Oral manifestation and root canal therapy of the patient with mucopolysaccharidosis
윤지혜, 이효일, 장지현, 최성현, 장훈상, 황윤찬, 황인남, 이빈나, 오원만,
소속 상세정보
윤지혜 ( Yoon Ji-Hye ) - Chonnam National University School of Dentistry Department of Conservative Dentistry
이효일 ( Lee Hyo-Il ) - Chonnam National University School of Dentistry Department of Conservative Dentistry
장지현 ( Jang Ji-Hyun ) - Kyung Hee University School of Dentistry Department of Conservative Dentistry
최성현 ( Choi Sung-Hyeon ) - Chonnam National University School of Dentistry Department of Conservative Dentistry
장훈상 ( Chang Hoon-Sang ) - Chonnam National University School of Dentistry Department of Conservative Dentistry
황윤찬 ( Hwang Yun-Chan ) - Chonnam National University School of Dentistry Department of Conservative Dentistry
황인남 ( Hwang In-Nam ) - Chonnam National University School of Dentistry Department of Conservative Dentistry
이빈나 ( Lee Bin-Na ) - Chonnam National University School of Dentistry Department of Conservative Dentistry
오원만 ( Oh Won-Mann ) - Chonnam National University School of Dentistry Department of Conservative Dentistry
Abstract
Mucopolysaccharidosis (MPS) is an inherited metabolic disorder caused by a deficiency in enzymes that participate in the degradation of glycosaminoglycans (GAGs) such as heparin sulfate and dermatan sulfate. Left untreated, patients show progressive mental and physical deterioration due to deposition of GAGs in organs. Death often occurs due to cardiac or respiratory failure before patients reach their early twenties. MPS has several oral and dental manifestations. An enlarged head, short neck, and open mouth associated with a large tongue are major characteristics of MPS patients. Dental complications can be severe, including unerupted dentition, dentigerous cyst-like follicles, malocclusions, condylar defects, and gingival hyperplasia. A 21-year-old female patient with MPS was described in this article, with special emphasis on oral manifestations and dental treatment.
키워드
Mucopolysaccharidosis; Oral manifestation; Root canal therapy
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