Bahadure Rakesh N, Dhote Vijaya, Sayed Kulsum Iqbal, Bailwad Sandeep, Kodate Poornima
Department of Pedodontics and Preventive Dentistry, Government Dental College and Hospital, Nagpur, Maharashtra, India.
Department of Pedodontics and Preventive Dentistry, Government Dental College and Hospital, Mumbai, Maharashtra, India.
Contemp Clin Dent. 2019 Jan-Mar;10(1):143-146. doi: 10.4103/ccd.ccd_137_18.
Glanzmann's thrombasthenia (GT) is a rare inherited bleeding disorder characterized by disturbed ability of the blood platelets to gather around the site of a broken blood vessel and fail to form a plug to stop bleeding due to deficiency of a glycoprotein IIb/IIIa in the process of blood clotting and results in moderate-to-severe bleeding on slightest injury to blood vessels. It is typically diagnosed in infancy or early childhood due to mucocutaneous bleeding tendencies. Treatment goals in GT are aimed at control of bleeding. Dentoalveolar trauma in patient with bleeding disorder poses a challenge in pediatric dentistry. The present article reports the successful management of subluxation dental injury in a 3-year-old child with "Glanzmann's thrombasthenia associated with sickle cell anemia." A narrative review of GT is given highlighting the importance of preventive dentistry and anticipatory guidance to reduce potential complications in such patients.
血小板无力症(GT)是一种罕见的遗传性出血性疾病,其特征是血小板在破损血管部位聚集的能力受到干扰,由于血液凝固过程中糖蛋白IIb/IIIa缺乏而无法形成堵塞物来止血,导致血管稍有损伤就会出现中度至重度出血。由于黏膜皮肤出血倾向,该病通常在婴儿期或幼儿期被诊断出来。GT的治疗目标是控制出血。出血性疾病患者的牙槽创伤给儿童牙科带来了挑战。本文报道了一名患有“血小板无力症合并镰状细胞贫血”的3岁儿童脱位性牙损伤的成功治疗。本文对GT进行了叙述性综述,强调了预防牙科和预期指导对于减少此类患者潜在并发症的重要性。