Multi-disciplinary team of the treatment of bilateral lung transplantation for pediatric cystic fibrosis
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摘要:
目的 通过多学科综合诊疗(MDT)模式提高双肺移植治疗儿童囊性纤维化的外科疗效。 方法 对1例国内罕见的终末期囊性纤维化10岁患儿进行肺移植术前MDT讨论,制定对应治疗方案。 结果 患儿确诊囊性纤维化5年,肺移植指征明确,经过MDT讨论决定行Clam-shell切口下双肺移植术,术中维持生命体征稳定,术后给予呼吸机辅助通气、抗感染、免疫抑制、抑酸和预防应激性溃疡等治疗,并根据患儿特点给予个体化治疗方案,术后恢复良好。 结论 通过肺移植术前MDT讨论,肺移植治疗儿童囊性纤维化的手术效果满意,减少了术后并发症的发生风险,值得临床推广。 -
关键词:
- 双肺移植 /
- 多学科综合诊疗 /
- 儿童肺移植 /
- 囊性纤维化 /
- 囊性纤维化跨膜电导调节因子(CFTR) /
- Ⅰ型呼吸衰竭 /
- 体外膜肺氧合 /
- 肺分配评分(LAS)
Abstract:Objective To improve the surgical efficacy of bilateral lung transplantation for pediatric cystic fibrosis through multi-disciplinary team (MDT). Methods Preoperative MDT consultation was delivered for a 10-year-old child with rare end-stage cystic fibrosis to establish the corresponding treatment protocol. Results The child was diagnosed with cystic fibrosis for 5 years, and the indication of lung transplantation were confirmed. After preoperative MDT consultation, bilateral lung transplantation via a Clam-shell incision was determined. The vital signs were maintained stable during operation. Postoperatively, ventilator-assisted ventilation, anti-infection, immunosuppression, acid suppression, prevention of stress ulceration and other treatments were delivered. Individualized treatment was given according to the characteristics of the child, and the child was well recovered. Conclusions Through preoperative MDT consultation, lung transplantation yields satisfactory surgical efficacy in treating pediatric cystic fibrosis and lowers the risk of postoperative complications, which deserves application in clinical practice. -
图 1 肺囊性纤维化患儿围手术期胸部影像学表现和支气管镜探查表现
注:A图示患儿术前胸部CT检查图像;B图示患儿术后第4日胸部CT检查图像;C图示患儿出院前胸部CT检查图像;D图示患儿术后3个月复查胸部CT检查图像;E图示患儿术后第1日X线胸片检查图像;F图示患儿术后第6日X线胸片检查图像;G图示患儿术前纤维支气管镜探查图像;H图示患儿术后第1日床边纤维支气管镜探查图像。
Figure 1. The perioperative chest imaging findings and bronchoscopy findings in the child with pulmonary cystic fibrosis
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[1] 安佳, 莫绪明. 儿童肺移植的现状及研究进展[J]. 中华器官移植杂志, 2020, 41(5): 313-316. DOI: 10.3760/cma.j.cn421203-20200517-00156.AN J, MO XM. Current situation and research progress of pediatric lung transplantation[J]. Chin J Organ Transplant, 2020, 41(5): 313-316. DOI: 10.3760/cma.j.cn421203-20200517-00156. [2] ELBORN JS. Cystic fibrosis[J]. Lancet, 2016, 388(10059): 2519-2531. DOI: 10.1016/S0140-6736(16)00576-6. [3] 晋晨晨, 杜洁仪, 黄雪莲, 等. CFTR对前脂肪细胞增殖和分化的影响[J]. 中山大学学报(医学科学版), 2019, 40(2): 161-171.JIN CC, DU JY, HUANG XL, et al. Effects of CFTR on preadipocyte proliferation and differentiation[J]. J Sun Yat-sen Univ(Med Sci), 2019, 40(2): 161-171. [4] NOH SR, LEE E, YOON J, et al. The first successful lung transplantation in a Korean child with cystic fibrosis[J]. J Korean Med Sci, 2017, 32(12): 2073-2078. DOI: 10.3346/jkms.2017.32.12.2073. [5] WEILL D, BENDEN C, CORRIS PA, et al. A consensus document for the selection of lung transplant candidates: 2014--an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation[J]. J Heart Lung Transplant, 2015, 34(1): 1-15. DOI: 10.1016/j.healun.2014.06.014. [6] ROSSANO JW, SINGH TP, CHERIKH WS, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: twenty-second pediatric heart transplantation report - 2019; focus theme: donor and recipient size match[J]. J Heart Lung Transplant, 2019, 38(10): 1028-1041. DOI: 10.1016/j.healun.2019.08.002. [7] 中华医学会器官移植学分会. 中国肺移植供肺获取与保护技术规范(2019版)[J/CD]. 中华移植杂志(电子版), 2019, 13 (2): 87-90. DOI: 10.3877/cma.j.issn.1674-3903.2019.02.002.Branch of Organ Transplantation of Chinese Medical Association. Technical specification for donor lung procurement and protection of lung transplantation in China (2019 edition)[J/CD]. Chin J Transplant (Electr Edit), 2019, 13(2): 87-90. DOI: 10.3877/cma.j.issn.1674-3903.2019.02.002. [8] MITILIAN D, SAGE E, PUYO P, et al. Techniques and results of lobar lung transplantations[J]. Eur J Cardiothorac Surg, 2014, 45(2): 365-370. DOI: 10.1093/ejcts/ezt353. [9] DATE H, AOE M, NAGAHIRO I, et al. Living-donor lobar lung transplantation for various lung diseases[J]. J Thorac Cardiovasc Surg, 2003, 126(2): 476-481. DOI: 10.1016/s0022-5223(03)00235-6. [10] 中华医学会器官移植学分会, 国家肺移植质量管理与控制中心. 