Biomaterial Database

Dental management of a patient with glycogen storage disease type I. 1985

S A Ralls, and E C Marshall

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.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D010510	Periodontal Diseases	Pathological processes involving the PERIODONTIUM including the gum (GINGIVA), the alveolar bone (ALVEOLAR PROCESS), the DENTAL CEMENTUM, and the PERIODONTAL LIGAMENT.	Parodontosis,Pyorrhea Alveolaris,Disease, Periodontal,Diseases, Periodontal,Parodontoses,Periodontal Disease
D003451	Cryoprotective Agents	Substances that provide protection against the harmful effects of freezing temperatures.	Cryoprotective Agent,Cryoprotective Effect,Cryoprotective Effects,Agent, Cryoprotective,Agents, Cryoprotective,Effect, Cryoprotective,Effects, Cryoprotective
D003730	Dental Care for Disabled	Dental care for the emotionally, mentally, or physically disabled patient. It does not include dental care for the chronically ill (	Dental Care for Handicapped,Dentistry for Disabled,Dentistry for Handicapped,Disabled, Dentistry for,Handicapped, Dentistry for
D005884	Gingival Hemorrhage	The flowing of blood from the marginal gingival area, particularly the sulcus, seen in such conditions as GINGIVITIS, marginal PERIODONTITIS, injury, and ASCORBIC ACID DEFICIENCY.	Hemorrhage, Gingival,Gingival Hemorrhages
D005953	Glycogen Storage Disease Type I	An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.	Glucose-6-Phosphatase Deficiency,Glucosephosphatase Deficiency,Glycogenosis 1,Hepatorenal Glycogen Storage Disease,von Gierke Disease,Deficiency, Glucosephosphatase,Gierke Disease,Gierke's Disease,Glycogen Storage Disease 1 (GSD I),von Gierke's Disease,Deficiencies, Glucose-6-Phosphatase,Deficiencies, Glucosephosphatase,Deficiency, Glucose-6-Phosphatase,Disease, Gierke,Disease, Gierke's,Disease, von Gierke,Disease, von Gierke's,Gierkes Disease,Glucose 6 Phosphatase Deficiency,Glucose-6-Phosphatase Deficiencies,Glucosephosphatase Deficiencies,von Gierkes Disease
D006474	Hemorrhagic Disorders	Spontaneous or near spontaneous bleeding caused by a defect in clotting mechanisms (BLOOD COAGULATION DISORDERS) or another abnormality causing a structural flaw in the blood vessels (HEMOSTATIC DISORDERS).	Hemorrhagic Diathesis,Diatheses, Hemorrhagic,Diathesis, Hemorrhagic,Disorder, Hemorrhagic,Disorders, Hemorrhagic,Hemorrhagic Diatheses,Hemorrhagic Disorder
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D012534	Dental Scaling	Removal of dental plaque and dental calculus from the surface of a tooth, from the surface of a tooth apical to the gingival margin accumulated in periodontal pockets, or from the surface coronal to the gingival margin.	Root Scaling,Scaling, Dental,Scaling, Root,Scaling, Subgingival,Scaling, Supragingival,Root Scalings,Scalings, Root,Subgingival Scaling,Supragingival Scaling