Page last updated: 2024-11-05

tiopronin and Cystinuria

tiopronin has been researched along with Cystinuria in 74 studies

Tiopronin: Sulfhydryl acylated derivative of GLYCINE.

Cystinuria: An inherited disorder due to defective reabsorption of CYSTINE and other BASIC AMINO ACIDS by the PROXIMAL RENAL TUBULES. This form of aminoaciduria is characterized by the abnormally high urinary levels of cystine; LYSINE; ARGININE; and ORNITHINE. Mutations involve the amino acid transport protein gene SLC3A1.

Research Excerpts

ExcerptRelevanceReference
"Appropriate dosing of cystine-binding thiol drugs in the management of cystinuria has been based on clinical stone activity."9.30Effect of increasing doses of cystine-binding thiol drugs on cystine capacity in patients with cystinuria. ( Asplin, JR; Enders, FT; Goldfarb, DS; Malieckal, DA; Mara, K; Modersitzki, F, 2019)
"Thirty two patients with cystinuria were enrolled in this long-term study and 16 patients were treated with tiopronin for 24 weeks."9.10[Long-term study of tiopronin in patients with cystinuria]. ( Koide, T; Miyake, O; Okuyama, A; Yachiku, S; Yoshioka, T, 2003)
" We have studied the effect of a sodium-restricted diet on the urinary excretion of cystine in 13 patients with cystinuria, 7 of whom were treated with the SH compound tiopronin (2-mercaptopropionylglycine)."9.08The effect of sodium intake on cystinuria with and without tiopronin treatment. ( Denneberg, T; Edholm, E; Jeppsson, JO; Lindell, A, 1995)
"We report the results of a biochemical evaluation of long-term treatment of cystinuria with the SH compound tiopronin (2-mercaptopropionylglycine)."9.08Urinary excretion of free cystine and the tiopronin-cysteine-mixed disulfide during long term tiopronin treatment of cystinuria. ( Denneberg, T; Jeppsson, JO; Lindell, A, 1995)
" Pediatricians should be aware of this adverse effect of tiopronin, and therefore, testing of the urine with strips or sulfosalicylic acid at least once weekly at home may be very helpful for early detection of proteinuria."7.77Nephrotic syndrome occurring during tiopronin treatment for cystinuria. ( Gucev, Z; Lozanovski, VJ; Ristoska-Bojkovska, N; Sahpazova, E; Tasic, V, 2011)
"A case of nephrotic syndrome with ascites and heart failure in a patient who had received tiopronin for the treatment of cystinuria is presented."7.71[Nephrotic syndrome and anasarca status, secondary to treatment with tiopronin in a case of cystinuria]. ( Alvarez Navascués, R; Herrera Pérez de Villar, J; Rodríguez Suarez, C; Suarez Heiva, M; Vidau Argüelles, P, 2001)
"From 1987 to 1990, 719 subjects (385 males and 334 females) were screened, by means of urocystin-kit, for cystinuria and cystine lithiasis, in Epirus district (North-West Greece)."7.69[Results of treatment of cystinuria and cystine lithiasis with alpha-mercaptopropionylglycine. Apropos of 40 patients]. ( Chambilomatis, P; Evangelou, A; Giannakis, D; Giannakopoulos, X; Kalfakakou, V; Kallistratos, G; Karkabounas, S; Tsoumanis, P, 1994)
"We have compared 2-mercaptopropionylglycine (MPG) with D-penicillamine in its capacity to reduce the urinary excretion of cystine by patients with cystinuria."7.67Comparison of 2-mercaptopropionylglycine and D-penicillamine in the treatment of cystinuria. ( Cusworth, DC; Harbar, JA; Lawes, LC; Wrong, OM, 1986)
" Drug dosage was adjusted to maintain free urine cystine level below 100 micromol/mmol creatinine."6.73Cystinuria in children and young adults: success of monitoring free-cystine urine levels. ( Dello Strologo, L; Laurenzi, C; Legato, A; Pastore, A, 2007)
"Potassium citrate was shown to be effective as an alkalizing agent and, in this respect, not significantly different from sodium bicarbonate."6.70A comparison of the effects of potassium citrate and sodium bicarbonate in the alkalinization of urine in homozygous cystinuria. ( Denneberg, T; Fjellstedt, E; Jeppsson, JO; Tiselius, HG, 2001)
"Cystinuria is an inherited autosomal recessive disease of the kidneys of recurring nature that contributes to frequent urinary tract infections due to bacterial growth and biofilm formation surrounding the stone microenvironment."5.91Chemical Modification of Tiopronin for Dual Management of Cystinuria and Associated Bacterial Infections. ( Estes Bright, LM; Garren, MRS; Handa, H; Kumar, A; Manuel, J; Shome, A, 2023)
