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Pediatr Dent. 2012 Sep-Oct;34(5):140-3.

Severe periodontal destruction in alpha-mannosidosis: a case series.

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  • 1Department of Restorative Dentistry, Croydon University Hospital, London, UK. jagdip82@hotmail.com

Abstract

Alpha-mannosidosis is a rare genetic lysosomal storage disorder that is inherited in an autosomal recessive pattern. Severe periodontal breakdown in alpha-mannosidosis patients has not previously been reported in the literature. The purposes of this paper are to: present the cases of 2 siblings diagnosed with alpha-mannosidosis, each of whom had varying severity of periodontal destruction; and provide an overview of alpha-mannosidosis, the possible reasons for the periodontal destruction, and the periodontal management in the 2 affected siblings. Both had preventive and nonsurgical periodontal therapy followed by a 5-year period of supportive therapy. Their pattern of bone loss was consistent with those with periodontitis as a manifestation of systemic diseases, with the extent of periodontal destruction being related to the severity of the alpha-mannosidosis. Alpha-mannosidosis patients present with social disfigurements and, to prevent tooth loss that can add to this, early periodontal diagnosis is important to optimize management and intervention.

PMID:
23211899
[PubMed - indexed for MEDLINE]
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