Volume 11 Issue 3
May  2020
Turn off MathJax
Article Contents
Chen Ao, Lian Qiaoyan, Xu Xin, et al. Lung transplantation for cystic fibrosis: a case report and literature review[J]. ORGAN TRANSPLANTATION, 2020, 11(3): 391-394,423. doi: 10.3969/j.issn.1674-7445.2020.03.012
Citation: Chen Ao, Lian Qiaoyan, Xu Xin, et al. Lung transplantation for cystic fibrosis: a case report and literature review[J]. ORGAN TRANSPLANTATION, 2020, 11(3): 391-394,423. doi: 10.3969/j.issn.1674-7445.2020.03.012

Lung transplantation for cystic fibrosis: a case report and literature review

doi: 10.3969/j.issn.1674-7445.2020.03.012
More Information
  • Corresponding author: Ju Chunrong, E-mail:juchunrong@126.com
  • Available Online: 2021-01-19
  • Publish Date: 2020-05-15
  •   Objective   To investigate the clinical efficacy and prognosis of lung transplantation in the treatment of cystic fibrosis (CF).   Methods   Clinical data of one patient with end-stage CF undergoing allogeneic bilateral lung transplantation were retrospectively analyzed. Clinical characteristics, diagnostic methods and treatment strategies of the CF recipient were summarized.  Results   The recipient had suffered from relevant symptoms since childhood including repeated cough and purulent sputum for 30 years, complicated with recurrent pulmonary infection combined with acute exacerbation, chronic sinusitis and extremely severe malnutrition. Prior to lung transplantation, the patient had to depend upon the invasive ventilator due to respiratory muscle weakness, and admitted to intensive care unit (ICU) for a long time. Imaging examination revealed multiple cystic columnar bronchiectasis accompanied with infection in bilateral lungs. The diagnosis of CF was further confirmed by sweat test and gene detection. The recipient underwent bilateral lung transplantation on August 17, 2017 and received rehabilitation treatment. The lung function was gradually restored to normal. The recipient had obtained the same quality of life to the healthy counterparts since the date of manuscript submission (over 2 years).   Conclusions   Lung transplantation is an efficacious treatment for end-stage CF, which can not only save patients' lives, but also significantly improve the quality of life of patients.

     