"Appropriate dosing of cystine-binding thiol drugs in the management of cystinuria has been based on clinical stone activity."5.30Effect of increasing doses of cystine-binding thiol drugs on cystine capacity in patients with cystinuria. ( Asplin, JR; Enders, FT; Goldfarb, DS; Malieckal, DA; Mara, K; Modersitzki, F, 2019)
"The frequency of cystinuria is in the order of 1:10,000."5.26[Cystinuria therapy by ascorbic acid (author's transl)]. ( Asper, R; Schmucki, O, 1982)
"Thirty two patients with cystinuria were enrolled in this long-term study and 16 patients were treated with tiopronin for 24 weeks."5.10[Long-term study of tiopronin in patients with cystinuria]. ( Koide, T; Miyake, O; Okuyama, A; Yachiku, S; Yoshioka, T, 2003)
" We have studied the effect of a sodium-restricted diet on the urinary excretion of cystine in 13 patients with cystinuria, 7 of whom were treated with the SH compound tiopronin (2-mercaptopropionylglycine)."5.08The effect of sodium intake on cystinuria with and without tiopronin treatment. ( Denneberg, T; Edholm, E; Jeppsson, JO; Lindell, A, 1995)
" Pediatricians should be aware of this adverse effect of tiopronin, and therefore, testing of the urine with strips or sulfosalicylic acid at least once weekly at home may be very helpful for early detection of proteinuria."3.77Nephrotic syndrome occurring during tiopronin treatment for cystinuria. ( Gucev, Z; Lozanovski, VJ; Ristoska-Bojkovska, N; Sahpazova, E; Tasic, V, 2011)
"Fifty-two cystinuric patients (cystine SF) entering our programme since 1970 were compared with 3215 SF without cystinuria (routine SF), of whom 114 had a single functioning kidney (routine SF + nephrectomy)."3.73Reduced renal function and benefits of treatment in cystinuria vs other forms of nephrolithiasis. ( Coe, FL; Evan, AP; Parks, JH; Worcester, EM, 2006)
" To improve the medical treatment of patients with cystinuria, this strategy was performed to guide the fluid intake and the administration of SH compounds (tiopronin, D-penicillamine, and MESNA)."3.71Cystine analyses of separate day and night urine as a basis for the management of patients with homozygous cystinuria. ( Christensson, A; Denneberg, T; Fjellstedt, E; Jeppsson, JO; Tiselius, HG, 2001)
"A case of nephrotic syndrome with ascites and heart failure in a patient who had received tiopronin for the treatment of cystinuria is presented."3.71[Nephrotic syndrome and anasarca status, secondary to treatment with tiopronin in a case of cystinuria]. ( Alvarez Navascués, R; Herrera Pérez de Villar, J; Rodríguez Suarez, C; Suarez Heiva, M; Vidau Argüelles, P, 2001)
"From 1987 to 1990, 719 subjects (385 males and 334 females) were screened, by means of urocystin-kit, for cystinuria and cystine lithiasis, in Epirus district (North-West Greece)."3.69[Results of treatment of cystinuria and cystine lithiasis with alpha-mercaptopropionylglycine. Apropos of 40 patients]. ( Chambilomatis, P; Evangelou, A; Giannakis, D; Giannakopoulos, X; Kalfakakou, V; Kallistratos, G; Karkabounas, S; Tsoumanis, P, 1994)
"We have compared 2-mercaptopropionylglycine (MPG) with D-penicillamine in its capacity to reduce the urinary excretion of cystine by patients with cystinuria."3.67Comparison of 2-mercaptopropionylglycine and D-penicillamine in the treatment of cystinuria. ( Cusworth, DC; Harbar, JA; Lawes, LC; Wrong, OM, 1986)
"During a 4-year period 9 cases of severe recurrent cystine stones and cystinuria were treated with mercaptopropionylglycine."3.65Mercaptopropionylglycine: a progress in cystine stone therapy. ( Hautmann, R; Lutzeyer, W; Stuhlsatz, HW; Terhorst, B, 1977)
" Drug dosage was adjusted to maintain free urine cystine level below 100 micromol/mmol creatinine."2.73Cystinuria in children and young adults: success of monitoring free-cystine urine levels. ( Dello Strologo, L; Laurenzi, C; Legato, A; Pastore, A, 2007)
"Cystinuria is the most common monogenic nephrolithiasis disorder."2.72[Cystinuria]. ( Courbebaisse, M; Daudon, M; Defazio, J; Dousseaux, MP; Knebelmann, B; Prot-Bertoye, C; Tostivint, I; Traxer, O, 2021)
"Potassium citrate was shown to be effective as an alkalizing agent and, in this respect, not significantly different from sodium bicarbonate."2.70A comparison of the effects of potassium citrate and sodium bicarbonate in the alkalinization of urine in homozygous cystinuria. ( Denneberg, T; Fjellstedt, E; Jeppsson, JO; Tiselius, HG, 2001)
