Dental Management in a Patient with Glanzmann’s Thrombasthenia : A Case Report
1
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
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.
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.
참고문헌 (15)
- Bellucci S, "Molecular basis of Glanzmann’s thrombasthenia and current strategics in treatment", Blood Rev, 16, pp.193~202 (2002)
- Caen JP, "Congenital bleeding disorders with long bleeding time and normal platelet count. I. Glanzmann’s thrombasthenia : Report of 15 patients", Am J Med, 41, pp.4~26 (1966)
- Ranjith A, "Glanzmann thrombasthenia : A rare hematological disorder with oral manifestations : A case report", J Contemp Dent Pract, 9, pp.107~113 (2008)
- George JN, "Glanzmann's thrombasthenia : The spectrum of clinical disease", Blood, 75, pp.1383~1395 (1990)
- Diana NM, "Dental considerations in the management of Glanzmann’s thrombasthenia", Int J Child Ped Dent, 3, pp.51~56 (2010)
- Brown DL, "Congenital bleeding disorders", Curr Probl Pediatr Adolesc Health Care, 35, pp.38~62 (2005)
- Toygar HU, "Excessive gingival bleeding in two patients with Glanzmann thrombasthenia", J Periodontol, 78, pp.1154~1158 (2007)
- Nurden AT, "Glanzmann thrombasthenia", Orphanet J Rare Dis, 1, pp.10~ (2006)
- Bellucci S, "Complete correction of Glanzmann’s thrombasthenia by allogeneic bone marrow transplantation", Br J Haematol, 59, pp.635~641 (1985)
- Doh YJ, "A case of Glanzmann's thrombasthenia diagnosed with flow cytometry and SDSPAGE analysis of platelet membrane glycoprotein", Korean J Hematol, 27, pp.443~451 (1992)
- 박정복, "혈소판 수혈에 내성을 보인 글란츠만씨 혈소판무력증을 가진 환자의 정형외과 수술 시 전신마취 경험", Korean Journal of Anesthesiology, 57(4), pp.507~510 (2009), 대한마취통증의학회
- Murphy MF, "Immunological aspects of platelet transfusions", Br J Haematol, 60, pp.409~414 (1985)
- Seligsohn U, "A Glanzmann's thrombasthenia cluster among Iraqi Jews in Israel", Thromb Haemost, 52, pp.230~231 (1984)
- Williams WJ, "Hematology", pp.1368~1376 (1977), McGraw-Hill
- Mehta BC, "Glanzmann’s thrombasthenia", J Postgrad Med, 26, pp.210~212 (1980)