Urologia Internationalis
Cystinurietherapie mit AscorbinsäureAsper R. · Schmucki O.Medizinisch-chemisches Zentrallaboratorium (Direktor: Prof. D.J. Vonderschmitt) und Urologische Klinik (Direktor: Prof. G. Mayor), Universitätsspital, Zürich, Schweiz
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Article / Publication Details
Published online: January 29, 2010
Issue release date: 1982
Number of Print Pages: 19
Number of Figures: 0
Number of Tables: 0
ISSN: 0042-1138 (Print)
eISSN: 1423-0399 (Online)
For additional information: https://www.karger.com/UIN
Abstract
At the beginning of the four chapters on phaenomena, analysis, pathophysiology and therapy of cystinuria the essentials of the published literature are summarized. The frequency of cystinuria is in the order of 1:10,000. Besides the cystine lithiasis occurring in nine tenths of all cystinuria patients neurological diseases may also be observed. All commonly applied methods to analyze cystine detect the sum of cystine and cysteine. Cystinuria is characterized by a higher cystine excretion, up to the 100-fold of the normal. Also the concentrations of lysine, arginine and ornithine in the urine of cystinuria patients are elevated, caused by intestinal and renal transport defects. Inevitable damage of renal parenchyma by multiple operations can drastically be reduced by the therapy with D-penicillamine or α-mercaptopro-pionylglycine. The disadvantages of that formation of soluble asymmetric disulfides are the side effects, such as nausea, gastric difficulties and dermatosis, occurring in up to 50% of the patients. Using the especially developed method with HPLC separation and electrochemical detector with a mercury electrode, cystine and cysteine are analyzed simultaneously. In the urine of healthy persons the molar concentration of cysteine is in the same order as cystine. But in cystinuria the cysteine concentration in urine is about a thousand times less than that of cystine. These results are evidence that a shifted redox-equilibrium of cystine-cysteine is also typical of cystinuria. The molar cysteine percentage of cysteine in healthy persons is increased from 30 to 50% by oral ascorbic acid administration. Therefore a vitamine C therapy for cystinuria is developed. 31 cystinuria patients who receive 5 g of vitamine C a day show a decrease in the cystine concentration of about 40%. Up to now, no side effects have been observed. The most obvious sign of the positive effect of the proposed vitamine C therapy for cystinuria is the missing cystine sediment in fresh urine.
© 1982 S. Karger AG, Basel
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Article / Publication Details
Published online: January 29, 2010
Issue release date: 1982
Number of Print Pages: 19
Number of Figures: 0
Number of Tables: 0
ISSN: 0042-1138 (Print)
eISSN: 1423-0399 (Online)
For additional information: https://www.karger.com/UIN
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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