title: Severe gingival hyperplasia in a child with I-cell disease.
abstract: I-cell disease (mucolipidosis II) is a rare metabolic disorder resulting from the deficiency of a specific lysosomal enzyme, N-acetylglucosamine-1-phosphotransferease. The disease presents as a mental and motor developmental delay with oral manifestations that include severe gingival hyperplasia usually seen before one year of age. The life expectancy of children with this condition is poor, with death usually occurring around the fifth year. A case report of a 3-year-old Pakistani male, with I-cell disease, is presented. The chief dental concerns of the parents were his swollen gums and delayed tooth eruption. Supportive treatment only was initiated. Differential diagnosis for severe gingival overgrowth in young patients should take account of this rare metabolic disorder in addition to hereditary and idiopathic fibromatosis and drug associated gingival overgrowth.
authors.affiliation: Paediatric Dentistry and Orthodontics, Faculty of Dentistry, University of Hong Kong, China.
authors.forename: W
authors.initials: W
authors.lastname: Lee
authors.forename: D
authors.initials: D
authors.lastname: O'Donnell
mesh_terms: D002675:Child, Preschool; D016572:Cyclosporine / Q000009:adverse effects; D019140:Dental Care for Children; D017742:Dental Care for Chronically Ill; D005885:Gingival Hyperplasia / Q000175:diagnosis / Q000209:etiology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects; D008297:Male; D009081:Mucolipidoses / Q000150:complications*; D014078:Tooth Eruption / Q000187:drug effects
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine