Restor Dent Endod.  2019 May;44(2):e14. 10.5395/rde.2019.44.e14.

Oral manifestation and root canal therapy of the patient with mucopolysaccharidosis

Affiliations
  • 1Department of Conservative Dentistry, School of Dentistry, Dental Science Research Institute, Chonnam National University, Gwangju, Korea. bnlee13@jnu.ac.kr, wmoh@chonnam.ac.kr
  • 2Department of Conservative Dentistry, School of Dentistry, Kyung Hee University, Seoul, Korea.

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.

Keyword

Mucopolysaccharidosis; Oral manifestation; Root canal therapy

MeSH Terms

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
Dermatan Sulfate
Glycosaminoglycans
Heparin
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