"Captopril was added for failure of or intolerance to D-penicillamine or alpha-mercaptopropionylglycine."2.68Medical treatment of cystinuria: results of contemporary clinical practice. ( Chow, GK; Streem, SB, 1996)
"Nephrotic syndrome was suggested and renal biopsy was performed."2.38A case of nephrotic syndrome due to alpha-mercaptopropionyl glycine in a patient with familial cystinuria. ( Ishimoto, F; Kodama, K; Murai, S; Nakano, H; Sakai, O; Shibasaki, T, 1990)
"Cystinuria is an inherited autosomal recessive disease of the kidneys of recurring nature that contributes to frequent urinary tract infections due to bacterial growth and biofilm formation surrounding the stone microenvironment."1.91Chemical Modification of Tiopronin for Dual Management of Cystinuria and Associated Bacterial Infections. ( Estes Bright, LM; Garren, MRS; Handa, H; Kumar, A; Manuel, J; Shome, A, 2023)
"To evaluate medical treatments, in terms of adverse events (AEs) and therapeutic goals, in a large series of patients with cystinuria."1.51Adverse events associated with currently used medical treatments for cystinuria and treatment goals: results from a series of 442 patients in France. ( Bataille, P; Bridoux, F; Brignon, P; Choquenet, C; Cochat, P; Combe, C; Conort, P; Courbebaisse, M; Daudon, M; Decramer, S; Doré, B; Dussol, B; Essig, M; Friedlander, G; Frimat, M; Gaunez, N; Jais, JP; Joly, D; Knebelmann, B; Le Toquin-Bernard, S; Lebbah, S; Lillo-Le Louët, A; Méjean, A; Meria, P; Morin, D; N'Guyen, HV; Normand, M; Pietak, M; Pontoizeau, C; Prot-Bertoye, C; Ronco, P; Saussine, C; Tostivint, I; Traxer, O; Tsimaratos, M, 2019)
"Cystinuria is an inherited disorder characterized by defective renal reabsorption of cystine and dibasic amino acids leading to nephrolithiasis."1.46Genotype and Phenotype Analysis in Pediatric Patients with Cystinuria. ( Cheong, HI; Ha, IS; Hyun, HS; Kang, HG; Kim, GH; Kim, JH; Lee, BH; Lee, JH; Park, E; Park, YS, 2017)
"Cystinuria was diagnosed by stone analysis and increased urinary excretion of cystine."1.39An uncommon cause of acute kidney injury in young children: cystinuria. ( Genc, G; Nalcacioglu, H; Ozden, E; Ozkaya, O; Sarikaya, S; Yakupoglu, YK, 2013)
"Cystinuria is an autosomal recessive disorder, and primary manifestation is the repeated formation of cystine calculi."1.30[Clinical study on cystinuria in children--the stone management and the prevention of calculi recurrence]. ( Asanuma, H; Kawamura, T; Nagakura, K; Nakai, H; Shishido, S; Takeda, M; Yamafuji, M, 1998)
"The pharmacokinetic disposition of 2-mercaptopropionylglycine (2-MPG) given as a single intravenous injection and/or as a single oral dose was studied in 9 normal and 13 cystinuric dogs."1.28Pharmacokinetics and bioavailability of 2-mercaptopropionylglycine administered intravenously and orally in dogs. ( Denneberg, T; Emanuelsson, BM; Hoppe, A; Kågedal, B; Lindgren, S, 1991)
" We concluded that 2-MPG is well tolerated and promising for treatment of cystinuric dogs, but the pharmacokinetic studies should be expanded to include different dosage regimens, and results of long-term treatment should be evaluated."1.27Treatment of clinically normal and cystinuric dogs with 2-mercaptopropionylglycine. ( Denneberg, T; Hoppe, A; Kågedal, B, 1988)
"Cystinuria is a complex hereditary disorder characterized by excessive urinary excretion of cystine."1.27Management of the patient with cystinuria. ( Pahira, JJ, 1987)
"The frequency of cystinuria is in the order of 1:10,000."1.26[Cystinuria therapy by ascorbic acid (author's transl)]. ( Asper, R; Schmucki, O, 1982)
"No recurrence of renal calculi was observed."1.26[Clinical aspects of cystinuria and its treatment by thiola (author's transl)]. ( Gründig, E; Latal, D; Müller, M; Zechner, O, 1976)
"The causes of the recurrences in the other 2 children are discussed."1.26Cystinuria in children. ( Dussini, N; Pagano, F; Passerini, G; Pavanello, L; Rizzoni, G; Tasca, A; Zacchello, G, 1981)
" A relatively high dosage resulted in a reduction of the cytinuria."1.25[Cystine stone therapy with mercapto-propyonyl-glycine (MPG) (Thiola) (author's transl)]. ( Stuhlsatz, HW; Terhorst, B, 1975)

