Oral manifestation and root canal therapy of the patient with mucopolysaccharidosis
- Author:
Ji Hye YOON
1
;
Hyo Il LEE
;
Ji Hyun JANG
;
Sung Hyeon CHOI
;
Hoon Sang CHANG
;
Yun Chan HWANG
;
In Nam HWANG
;
Bin Na LEE
;
Won Mann OH
Author Information
- Publication Type:Case Report
- Keywords: Mucopolysaccharidosis; Oral manifestation; Root canal therapy
- MeSH: Dental Pulp Cavity; Dentition; Dermatan Sulfate; Female; Gingival Hyperplasia; Glycosaminoglycans; Head; Heparin; Humans; Malocclusion; Mouth; Mucopolysaccharidoses; Neck; Oral Manifestations; Respiratory Insufficiency; Root Canal Therapy; Tongue; Young Adult
- From:Restorative Dentistry & Endodontics 2019;44(2):e14-
- CountryRepublic of Korea
- Language:English
- 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.
