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结节性硬化症相关的肺淋巴管平滑肌瘤病:肺康复的作用——一例报告

Tuberous sclerosis-associated pulmonary lymphangioleiomyomatosis: The role of pulmonary rehabilitation - A case report.

作者信息

Castro-Rodriguez Flor, Rodriguez-Gallo Yakdiel

机构信息

Department of Critical Care Medicine, National Hospital of El Salvador, Avenida la Revolución 222, Ex-Cifo., CP 01101, San Salvador, El Salvador.

Faculty of Biomedicine, Don Bosco University, Calle a Plan del Pino Km 1 1/2, Soyapango, 1874, El Salvador.

出版信息

Respir Med Case Rep. 2024 Oct 5;52:102128. doi: 10.1016/j.rmcr.2024.102128. eCollection 2024.

Abstract

Lymphangioleiomyomatosis (LAM) is a rare lung disease that primarily affects women. A patient with a medical history of Tuberous Sclerosis-Associated Lymphangioleiomyomatosis (TSC-LAM), a prior thyroidectomy for thyroid cancer, chronic hypertension, and a previous pulmonary thromboembolism was admitted to the hospital. Following the stabilization of her clinical condition, diaphragmatic exercises and incentive spirometers were implemented. This intervention significantly enhanced her respiratory status, prevented the need for invasive mechanical ventilation, and expediting pulmonary functional recovery. While further studies are needed, pulmonary rehabilitation has the potential to influence the clinical course of TSC-LAM patients in the ICU by improving respiratory capacity and reducing hospitalization time.

摘要

淋巴管平滑肌瘤病(LAM)是一种主要影响女性的罕见肺部疾病。一名患有结节性硬化症相关淋巴管平滑肌瘤病(TSC-LAM)、曾因甲状腺癌接受甲状腺切除术、患有慢性高血压且既往有肺血栓栓塞史的患者入院。在其临床状况稳定后,实施了膈肌锻炼和激励式肺量计训练。这种干预显著改善了她的呼吸状况,避免了有创机械通气的需要,并加速了肺功能恢复。虽然还需要进一步研究,但肺康复有可能通过提高呼吸能力和缩短住院时间来影响重症监护病房(ICU)中TSC-LAM患者的临床病程。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8a93/11497434/520f21fe16b5/gr1.jpg

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