title: Severe periodontal destruction in alpha-mannosidosis: a case series.
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.
authors.affiliation: Department of Restorative Dentistry, Croydon University Hospital, London, UK. jagdip82@hotmail.com
authors.forename: Jagdip S
authors.initials: JS
authors.lastname: Kalsi
authors.forename: Gita
authors.initials: G
authors.lastname: Auplish
authors.forename: Adele R
authors.initials: AR
authors.lastname: Johnson
authors.forename: Ulpee R
authors.initials: UR
authors.lastname: Darbar
mesh_terms: D000293:Adolescent; D016976:Aggregatibacter actinomycetemcomitans / Q000302:isolation & purification; D016301:Alveolar Bone Loss / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D002648:Child; D003773:Dental Plaque / Q000382:microbiology; D005260:Female; D019214:Gingival Overgrowth / Q000209:etiology; D006801:Humans; D008297:Male; D010518:Periodontitis / Q000209:etiology* / Q000382:microbiology / Q000473:pathology / Q000628:therapy; D035781:Siblings; D008363:alpha-Mannosidosis / Q000150:complications*