Glycogen storage disease and von Willebrand's disease implications for dental treatment: Dental management of a pediatric patient

Abstract

Glycogen storage diseases (GSD) are metabolic disorders which impair the body's ability to store glucose and utilize it later, requiring patients to take multiple daily dietary supplementation with a high carbohydrate content. Patients undergoing this treatment modality are placed at increased risk for gross dental caries and other oral abnormalities. Additionally, GSD may prolong the patient's bleeding time, which may necessitate consultation with the treating physician. In the following case, our patient required a multidisciplinary approach to address not only her dental needs, but also to coordinate the management of both her GSD and an additional complication, von Willebrand's disease. This was best achieved in a hospital setting.

Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics

“…glucose administration. 145,146 In addition, the use of a fibrinolytic inhibitor, such as ε-aminocaproic acid (Amicar), can be used as an adjunctive medication if there is mucosal-associated bleeding. For oral hemorrhage, Amicar can be given as a solution to "swish for 30 seconds and spit" at a dose of 1.25 g four times daily.…”

Decreased plasma concentration of von Willebrand factor antigen (VWF:Ag) in patients with glycogen storage disease type Ia

“…This was originally attributed to impaired platelet function (Corby et al 1974;Czapek et al 1973), but some anectodal reports have found a bleeding disorder resembling von Willebrand disease (Heyne et al 1984;Marti et al 1986). Administration of 1-deamino-8-D-arginine vasopressin (DDAVP) has been shown to correct this condition effectively and, therefore, has been recommended for GSD Ia patients prior to elective surgical procedures (Dellinger et al 1998;Marti et al 1986).…”

Oral Manifestations in Patients with Glycogen Storage Disease: A Systematic Review of the Literature

“…Three articles [8][9][10] described oral manifestations in patients with GSD Ia. Avsar [8] describes a single case of a 10-year-old boy who presented a tooth eruption delay, reduced dimensions of the craniofacial complex, taurodontism and multiple caries.…”

“…Avsar [8] describes a single case of a 10-year-old boy who presented a tooth eruption delay, reduced dimensions of the craniofacial complex, taurodontism and multiple caries. DeIIinger et al [9] reported a low level of oral hygiene of the patient with the presence of multiple caries. Duplan et al [10] evaluated dental and periodontal health in 60 patients with GSD and, among them, 25 had GSD Ia.…”