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    Perioperative risk assessment for successful kidney transplant in leigh syndrome: a case report

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    Author
    Ducharlet, K; Thyagarajan, D; Ierino, F; McMahon, LP; Lee, D
    Date
    2018-02-01
    Source Title
    BMC Nephrology
    Publisher
    BIOMED CENTRAL LTD
    University of Melbourne Author/s
    Ducharlet, Kathryn; Ierino, Francesco
    Affiliation
    Medicine and Radiology
    Medical Education
    Metadata
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    Document Type
    Journal Article
    Citations
    Ducharlet, K., Thyagarajan, D., Ierino, F., McMahon, L. P. & Lee, D. (2018). Perioperative risk assessment for successful kidney transplant in leigh syndrome: a case report. BMC NEPHROLOGY, 19 (1), https://doi.org/10.1186/s12882-018-0816-6.
    Access Status
    Open Access
    URI
    http://hdl.handle.net/11343/256012
    DOI
    10.1186/s12882-018-0816-6
    Abstract
    BACKGROUND: Leigh syndrome (LS) is a rare neurodegenerative mitochondrial disorder which typically presents in childhood but has a varied clinical course. Renal involvement such as proximal tubulopathy in patients with mitochondrial disorders has been described. However, end stage renal disease (ESRD) is uncommon and literature regarding patients undergoing kidney transplantation is limited. Successful deceased donor renal transplant has not been previously described in a patient with Leigh Syndrome. CASE PRESENTATION: We report a 21-year-old Han Chinese man who presented with limb weakness and unsteady gait, which progressed rapidly over a period of months until he was wheelchair-bound. He subsequently developed ESRD and was commenced on hemodialysis. Investigations revealed a m.13513G > A mutation with clinical and radiological features consistent with LS. His mitochondrial disease stabilised and he underwent a multidisciplinary assessment for deceased donor kidney transplantation to identify and minimise the LS-associated perioperative risks and potential negative effects of immunosuppressants on his LS. Successful kidney transplantation followed with excellent graft function three and a half years post-transplant and improvement in the patient's physical function. CONCLUSION: This case highlights the importance of careful pre-transplant perioperative risk assessment and post-transplant care in a rare and heterogeneous neurological disease to achieve an ultimately excellent clinical outcome. To our knowledge, this is the first report of successful deceased donor kidney transplant in a patient with known LS.

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