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Management of Cystine Nephrolithiasis with Alpha-Mercaptopropionylglycine

1986; Lippincott Williams & Wilkins; Volume: 136; Issue: 5 Linguagem: Inglês

10.1016/s0022-5347(17)45188-3

1527-3792

Charles Y.C. Pak, Cindy J. Fuller, Khashayar Sakhaee, Joseph E. Zerwekh, Beverley V. Adams,

Urinary Bladder and Prostate Research

No AccessJournal of Urology1 Nov 1986Management of Cystine Nephrolithiasis with Alpha-Mercaptopropionylglycine Charles Y.C. Pak, Cindy Fuller, Khashayar Sakhaee, Joseph E. Zerwekh, Beverley V. Adams, and With Investigators From Collaborating Units* Charles Y.C. PakCharles Y.C. Pak More articles by this author , Cindy FullerCindy Fuller More articles by this author , Khashayar SakhaeeKhashayar Sakhaee More articles by this author , Joseph E. ZerwekhJoseph E. Zerwekh More articles by this author , Beverley V. AdamsBeverley V. Adams More articles by this author , and With Investigators From Collaborating Units* Collaborating investigators: Steven R. Alexander, Department of Pediatrics, The Oregon Health Sciences University, Portland, Oregon; Chaim Charytan and Joel H. Rascoff, Booth Memorial Medical Center, Flushing, New York; James W. Edmonson, IU Medical Center, CRC University Hospital, Indianapolis, Indiana; Keith A. Hruska, Department of Nephrology, The Jewish Hospital-Washington University, St. Louis, Missouri; Gary Linn, 1915 Six Avenue, Neptune, New Jersey; Robert W. Rosenbaum, Hurley Medical Center, Flint, Michigan; Leon Rosenberg, Department of Human Genetics, Yale University School of Medicine, New Haven, Connecticut, and Christian J. Vandenberg, Departments of Nephrology and Internal Medicine, The Mayo Clinic, Rochester, Minnesota. More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(17)45188-3AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail The effect of long-term treatment with alpha-mercaptopropionylglycine was examined in 66 patients with cystinuria. Of the patients 49 took D-penicillamine before therapy, whereas 17 did not. Over-all side effects to alpha-mercaptopropionylglycine were common, and occurred in 75.5 per cent of the patients with and 64.7 per cent without a history of D-penicillamine treatment, compared to 83.7 per cent who suffered toxicity to D-penicillamine. Serious adverse reactions requiring cessation of therapy were less common with alpha-mercaptopropionylglycine. Among the patients who took both drugs 30.6 per cent had to stop taking alpha-mercaptopropionylglycine, whereas 69.4 per cent could not tolerate D-penicillamine. Of the latter group with toxicity to D-penicillamine therapy only 5.9 per cent had side effects to alpha-mercaptopropionylglycine of sufficient severity to require withdrawal. Alpha-mercaptopropionylglycine was equally as effective as D-penicillamine in reducing cystine excretion. During long-term treatment with alpha-mercaptopropionylglycine (average dose 1,193mg. per day) urinary cystine levels were maintained at 350 to 560mg. per day and urinary cystine was kept at undersaturated levels. Commensurate with these changes, alpha-mercaptopropionylglycine produced remission of stone formation in 63 to 71 per cent of the patients and reduced individual stone formation rate in 81 to 94 per cent. Thus, alpha-mercaptopropionylglycine has a definite therapeutic role in cystinuric patients with toxicity to D-penicillamine. © 1986 by The American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetailsCited ByAssimos D (2020) Re: Assessment of Health-Related Quality of Life in Patients with Cystinuria on Tiopronin TherapyJournal of Urology, VOL. 204, NO. 1, (180-180), Online publication date: 1-Jul-2020.Pearle M, Goldfarb D, Assimos D, Curhan G, Denu-Ciocca C, Matlaga B, Monga M, Penniston K, Preminger G, Turk T and White J (2014) Medical Management of Kidney Stones: AUA GuidelineJournal of Urology, VOL. 192, NO. 2, (316-324), Online publication date: 1-Aug-2014.Asplin D and Asplin J (2012) The Interaction of