Cystinuria].

Caroline Prot-Bertoye, Michel Daudon, Isabelle Tostivint, Marie-Paule Dousseaux, Jérôme Defazio, Olivier Traxer, Bertrand Knebelmann, Marie Courbebaisse

Nephrologie & therapeutique 2021 Apr

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Cystinuria is the most common monogenic nephrolithiasis disorder. Because of its poor solubility at a typical urine pH of less than 7, cystine excretion results in recurrent urinary cystine stone formation. A high prevalence of high blood pressure and of chronic kidney disease has been reported in these patients. Alkaline hyperdiuresis remains the cornerstone of the preventive medical treatment. To reach a urine pH between 7.5 and 8 and a urine specific gravity less than or equal to 1.005 should be the goal of medical treatment. D-penicillamine and tiopronin, two cysteine-binding thiol agents, should be considered as second line treatments with frequent adverse events that should be closely monitored. Copyright © 2020 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.

Citation

Caroline Prot-Bertoye, Michel Daudon, Isabelle Tostivint, Marie-Paule Dousseaux, Jérôme Defazio, Olivier Traxer, Bertrand Knebelmann, Marie Courbebaisse. Cystinuria]. Nephrologie & therapeutique. 2021 Apr;17S:S100-S107