  • loading
  • [1]
    ELBORN JS. Cystic fibrosis[J]. Lancet, 2016, 388(10059): 2519-2531. DOI: 10.1016/S0140-6736(16)00576-6.
    [2]
    REY MM, BONK MP, HADJILIADIS D. Cystic fibrosis: emerging understanding and therapies[J]. Annu Rev Med, 2019, 70:197-210. DOI: 10.1146/annurev-med-112717-094536.
    [3]
    WIENCEK JR, LO SF. Advances in the diagnosis and management of cystic fibrosis in the genomic era[J]. Clin Chem, 2018, 64(6):898-908. DOI: 10.1373/clinchem. 2017.274670.
    [4]
    ROSENBLATT RL. Lung transplantation in cystic fibrosis[J]. Respir Care, 2009, 54(6):777-787. doi: 10.4187/002013209790983197
    [5]
    RATJEN F, DÖRING G. Cystic fibrosis[J]. Lancet, 2003, 361(9358):681-689. doi: 10.1016/S0140-6736(03)12567-6
    [6]
    KEREM B, ROMMENS JM, BUCHANAN JA, et al. Identification of the cystic fibrosis gene: genetic analysis[J]. Science, 1989, 245(4922):1073-1080. doi: 10.1126/science.2570460
    [7]
    FARRELL PM, WHITE TB, REN CL, et al. Diagnosis of cystic fibrosis: consensus guidelines from the cystic fibrosis foundation[J]. J Pediatr, 2017, 181S:S4-S15. DOI: 10.1016/j.jpeds.2016.09.064.
    [8]
    DE BOECK K, ZOLIN A, CUPPENS H, et al. The relative frequency of CFTR mutation classes in European patients with cystic fibrosis[J]. J Cyst Fibros, 2014, 13(4):403-409. DOI: 10.1016/j.jcf.2013.12.003.
    [9]
    SINGH M, REBORDOSA C, BERNHOLZ J, et al. Epidemiology and genetics of cystic fibrosis in Asia: in preparation for the next-generation treatments[J]. Respirology, 2015, 20(8):1172-1181. DOI: 10.1111/resp.12656.
    [10]
    CASTELLANI C, CUPPENS H, MACEK M JR, et al. Consensus on the use and interpretation of cystic fibrosis mutation analysis in clinical practice[J]. J Cyst Fibros, 2008, 7(3):179-196. DOI: 10.1016/j.jcf.2008.03.009.
    [11]
    MORRAL N, BERTRANPETIT J, ESTIVILL X, et al. The origin of the major cystic fibrosis mutation (delta F508) in European populations[J]. Nat Genet, 1994, 7(2):169-175. doi: 10.1038/ng0694-169
    [12]
    BELL SC, MALL MA, GUTIERREZ H, et al. The future of cystic fibrosis care: a global perspective[J]. Lancet Respir Med, 2020, 8(1):65-124. DOI: 10.1016/S2213-2600(19)30337-6.
    [13]
    SANTI C, GREENE CM. Challenges facing microRNA therapeutics for cystic fibrosis lung disease[J]. Epigenomics, 2020, 12(3): 179-181. DOI: 10.2217/epi-2019-0395.
    [14]
    DAVIS PB. Cystic fibrosis since 1938[J]. Am J Respir Crit Care Med, 2006, 173(5):475-482. doi: 10.1164/rccm.200505-840OE
    [15]
    MURPHY KP, MAHER MM, O'CONNOR OJ. Imaging of cystic fibrosis and pediatric bronchiectasis[J]. AJR Am J Roentgenol, 2016, 206(3):448-454. DOI: 10.2214/AJR.15.14437.
    [16]
    LEWIS PA, MORISON S, DODGE JA, et al. Survival estimates for adults with cystic fibrosis born in the United Kingdom between 1947 and 1967. the UK CysticFibrosis Survey Management Committee[J]. Thorax, 1999, 54(5):420-422. doi: 10.1136/thx.54.5.420
    [17]
    SHEN Y, LIU J, ZHONG L, et al. Clinical phenotypes and genotypic spectrum of cystic fibrosis in Chinese children[J]. J Pediatr, 2016, 171:269-276. DOI: 10.1016/ j.jpeds.2015.12.025.
    [18]
    SAVANT AP, MCCOLLEY SA. Cystic fibrosis year in review 2018, part 1[J]. Pediatr Pulmonol, 2019, 54(8):1117- 1128. DOI: 10.1002/ppul.24361.
    [19]
    MARTINIANO SL, TOPRAK D, ONG T, et al. Highlights from the 2017 North American Cystic Fibrosis Conference[J]. Pediatr Pulmonol, 2018, 53(7):979-986. DOI: 10.1002/ppul.24000.
    [20]
    RAFEEQ MM, MURAD HAS. Cystic fibrosis: current therapeutic targets and future approaches[J]. J Transl Med, 2017, 15(1):84. DOI: 10.1186/s12967-017-1193-9.
    [21]
    STRUG LJ, STEPHENSON AL, PANJWANI N, et al. Recent advances in developing therapeutics for cystic fibrosis[J]. Hum Mol Genet, 2018, 27(R2):R173-R186. DOI: 10.1093/hmg/ddy188.
    [22]
    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 heartlung transplant report-2018; focus theme: multiorgantransplantation[J]. J Heart Lung Transplant, 2018, 37(10):1169-1183. DOI: 10.1016/j.healun.2018.07.020.
    [23]
    RAMOS KJ, QUON BS, HELTSHE SL, et al. Heterogeneity in survival in adult patients with cystic fibrosis with FEV1 < 30% of predicted in the United States[J]. Chest, 2017, 151(6):1320-1328. DOI: 10.1016/j.chest.2017.01.019.
    [24]
    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.
    [25]
    HIRCHE TO, KNOOP C, HEBESTREIT H, et al. Practical guidelines: lung transplantation in patients with cystic fibrosis[J]. Pulm Med, 2014:621342. DOI: 10.1155/2014/621342.
    [26]
    SNELL G, REED A, STERN M, et al. The evolution of lung transplantation for cystic fibrosis: a 2017 update[J]. J Cyst Fibros, 2017, 16(5):553-564. DOI: 10.1016/j.jcf. 2017.06.008.
  • 加载中

Catalog

    通讯作者: 陈斌, bchen63@163.com
    • 1. 

      沈阳化工大学材料科学与工程学院 沈阳 110142

    1. 本站搜索
    2. 百度学术搜索
    3. 万方数据库搜索
    4. CNKI搜索

    Figures(2)

    Article Metrics

    Article views (230) PDF downloads(21) Cited by()
    Proportional views
    Related

    /

    DownLoad:  Full-Size Img  PowerPoint
    Return
    Return