Research

Studies (74)

TimeframeStudies, this research(%)All Research%
pre-199033 (44.59)18.7374
1990's18 (24.32)18.2507
2000's11 (14.86)29.6817
2010's5 (6.76)24.3611
2020's7 (9.46)2.80

Authors

AuthorsStudies
Micallef, S1
Delicata, L1
Agius, JF1
Holwill, S1
Mikel, CC1
Goldfarb, DS4
Ponte, A1
Steigelman, K1
Latyshev, S1
Kumar, A1
Estes Bright, LM1
Garren, MRS1
Manuel, J1
Shome, A1
Handa, H1
Modersitzki, F2
Goldstein, RL1
Sur, RL1
Penniston, KL1
Melessen, IM1
Henderickx, MM1
Merkx, MM1
van Etten-Jamaludin, FS1
Homan van der Heide, JJ1
Kamphuis, GM1
Assimos, DG1
Prot-Bertoye, C2
Daudon, M2
Tostivint, I2
Dousseaux, MP1
Defazio, J1
Traxer, O2
Knebelmann, B2
Courbebaisse, M2
Lebbah, S1
Jais, JP1
Lillo-Le Louët, A1
Pontoizeau, C1
Cochat, P1
Bataille, P1
Bridoux, F1
Brignon, P1
Choquenet, C1
Combe, C1
Conort, P1
Decramer, S1
Doré, B1
Dussol, B1
Essig, M1
Frimat, M1
Gaunez, N1
Joly, D1
Le Toquin-Bernard, S1
Méjean, A1
Meria, P1
Morin, D1
N'Guyen, HV1
Normand, M1
Pietak, M1
Ronco, P1
Saussine, C1
Tsimaratos, M1
Friedlander, G1
Malieckal, DA1
Mara, K1
Enders, FT1
Asplin, JR3
Kim, JH1
Park, E1
Hyun, HS1
Lee, BH1
Kim, GH1
Lee, JH1
Park, YS1
Kang, HG1
Ha, IS1
Cheong, HI1
Tasic, V1
Lozanovski, VJ1
Ristoska-Bojkovska, N1
Sahpazova, E1
Gucev, Z1
Nalcacioglu, H1
Ozden, E1
Genc, G1
Yakupoglu, YK1
Sarikaya, S1
Ozkaya, O1
Koide, T1
Yoshioka, T1
Miyake, O1
Okuyama, A1
Yachiku, S1
Knoll, T1
Janitzky, V1
Michel, MS1
Alken, P1
Köhrmann, KU1
Fjellstedt, E3
Harnevik, L1
Jeppsson, JO7
Tiselius, HG4
Söderkvist, P1
Denneberg, T14
Parvex, P1
Rozen, R1
Dziarmaga, A1
Goodyer, P1
Dolin, DJ1
Flagel, L1
Grasso, M1
Worcester, EM1
Coe, FL3
Evan, AP1
Parks, JH2
Dello Strologo, L1
Laurenzi, C1
Legato, A1
Pastore, A1
Stenberg, P1
Kallistratos, G5
Dimopoulos, C2
Kalfakakou-Vadalouka, V1
Evangelou, A2
Stockidis, D1
Vezyraki, P1
Charalambopoulos, C1
Mita, I2
Hales, DS1
Scott, R1
Lewi, HJ1
Mansell, MA1
Lucky, PA1
Skovby, F1
Thier, SO1
Smith, A1
Vidler, J1
Asper, R1
Schmucki, O1
Bruno, M1
Fruttero, B1
Marangella, M1
Linari, F1
Pavanello, L2
Rizzoni, G2
Dussini, N2
Zacchello, G2
Passerini, G1
Tasca, A1
Pagano, F1
Johansen, K1
Gammelgård, PA1
Jørgensen, FS1
Stokidis, D1
Sidéris, C1
Kyriakidis, A1
Kollias, G1
Joost, J1
Jarosch, E1
Lindell, A5
Hellgren, E1
Giannakopoulos, X1
Kalfakakou, V1
Tsoumanis, P1
Karkabounas, S1
Giannakis, D1
Chambilomatis, P1
Persu, A1
Pirson, Y1
Hoppe, A4
Frank, A1
Kågedals, B1
Petersson, LR1
Edholm, E1
Chow, GK1
Streem, SB1
Pierides, AM1
Deltas, CC1
Owada, M1
Watanabe, S1
Tsuda, M1
Berio, A3
Piazzi, A1
Asanuma, H1
Nakai, H1
Takeda, M1
Shishido, S1
Kawamura, T1
Nagakura, K1
Yamafuji, M1
Akakura, K1
Egoshi, K1
Ueda, T1
Nozumi, K1
Kotake, T1
Masai, M1
Ito, H1
Nakagawa, Y1
Clark, C1
Alvarez Navascués, R1
Vidau Argüelles, P1
Rodríguez Suarez, C1
Herrera Pérez de Villar, J1
Suarez Heiva, M1
Christensson, A1
Perfumo, F1
Basile, G1
Ginevri, F1
Gusmano, R1
Barbero, S1
Domeneghetti, G1
Sardi, R1
Chiandetti, L1
Vadaloyka-Kalfakakou, V1
Terhorst, B2
Stuhlsatz, HW2
Hautmann, R1
Lutzeyer, W1
Zechner, O1
Latal, D1
Gründig, E1
Müller, M1
Carratù, A1
Innocenti, MG1
Rossi, R1
Remien, A1
Burchardt, P1
Siskind, MS1
Popovtzer, MM1
Emanuelsson, BM1
Kågedal, B4
Lindgren, S1
Eneström, S1
Fich, C1
Skogh, T1
Shibasaki, T1
Murai, S1
Kodama, K1
Nakano, H1
Ishimoto, F1
Sakai, O1
Mårtensson, J2
Textorius, O1
Jaeger, P1
de Temple, R1
Brien, G1
Hellmann, C1
Otting, U1
Schöpke, W1
Schönberger, B1
Pak, CY1
Fuller, C1
Sakhaee, K1
Zerwekh, JE1
Adams, BV1
Pahira, JJ1
Andersson, T1
Carlsson, M1
Harbar, JA1
Cusworth, DC1
Lawes, LC1
Wrong, OM1
Grunfeld Avellan, JA1

Clinical Trials (1)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Phase II Study of the Effects of Tiopronin on 3-Aminopropanal Level & Neurologic Outcome After Aneurysmal Subarachnoid Hemorrhage[NCT01095731]Phase 260 participants (Actual)Interventional2010-04-30Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Reviews

8 reviews available for tiopronin and Cystinuria

ArticleYear
The effect of additional drug therapy as metaphylaxis in patients with cystinuria: a systematic review.
    Minerva urologica e nefrologica = The Italian journal of urology and nephrology, 2020, Volume: 72, Issue:4

    Topics: Captopril; Cystine; Cystinuria; Drug Therapy, Combination; Evidence-Based Medicine; Female; Humans;

2020
[Cystinuria].
    Nephrologie & therapeutique, 2021, Volume: 17S

    Topics: Cystine; Cystinuria; Humans; Kidney Calculi; Penicillamine; Tiopronin

2021
[Cystinuria - Cystine Stones: Recommendations for Diagnosis, Therapy and Follow-up].
    Aktuelle Urologie, 2003, Volume: 34, Issue:2

    Topics: Adult; Age Factors; Angiotensin-Converting Enzyme Inhibitors; Ascorbic Acid; Captopril; Chelating Ag

