Case-based review of dietary management of cystinuria.

PURPOSE: The currently recommended treatment strategy for cystine stone formers is based on a progressive approach that starts with the most conservative measures. In patients with cystinuria, increased patient compliance with dietary management and medical treatment is associated with fewer stone interventions. In this case-based review, the dietary management of cystine stone former was reviewed under the guidance of evidence-based medicine. METHODS: The dietary management of the 13-year-old cystinuria patient, who underwent 18 endourological stone interventions, was reviewed in the light of evidence-based medicine. A literature search was performed in Pubmed, MEDLINE, Embase, and Cochrane Library databases according to PRISMA guidelines published from 1993 to September 2022. A total of 304 articles were included in this paper. RESULTS: In managing patients with cystinuria, hyperhydration, and alkalinization of the urine with medical treatment, the rational use of cystine-binding drugs by taking into account individual situations has come to the fore. A limited study has argued that a vegetarian diet is effective as the alkaline load from fruits and vegetables can reduce the amount of alkalizing substances required to achieve urinary alkalinization above pH 7.5, making it particularly suitable for the dietary treatment of cystine stone disease. CONCLUSION: Life-long follow-up with dietary modification, hyperhydration, and personalized medical therapy (alkalinization and cystine-binding drugs) are critical in preventing chronic kidney disease and kidney failure in cystinuria.