Laboratory parameters during the treatment and follow-up of the patient
| Laboratory parameter | On admission | 7th day of hospitalization | After one month |
|---|---|---|---|
| 24 hours proteinuria (normal < 0.1 g/l) | 6.6 g/l | 0.18 g/l | 0.2 g/day |
| Serum albumin (normal 35–50 g/l ) | 19 g/l | 31 g/l | 49 g/l |
| Total proteins (normal 63–83 g/l) | 41 g/l | 56 g/l | 73 g/l |
| Total cholesterol (normal < 5.17 mmol/l) | 6.6 mmol/l | 4.8 mmol/l | 3.76 mmol/l |
| Serum creatinine (normal 45–109 μmol/l) | 75 μmol/l | 71 μmol/l | 73 μmol/l |
Reported cases of Tiopronin-induced nephrotic syndrome in patients with cystinuria
| Authors | Age (years) | sex | Dosage | Occurrence time after initiation of tiopronin | Clinical presentation | Renal biopsy findings | Therapy | Outcome |
|---|---|---|---|---|---|---|---|---|
| Our case | 18 | male | 10 mg/kg/day | 3 months | nephrotic syndrome with proteinuria of 6.6 g/l | No | tiopronin withdrawal | complete remission after 7 days |
| Tasic V et al (2011)16 | 3 | male | 10 mg/kg/day | 9 months | nephrotic syndrome | No | tiopronin withdrawal | complete remission after 10 days |
| Vidoviæ L et al (2021)5 | 22 | female | no data | 4 months | nephrotic syndrome with proteinuria of 4.4 g/day | acute interstitial nephritis | tiopronin withdrawal and prednisone 60 mg | remission with proteinuria 0.4 g/24 h after 7 days |
| Iwanaka Y et al (2024)18 | 13 | female | 9.1 mg/kg/day | 44 months | nephrotic syndrome | No | tiopronin withdrawal | complete remission after 10 days |
| Kalbani Al et al (2020)19 | 13 | male | 12 mg/kg/day | 3 months | nephrotic syndrome | No | tiopronin withdrawal | complete remission after 1 month |
| Micallef S et al (2022)20 | 16 | male | 12 mg/kg/day | 16 months | acute kidney injury and nephrotic syndrome with proteinuria 6 g/day | membranous nephropathy, likely drug-induced. | tiopronin withdrawal | serum 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)21 | 73 | male | no data | 6 weeks | severe nephrotic syndrome | minimal glomerular lesions | tiopronin withdrawal | complete remission 5 weeks after withdrawal |
| Shibasaki T et al (1990)22 | 26 | male | no data | 36 months | nephrotic syndrome | membranous glomerulonephritis (MN) at stage I | tiopronin withdrawal and prednisone | complete remission |
| Yamashita J et al (2024)14 | 41 | female | 600–1600 mg/day | 6 months | nephrotic syndrome with proteinuria of 5.5 g/gCre (normal < 0.2 g/gCre) | membranous nephropathy with strong deposition of immunoglobulin G4 | tiopronin withdrawal | remission 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)15 | 53 | male | no data | 7 months | nephrotic syndrome with proteinuria of 4.6 g/day and slightly elevated serum creatinine | Segmental membranous nephropathy, NELL-1 positive | tiopronin withdrawal, telmisartan and dapagliflozin | After 6 months normal serum creatinine and albumin, but a protein-to-creatinine ratio of 2.08 g/g (patient with diabetes, obesity) |