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- Title
Dental management of a rare hematologic disorder - Glanzmann's thrombasthenia: A case report.
- Authors
Pires, Vernon
- Abstract
Introduction: Glanzmann's Thrombasthenia (GT) is rare, genetic haematologic disorder where the platelet glycoprotein integrin membrane glycoproteins IIb/IIIa complex is either dysfunctional or deficient, which results in faulty platelet aggregation and diminished clot retraction. It has been more specifically described as an autosomal recessive genetic disorder with a global incidence of 1/10,00,000. Spontaneous mucocutaneous bleeding is common and can lead to fatal bleeding episodes. Case Report: This is a case report of a 5-year-old boy who has a known case of GT that reported to our department for dental treatment. The mother's chief complaint was that her son had several decayed teeth. After consultation with the child's pediatrician and their consulting hematologist, treatment was planned that included restorations and pulpectomy. Clinical Significance/Unique Feature: The treatment of GT remains unsatisfactory. The quality of life of patients with GT remains impaired by multiple, spontaneous mucocutaneous bleeding episodes, and the high risk of hemorrhaging with surgery or any trauma. The available therapies, aside from the costly and risky allogeneic stem cell transplantation, are focused on treating bleeding episodes, and none of them provide a cure. Awareness about this condition, and proper medical as well as dental management is of importance.
- Subjects
STEM cell transplantation; MEMBRANE glycoproteins; BLOOD platelet aggregation; GENETIC disorders; MOTHERS; BLOOD platelet disorders
- Publication
Journal of the Indian Society of Pedodontics & Preventive Dentistry, 2024, Vol 42, pS131
- ISSN
0970-4388
- Publication type
Case Study
- DOI
10.4103/jisppd.jisppd_59_24