title: Oral manifestations and dental management of patients with hereditary angioedema.
abstract: Hereditary angioedema (HAE) is a genetic disorder in which affected individuals develop extensive, spontaneous angioedema of the extremities, gastrointestinal tract, and oropharynx. Dental treatment of unmedicated patients with HAE can trigger life-threatening pharyngeal edema. Previously, it was demonstrated that the administration of fresh frozen plasma (FFP) before surgery prevented angioedema attacks in 6 patients undergoing dental extractions. The present study examines the long term effectiveness of FFP in preventing angioedema from developing in 53 patients with HAE undergoing all types of dental treatment over a ten-year period. Only 3 of 45 patients (6.7%) covered with FFP had a minor angioedema attack after dental therapy in 10 yr. No attacks of moderate or severe swelling were seen. Attacks occurred independently of the disease activity of the patient and the trauma of the dental procedure. The use of fresh frozen plasma is effective in preventing attacks of angioedema in HAE patients undergoing all types of dental procedures.
authors.affiliation: Clinical Investigations and Patient Care Branch, National Institute of Dental Research, National Institutes of Health, Bethesda, MD 20892.
authors.forename: J C
authors.initials: JC
authors.lastname: Atkinson
authors.forename: M M
authors.initials: MM
authors.lastname: Frank
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000799:Angioedema / Q000150:complications / Q000235:genetics* / Q000517:prevention & control; D001803:Blood Transfusion; D002648:Child; D003730:Dental Care for Persons with Disabilities; D003777:Dental Prophylaxis / Q000009:adverse effects; D003793:Dental Restoration, Permanent / Q000009:adverse effects; D005260:Female; D006801:Humans; D007570:Jaw Cysts / Q000150:complications / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009059:Mouth Diseases / Q000150:complications* / Q000628:therapy; D010949:Plasma; D011183:Postoperative Complications; D014081:Tooth Extraction / Q000009:adverse effects