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