肺移植围手术期体外膜肺氧合应用指南(2019版)[J]. 器官移植, 2019, 10(4): 402-409. DOI: 10.3969/j.issn.1674-7445.2019.04.009.Branch of Organ Transplantation of Chinese Medical Association, National Quality Management and Control Center for Lung Transplantation. Guideline on the application of extracorporeal membrane oxygenation during the perioperative period of lung transplantation(2019 edition)[J]. Organ Transplant, 2019, 10(4): 402-409. DOI: 10.3969/j.issn.1674-7445.2019.04.009. [11] LOOR G, SIMPSON L, PARULEKAR A. Bridging to lung transplantation with extracorporeal circulatory support: when or when not?[J]. J Thorac Dis, 2017, 9(9): 3352-3361. DOI: 10.21037/jtd.2017.08.117. [12] IUS F, SOMMER W, TUDORACHE I, et al. Five-year experience with intraoperative extracorporeal membrane oxygenation in lung transplantation: indications and midterm results[J]. J Heart Lung Transplant, 2016, 35(1): 49-58. DOI: 10.1016/j.healun.2015.08.016. [13] CHAMBERS DC, CHERIKH WS, GOLDFARB SB, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: thirty-fifth adult lung and heart-lung transplant report-2018; focus theme: multiorgan transplantation[J]. J Heart Lung Transplant, 2018, 37(10): 1169-1183. DOI: 10.1016/j.healun.2018.07.020. [14] GOLDFARB SB, HAYES D JR, LEVVEY BJ, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: twenty-first pediatric lung and hear lung transplantation report-2018; focus theme: multiorgan transplantation[J]. J Heart Lung Transplant, 2018, 37(10): 1196-1206. DOI: 10.1016/j.healun.2018.07.021. [15] FRASER CD 3RD, ZHOU X, GRIMM JC, et al. Size mismatching increases mortality after lung transplantation in preadolescent patients[J]. Ann Thorac Surg, 2019, 108(1): 130-137. DOI: 10.1016/j.athoracsur. 2019.01.015. [16] MUELLER C, HANSEN G, BALLMANN M, et al. Size reduction of donor organs in pediatric lung transplantation[J]. Pediatr Transplant, 2010, 14(3): 364-368. DOI: 10.1111/j.1399-3046.2009.01242.x. [17] EGAN TM, MURRAY S, BUSTAMI RT, et al. Development of the new lung allocation system in the United States[J]. Am J Transplant, 2006, 6(5 Pt 2): 1212-1227. DOI: 10.1111/j.1600-6143.2006.01276.x. [18] 中华医学会器官移植学分会. 中国肺移植受者选择与术前评估技术规范(2019版)[J/CD]. 中华移植杂志(电子版), 2019, 13 (2): 81-86. DOI: 10.3877/cma.j.issn.1674-3903.2019.02.001.Branch of Organ Transplantation of Chinese Medical Association. Techical specification for the selection of recipient and preoperative evaluation of lung transplantation in China (2019 edition)[J/CD]. Chin J Transplant (Electr Edit), 2019, 13(2): 81-86. DOI: 10.3877/cma.j.issn.1674-3903.2019.02.001. [19] YUE B, WU B, ZHANG J, et al. Pediatric lung transplantation in the largest lung transplantation center of China: embarking on a long road[J]. Sci Rep, 2020, 10(1): 12471. DOI: 10.1038/s41598-020-69340-0. [20] AMIN R, RATJEN F. Emerging drugs for cystic fibrosis[J]. Expert Opin Emerg Drugs, 2014, 19(1): 143-155. DOI: 10.1517/14728214.2014.882316. [21] YEUNG JC, MACHUCA TN, CHAPARRO C, et al. Lung transplantation for cystic fibrosis[J]. J Heart Lung Transplant, 2020, 39(6): 553-560. DOI: 10.1016/j.healun.2020.02.010. [22] MORRELL MR, KIEL SC, PILEWSKI JM. Organ transplantation for cystic fibrosis[J]. Semin Respir Crit Care Med, 2019, 40(6): 842-856. DOI: 10.1055/s-0039-3399554. [23] GOLDFARB SB, LEVVEY BJ, EDWARDS LB, et al. The Registry of the International Society for Heart and Lung Transplantation: nineteenth pediatric lung and heart-lung transplantation report-2016; focus theme: primary diagnostic indications for transplant[J]. J Heart Lung Transplant, 2016, 35(10): 1196-1205. DOI: 10.1016/j.healun.2016.08.019. [24] 陈奥, 练巧燕, 徐鑫, 等. 肺移植治疗囊性纤维化1例并文献复习[J]. 器官移植, 2020, 11(3): 391-394, 423. DOI: 10.3969/j.issn.1674-7445.2020.03.012.CHEN A, LIAN QY, XU X, et al. Lung transplantation for cystic fibrosis: a case report and literature review[J]. Organ Transplant, 2020, 11(3): 391-394, 423. DOI: 10.3969/j.issn.1674-7445.2020.03.012. [25] BENZIMRA M, CALLIGARO GL, GLANVILLE AR. Acute rejection[J]. J Thorac Dis, 2017, 9(12): 5440-5457. DOI: 10.21037/jtd.2017.11.83. [26] BENDEN C. Pediatric lung transplantation[J]. J Thorac Dis, 2017, 9(8): 2675-2683. DOI: 10.21037/jtd.2017.07.84.