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囊性纤维化患者肺移植候选者的时机和优先事项

Timing and priorities for cystic fibrosis patients candidates to lung transplantation.

作者信息

Venuta F, Rendina E A, De Giacomo T, Quattrucci S, Vizza D, Ciccone A M, Guarino E, Della Rocca G, Ricci C

机构信息

Department of Thoracic Surgery, University of Rome La Sapienza, Italy.

出版信息

Eur J Pediatr Surg. 1998 Oct;8(5):274-7. doi: 10.1055/s-2008-1071213.

DOI:10.1055/s-2008-1071213
PMID:9825236
Abstract

Bilateral lung transplantation is actually considered a valuable option for patients with endstage lung disease related to cystic fibrosis. Timing is crucial to transplant successfully as many patients as possible and it is mainly based on the progressive worsening of pulmonary function tests and quality of life. We reviewed the charts of all patients accepted for lung transplantation at our institution, in order to assess the role of several functional and demographic parameters; we compared the group of patients able to successfully wait for transplantation (Group A) with patients dying on the waiting list (Group B). Twenty-eight patients were accepted: 15 were successfully transplanted (2 at other institutions) (mean waiting time: 117 days), 7 died waiting (mean waiting time: 108 days) and 6 are still on the list. We recorded FEV-1, FVC, PaO2, PaCO2, supplemental O2 requirement, 6-minute walking test, right ventricular ejection fraction (RVEF) and cardio-pulmonary hemodynamics measured at right heart catheterization; we recorded also age at time of diagnosis and at time of evaluation, sex, weight and Schwachman score. These parameters were compared between Group A and B. Age at time of evaluation, sex, weight and Schwachman score did not present any difference between the two groups, as well as pulmonary function tests, PaO2, 6-minute walk test and RVEF. A statistically significant difference was found in terms of PaCO2 (43.9 +/- 9.3 in Group A vs 69.1 +/- 32.4 in Group B, heart rate at rest (102 +/- 21 vs 131 +/- 12) mean pulmonary artery pressure (20.6 +/- 2.9 vs 36 +/- 15.7), pulmonary vascular resistances (350 +/- 96 vs 460 +/- 119.4), cardiac index (3.2 +/- 0.6 vs 5.4 +/- 0.9). On the base of our initial experience we conclude that a careful evaluation of CF candidates for lung transplantation is recommended. A deterioration of pulmonary function tests and quality of life are useful parameters to accept patients in the waiting list; however priority should be attributed also on the base of cardio-pulmonary hemodynamics. A larger series of patients is required to draw definitive conclusions.

摘要

对于患有与囊性纤维化相关的终末期肺病的患者,双侧肺移植实际上被认为是一种有价值的选择。时机对于尽可能成功地为众多患者进行移植至关重要,这主要基于肺功能测试和生活质量的逐渐恶化。我们回顾了在我们机构接受肺移植的所有患者的病历,以评估几个功能和人口统计学参数的作用;我们将能够成功等待移植的患者组(A组)与在等待名单上死亡的患者(B组)进行了比较。共有28名患者被接受:15名成功接受了移植(2名在其他机构)(平均等待时间:117天),7名在等待中死亡(平均等待时间:108天),6名仍在等待名单上。我们记录了第一秒用力呼气容积(FEV-1)、用力肺活量(FVC)、动脉血氧分压(PaO2)、动脉血二氧化碳分压(PaCO2)、补充氧气需求、6分钟步行试验、右心室射血分数(RVEF)以及通过右心导管检查测量的心肺血流动力学;我们还记录了诊断时和评估时的年龄、性别、体重和施瓦赫曼评分。对A组和B组之间的这些参数进行了比较。评估时的年龄、性别、体重和施瓦赫曼评分在两组之间没有差异,肺功能测试、PaO2、6分钟步行试验和RVEF也是如此。在PaCO2(A组为43.9±9.3,B组为69.1±32.4)、静息心率(102±21对131±12)、平均肺动脉压(20.6±2.9对36±15.7)、肺血管阻力(350±96对460±119.4)、心脏指数(3.2±0.6对5.4±0.9)方面发现了统计学上的显著差异。基于我们最初的经验,我们得出结论,建议对囊性纤维化肺移植候选者进行仔细评估。肺功能测试和生活质量的恶化是将患者纳入等待名单的有用参数;然而,也应根据心肺血流动力学来确定优先级。需要更多的患者系列来得出明确的结论。

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Semin Respir Crit Care Med. 2015 Apr;36(2):299-320. doi: 10.1055/s-0035-1547347. Epub 2015 Mar 31.