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Open AccessCase Report
Dent. J. 2018, 6(4), 53; https://doi.org/10.3390/dj6040053

Glycogen Storage Disease Ib and Severe Periodontal Destruction: A Case Report

1
Private Practice, 1047 Old Post Road, Fairfield, CT 06824, USA
2
210-11808 Saint Albert Trail, Edmonton, AB T5L 4G4, Canada
3
Stony Brook University School of Dental Medicine, South Drive, Stony Brook, NY 11794, USA
4
Department of Periodontics and Dental Hygiene, The University of Texas School of Dentistry at Houston, 7500 Cambridge Street, Suite 6427, Houston, TX 77054, USA
5
Department of Periodontology, Stony Brook University School of Dental Medicine, South Drive, Stony Brook, NY 11794, USA
*
Author to whom correspondence should be addressed.
Received: 1 August 2018 / Revised: 27 September 2018 / Accepted: 28 September 2018 / Published: 3 October 2018
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Abstract

Background: Glycogen storage diseases (GSDs) are genetic disorders that result from defects in the processing of glycogen synthesis or breakdown within muscles, liver, and other cell types. It also manifests with impaired neutrophil chemotaxis and neutropenic episodes which results in severe destruction of the supporting dental tissues, namely the periodontium. Although GSD Type Ib cannot be cured, associated symptoms and debilitating oral manifestations of the disease can be managed through collaborative medical and dental care where early detection and intervention is of key importance. This objective of the case report was to describe a child with GSD Ib and its associated oral manifestations with microbial, immunological and histological appearances. Case Presentation: An eight-year-old Hispanic male with a history of GSD type Ib presented with extensive intraoral generalized inflammation of the gingiva, ulcerations and bleeding, and intraoral radiographic evidence of bone loss. Tannerella forsythia was readily identifiable from the biofilm samples. Peripheral blood neutrophils were isolated and a deficient host response was observed by impaired neutrophil migration. Histological evaluation of the soft and hard tissues of the periodontally affected primary teeth showed unaffected dentin and cementum. Conclusions: This case illustrates the association between GSD Ib and oral manifestations of the disease. A multi-disciplinary treatment approach was developed in order to establish healthy intraoral conditions for the patient. Review of the literature identified several cases describing GSD and its clinical and radiographic oral manifestations; however, none was identified where also microbial, immunological, and histological appearances were described. View Full-Text
Keywords: glycogen storage disease; neutrophils; chemotaxis; periodontitis; oral manifestations glycogen storage disease; neutrophils; chemotaxis; periodontitis; oral manifestations
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Ma, R.; Moein Vaziri, F.; Sabino, G.J.; Sarmast, N.D.; Zove, S.M.; Iacono, V.J.; Carrion, J.A. Glycogen Storage Disease Ib and Severe Periodontal Destruction: A Case Report. Dent. J. 2018, 6, 53.

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