Thiol Drugs and Urine pH in the Treatment of CystinuriaJournal of Urology, VOL. 189, NO. 6, (2147-2151), Online publication date: 1-Jun-2013.Pak C (2008) Medical Stone Management: 35 Years of AdvancesJournal of Urology, VOL. 180, NO. 3, (813-819), Online publication date: 1-Sep-2008.PAREEK G, STEELE T and NAKADA S (2018) UROLOGICAL INTERVENTION IN PATIENTS WITH CYSTINURIA IS DECREASED WITH MEDICAL COMPLIANCEJournal of Urology, VOL. 174, NO. 6, (2250-2252), Online publication date: 1-Dec-2005.PIETROW P, AUGE B, WEIZER A, DELVECCHIO F, SILVERSTEIN A, MATHIAS B, ALBALA D and PREMINGER G (2018) Durability Of the Medical Management Of CystinuriaJournal of Urology, VOL. 169, NO. 1, (68-70), Online publication date: 1-Jan-2003.TEKIN A, TEKGUL S, ATSU N, SAHIN A and BAKKALOGLU M (2018) CYSTINE CALCULI IN CHILDREN: THE RESULTS OF A METABOLIC EVALUATION AND RESPONSE TO MEDICAL THERAPYJournal of Urology, VOL. 165, NO. 6 Part 2, (2328-2330), Online publication date: 1-Jun-2001.COE F, CLARK C, PARKS J and ASPLIN J (2018) SOLID PHASE ASSAY OF URINE CYSTINE SUPERSATURATION IN THE PRESENCE OF CYSTINE BINDING DRUGSJournal of Urology, VOL. 166, NO. 2, (688-693), Online publication date: 1-Aug-2001.BARBEY F, JOLY D, RIEU P, MÉJEAN A, DAUDON M and JUNGERS P (2018) MEDICAL TREATMENT OF CYSTINURIA: CRITICAL REAPPRAISAL OF LONG-TERM RESULTSJournal of Urology, VOL. 163, NO. 5, (1419-1423), Online publication date: 1-May-2000.Chow G and Streem S (2018) Medical Treatment of Cystinuria: Results of Contemporary Clinical PracticeJournal of Urology, VOL. 156, NO. 5, (1576-1578), Online publication date: 1-Nov-1996.Fine J, Pak C, Preminger G, Segura J and Marberger M (2018) Effect of Medical Management and Residual Fragments on Recurrent Stone Formation Following Shock Wave LithotripsyJournal of Urology, VOL. 153, NO. 1, (27-33), Online publication date: 1-Jan-1995.Cohen T, Streem S and Hall P (2018) Clinical Effect of Captopril on the Formation and Growth of Cystine CalculiJournal of Urology, VOL. 154, NO. 1, (164-166), Online publication date: 1-Jul-1995.Norman R and Manette W (2018) Dietary Restriction of Sodium as a Means of Reducing Urinary CystineJournal of Urology, VOL. 143, NO. 6, (1193-1195), Online publication date: 1-Jun-1990.Sakhaee K, Poindexter J and Pak C (2018) The Spectrum of Metabolic Abnormalities in Patients with Cystine NephrolithiasisJournal of Urology, VOL. 141, NO. 4, (819-821), Online publication date: 1-Apr-1989.Preminger G (2018) Editorial CommentJournal of Urology, VOL. 142, NO. 6, (1524-1524), Online publication date: 1-Dec-1989.Singer A and Das S (2018) Cystinuria: A Review of the Pathophysiology and ManagementJournal of Urology, VOL. 142, NO. 3, (669-673), Online publication date: 1-Sep-1989. Volume 136Issue 5November 1986Page: 1003-1008 Advertisement Copyright & Permissions© 1986 by The American Urological Association Education and Research, Inc.MetricsAuthor Information Charles Y.C. Pak More articles by this author Cindy Fuller More articles by this author Khashayar Sakhaee More articles by this author Joseph E. Zerwekh More articles by this author Beverley V. Adams More articles by this author With Investigators From Collaborating Units* Collaborating investigators: Steven R. Alexander, Department of Pediatrics, The Oregon Health Sciences University, Portland, Oregon; Chaim Charytan and Joel H. Rascoff, Booth Memorial Medical Center, Flushing, New York; James W. Edmonson, IU Medical Center, CRC University Hospital, Indianapolis, Indiana; Keith A. Hruska, Department of Nephrology, The Jewish Hospital-Washington University, St. Louis, Missouri; Gary Linn, 1915 Six Avenue, Neptune, New Jersey; Robert W. Rosenbaum, Hurley Medical Center, Flint, Michigan; Leon Rosenberg, Department of Human Genetics, Yale University School of Medicine, New Haven, Connecticut, and Christian J. Vandenberg, Departments of Nephrology and Internal Medicine, The Mayo Clinic, Rochester, Minnesota. More articles by this author Expand All Advertisement Loading ...