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- Title
Multiple Dentigerous Cysts as a Rare Presentation of Maroteaux-Lamy Syndrome.
- Authors
Shah, Ajaz Ahmad; Hakim, Tajamul Ahmad; Farooq, Shahid; Younis, Mubashir; Kosar, Shamina; Malik, Altaf; Najar, Latief
- Abstract
Maroteaux-Lamy syndrome is one of the genetic disorders involving disturbances in mucopolysaccaride metabolism, due to deficiency of aryl sulfatase-B which leads to accumulation of dermatan sulfate in tissues and their excretion in urine. The diseases has several oral and dental manifestations, is first diagnosed on the basis of clinical findings. It is characterized by coarse facial features, normal intelligence, organomegaly, enlarged head, short neck, corneal clouding, enlarged tongue, and prominent metachromatic inclusions in leukocytes. Death is usually a result of either respiratory tract infection or cardiac disease, which is caused by the deposition of mucopolysaccharides.An 18-year-old with Maroteaux-Lamy syndrome is described in this article with multiple dentigerous cysts as the first presentation.
- Subjects
MAROTEAUX-Lamy syndrome; DENTIGEROUS cyst
- Publication
Annals of Maxillofacial Surgery, 2017, Vol 7, Issue 2, p282
- ISSN
2231-0746
- Publication type
Case Study
- DOI
10.4103/ams.ams_13_17