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Year : 2017  |  Volume : 7  |  Issue : 2  |  Page : 282-286

Multiple dentigerous cysts as a rare presentation of maroteaux– Lamy syndrome

Department of Oral and Maxillofacial Surgery, Government Dental College, Srinagar, Jammu and Kashmir, India

Correspondence Address:
Tajamul Ahmad Hakim
Room No. 202, Department of Oral and Maxillofacial Surgery, Government Dental College, Srinagar - 190 001, Jammu and Kashmir
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DOI: 10.4103/ams.ams_13_17

PMID: 29264299

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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.

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