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PERIO - Periodontal Practice Today
PERIO - Periodontal Practice Today 2 (2005), No. 1     11. Apr. 2005
PERIO - Periodontal Practice Today 2 (2005), No. 1  (11.04.2005)

Page 39-48

Periodontal and Mucosal Finding in Glycogen-Storage Disease Type Ib:
A 12-Year Case Report

Hornecker, Else
Some genetic disorders are associated with recurrent inflammation of the oral mucosa and with periodontal disease. Of the various incarnations of glycogen-storage diseases (GSD), type Ib is such a disorder. It is caused by a lack of polymorphous nuclear neutrophilic granulocytes (PMN) and PMN dysfunction. The present case report relates to a patient suffering from GSD type Ib and describes the oral findings between the ages of seven and 19 years. His oral situation was characterized by frequent ulcerations in various regions of the oral mucosa, persistent gingivitis, and advanced periodontal disease, initially affecting the deciduous molars (F3 furcation involvement). A comprehensive preventive dental-care program and excellent compliance on the part of the patient could not prevent periodontal destruction of the mandibular right first permanent molar (4 mm attachment loss, F2 furcation involvement).

Keywords: GSD type Ib, glycogen-storage disease type Ib, neutropenia, neutrophilic dysfunction, immunodeficiency, periodontitis, oral complications