Summary
The “Urocystin Test” has been used as a screening procedure for the diagnosis of the incidence of cystinuria in Ioannina District (Northwest Greece). From the 210 investigated urine samples, eight were positive and four of them were also L-cystine stone formers. All positive cases belong to two cystinuric families. The pedigree in village “Kato-Lapsista” is a genetic type of a “completely recessive cystinuria” while the other one in “Marmara” is the type of an “incompletely recessive cystinuria”. All patients with L-cystine urolithiasis, except one child, were treated with the drug Thiola.
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References
Berio A (1979) La cistin-lisin-ornithin-argininuria. Minerva Pediatr 31:1443–1452
Bersin T, Steudel J (1938) Polarimetrische Untersuchungen über das Thiol-Disulfid System. Chem Ber 71B:1015
Crawhall JC, Scowen EF, Watts RWE (1963) Effect of penicillamine on cystinuria. Br Med J 1:588
Dent CE, Harris H (1951) The genetics of Cystinuria. Ann Eugen 16:60
Dent CE, Senior B (1955) Studies on the treatment of cystinuria. Br J Urol 27:317
Dimopoulos K, Stockidis D, Kallistratos G, Sideris C, Kyriakidis A, Kollias G (1981) L'emploi de la mercapto-propionylglycine (MPG) dans le traitement des lithiases cystiniques. J Urol (Paris) 87:265–268
Dimopoulos K, Kyriakidis A, Stockidis D, Karayannis A, Kallistratos G (1982) Metabolic disturbances in cystinuria. Eur Urol 8:367–369
Halperin EC, Thier SO, Rosenberg LE (1981) The use of D-penicillamine in cystinuria: efficacy and untoward reactions. Yale J Biol Med 54:439–446
Hautmann R, Terhorst B, Stuhlsatz H, Lutzeyer W (1977) mercapto-propionyl-glycine: A progress in cystine stone therapy. J urol 117:628–630
Hayase Y, Fukatsu H, Segawa A (1980) The dissolution of cystine stones by irrigated Tiopronin solution. J Urol 124: 775–778
Hales DSM, Scott R, Lewi HJE (1982) Myopathy due to mercapto-propionyl-glycine. Br Med J 285:939
Johansen K, Gammelgard PA, Schönau Jørgensen F (1980) Treatment of cystinuria with alpha-mercaptopropionylglycine. Scand J Urol Nephrol 14:189–192
Kallistratos G, Timmermann A (1963) Die Thiol-Disulfidaustauschreaktion und ihre Anwendung zur Cystinsteinauflösung. Naturwissenschaften 50:691
Kallistratos G, Timmermann A (1966) Die chemische Auflösung von Cystinsteinen durch Mercaptoverbindungen. Drug Res 16:781–782
Kallistratos G, Timmermann A (1968) Report on Thiola. Naturwissenschaften 55:647
Kallistratos G, Malorny G (1972) Experimentelle Untersuchungen zur Frage der chemischen Auflösung von Cystinsteinen. Drug Res 22:1434–1444
Kallistratos G (1973) Litholytic agents: Preventive and curative drugs for nephrolithiasis. In: Drug Design, vol 11IV. Academic Press, New York, pp 237–283
Kallistratos G, Panteliadis C, Marketos S (1977) Cystinurienachweis mit Hilfe des “Urocystin Tests”., Fortschr Urol Nephrol 9:210–212
Kallistratos G, Mita I (1977) Synthese neuer potentieller L-cystin-lösender Verbindungen. Fortschr Urol Nephrol 9:324–334
Kallistratos G, Burchardt P, Freerksen P (1978) Recent advances in the diagnosis and treatment of L-cystin stone disease. Folia Biochim Biol Greaca 15:21–31
Kallistratos G, Mita I, Vadalouka-Kalfakakou V (1979) Management of cystinuric disorders with sulfhydryl drugs. Prog Clin Biol Res 34:255–264
King S (1968) Treatment of cystinuria with a-Mercaptopropionyl-glycine. Proc Soc Exp Biol Med 129:927
Koide T, Kinoshita K, Takemoto M, Yashiku S, Sonoda T (1982) Conservative treatment of cystine calculi: Effect of oral alpha-mercaptopropionylglycine on cystine stone dissolution and on prevention of stone recurrence. J Urol 128:513–516
Miano L, Gallucci M, Petta S (1979) Results of medical treatment of cystine lithiasis. Eur Urol 5:265–272
Pasero G, Pellegrini P, Ambanelli U, Ciompi ML, Colamussi V, Ferraccioli G, Barbiery P, Mazzoni MR, Menegale G, Trippi D (1982) Controlled multicenter trial of Tiopronin and D-penicillamine for rheumatoid arthritis. Arthritis Rheum 25:923–929
Remien A, Kallistratos G, Burchardt P (1975) Die Behandlung der Cystinurie mit Thiola. Fortschr Urol Nephrol 7:198
Remien A, Kallistratos G, Burchardt P (1975) Treatment of cystinuria with Thiola. Eur Urol 1:227–228
Reveillaud RJ, Blanc G, Daudon M (1978) Syndrom nephrotique et troubles cutanes survenus au cours du traitment de deux cas de lithiase cystinique par l'alpha-mercaptopropionylglycine. J Urol (Paris) 84:663–667
Rizzoni G, Pavanello L, Dussini N, Chiandetti L, Zachello G (1979) Nephrotic syndrome during treatment with alphamercaptopropionyl-glycine. J. Urol 122:381–382
Smith A, Vidler J (1981) Evaluation of technics to estimate binding of Thiola to cystine in cystinuric patients. Aust NZ J Med 11:251–254
Stockidis D (1981) Cystinuria. Thesis, Athens University, Greece
Terhorst B, Hautmann R (1976) Medikamentöse Harnsteinprophylaxe. Therapiewoche 26:5908–5918
Thomas J, Charpentier C, Thomas E, Lemmonnier A, Steg A, Aboulker P (1976) Traitement de la lithiase cystinique par la 2-mercapto-propionyl-glycine. Therapie 31:623–629
Timmermann A, Kallistratos G, Fenner O, Sommer-Tsilenis E (1968).A tentative map suggesting the possible role of urinary minerals for the formation of renal stones. Renal Stone Research Symposium. Leeds 1968. Congress report, pp 273–288
Viville C, Petriconi R (1980) Cystinurie, lithiase cystinique et D-penicillamine. J Urol (Paris) 86:21–32
Zechner O, Latal D, Gründig E, Müller M (1976) Klinische Aspekte der Cystinurie und ihre Behandlung mit Thiola. Wien Klin Wochenschr 88:101–105
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Kallistratos, G., Dimopoulos, C., Kalfakakou-Vadalouka, V. et al. Familial cystinuria in Ioannina District (Greece) diagnosis and treatment. Urol. Res. 11, 291–296 (1983). https://doi.org/10.1007/BF00256349
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DOI: https://doi.org/10.1007/BF00256349