Gopalakrishnan Anjana, Veeraraghavan Ravi, Panicker Prasanth
Department of Pediatric Dentistry, Amrita School of Dentistry, Kochi, Kerala, India.
J Indian Soc Pedod Prev Dent. 2014 Apr-Jun;32(2):181-4. doi: 10.4103/0970-4388.131000.
Glanzmann's thrombasthenia (GT) is a rare, congenital, and moderate to severe platelet disorder. The bleeding time is increased, due to lack of platelet aggregation, since the patients with GT have deficient or dysfunctional integrin membrane glycoproteins IIb and IIIa essential for platelet aggregation. Children with GT are mostly diagnosed very early in life due to the spontaneous and unexplained mucocutaneous bleeding. It is quite a challenging task when any surgery is indicated for children with GT. This case report is about the medical and surgical management of an 11-year-old girl diagnosed with Glannzmann's thrombasthenia who had to undergo a maxillary cyst enucleation.
Glanzmann血小板无力症（GT）是一种罕见的先天性中重度血小板疾病。由于GT患者缺乏对血小板聚集至关重要的整合素膜糖蛋白IIb和IIIa或其功能异常，导致血小板聚集缺乏，出血时间延长。GT患儿大多在生命早期因自发性和不明原因的黏膜皮肤出血而被诊断出来。对于患有GT的儿童，如需进行任何手术，都是一项颇具挑战性的任务。本病例报告讲述的是一名被诊断为Glannzmann血小板无力症的11岁女孩的医疗和手术治疗情况，她不得不接受上颌囊肿摘除术。
