中度血友病 A 患儿自发性血心包并发心肌填塞:基于病例的综述》(Spontaneous Hemopericardium Complicated by Tamponade in a Child with Moderate Hemophilia A: Case-Based Review.

IF 1.1 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS World Journal for Pediatric and Congenital Heart Surgery Pub Date : 2023-07-01 Epub Date: 2023-04-26 DOI:10.1177/21501351231168868
Seyfeddine Zayani, Farah Thabet, Majdi Ben Massoud, Chokri Chouchane, Habib Gamra, Slaheddine Chouchane
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引用次数: 0

摘要

血友病 A 是最常见的严重先天性出血性疾病。它是一种 X 连锁隐性遗传出血性疾病,其特征是第八因子定性和/或定量缺乏。该病的临床表现为出血,可累及各个器官,尤其是关节(血肿)和肌肉(血肿)。一些严重但罕见的出血可危及生命,尤其是中枢神经系统出血和血心包。我们报告了一例罕见的中度 A 型血友病患儿自发性血心包并发心包填塞的病例,患儿接受了因子 VIII 替代输注和心包引流治疗,结果良好。据我们所知,这是文献中描述的第二例发生在 A 型血友病患儿身上的自发性血心包积血病例。我们的病例表明,在拔除心包引流管后维持 50 IU/kg/8 h 的 VIII 因子剂量长达一天似乎就足以确保正确止血,但这一印象还需要进一步的证据来证实。
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Spontaneous Hemopericardium Complicated by Tamponade in a Child With Moderate Hemophilia A: Case-Based Review.

Hemophilia A is the most common severe innate bleeding disorder. It is an X-linked recessive inherited bleeding disorder characterized by a qualitative and/or quantitative deficiency of factor VIII. The clinical manifestation of this disease is hemorrhaging that can affect every organ, in particular joints (hemarthrosis) and muscles (hematoma). Some serious but rare hemorrhages can be life-threatening, in particular hemorrhage of the central nervous system and hemopericardium. We report a rare case of spontaneous hemopericardium complicated by tamponade in a child with moderate hemophilia A treated with Factor VIII replacement infusion and pericardial drainage, with a favorable outcome. To our knowledge, this is the second case described in the literature of spontaneous hemopericardium occurring in a child with hemophilia A. Our case suggests that a dose of 50 IU/kg/8 h of factor VIII maintained for up to one day after removal of the pericardial drain seems to be sufficient to ensure correct hemostasis, though further evidence is needed to confirm this impression.

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来源期刊
CiteScore
1.80
自引率
11.10%
发文量
128
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