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Detecting Glycogen in Peripheral Blood Mononuclear Cells with Periodic Acid Schiff Staining
Published on: December 23, 2014
Glycogen Storage Disease type Ib: a paediatric case report
A Bartoli1, M Bossù, G Sfasciotti
1Department of Pediatric Dentistry, University of Rome La Sapienza, Italy. adriano.bartoli@libero.it
European Journal of Paediatric Dentistry
|December 16, 2006
Summary
This study highlights oral health challenges in pediatric Glycogen Storage Disease (GSD) type Ib, detailing unique enamel hypomineralization and oral ulcers. Early dental intervention and management improve quality of life for these patients.
Area of Science:
- Pediatric Dentistry
- Metabolic Disorders
- Oral Medicine
Background:
- Glycogen Storage Disease (GSD) type Ib presents unique challenges in pediatric oral health.
- Increased susceptibility to infections, caries, and bleeding necessitates specialized dental management.
Observation:
- A 9-year-old boy with GSD type Ib exhibited severe oral ulcers and enamel hypomineralization.
- Scanning Electron Microscopy revealed dark spots indicative of hypomineralization on deciduous tooth enamel.
Findings:
- Oral ulcers may stem from impaired neutrophil-related immune function in GSD type Ib.
- Enamel hypomineralization represents a novel finding linked to increased dental caries risk in GSD type Ib.
Implications:
- Multidisciplinary management, including pharmacologic, hygienic, dietary, and surgical measures, is crucial for oral health.
- Dentists must be prepared for surgical complications, with hospital settings offering enhanced safety.
- Improved oral care significantly enhances the quality of life for children with GSD type Ib.
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