2003
[Current aspects of the medical treatment of cystinuria].
    Acta urologica Belgica, 1994, Volume: 62, Issue:2

    Topics: Ascorbic Acid; Captopril; Cystine; Cystinuria; Humans; Hydrogen-Ion Concentration; Penicillamine; So

1994
[Cystinuria].
    Ryoikibetsu shokogun shirizu, 1998, Issue:19 Pt 2

    Topics: Amino Acid Transport Systems, Basic; Carrier Proteins; Citric Acid; Cystinuria; Diagnosis, Different

1998
[The hyperaminoacidurias with special reference to cystinuria].
    Minerva medica, 1979, Oct-13, Volume: 70, Issue:44

    Topics: Amino Acids; Biological Transport; Carrier Proteins; Child; Cystinuria; Female; Humans; Microvilli;

1979
A case of nephrotic syndrome due to alpha-mercaptopropionyl glycine in a patient with familial cystinuria.
    Nihon Jinzo Gakkai shi, 1990, Volume: 32, Issue:8

    Topics: Adult; Cystinuria; Glomerulonephritis, Membranous; Humans; Male; Nephrotic Syndrome; Tiopronin

1990
Cystinuria: pathophysiology and treatment.
    Advances in nephrology from the Necker Hospital, 1989, Volume: 18

    Topics: Captopril; Cystinuria; Humans; Penicillamine; Tiopronin

1989

Trials

9 trials available for tiopronin and Cystinuria

ArticleYear
Effect of increasing doses of cystine-binding thiol drugs on cystine capacity in patients with cystinuria.
    Urolithiasis, 2019, Volume: 47, Issue:6

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cross-Over Studies; Cystine; Cystinuria; Dose-Response R

2019
[Long-term study of tiopronin in patients with cystinuria].
    Hinyokika kiyo. Acta urologica Japonica, 2003, Volume: 49, Issue:2

    Topics: Adult; Aged; Anorexia; Constipation; Cystinuria; Dose-Response Relationship, Drug; Drug Administrati

2003
Cystinuria in children and young adults: success of monitoring free-cystine urine levels.
    Pediatric nephrology (Berlin, Germany), 2007, Volume: 22, Issue:11

    Topics: Adolescent; Adult; Alkalies; Chelating Agents; Child; Child, Preschool; Cystine; Cystinuria; Diureti

2007
Clinical course and cystine stone formation during tiopronin treatment.
    Urological research, 1995, Volume: 23, Issue:2

    Topics: Adolescent; Adult; Child; Child, Preschool; Cystinuria; Female; Humans; Kidney Calculi; Male; Middle

1995
Urinary excretion of free cystine and the tiopronin-cysteine-mixed disulfide during long term tiopronin treatment of cystinuria.
    Nephron, 1995, Volume: 71, Issue:3

    Topics: Adolescent; Adult; Aged; Cystine; Cystinuria; Disulfides; Dose-Response Relationship, Drug; Female;

1995
The effect of sodium intake on cystinuria with and without tiopronin treatment.
    Nephron, 1995, Volume: 71, Issue:4

    Topics: Adolescent; Adult; Creatinine; Cystine; Cystinuria; Diet, Sodium-Restricted; Female; Humans; Kidney

1995
Medical treatment of cystinuria: results of contemporary clinical practice.
    The Journal of urology, 1996, Volume: 156, Issue:5

    Topics: Adolescent; Adult; Angiotensin-Converting Enzyme Inhibitors; Captopril; Child; Child, Preschool; Cys

1996
A comparison of the effects of potassium citrate and sodium bicarbonate in the alkalinization of urine in homozygous cystinuria.
    Urological research, 2001, Volume: 29, Issue:5

    Topics: Adult; Aged; Alkalies; Cystinuria; Female; Homozygote; Humans; Hydrogen-Ion Concentration; Male; Mid

2001
Management of cystine nephrolithiasis with alpha-mercaptopropionylglycine.
    The Journal of urology, 1986, Volume: 136, Issue:5

    Topics: Adolescent; Adult; Aged; Amino Acids, Sulfur; Child; Clinical Trials as Topic; Cystine; Cystinuria;

1986

Other Studies

57 other studies available for tiopronin and Cystinuria

ArticleYear
Tiopronin-induced membranous nephropathy in a patient with refractory familial cystinuria.
    British journal of hospital medicine (London, England : 2005), 2022, 08-02, Volume: 83, Issue:8

    Topics: Cystinuria; Glomerulonephritis, Membranous; Humans; Nephrotic Syndrome; Tiopronin

2022
Accurate 24-h urine cystine quantification for patients on cystine-binding thiol drugs.
    Urolithiasis, 2022, Volume: 50, Issue:6

    Topics: Cysteine; Cystine; Cystinuria; Humans; Quality of Life; Sulfhydryl Compounds; Tandem Mass Spectromet

2022
Chemical Modification of Tiopronin for Dual Management of Cystinuria and Associated Bacterial Infections.
    ACS applied materials & interfaces, 2023, Sep-20, Volume: 15, Issue:37

    Topics: Animals; Bacterial Infections; Cystine; Cystinuria; Disulfides; Dogs; Escherichia coli; Mice; Nitric

2023
Assessment of health-related quality of life in patients with cystinuria on tiopronin therapy.
    Urolithiasis, 2020, Volume: 48, Issue:4

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cross-Sectional Studies; Cystinuria; Female; Humans; Kid

