Dental management of a patient with glycogen storage disease type I

Patients with glycogen storage disease type I (GSD I) have varied medical problems, including a bleeding diathesis characterized by a prolonged bleeding time. A case of a patient with GSD I having a history of medical problems, some of which were related to oral and dental bleeding, was presented. The patient was cariesfree and had generalized moderate to severe periodontal breakdown. A treatment regimen in a hospital setting, using cryoprecipitate and aminocaproic acid, permitted dental care and control of oral bleeding and associated complications.