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囊性纤维化:从确诊到肺移植,单中心经验

Cystic Fibrosis: From Qualification to Lung Transplantation, a Single Center Experience.

作者信息

Ochman Marek, Latos Magdalena, Urlik Maciej, Stącel Tomasz, Nęcki Mirosław, Tatoj Zofia, Zawadzki Fryderyk, Wajda-Pokrontka Marta, Przybyłowski Piotr, Zembala Marian

机构信息

Department of Cardiac, Vascular and Endovascular Surgery and Transplantology, Medical University of Silesia, Katowice, Poland.

Institute of Cardiology, Jagiellonian University, Medical College, Cracow, Poland.

出版信息

Ann Transplant. 2019 Apr 5;24:185-190. doi: 10.12659/AOT.914328.

Abstract

BACKGROUND Cystic fibrosis (CF) is congenital multisystem disorder, that leads to gradual deterioration of pulmonary function. Advancements in therapy of CF-related lung disease have delayed its progression. However, lung transplantation remains the only therapeutic option for majority of such patients. Aim of the study was to assess qualification process and outcome of lung transplantation as a treatment of CF patients qualified in a single center between 2011 and 2018. MATERIAL AND METHODS This retrospective study assessed 41 patients who were qualified to be treated by means of lung transplantation due to CF in Lung Transplant Program of Silesian Center for Heart Diseases between 2011 and 2018. Analysis of patients during qualification process and after lung transplantation was performed. Lung recipients were observed during 1-year follow-up by means of pulmonary function tests. RESULTS 1-year survival was noted among 80% of the patients; 3-year survival and 5-year survival were noted among 70% of the recipients. Mean forced expiratory volume in 1 second (FEV1) increased after lung transplantation: 21.19% at qualification; and 76.67% at 12 months after lung transplantation. Mean forced vital capacity (FVC) results also improved: 34.18% at qualification and 78.34% at 12 months after lung transplantation. The 6-minute walk test (6MWT) before and after treatment noted an increase of 175.55 m. CONCLUSIONS Lung transplantation improves respiratory capacity of CF patients and prolongs their life.

摘要

背景 囊性纤维化(CF)是一种先天性多系统疾病,可导致肺功能逐渐恶化。CF相关肺部疾病治疗方法的进步延缓了其进展。然而,肺移植仍然是大多数此类患者的唯一治疗选择。本研究的目的是评估2011年至2018年间在单一中心符合条件的CF患者接受肺移植治疗的资格评估过程和结果。

材料与方法 这项回顾性研究评估了2011年至2018年间在西里西亚心脏病中心肺移植项目中因CF有资格接受肺移植治疗的41例患者。对患者资格评估过程中和肺移植后的情况进行了分析。通过肺功能测试对肺移植受者进行了1年的随访观察。

结果 80%的患者观察到1年生存率;70%的受者观察到3年生存率和5年生存率。肺移植后1秒用力呼气容积(FEV1)平均值增加:资格评估时为21.19%;肺移植后12个月时为76.67%。用力肺活量(FVC)平均结果也有所改善:资格评估时为34.18%,肺移植后12个月时为78.34%。治疗前后的6分钟步行试验(6MWT)显示增加了175.55米。

结论 肺移植可改善CF患者的呼吸功能并延长其寿命。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fcac/6467174/426d83784a3d/anntransplant-24-185-g001.jpg

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