2020
Re: Assessment of Health-Related Quality of Life in Patients with Cystinuria on Tiopronin Therapy.
    The Journal of urology, 2020, Volume: 204, Issue:1

    Topics: Cystinuria; Humans; Quality of Life; Tiopronin

2020
Adverse events associated with currently used medical treatments for cystinuria and treatment goals: results from a series of 442 patients in France.
    BJU international, 2019, Volume: 124, Issue:5

    Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Cystinuria; Drug-Related Side Effects and Adverse

2019
Genotype and Phenotype Analysis in Pediatric Patients with Cystinuria.
    Journal of Korean medical science, 2017, Volume: 32, Issue:2

    Topics: Adolescent; Amino Acid Transport Systems, Basic; Amino Acid Transport Systems, Neutral; Asian People

2017
Nephrotic syndrome occurring during tiopronin treatment for cystinuria.
    European journal of pediatrics, 2011, Volume: 170, Issue:2

    Topics: Amino Acids, Sulfur; Benzenesulfonates; Child, Preschool; Cystinuria; Edema; Humans; Lithotripsy; Ma

2011
An uncommon cause of acute kidney injury in young children: cystinuria.
    Journal of pediatric urology, 2013, Volume: 9, Issue:1

    Topics: Acute Kidney Injury; Anuria; Child, Preschool; Cystinuria; Diagnosis, Differential; Diuretics; Femal

2013
Urinary excretion of total cystine and the dibasic amino acids arginine, lysine and ornithine in relation to genetic findings in patients with cystinuria treated with sulfhydryl compounds.
    Urological research, 2003, Volume: 31, Issue:6

    Topics: Adolescent; Adult; Aged; Aged, 80 and over; Alleles; Amino Acid Transport Systems, Basic; Amino Acid

2003
Studies of urinary cystine precipitation in vitro: ontogeny of cystine nephrolithiasis and identification of meso-2,3-dimercaptosuccinic acid as a potential therapy for cystinuria.
    Molecular genetics and metabolism, 2003, Volume: 80, Issue:4

    Topics: Adolescent; Age Factors; Chemical Precipitation; Child; Child, Preschool; Cystine; Cystinuria; Dose-

2003
Effect of cystine-binding thiol drugs on urinary cystine capacity in patients with cystinuria.
    Journal of endourology, 2005, Volume: 19, Issue:3

    Topics: Administration, Oral; Adult; Captopril; Case-Control Studies; Cysteine; Cystinuria; Dose-Response Re

2005
Reduced renal function and benefits of treatment in cystinuria vs other forms of nephrolithiasis.
    BJU international, 2006, Volume: 97, Issue:6

    Topics: Adult; Chelating Agents; Cystine; Cystinuria; Drinking; Female; Humans; Male; Middle Aged; Nephrecto

2006
Alternative treatment of cystinuria with alpha-merkaptopropionylglycine, Thiola.
    Proceedings of the European Dialysis and Transplant Association. European Dialysis and Transplant Association, 1983, Volume: 20

    Topics: Adolescent; Adult; Aged; Amino Acids, Sulfur; Cystine; Cystinuria; Dipeptides; Female; Humans; Male;

1983
Familial cystinuria in Ioannina District (Greece). Diagnosis and treatment.
    Urological research, 1983, Volume: 11, Issue:6

    Topics: Adolescent; Adult; Child; Cystinuria; Female; Greece; Humans; Male; Middle Aged; Pedigree; Tiopronin

1983
Myopathy due to mercaptopropionyl glycine.
    British medical journal (Clinical research ed.), 1982, Oct-02, Volume: 285, Issue:6346

    Topics: Adult; Amino Acids, Sulfur; Cystinuria; Female; Humans; Muscular Diseases; Tiopronin

1982
Myopathy due to mercaptopropionyl glycine.
    British medical journal (Clinical research ed.), 1982, Nov-06, Volume: 285, Issue:6351

    Topics: Amino Acids, Sulfur; Cystinuria; Humans; Muscular Diseases; Penicillamine; Tiopronin

1982
Pemphigus foliaceus and proteinuria induced by alpha-mercaptopropionylglycine.
    Journal of the American Academy of Dermatology, 1983, Volume: 8, Issue:5

    Topics: Amino Acids, Sulfur; Cystinuria; Humans; Male; Middle Aged; Pemphigus; Proteinuria; Skin; Tiopronin

1983
Evaluation of techniques to estimate binding of thiola to cystine in cystinuric patients.
    Australian and New Zealand journal of medicine, 1981, Volume: 11, Issue:3

    Topics: Amino Acids; Amino Acids, Sulfur; Chromatography, Paper; Creatine; Crystallization; Cystine; Cystinu

1981
[Cystinuria therapy by ascorbic acid (author's transl)].
    Urologia internationalis, 1982, Volume: 37, Issue:2

    Topics: Ascorbic Acid; Cysteine; Cystine; Cystinuria; Humans; Intestinal Absorption; Kidney; Penicillamine;

1982
[Cystinuria: clinical and therapeutic aspects].
    Bollettino chimico farmaceutico, 1982, Volume: 121, Issue:2

    Topics: Adolescent; Adult; Aged; Child; Cystinuria; Drug Evaluation; Glycine; Humans; Kinetics; Middle Aged;

1982
Cystinuria in children.
    European urology, 1981, Volume: 7, Issue:3

    Topics: Amino Acids, Sulfur; Child; Child, Preschool; Cystinuria; Female; Humans; Infant; Kidney Calculi; Ma

