J Korean Acad Pediatr Dent.  2020 Aug;47(3):352-358. 10.5933/JKAPD.2020.47.3.352.

Dental Management in a Patient with Glanzmann’s Thrombasthenia : A Case Report

Affiliations
  • 1Department of Pediatric Dentistry, Jukjeon Dental Hospital, Dankook University, Yongin, Korea
  • 2Department of Pediatric Dentistry, College of Dentistry, Dankook University, Cheonan, Korea

Abstract

Glanzmann’s thrombasthenia (GT) is a rare, autosomal recessive inherited congenital disorder, characterized by impaired blood coagulation due to platelet dysfunction. It was first reported by the pediatrician Glanzmann in 1918. GT affects both males and females, and it is more common in regions of the Middle East, India, and France, where intermarriage is common. It has an incidence of about 1 in 1,000,000 people. In South Korea, according to the Division of Rare Diseases, Korea Centers for Disease Control and Prevention, around 200 cases have been reported in 2018. Clinical symptoms include petechia, ecchymosis, epistaxis, and gingival bleeding. The spontaneous loss of deciduous teeth can result in excessive bleeding with that blood transfusion should be considered. Preventing hemorrhages and hemostasis are most important factors in dental treatment. Local bleeding can be controlled by compression, but platelet transfusion can be required by prolonged bleeding. Pediatric dentists can minimize the gingival bleeding by control of the oral hygiene to prevent gingivitis and dental caries. The importance of oral hygiene and periodic recall check-up should be emphasized. During dental treatment, the examination and the treatment plan of patient should be modified to prevention of hemorrhages carefully. A 6-year-old girl with GT was referred for the treatment of dental caries, and resin restoration was performed under nitrous oxide inhalation sedation. After treatment, compression was required for the bleeding control.

Keyword

Glanzmann thrombasthenia; Bleeding control; Dental management
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