Francesca Caprio, Giuseppe Orefice, Floriana Secondulfo, Filippo Carone Fabiani, A. Iervolino, Giovambattista Capasso, M. Simeoni, M. Zacchia, F. Trepiccione, G. Capolongo
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Renal cystinuria and immune cells (T lymphocytes) dysfunction, what we know about?
Cystinuria (CYS) is the most common monogenic kidney stone disease. Starting from an unusual case of CYS associated to Primary Sclerosing Cholangitis, inflammatory bowel disease (IBD) and autoimmune hepatitis in a young male, we carefully review the literature and propose here a working hypothesis regarding the potential risk of cystinuric patients to develop conditions due to immune system dysregulation. To corroborate this hypothesis, we retrospectively evaluate the frequency of dysimmunity in a cohort of cystinuric patients compared to healthy and disease controls. Further studies are needed to define the relationship between proximal tubular transport defect of CYS and dysregulated immunity.
期刊介绍:
''Nephron'' comprises three sections, which are each under the editorship of internationally recognized leaders and served by specialized Associate Editors. Apart from high-quality original research, ''Nephron'' publishes invited reviews/minireviews on up-to-date topics. Papers undergo an innovative and transparent peer review process encompassing a Presentation Report which assesses and summarizes the presentation of the paper in an unbiased and standardized way.