1981
Treatment of cystinuria with alpha-mercaptopropionylglycine.
    Scandinavian journal of urology and nephrology, 1980, Volume: 14, Issue:2

    Topics: Adult; Amino Acids, Sulfur; Cystine; Cystinuria; Female; Humans; Male; Recurrence; Tiopronin; Urinar

1980
[The use of mercapto-propionyl-glycine in the treatment of cystine lithiasis (author's transl)].
    Journal d'urologie, 1981, Volume: 87, Issue:5

    Topics: Adult; Amino Acids, Sulfur; Cystinuria; Female; Humans; Kidney Calculi; Male; Recurrence; Tiopronin;

1981
Glutamine: a new anticystinuric drug?
    European urology, 1981, Volume: 7, Issue:6

    Topics: Adult; Ammonia; Cystinuria; Drug Combinations; Female; Glutamine; Humans; Time Factors; Tiopronin

1981
[Results of treatment of cystinuria and cystine lithiasis with alpha-mercaptopropionylglycine. Apropos of 40 patients].
    Journal d'urologie, 1994, Volume: 100, Issue:3

    Topics: Adult; Aged; Cystine; Cystinuria; Female; Follow-Up Studies; Humans; Male; Middle Aged; Recurrence;

1994
Urinary excretion of metals during treatment with D-penicillamine and 2-mercaptopropionylglycine in normal and cystinuric dogs.
    Journal of veterinary pharmacology and therapeutics, 1993, Volume: 16, Issue:1

    Topics: Administration, Oral; Animals; Creatinine; Cystinuria; Dog Diseases; Dogs; Injections, Intravenous;

1993
Clinical aspects of cystinuria.
    Contributions to nephrology, 1997, Volume: 122

    Topics: Adolescent; Adult; Chelating Agents; Child; Child, Preschool; Cystine; Cystinuria; Diet; Female; Hum

1997
Prophylaxis of cystine calculi by low dose of alpha-mercapto-propionyl-glycine administered every other day.
    Panminerva medica, 1998, Volume: 40, Issue:3

    Topics: Adult; Cystinuria; Dose-Response Relationship, Drug; Drug Administration Schedule; Humans; Kidney Ca

1998
[Clinical study on cystinuria in children--the stone management and the prevention of calculi recurrence].
    Nihon Hinyokika Gakkai zasshi. The japanese journal of urology, 1998, Volume: 89, Issue:9

    Topics: Adolescent; Adult; Child; Child, Preschool; Cystinuria; Female; Follow-Up Studies; Humans; Infant; L

1998
The long-term outcome of cystinuria in Japan.
    Urologia internationalis, 1998, Volume: 61, Issue:2

    Topics: Adult; Chelating Agents; Cystine; Cystinuria; Female; Follow-Up Studies; Humans; Hydrogen-Ion Concen

1998
A modified cyanide-nitroprusside method for quantifying urinary cystine concentration that corrects for creatinine interference.
    Clinica chimica acta; international journal of clinical chemistry, 1999, Volume: 289, Issue:1-2

    Topics: Adult; Artifacts; Ascorbic Acid; Captopril; Chlorides; Colorimetry; Creatinine; Cyanides; Cysteine;

1999
Solid phase assay of urine cystine supersaturation in the presence of cystine binding drugs.
    The Journal of urology, 2001, Volume: 166, Issue:2

    Topics: Buffers; Captopril; Chemical Phenomena; Chemistry; Crystallization; Cystinuria; Humans; Penicillamin

2001
[Nephrotic syndrome and anasarca status, secondary to treatment with tiopronin in a case of cystinuria].
    Archivos espanoles de urologia, 2001, Volume: 54, Issue:5

    Topics: Adult; Ascites; Cystinuria; Edema; Female; Humans; Nephrotic Syndrome; Tiopronin

2001
Cystine analyses of separate day and night urine as a basis for the management of patients with homozygous cystinuria.
    Urological research, 2001, Volume: 29, Issue:5

    Topics: Adult; Aged; Circadian Rhythm; Cystinuria; Dose-Response Relationship, Drug; Female; Homozygote; Hum

2001
[Cystinuria. Description of a case].
    Minerva pediatrica, 1979, Feb-15, Volume: 31, Issue:3

    Topics: Child, Preschool; Cystinuria; Female; Humans; Tiopronin; Urinary Calculi

1979
Nephrotic syndrome during treatment with alpha-mercaptopropionylglycine.
    The Journal of urology, 1979, Volume: 122, Issue:3

    Topics: Amino Acids, Sulfur; Child; Child, Preschool; Cystinuria; Humans; Male; Nephrotic Syndrome; Proteinu

1979
Management of cystinuric disorders with sulfhydryl drugs.
    Progress in clinical and biological research, 1979, Volume: 34

    Topics: Cystinuria; Humans; Sulfhydryl Compounds; Tiopronin

1979
[Cystine-lysine-ornithine-argininuria. Current genetic and clinico-therapeutic problems].
    Minerva pediatrica, 1979, Oct-31, Volume: 31, Issue:20

    Topics: Adolescent; Amino Acid Metabolism, Inborn Errors; Amino Acids, Sulfur; Arginine; Child; Child, Presc

1979
[Cystine stone therapy with mercapto-propyonyl-glycine (MPG) (Thiola) (author's transl)].
    Der Urologe. Ausg. A, 1975, Volume: 14, Issue:4

    Topics: Adult; Amino Acids; Amino Acids, Sulfur; Cystinuria; Drug Tolerance; Female; Humans; Long-Term Care;

