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资源有限国家中一名甲型血友病患者自发性颅内出血的围手术期管理

Perioperative Management of Spontaneous Intracranial Hemorrhage in a Patient With Hemophilia A in a Resource Limited Country.

作者信息

Chopra Puneet, Singh Manraj, Singh Adityabikram, Masi Athena, Yurkofsky Judith, Zaita Brittany, Kaur Gurjinder

机构信息

Critical Care, Satguru Partap Singh (SPS) Hospital, Ludhiana, IND.

Basic Biomedical Sciences, Dayanand Medical College and Hospital, Ludhiana, IND.

出版信息

Cureus. 2023 Aug 14;15(8):e43485. doi: 10.7759/cureus.43485. eCollection 2023 Aug.

Abstract

Intracranial hemorrhage (ICH) is a serious complication of hemophilia A with high morbidity and mortality. The management of such cases is complicated by nonspecific and often delayed presentation, increased frequency of rebleeding, low awareness regarding clotting factor replacement, and debate regarding the efficacy of surgical interventions. We report a case of an 18-year-old male patient with hemophilia A, who first presented to the emergency department in India in a comatose state. Neuroimaging revealed subdural hematoma with midline shift and uncal herniation. The patient was successfully managed with perioperative cryoprecipitate and factor VIII replacement, tiered intracranial pressure lowering strategies, and early decompressive craniectomy with clot evacuation. In India, there are no standardized guidelines for screening and routine care for hereditary diseases like hemophilia. In a resource-deficient country, management was complicated by the limited availability of factor VIII in the emergent setting, as well as the inability to obtain serial factor levels in the postoperative period. We hope that this article helps to guide the management of ICH and hemophilia in resource-limited countries.

摘要

颅内出血(ICH)是甲型血友病的一种严重并发症,发病率和死亡率都很高。此类病例的管理因以下因素而变得复杂:症状不具特异性且往往出现延迟、再出血频率增加、对凝血因子替代治疗的认识不足,以及关于手术干预疗效的争议。我们报告一例18岁甲型血友病男性患者,该患者最初在印度以昏迷状态被送至急诊科。神经影像学检查显示硬膜下血肿伴中线移位和钩回疝。该患者通过围手术期冷沉淀和凝血因子VIII替代治疗、分级降低颅内压策略以及早期减压性颅骨切除术并清除血块,成功得到救治。在印度,对于血友病等遗传性疾病,没有标准化的筛查和常规护理指南。在一个资源匮乏的国家,管理工作因紧急情况下凝血因子VIII供应有限以及术后无法获得连续的因子水平而变得复杂。我们希望本文有助于指导资源有限国家对颅内出血和血友病的管理。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/238d/10499499/60274840f16d/cureus-0015-00000043485-i01.jpg

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