Volume 15 Issue 2
Mar.  2024
Turn off MathJax
Article Contents
Article Navigation >  ORGAN TRANSPLANTATION  > 2024  >  15(2): 257-262
Zhang Meng, Wang Yibin, Wang Yuchen, et al. Kidney transplantation from donors with Marfan syndrome: report of 2 cases and literature review[J]. ORGAN TRANSPLANTATION, 2024, 15(2): 257-262. doi: 10.3969/j.issn.1674-7445.2023239
Citation: Zhang Meng, Wang Yibin, Wang Yuchen, et al. Kidney transplantation from donors with Marfan syndrome: report of 2 cases and literature review[J]. ORGAN TRANSPLANTATION, 2024, 15(2): 257-262. doi: 10.3969/j.issn.1674-7445.2023239
shu

Kidney transplantation from donors with Marfan syndrome: report of 2 cases and literature review

doi: 10.3969/j.issn.1674-7445.2023239

  • Department of Organ Transplantation, Nanfang Hospital of Southern Medical University, Guangzhou 510515, China

More Information
  • Corresponding author: Miao Yun, Email:miaoyunecho@126.com
  • Received Date: 2023-11-07
  • Accepted Date: 2023-12-25
    • Available Online: 2024-01-05
  • Publish Date: 2024-03-15
    Abstract
  •   Objective  To investigate the feasibility and clinical experience of kidney transplantation from donors with Marfan syndrome (MFS).   Methods  Clinical data of 2 recipients undergoing kidney transplantation from the same MFS patient were retrospectively analyzed and literature review of 2 cases was conducted. Characteristics and clinical diagnosis and treatment of kidney transplantation from MFS patients were summarized.   Results  The Remuzzi scores of the left and right donor kidneys of the MFS patient during time-zero biopsy were 1 and 2. No significant difference was observed in the renal arteriole wall compared with other donors of brain death and cardiac death. Two recipients who received kidney transplantation from the MFS patient suffered from postoperative delayed graft function. After short-term hemodialysis, the graft function of the recipients received the left and right kidney began to gradually recover at postoperative 10 d and 20 d. After discharge, serum creatinine level of the recipient received the left kidney was ranged from 80 to 90 μmol/L, whereas that of the recipient received the right kidney kept declining, and the lowest serum creatinine level was 232 μmol/L before the submission date (at postoperative 43 d). Through literature review, two cases successfully undergoing kidney transplantation from the same MFS donor were reported. Both two recipients experienced delayed graft function, and then renal function was restored to normal. Until the publication date, 1 recipient has survived for 6 years, and the other recipient died of de novo cerebrovascular disease at postoperative 2 years.   Conclusions  MFS patients may serve as an acceptable source of kidney donors. However, the willingness and general conditions of the recipients should be carefully evaluated before kidney transplantation. Intraoperatively, potential risk of tear of renal arterial media should be properly treated. Extensive attention should be paid to the incidence of postoperative complications.

     

    • FullText(HTML)
  • loading
    • References(38)
  • [1]
    CONNOLLY HM, NIAZ T, BOWEN JM. What is Marfan syndrome?[J]. JAMA, 2023, 329(18): 1618. DOI: 10.1001/jama.2023.3826.
    [2]
    SAKAI LY, KEENE DR, RENARD M, et al. FBN1: the disease-causing gene for Marfan syndrome and other genetic disorders[J]. Gene, 2016, 591(1): 279-291. DOI: 10.1016/j.gene.2016.07.033.
    [3]
    MILEWICZ DM, BRAVERMAN AC, DE BACKER J, et al. Marfan syndrome[J]. Nat Rev Dis Primers, 2021, 7(1): 64. DOI: 10.1038/s41572-021-00298-7.
    [4]
    ZEIGLER SM, SLOAN B, JONES JA. Pathophysiology and pathogenesis of Marfan syndrome[J]. Adv Exp Med Biol, 2021, 1348: 185-206. DOI: 10.1007/978-3-030-80614-9_8.
    [5]
    DU Q, ZHANG D, ZHUANG Y, et al. The molecular genetics of Marfan syndrome[J]. Int J Med Sci, 2021, 18(13): 2752-2766. DOI: 10.7150/ijms.60685.
    [6]
    PEETERS S, DE KINDEREN P, MEESTER JAN, et al. The fibrillinopathies: new insights with focus on the paradigm of opposing phenotypes for both FBN1 and FBN2[J]. Hum Mutat, 2022, 43(7): 815-831. DOI: 10.1002/humu.24383.
    [7]
    赵立玲, 刘升平, 胡文沐, 等. FBN1基因新变异所致马凡综合征患儿1例的分析[J]. 中华医学遗传学杂志, 2023, 40(1): 62-65. DOI: 10.3760/cma.j.cn511374-20210630-00554.

