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首页> 外文期刊>Journal of Clinical and Diagnostic Research >Total Extraction as a Treatment for Anaemia in a Patient of Glanzmann's Thrombasthenia with Chronic Gingival Bleed: Case Report
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Total Extraction as a Treatment for Anaemia in a Patient of Glanzmann's Thrombasthenia with Chronic Gingival Bleed: Case Report

机译:总提取物治疗患有慢性牙龈出血的格兰仕曼血小板减少症患者的贫血:病例报告

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摘要

Glanzmann?s Thrombasthenia (GT) is a rare autosomal recessive bleeding disorder affecting the megakaryocyte lineage and is characterized by lack of platelet aggregation on stimulation. The molecular basis is linked to quantitative and qualitative abnormalities of aIIb?3 integrin. Most of the patients with severe Glanzmann?s thrombasthenia have spontaneous gum bleeding and persistent low haemoglobin levels. Often these patients are addressed with local haemostatic measures and platelet coverage. We report a case of a severe Glanzmann?s thrombasthenia with chronic gingivitis and associated spontaneous gum bleed with chronic low haemoglobin levels, managed subsequently with total dental extraction under appropriate platelet and recombinant factor VIIa coverage. Further follow up of the patient substantiated the treatment protocol with increased and stable haemoglobin levels, thus emphasizing the need for total dental extraction in patients with severe Glanzmann?s with chronic spontaneous gum bleed, as a definitive treatment option, which has not been reported so far in the literature.
机译:格兰兹曼血小板减少症(GT)是一种罕见的常染色体隐性遗传性出血性疾病,会影响巨核细胞谱系,其特征是刺激后血小板聚集不足。分子基础与aIIb?3整联蛋白的定量和定性异常有关。大多数患有严重的格兰兹曼血栓性衰弱的患者都有自发性牙龈出血和持续的低血红蛋白水平。通常这些患者可以通过局部止血措施和血小板覆盖来治疗。我们报告了一例严重的格兰兹曼血栓性衰弱伴有慢性牙龈炎,伴有慢性低血红蛋白水平的自发性牙龈出血,随后在适当的血小板和重组因子VIIa覆盖下进行了全牙拔除。对患者的进一步随访证实了治疗方案的血红蛋白水平升高且稳定,从而强调了对于患有慢性自发性牙龈出血的严重格兰仕曼氏病患者的彻底拔牙的需要,作为确定的治疗选择,至今尚未见报道。在文献中没有。

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