1975
Mercaptopropionylglycine: a progress in cystine stone therapy.
    The Journal of urology, 1977, Volume: 117, Issue:5

    Topics: Adult; Amino Acids, Sulfur; Cystine; Cystinuria; Female; Follow-Up Studies; Humans; Male; Penicillam

1977
[Clinical aspects of cystinuria and its treatment by thiola (author's transl)].
    Wiener klinische Wochenschrift, 1976, Feb-06, Volume: 88, Issue:3

    Topics: Amino Acids; Amino Acids, Sulfur; Biological Transport; Cystinuria; Humans; Kidney Calculi; Tioproni

1976
[Cystine lithiasis. Report of a case with severe neuro-psychic disturbances. Discussion of the effects of anti-lithiasic treatment].
    Minerva pediatrica, 1976, Dec-15, Volume: 28, Issue:39

    Topics: Amino Acids, Sulfur; Child; Cystinuria; Epilepsy; Humans; Male; Mental Disorders; Tiopronin; Urinary

1976
Treatment of cystinuria with Thiola (alpha-mercaptopropionyl glycine).
    European urology, 1975, Volume: 1, Issue:5

    Topics: Amino Acids, Sulfur; Cystinuria; Dose-Response Relationship, Drug; Female; Humans; Male; Recurrence;

1975
Hyperlipidemia associated with alpha-mercaptopropionylglycine therapy for cystinuria.
    American journal of kidney diseases : the official journal of the National Kidney Foundation, 1992, Volume: 19, Issue:2

    Topics: Adult; Cystinuria; Female; Humans; Hyperlipidemias; Tiopronin

1992
Pharmacokinetics and bioavailability of 2-mercaptopropionylglycine administered intravenously and orally in dogs.
    Journal of veterinary pharmacology and therapeutics, 1991, Volume: 14, Issue:4

    Topics: Administration, Oral; Animals; Biological Availability; Blood Proteins; Cystinuria; Dog Diseases; Do

1991
Membranous glomerulonephritis induced by 2-mercaptopropionylglycine (2-MPG).
    Clinical nephrology, 1990, Volume: 34, Issue:3

    Topics: Antibodies, Antinuclear; Biopsy; Cystinuria; Female; Glomerulonephritis, Membranous; Humans; Kidney

1990
Sulfur amino acid metabolism in cystinuria: a biochemical and clinical study of patients.
    Kidney international, 1990, Volume: 37, Issue:1

    Topics: Acetylcysteine; Adult; Amino Acids, Sulfur; Cysteine; Cystinuria; Female; Homozygote; Humans; Male;

1990
[Treatment and metaphylaxis of cystine urolithiasis in children].
    Kinderarztliche Praxis, 1989, Volume: 57, Issue:9

    Topics: Acetylcysteine; Administration, Oral; Adolescent; Cystinuria; Female; Humans; Kidney Calculi; Nephro

1989
Tiopronin for cystinuria.
    The Medical letter on drugs and therapeutics, 1989, Jan-27, Volume: 31, Issue:784

    Topics: Cystine; Cystinuria; Gastrointestinal Diseases; Humans; Leukopenia; Solubility; Tiopronin; Urinary C

1989
Treatment of clinically normal and cystinuric dogs with 2-mercaptopropionylglycine.
    American journal of veterinary research, 1988, Volume: 49, Issue:6

    Topics: Administration, Oral; Amino Acids, Sulfur; Animals; Cystinuria; Dog Diseases; Dogs; Drug Tolerance;

1988
Management of the patient with cystinuria.
    The Urologic clinics of North America, 1987, Volume: 14, Issue:2

    Topics: Adult; Alkalies; Child; Cystinuria; Dietary Proteins; Female; Fluid Therapy; Humans; Kidney Calculi;

1987
High-performance liquid chromatography of 2-mercaptopropionylglycine and its metabolite 2-mercaptopropionic acid in plasma and urine after treatment with thiopronine.
    Journal of chromatography, 1987, Jul-03, Volume: 417, Issue:2

    Topics: Amino Acids, Sulfur; Animals; Chromatography, High Pressure Liquid; Cystinuria; Dogs; Humans; Specie

1987
Comparison of 2-mercaptopropionylglycine and D-penicillamine in the treatment of cystinuria.
    The Journal of urology, 1986, Volume: 136, Issue:1

    Topics: Adult; Amino Acids, Sulfur; Cystinuria; Female; Humans; Male; Middle Aged; Penicillamine; Tiopronin

1986
2-Mercaptopropionate, a novel metabolite formed during treatment with 2-mercaptopropionyl-glycine in cystinuria.
    European journal of clinical pharmacology, 1986, Volume: 31, Issue:1

    Topics: Amino Acids, Sulfur; Chromatography, Gas; Cystinuria; Humans; Mass Spectrometry; Sulfhydryl Compound

1986
[Prophylaxis of cystinic calculosis with alpha mercapto propionylglycine. Personal cases].
    Minerva pediatrica, 1985, Dec-31, Volume: 37, Issue:23-24

    Topics: Adolescent; Adult; Amino Acids, Sulfur; Cystinuria; Electrophoresis; Humans; Kidney Calculi; Male; M

1985
[Cystine lithiasis in the adult. Apropos of an isolated case without familial antecedents].
    Journal d'urologie, 1985, Volume: 91, Issue:4

    Topics: Adult; Cystinuria; Diuresis; Female; Humans; Prognosis; Recurrence; Tiopronin; Ureteral Calculi; Uri

1985