{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T11:05:03Z","timestamp":1793271903105,"version":"4.5.0"},"reference-count":22,"publisher":"Walter de Gruyter GmbH","issue":"1","license":[{"start":{"date-parts":[[2025,6,1]],"date-time":"2025-06-01T00:00:00Z","timestamp":1748736000000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/3.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,6,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Cystinuria is a rare, lifelong, autosomal recessive disorder characterized by high urine cystine excretion, leading to chronic and recurrent kidney stone formation. This inherited metabolic disorder occurs due to defective cystine, lysine, ornithine, and arginine reabsorption in the brush border membrane of the proximal renal tubule (S3 segment) and the gastrointestinal epithelial cells. Tiopronin is a thiol agent used in the treatment of severe homozygous cystinuria in patients who are resistant to conservative measures. We report an 18-year-old male with cystinuria confirmed by genetic testing, who was treated with conservative measures such as high fluid intake, low sodium diet, and oral potassium citrate since the diagnosis was made at four years of age. However, due to the recurrence of several calculi in both kidneys, tiopronin treatment was initiated at the dose of 250 mg three times a day. After three months of therapy, the patient developed nephrotic syndrome with proteinuria of 6.6 g\/l, hypoproteinemia, hyperlipidemia, and edema. Considering the adverse effect of tiopronin, the drug was immediately withdrawn and the patient was managed with human albumin substitution, diuretics, and angiotensin receptor blocker, without corticosteroids. Complete clinical and biochemical remission was achieved within 7 days. Clinicians should be aware of this rare but serious adverse effect of tiopronin, and monitor patients receiving tiopronin carefully for the possible occurrence of edema, proteinuria, with the aim of timely intervention and tiopronin discontinuation.<\/jats:p>","DOI":"10.2478\/bjmg-2025-0012","type":"journal-article","created":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T05:00:04Z","timestamp":1759986004000},"source":"Crossref","is-referenced-by-count":2,"title":["Nephrotic Syndrome Induced by Tiopronin in a Male Patient with Cystinuria"],"prefix":"10.2478","volume":"28","author":[{"given":"V","family":"Karanfilovski","sequence":"first","affiliation":[{"name":"University Hospital of Nephrology, Faculty of Medicine , Ss. Cyril and Methodius , Skopje , R. North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A","family":"Severova Stojanoska","sequence":"additional","affiliation":[{"name":"University Hospital of Nephrology, Faculty of Medicine , Ss. Cyril and Methodius , Skopje , R. North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V","family":"Ristovska","sequence":"additional","affiliation":[{"name":"University Hospital of Nephrology, Faculty of Medicine , Ss. Cyril and Methodius , Skopje , R. North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Gjorgjievska","sequence":"additional","affiliation":[{"name":"University Children Hospital, Faculty of Medicine , Ss. Cyril and Methodius , Skopje , R. North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V","family":"Tasic","sequence":"additional","affiliation":[{"name":"University Children Hospital, Faculty of Medicine , Ss. Cyril and Methodius , Skopje , R. North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T","family":"Arsov","sequence":"additional","affiliation":[{"name":"Faculty of Medical Sciences, Goce Delcev University , Shtip , North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P","family":"Dzekova-Vidimliski","sequence":"additional","affiliation":[{"name":"University Hospital of Nephrology, Faculty of Medicine , Ss. Cyril and Methodius , Skopje , R. North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D","family":"Plaseska-Karanfilska","sequence":"additional","affiliation":[{"name":"Research Center for Genetic engineering and Biotechnology\u201d Georgi Efremov\u201d Academy of Science and Arts , Skopje RN Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I","family":"Nikolov","sequence":"additional","affiliation":[{"name":"University Hospital of Nephrology, Faculty of Medicine , Ss. Cyril and Methodius , Skopje , R. North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N","family":"Gjorgjievski","sequence":"additional","affiliation":[{"name":"University Hospital of Nephrology, Faculty of Medicine , Ss. Cyril and Methodius , Skopje , R. North Macedonia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2025,10,8]]},"reference":[{"key":"2026042716344802137_j_bjmg-2025-0012_ref_001","doi-asserted-by":"crossref","unstructured":"Servais A, Thomas K, Dello Strologo L. et al. Cystinuria: clinical practice recommendation. Kidney Int. 2021;99(1):48\u201358.","DOI":"10.1016\/j.kint.2020.06.035"},{"key":"2026042716344802137_j_bjmg-2025-0012_ref_002","doi-asserted-by":"crossref","unstructured":"Chillar\u00f3n J, Font-Llitj\u00f3s M, Fort J, Zorzano A, Goldfarb DS, Nunes V, Palac\u00edn M. Pathophysiology and treatment of cystinuria. Nat Rev Nephrol. 2010 Jul;6(7):424\u2013434.","DOI":"10.1038\/nrneph.2010.69"},{"key":"2026042716344802137_j_bjmg-2025-0012_ref_003","doi-asserted-by":"crossref","unstructured":"Sahota A, Tischfield JA, Goldfarb DS, Ward MD, Hu L. Cystinuria: genetic aspects, mouse models, and a new approach to therapy. Urolithiasis. 2019;47(1):57\u201366.","DOI":"10.1007\/s00240-018-1101-7"},{"key":"2026042716344802137_j_bjmg-2025-0012_ref_004","doi-asserted-by":"crossref","unstructured":"Dello Strologo L, Pras E, Pontesilli C, Beccia E, Ricci-Barbini V, de Sanctis L, Ponzone A, Gallucci M et al. Comparison between SLC3A1 and SLC7A9 cystinuria patients and carriers: a need for a new classification. J Am Soc Nephrol. 2002 Oct;13(10):2547\u20132553.","DOI":"10.1097\/01.ASN.0000029586.17680.E5"},{"key":"2026042716344802137_j_bjmg-2025-0012_ref_005","unstructured":"Vidovi\u0107 L, Josipovi\u0107 J, \u0160enjug P. Tiopronin and\/or NSAID? A case of nephrotic syndrome and acute interstitial nephritis in a young woman with cystinuria. Acta Clin Croat (Suppl 1) 2021; 60: 125\u2013127."},{"key":"2026042716344802137_j_bjmg-2025-0012_ref_006","doi-asserted-by":"crossref","unstructured":"Zhong H, Wang L, Gu ZC, Cui M, Liu XY, Pang XY. Minimal change disease induced by tiopronin: a rare case report and a review of the literature. Ann Transl Med. 2019 Aug;7(16):398.","DOI":"10.21037\/atm.2019.07.42"},{"key":"2026042716344802137_j_bjmg-2025-0012_ref_007","doi-asserted-by":"crossref","unstructured":"Li XP, Wen F, Yang W, et al. The role of tiopronin for the prevention of chemotherapy-related liver toxicity in advanced colorectal cancer patients treated with mFOLFOX7: a prospective analysis. Tumori 2014;100:446\u2013451.","DOI":"10.1177\/1636.17908"},{"key":"2026042716344802137_j_bjmg-2025-0012_ref_008","unstructured":"Tang MC, Cheng L, Qiu L, et al. Efficacy of Tiopronin in treatment of severe non-alcoholic fatty liver disease. 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