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Severe gingival hyperplasia in a child with I‐cell disease
Author(s) -
Lee W.,
O'Donnell D.
Publication year - 2003
Publication title -
international journal of paediatric dentistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.183
H-Index - 62
eISSN - 1365-263X
pISSN - 0960-7439
DOI - 10.1046/j.1365-263x.2003.00419.x
Subject(s) - medicine , disease , gingival enlargement , life expectancy , pediatrics , hyperplasia , gingivectomy , dermatology , dentistry , pathology , population , environmental health
Summary. 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.