뮤코다당증(Mucopolysaccharidosis)환아의 치은 증식

Abstract

Mucopolysaccharidosis (MPS) is a disorder of storage in which there is excessive accumulation of glycosaminoglycans (GAGs) from lysosomal enzyme defect. Lysosomal accumulation of GAGs eventually results in cell, tissue and organ dysfunction. This patient may manifest mental retardation and physical disorders. This clinical report presents a girl with MPS having severe gingival hyperplasia. Gingivectomy was performed under general anesthesia. The pediatric dentist must be aware of oral manifestations present in the MPS. The approach to dental management will require teamwork between the dentist and the patient's physician.