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器官移植治疗囊性纤维化。

Organ Transplantation for Cystic Fibrosis.

机构信息

Lung Transplant Program, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

Pulmonary, Allergy, and Critical Care Medicine Division, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.

出版信息

Semin Respir Crit Care Med. 2019 Dec;40(6):842-856. doi: 10.1055/s-0039-3399554. Epub 2019 Dec 30.

DOI:10.1055/s-0039-3399554
PMID:31887769
Abstract

Cystic fibrosis (CF) remains the most common indication for lung transplantation in children and the third most common in adults and has the highest median survival posttransplant for all pretransplant diagnoses. Criteria for transplant in patients with CF vary widely among transplant centers and early referral to multiple centers may be needed to maximize opportunities for lung transplantation. Comorbidities unique to CF such as resistant and atypical pathogens like and , and cirrhosis require special consideration for lung transplantation but should not be considered as absolute contraindications. For those patients who are listed for lung transplantation, mechanical support with extracorporeal membrane oxygenation and mechanical ventilation can be efficacious as bridges to lung transplantation in experienced centers with adequate resources. Liver and pancreas transplantations are also acceptable options for end-organ disease related to CF and can provide improvements in both quantity and quality of life.

摘要

囊性纤维化(CF)仍然是儿童肺移植最常见的适应证,也是成人第三常见的适应证,并且在所有移植前诊断中具有最高的中位移植后生存率。CF 患者的移植标准在移植中心之间差异很大,可能需要及早向多个中心转诊,以最大限度地增加肺移植的机会。CF 特有的合并症,如耐药和非典型病原体,如 和 ,以及肝硬化,需要特别考虑肺移植,但不应被视为绝对禁忌。对于那些被列入肺移植名单的患者,在经验丰富的中心,使用体外膜氧合和机械通气等机械支持可以作为肺移植的有效桥梁,这些中心具备充足的资源。肝和胰腺移植也是 CF 相关终末器官疾病的可接受选择,可以提高生活质量和数量。

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A case of localized paranasal sinusitis associated with ST 1880 in a cystic fibrosis patient.一名囊性纤维化患者发生与ST 1880相关的局限性鼻窦炎病例。
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