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Oral health of a child being treated for subtype I Gaucher's disease
Author(s) -
Sousa Lucas Lopes Araújo,
Rocha Moema Modesto Fonseca,
Mendes Regina Ferraz,
Junior Raimundo Rosendo Prado
Publication year - 2013
Publication title -
special care in dentistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.328
H-Index - 41
eISSN - 1754-4505
pISSN - 0275-1879
DOI - 10.1111/scd.12026
Subject(s) - medicine , disease , enzyme replacement therapy , oral cavity , oral health , pediatrics , family medicine , dentistry
ABSTRACT Gaucher disease (GD) is an autosomal recessive disorder characterized by the absence of glucosylceramidase. The accumulation of substrates of this enzyme in the cytoplasm of cells of the phagocytary system causes skeletal and hematologic disorders, and has oral repercussions. This report describes the findings of the oral cavity of an 8‐year‐old diagnosed with subtype I GD who has been receiving enzyme replacement therapy for the past 6 years without interruption. The report highlights that the child had none of the most common oral disease signs and symptoms, which demonstrates the importance of early diagnosis and continuous treatment. The article also emphasizes that it is important for dentists to recognize oral manifestations of GD (in order to assist in early diagnosis) and understand how to treat the likely oral health conditions of GD patients.