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血小板无力症的口腔侵入性操作:一项回顾性观察研究。

Oral invasive procedures in Glanzmann thrombasthenia: a retrospective observational study.

作者信息

Delarue Maxime, Severac François, Soell Martine, Pierre Léa, Desprez Dominique, Bornert Fabien

机构信息

University of Strasbourg, Faculty of Dental Surgery, Strasbourg, France.

University Hospital Strasbourg, Department of Oral Surgery, Strasbourg, France.

出版信息

Res Pract Thromb Haemost. 2024 Nov 4;8(8):102619. doi: 10.1016/j.rpth.2024.102619. eCollection 2024 Nov.

DOI:10.1016/j.rpth.2024.102619
PMID:39660077
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11629330/
Abstract

BACKGROUND

Glanzmann thrombasthenia (GT) is a very rare autosomal inherited bleeding disease affecting megakaryocyte lineage with impacts on oral health such as gingival bleeding, which requires specific management protocols. Very few clinical cases have been published in the dental and hematologic literature.

OBJECTIVES

This study focuses on a series of 21 patients affected specifically by GT and their hemorrhagic prophylaxis management with the use of recombinant activated factor VII (rFVIIa) for dental extractions and full-mouth debridement.

METHODS

Data were collected from medical and dental records. rFVIIa was administered prophylactically for oral procedures, following a standardized protocol. Each sessions were performed by experienced oral surgeons, and outcomes were analyzed with a focus on bleeding complications and adverse events.

RESULTS

Forty-one full-mouth debridements and 176 dental extractions were performed during 102 sessions of dental care in an outpatient setting. A total of 226 injections of rFVIIa was delivered. The mean number of injections was 2.2 (range, 1-4) per dental procedure. The overall rate of bleeding complications was 5.9% (n = 6). All 6 hemorrhagic complications were classified as minor bleeding. No thromboembolic event or allergic reaction was observed.

CONCLUSION

The data presented in this retrospective observational study support the efficacy and safety of rFVIIa for the prevention of bleeding during invasive dental procedures in patients affected by GT. The rFVIIa protocol presented here seems to be a safe and efficient protocol for the prevention of bleeding during invasive oral procedures.

摘要

背景

Glanzmann血小板无力症(GT)是一种非常罕见的常染色体遗传性出血性疾病,影响巨核细胞谱系,对口腔健康有影响,如牙龈出血,这需要特定的管理方案。牙科和血液学文献中发表的临床病例非常少。

目的

本研究聚焦于一系列21例受GT影响的患者,以及他们在拔牙和全口清创术中使用重组活化因子VII(rFVIIa)进行出血预防管理的情况。

方法

从医疗和牙科记录中收集数据。按照标准化方案,在口腔手术前预防性使用rFVIIa。每次手术均由经验丰富的口腔外科医生进行,重点分析出血并发症和不良事件的结果。

结果

在门诊环境下的102次牙科护理中,进行了41次全口清创术和176次拔牙。总共注射了226次rFVIIa。每次牙科手术的平均注射次数为2.2次(范围为1 - 4次)。出血并发症的总体发生率为5.9%(n = 6)。所有6例出血并发症均被归类为轻微出血。未观察到血栓栓塞事件或过敏反应。

结论

这项回顾性观察研究中呈现的数据支持rFVIIa在预防GT患者侵入性牙科手术期间出血方面的有效性和安全性。这里提出的rFVIIa方案似乎是预防侵入性口腔手术期间出血的一种安全有效的方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/bea4ec1c00b2/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/ba5672b4081a/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/ea0c1dfb1bcf/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/b4f058bf5972/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/bea4ec1c00b2/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/ba5672b4081a/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/ea0c1dfb1bcf/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/b4f058bf5972/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2570/11629330/bea4ec1c00b2/gr4.jpg

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本文引用的文献

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Haemophilia. 2024 Jul;30(4):943-949. doi: 10.1111/hae.15055. Epub 2024 Jun 2.
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Glanzmann Thrombasthenia 10 Years Later: Progress Made and Future Directions.10年后的Glanzmann血小板无力症:取得的进展与未来方向
Semin Thromb Hemost. 2025 Mar;51(2):196-208. doi: 10.1055/s-0044-1782519. Epub 2024 Mar 18.
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Apixaban for treatment of venous thromboembolism in an obese patient with Glanzmann thrombasthenia.
阿哌沙班用于治疗患有Glanzmann血小板无力症的肥胖患者的静脉血栓栓塞症。
Res Pract Thromb Haemost. 2023 May 20;7(4):100183. doi: 10.1016/j.rpth.2023.100183. eCollection 2023 May.
4
Current status and future prospects of activated recombinant coagulation factor VIIa, NovoSeven®, in the treatment of haemophilia and rare bleeding disorders.活化重组凝血因子 VIIa(诺其)在治疗血友病和罕见出血性疾病中的现状和未来前景。
Ann Hematol. 2024 Aug;103(8):2647-2658. doi: 10.1007/s00277-023-05287-2. Epub 2023 Jun 30.
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Emergency management of patients with Glanzmann thrombasthenia: consensus recommendations from the French reference center for inherited platelet disorders.Glanzmann 血小板无力症患者的紧急处理:法国遗传性血小板疾病参考中心的共识建议。
Orphanet J Rare Dis. 2023 Jun 29;18(1):171. doi: 10.1186/s13023-023-02787-2.
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Perioperative Management of Oral Antithrombotics in Dentistry and Oral Surgery: Part 2.口腔颌面外科学中抗栓药物的围手术期管理:第 2 部分。
Anesth Prog. 2023 Mar 1;70(1):37-48. doi: 10.2344/anpr-70-01-06.
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