    ZHAO LL, LIU SP, HU WM, et al. Analysis of a child with Marfan syndrome due to a novel variant of FBN1 gene[J]. Chin J Med Genet, 2023, 40(1): 62-65. DOI: 10.3760/cma.j.cn511374-20210630-00554.
    [8]
    ȘULEA CM, MÁRTONFALVI Z, CSÁNYI C, et al. Nanoscale structural comparison of fibrillin-1 microfibrils isolated from Marfan and non-Marfan syndrome human aorta[J]. Int J Mol Sci, 2023, 24(8): 7561. DOI: 10.3390/ijms24087561.
    [9]
    CHEN ZX, JIA WN, JIANG YX. Genotype-phenotype correlations of Marfan syndrome and related fibrillinopathies: phenomenon and molecular relevance[J]. Front Genet, 2022, 13: 943083. DOI: 10.3389/fgene.2022.943083.
    [10]
    WANG L, XU X, ZHANG M, et al. Prevalence of chronic kidney disease in china: results from the sixth china chronic disease and risk factor surveillance[J]. JAMA Intern Med, 2023, 183(4): 298-310. DOI: 10.1001/jamainternmed.2022.6817.
    [11]
    黄洁夫. 中国器官移植发展报告(2019)[M]. 北京: 清华大学出版社, 2020.
    [12]
    HU XP, YIN H, ZHANG XD. Evaluation and treatment of marginal grafts with surgical diseases in kidney transplantation[J]. Chin Med J (Engl), 2012, 125(3): 461-464.
    [13]
    SHARMA N, MAHAJAN A, QAZI YA. Marginal kidney transplantation: the road less traveled[J]. Curr Opin Organ Transplant, 2019, 24(1): 92-96. DOI: 10.1097/MOT.0000000000000603.
    [14]
    FARESE S, VOGT B, FREY FJ, et al. Successful kidney transplantation from donor with Marfan's syndrome[J]. Am J Transplant, 2006, 6(8): 1972-1974. DOI: 10.1111/j.1600-6143.2006.01389.x.
    [15]
    MILLERON O, ARNOULT F, DELORME G, et al. Pathogenic FBN1 genetic variation and aortic dissection in patients with Marfan syndrome[J]. J Am Coll Cardiol, 2020, 75(8): 843-853. DOI: 10.1016/j.jacc.2019.12.043.
    [16]
    ROMAN MJ, DEVEREUX RB. Aortic dissection risk in Marfan syndrome[J]. J Am Coll Cardiol, 2020, 75(8): 854-856. DOI: 10.1016/j.jacc.2019.12.042.
    [17]
    BRAVERMAN AC. Aortic dissection in Marfan syndrome: protect the tube of life[J]. J Am Coll Cardiol, 2023,DOI: 10.1016/j.jacc.2023.08.054[Epub ahead of print
    [18]
    VON KODOLITSCH Y, DEMOLDER A, GIRDAUSKAS E, et al. Features of Marfan syndrome not listed in the Ghent nosology - the dark side of the disease[J]. Expert Rev Cardiovasc Ther, 2019, 17(12): 883-915. DOI: 10.1080/14779072.2019.1704625.
    [19]
    STENGL R, ÁGG B, SZILVESZTER B, et al. Case report: morphological characterization and long-term observation of bilateral sequential internal mammary artery aneurysms in a patient with confirmed FBN1 mutation[J]. Front Cardiovasc Med, 2021, 8: 697591. DOI: 10.3389/fcvm.2021.697591.
    [20]
    AWAIS M, WILLIAMS DM, DEEB GM, et al. Aneurysms of medium-sized arteries in Marfan syndrome[J]. Ann Vasc Surg, 2013, 27(8): 1188.e5-e7. DOI: 10.1016/j.avsg.2012.12.002.
    [21]
    DE SOUZA RB, LEMES RB, FORESTO-NETO O, et al. Extracellular matrix and vascular dynamics in the kidney of a murine model for Marfan syndrome[J]. PLoS One, 2023, 18(5): e0285418. DOI: 10.1371/journal.pone.0285418.
    [22]
    BOSEMAN P, LEWIN M, DILLON J, et al. Marfan syndrome, MPGN, and bacterial endocarditis[J]. Am J Kidney Dis, 2008, 51(4): 697-701. DOI: 10.1053/j.ajkd.2007.08.031.
    [23]
    CHOW K, PYERITZ RE, LITT HI. Abdominal visceral findings in patients with Marfan syndrome[J]. Genet Med, 2007, 9(4): 208-212. DOI: 10.1097/gim.0b013e3180423cb3.
    [24]
    BOULETI C, FLAMANT M, ESCOUBET B, et al. Risk of ascending aortic aneurysm in patients with autosomal dominant polycystic kidney disease[J]. Am J Cardiol, 2019, 123(3): 482-488. DOI: 10.1016/j.amjcard.2018.10.030.
    [25]
    SBAR GD, VENKATASESHAN VS, HUANG Z, et al. Renal disease in Marfan syndrome[J]. Am J Nephrol, 1996, 16(4): 320-326. DOI: 10.1159/000169017.
    [26]
    LI L, LIAO J, YUAN Q, et al. Fibrillin-1-enriched microenvironment drives endothelial injury and vascular rarefaction in chronic kidney disease[J]. Sci Adv, 2021, 7(5): eabc7170. DOI: 10.1126/sciadv.abc7170.
    [27]
    MIAO C, ZHU X, WEI X, et al. Pro- and anti-fibrotic effects of vascular endothelial growth factor in chronic kidney diseases[J]. Ren Fail, 2022, 44(1): 881-892. DOI: 10.1080/0886022X.2022.2079528.
    [28]
    HARTNER A, SCHAEFER L, PORST M, et al. Role of fibrillin-1 in hypertensive and diabetic glomerular disease[J]. Am J Physiol Renal Physiol, 2006, 290(6): F1329-F1336. DOI: 10.1152/ajprenal.00284.2005.
    [29]
    中华医学会器官移植学分会. 肾移植术后移植物功能延迟恢复诊疗技术规范(2019版)[J]. 器官移植, 2019, 10(5): 521-525. DOI: 10.3969/j.issn.1674-7445.2019.05.010.

