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Nephrotic Syndrome Induced by Tiopronin in a Male Patient with Cystinuria

Open Access
|Oct 2025

Figures & Tables

Laboratory parameters during the treatment and follow-up of the patient

Laboratory parameterOn admission7th day of hospitalizationAfter one month
24 hours proteinuria (normal < 0.1 g/l)6.6 g/l0.18 g/l0.2 g/day
Serum albumin (normal 35–50 g/l )19 g/l31 g/l49 g/l
Total proteins (normal 63–83 g/l)41 g/l56 g/l73 g/l
Total cholesterol (normal < 5.17 mmol/l)6.6 mmol/l4.8 mmol/l3.76 mmol/l
Serum creatinine (normal 45–109 μmol/l)75 μmol/l71 μmol/l73 μmol/l

Reported cases of Tiopronin-induced nephrotic syndrome in patients with cystinuria

AuthorsAge (years)sexDosageOccurrence time after initiation of tioproninClinical presentationRenal biopsy findingsTherapyOutcome
Our case18male10 mg/kg/day3 monthsnephrotic syndrome with proteinuria of 6.6 g/lNotiopronin withdrawalcomplete remission after 7 days
Tasic V et al (2011)163male10 mg/kg/day9 monthsnephrotic syndromeNotiopronin withdrawalcomplete remission after 10 days
Vidoviæ L et al (2021)522femaleno data4 monthsnephrotic syndrome with proteinuria of 4.4 g/dayacute interstitial nephritistiopronin withdrawal and prednisone 60 mgremission with proteinuria 0.4 g/24 h after 7 days
Iwanaka Y et al (2024)1813female9.1 mg/kg/day44 monthsnephrotic syndromeNotiopronin withdrawalcomplete remission after 10 days
Kalbani Al et al (2020)1913male12 mg/kg/day3 monthsnephrotic syndromeNotiopronin withdrawalcomplete remission after 1 month
Micallef S et al (2022)2016male12 mg/kg/day16 monthsacute kidney injury and nephrotic syndrome with proteinuria 6 g/daymembranous nephropathy, likely drug-induced.tiopronin withdrawalserum creatinine dropped to 117 μmol/l and albumin:creatinine ratio to 47.7 mg/g (normal range 1–20 mg/g) within 2 months
Lecoules S et al (1999)2173maleno data6 weekssevere nephrotic syndromeminimal glomerular lesionstiopronin withdrawalcomplete remission 5 weeks after withdrawal
Shibasaki T et al (1990)2226maleno data36 monthsnephrotic syndromemembranous glomerulonephritis (MN) at stage Itiopronin withdrawal and prednisonecomplete remission
Yamashita J et al (2024)1441female600–1600 mg/day6 monthsnephrotic syndrome with proteinuria of 5.5 g/gCre (normal < 0.2 g/gCre)membranous nephropathy with strong deposition of immunoglobulin G4tiopronin withdrawalremission after 1 year. After 5 years tiopronin was reintroduced, nephritic syndrome appeared again, and tiopronin was stopped with remission after 6 months
Santoriello D et al (2023)1553maleno data7 monthsnephrotic syndrome with proteinuria of 4.6 g/day and slightly elevated serum creatinineSegmental membranous nephropathy, NELL-1 positivetiopronin withdrawal, telmisartan and dapagliflozinAfter 6 months normal serum creatinine and albumin, but a protein-to-creatinine ratio of 2.08 g/g (patient with diabetes, obesity)
Language: English
Published on: Oct 8, 2025
Published by: Macedonian Academy of Sciences and Arts
In partnership with: Paradigm Publishing Services
Publication frequency: 2 times per year

© 2025 V Karanfilovski, A Severova Stojanoska, V Ristovska, J Gjorgjievska, V Tasic, T Arsov, P Dzekova-Vidimliski, D Plaseska-Karanfilska, I Nikolov, N Gjorgjievski, published by Macedonian Academy of Sciences and Arts
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.