Biomaterial Database

Severe periodontal destruction in alpha-mannosidosis: a case series. 2012

Jagdip S Kalsi, and Gita Auplish, and Adele R Johnson, and Ulpee R Darbar

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

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.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008363	alpha-Mannosidosis	An inborn error of metabolism marked by a defect in the lysosomal isoform of ALPHA-MANNOSIDASE activity that results in lysosomal accumulation of mannose-rich intermediate metabolites. Virtually all patients have psychomotor retardation, facial coarsening, and some degree of dysostosis multiplex. It is thought to be an autosomal recessive disorder.	Mannosidosis, alpha B, Lysosomal,Alpha-D-Mannosidosis,Alpha-Mannosidase B Deficiency,Alpha-Mannosidosis, Type I,Lysosomal Alpha B Mannosidosis,Lysosomal alpha-D-Mannosidase Deficiency,Mannosidosis, alpha B Lysosomal,alpha Mannosidase B Deficiency,alpha-Mannosidase Deficiency,Deficiencies, Lysosomal alpha-D-Mannosidase,Deficiencies, alpha-Mannosidase,Deficiency, Lysosomal alpha-D-Mannosidase,Deficiency, alpha-Mannosidase,Lysosomal alpha D Mannosidase Deficiency,Lysosomal alpha-D-Mannosidase Deficiencies,alpha Mannosidase Deficiency,alpha Mannosidosis,alpha-D-Mannosidase Deficiencies, Lysosomal,alpha-D-Mannosidase Deficiency, Lysosomal,alpha-Mannosidase Deficiencies,alpha-Mannosidoses
D010518	Periodontitis	Inflammation and loss of connective tissues supporting or surrounding the teeth. This may involve any part of the PERIODONTIUM. Periodontitis is currently classified by disease progression (CHRONIC PERIODONTITIS; AGGRESSIVE PERIODONTITIS) instead of age of onset. (From 1999 International Workshop for a Classification of Periodontal Diseases and Conditions, American Academy of Periodontology)	Pericementitis,Pericementitides,Periodontitides
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003773	Dental Plaque	A film that attaches to teeth, often causing DENTAL CARIES and GINGIVITIS. It is composed of MUCINS, secreted from salivary glands, and microorganisms.	Plaque, Dental
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D016301	Alveolar Bone Loss	Resorption or wasting of the tooth-supporting bone (ALVEOLAR PROCESS) in the MAXILLA or MANDIBLE.	Alveolar Resorption,Bone Loss, Alveolar,Bone Loss, Periodontal,Periodontal Bone Loss,Periodontal Resorption,Alveolar Bone Atrophy,Alveolar Process Atrophy,Alveolar Bone Atrophies,Alveolar Bone Losses,Alveolar Process Atrophies,Alveolar Resorptions,Bone Atrophies, Alveolar,Bone Atrophy, Alveolar,Bone Losses, Periodontal,Periodontal Bone Losses,Periodontal Resorptions,Resorption, Alveolar,Resorption, Periodontal,Resorptions, Alveolar
D016976	Aggregatibacter actinomycetemcomitans	A species of Gram-negative, facultatively anaerobic spherical or rod-shaped bacteria indigenous to dental surfaces. It is associated with PERIODONTITIS; BACTERIAL ENDOCARDITIS; and ACTINOMYCOSIS.	Actinobacillus actinomycetemcomitans,Bacterium acetinomycetum comitans,Bacterium comitans,Haemophilus actinomyceticomitans,Haemophilus actinomycetemcomitans