title: Oral manifestations in glycogen storage disease type 1b.
abstract: Glycogen storage disease type 1b is a rare metabolic disorder which affects the transport system of glucose-6-phosphatase metabolism. As a result, hepatomegaly, failure to thrive, renal dysfunction and recurrent infections occur in affected patients. In this paper, the oral complications in three children with glycogen storage disease type 1b are discussed. Oral ulcers were a common finding, probably due to severe neutropenia and impaired neutrophil migration which characterises the onset of this rare disorder.
authors.affiliation: Department of Pediatrics, Medical School, University of Athens, Greece.
authors.forename: Y
authors.initials: Y
authors.lastname: Salapata
authors.forename: G
authors.initials: G
authors.lastname: Laskaris
authors.forename: E
authors.initials: E
authors.lastname: Drogari
authors.forename: E
authors.initials: E
authors.lastname: Harokopos
authors.forename: J
authors.initials: J
authors.lastname: Messaritakis
mesh_terms: D002675:Child, Preschool; D005260:Female; D005882:Gingival Diseases / Q000473:pathology; D005929:Glossitis, Benign Migratory / Q000473:pathology; D005953:Glycogen Storage Disease Type I / Q000473:pathology*; D006529:Hepatomegaly / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008047:Lip Diseases / Q000473:pathology; D008297:Male; D009059:Mouth Diseases / Q000473:pathology*; D010159:Palate / Q000473:pathology; D014456:Ulcer / Q000473:pathology