哈钦森-吉尔福德早衰综合征心肺搭桥术的麻醉管理:病例报告。

IF 0.5 Q4 ANESTHESIOLOGY A&A practice Pub Date : 2024-08-23 eCollection Date: 2024-08-01 DOI:10.1213/XAA.0000000000001842
Katherine L Zaleski, Gregory S Matte, Monica E Kleinman, Ashwin Prakash, Mary Lyn Stein
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引用次数: 0

摘要

哈钦森-吉尔福德早衰综合征(HGPS)是一种节段性早衰的罕见疾病,与晚期动脉粥样硬化和严重的心脑血管疾病的发生有关。使用乐伐尼治疗可提高 HGPS 患者的生存率;然而,在长期的纵向随访中,快速进展性钙化性主动脉瓣狭窄的患病率有所上升。HGPS 病程的不断发展促使人们重新考虑保守治疗,并开发了解剖治疗策略。在本病例报告中,我们介绍了对接受心肺旁路主动脉瓣狭窄手术治疗的 HGPS 患者的麻醉管理。
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Anesthetic Management of Cardiopulmonary Bypass in Hutchinson-Gilford Progeria Syndrome: A Case Report.

Hutchinson-Gilford Progeria Syndrome (HGPS) is an ultrarare disorder of segmental premature aging that is associated with the development of advanced atherosclerosis and significant cardiovascular and cerebrovascular disease. Treatment with lonafarnib has improved survival in patients with HGPS; however, in extended longitudinal follow-up, there has been an increase in the prevalence of rapidly progressive calcific aortic stenosis. The evolving course of HGPS has prompted reconsideration of conservative management and led to the development of strategies for anatomic treatment. In this case report, we describe the anesthetic management of patients with HGPS undergoing surgical management of aortic stenosis with cardiopulmonary bypass.

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来源期刊
A&A practice
A&A practice Medicine-Medicine (all)
CiteScore
1.40
自引率
0.00%
发文量
126
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