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    Controversies and research agenda in nephropathic cystinosis: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference

    Access Status
    Fulltext not available
    Authors
    Langman, C.
    Barshop, B.
    Deschênes, G.
    Emma, F.
    Goodyer, P.
    Lipkin, G.
    Midgley, J.
    Ottolenghi, C.
    Servais, A.
    Soliman, N.
    Thoene, J.
    Levtchenko, E.
    Amon, O.
    Ariceta, G.
    Basurto, M.
    Belmont-Martínez, L.
    Bertholet-Thomas, A.
    Bos, M.
    Brown, T.
    Cherqui, S.
    Cornelissen, E.
    Del Monte, M.
    Ding, J.
    Dohil, R.
    Doyle, M.
    Elenberg, E.
    Gahl, W.
    Gomez, V.
    Greco, M.
    Greeley, C.
    Greenbaum, L.
    Grimm, P.
    Hohenfellner, K.
    Holm, T.
    Hotz, V.
    Janssen, M.
    Kaskel, F.
    Magriço, R.
    Nesterova, G.
    Newsholme, Philip
    Niaudet, P.
    Rioux, P.
    Sarwal, M.
    Schneider, J.
    Topaloglu, R.
    Trauner, D.
    Vaisbich, M.
    van den Heuvel, L.
    Van't Hoff, W.
    Date
    2016
    Type
    Journal Article
    
    Metadata
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    Citation
    Langman, C. and Barshop, B. and Deschênes, G. and Emma, F. and Goodyer, P. and Lipkin, G. and Midgley, J. et al. 2016. Controversies and research agenda in nephropathic cystinosis: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference. Kidney International. 89 (6): pp. 1192-1203.
    Source Title
    Kidney International
    DOI
    10.1016/j.kint.2016.01.033
    ISSN
    0085-2538
    School
    School of Biomedical Sciences
    URI
    http://hdl.handle.net/20.500.11937/30119
    Collection
    • Curtin Research Publications
    Abstract

    Nephropathic cystinosis is an autosomal recessive metabolic, lifelong disease characterized by lysosomal cystine accumulation throughout the body that commonly presents in infancy with a renal Fanconi syndrome and, if untreated, leads to end-stage kidney disease (ESKD) in the later childhood years. The molecular basis is due to mutations in CTNS, the gene encoding for the lysosomal cystine-proton cotransporter, cystinosin. During adolescence and adulthood, extrarenal manifestations of cystinosis develop and require multidisciplinary care. Despite substantial improvement in prognosis due to cystine-depleting therapy with cysteamine, no cure of the disease is currently available. Kidney Disease: Improving Global Outcomes (KDIGO) convened a Controversies Conference on cystinosis to review the state-of-the-art knowledge and to address areas of controversies in pathophysiology, diagnostics, monitoring, and treatment in different age groups. More importantly, promising areas of investigation that may lead to optimal outcomes for patients afflicted with this lifelong, systemic disease were discussed with a research agenda proposed for the future.

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