    Branch of Organ Transplantation of Chinese Medical Association. Technical specification for the diagnosis and treatment on delayed graft function after renal transplantation (2019 edition)[J]. Organ Transplant, 2019, 10(5): 521-525. DOI: 10.3969/j.issn.1674-7445.2019.05.010.
    [30]
    WARMUZIŃSKA N, ŁUCZYKOWSKI K, BOJKO B. A review of current and emerging trends in donor graft-quality assessment techniques[J]. J Clin Med, 2022, 11(3): 487. DOI: 10.3390/jcm11030487.
    [31]
    VILLANEGO F, VIGARA LA, CAZORLA JM, et al. Evaluation of expanded criteria donors using the kidney donor profile index and the preimplantation renal biopsy[J]. Transpl Int, 2022, 35: 10056. DOI: 10.3389/ti.2022.10056.
    [32]
    SHARIF A. Deceased donor characteristics and kidney transplant outcomes[J]. Transpl Int, 2022, 35: 10482. DOI: 10.3389/ti.2022.10482.
    [33]
    HAI Y, CHONG W, LIU JB, et al. The diagnostic value of contrast-enhanced ultrasound for monitoring complications after kidney transplantation-a systematic review and meta-analysis[J]. Acad Radiol, 2021, 28(8): 1086-1093. DOI: 10.1016/j.acra.2020.05.009.
    [34]
    BAHL D, HADDAD Z, DATOO A, et al. Delayed graft function in kidney transplantation[J]. Curr Opin Organ Transplant, 2019, 24(1): 82-86. DOI: 10.1097/MOT.0000000000000604.
    [35]
    GAO F, CHEN Z, GAO F, et al. Spontaneous renal artery dissection complicated by renal infarction: description of two cases[J]. Quant Imaging Med Surg, 2022, 12(10): 4972-4978. DOI: 10.21037/qims-22-342.
    [36]
    CAÑO-VELASCO J, POLANCO-PUJOL L, GONZÁLEZ-GARCÍA J, et al. Renal artery infectious (mycotic) pseudoaneurysms in renal transplantation recipients[J]. Actas Urol Esp (Engl Ed), 2021, 45(5): 335-344. DOI: 10.1016/j.acuroe.2021.04.009.
    [37]
    王湘, 李刚, 马琼, 等. 马凡综合征合并主动脉夹层患者的外科治疗[J]. 岭南心血管病杂志, 2022, 28(5): 423-426. DOI: 10.3969/j.issn.1007-9688.2022.05.08.

    WANG X, LI G, MA Q, et al. Surgical treatment of patients with Marfan syndrome and aortic dissection[J]. South China J Cardiovasc Dis, 2022, 28(5): 423-426. DOI: 10.3969/j.issn.1007-9688.2022.05.08.
    [38]
    MAYHEW M, SOLOMON R, LAGUARDIA H, et al. Vascular complications in renal transplantation: surgical salvage of renal artery dissection[J]. Transplant Direct, 2022, 8(6): e1340. DOI: 10.1097/TXD.00000000000 01340.
    • Relative Articles
    • Supplements(0)
    • Cited By
  • 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(8)

    Get Citation
    PDF
    XML

    Article Metrics

    Article views (217) PDF downloads(36) Cited by()
    Proportional views
    Related

    WeChat Subscription Account

    Wechat Service Account

    Contact Us
    • Tel：020-38736410
    • Email: organtranspl@163.com
    • Address：The Third Affiliated Hospital of Sun Yat-sen University, No. 600, Tianhe Road, Tianhe District, Guangzhou
    • Editorial Office of Organ Transplantation  粤ICP备2021030844号
    Links

    Supported by: Beijing Renhe Information Technology Co. Ltd    

    /

    DownLoad:  Full-Size Img  PowerPoint
    Return
    Return