Mathew Mebin George
Senior Lecturer, Saveetha Dental College, Saveetha Institute of Medical and Technical Sciences, Chennai, Tamil Nadu, India.
J Family Med Prim Care. 2020 Mar 26;9(3):1733-1735. doi: 10.4103/jfmpc.jfmpc_1083_19. eCollection 2020 Mar.
Glanzmann's thrombasthenia is a rare, genetically inherited platelet disorder characterized by a lack of platelet aggregation. Until date, only close to 500 cases have been reported. GT is associated with clinical variability: some patients have only minimal bruising while others have frequent, severe and potentially fatal hemorrhages often making diagnosis difficult. Children are mostly diagnosed very early in life due to the spontaneous and unexplained mucocutaneous bleeding. The present case report deals with two siblings who reported with spontaneous gingival bleeding who were successfully managed by removal of local irritant factors and proper supportive care.
Glanzmann血小板无力症是一种罕见的遗传性血小板疾病,其特征是血小板聚集功能缺乏。迄今为止,仅报告了近500例病例。Glanzmann血小板无力症具有临床变异性:一些患者仅有轻微瘀伤,而另一些患者则经常出现严重且可能致命的出血,这常常使诊断变得困难。由于自发性和不明原因的黏膜皮肤出血,儿童大多在生命早期就被诊断出来。本病例报告涉及两名出现自发性牙龈出血的兄弟姐妹,他们通过去除局部刺激因素和适当的支持性护理而得到成功治疗。