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Dental Management of Glanzmann's Thrombasthenia in a 4-Year-Old Child With Rampant Caries: A Case Report and
Bahareh Nazemisalman1, Gisoo Bahmani2, Kambiz Davari3
1Department of Pediatric Dentistry, School of Dentistry Zanjan University of Medical Sciences Zanjan Iran.
Abstract:
Glanzmann's Thrombasthenia (GT) is a rare congenital bleeding disorder in children, caused by the absence or dysfunction of glycoprotein (GP) IIb/IIIa, a receptor located on the platelet membrane. Proper oral health and regular dental appointments are crucial for these patients. However, maintaining good oral hygiene is challenging among them. Due to excessive gingival bleeding, patients refrain from brushing their teeth, which leads to poor oral hygiene and severe dental caries. Furthermore, gingival inflammation caused by dental plaque may exacerbate the bleeding and create a vicious cycle. A 4-year-old girl with the chief complaint of pain and discomfort in the upper left deciduous molars was examined in April 2024. According to her past medical history, she was previously diagnosed with GT. She was the firstborn child of parents of a consanguineous marriage with no other siblings. Physical examination revealed petechiae and ecchymosis on her face, trunk, abdomen, legs, and limbs that resolved spontaneously over time. Clinical intraoral examination revealed rampant dental caries and generalized gingivitis. The patient was hospitalized 1 day before the surgery and received 1 dose of Recombinant Activated Factor VII preoperatively. Dental caries were removed and restored under general anesthesia. However, 1 week after the dental operation, the patient experienced sudden and severe bleeding, requiring two units of blood transfusions at that time. Dental care for patients with GT might be challenging due to the risk of excessive bleeding. It's essential to consider platelet transfusions in case of a significant risk of bleeding during surgery, and with extreme caution. Furthermore, severe delayed bleeding requiring transfusion as a postoperative complication should be considered and managed. Dental procedures should be performed with minimal tissue trauma to prevent bleeding.
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