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STUDIES ON THE TREATMENT OF CYSTINURIA 1
Author(s) -
Dent C. E.,
Senior B.
Publication year - 1955
Publication title -
british journal of urology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.773
H-Index - 148
eISSN - 1464-410X
pISSN - 0007-1331
DOI - 10.1111/j.1464-410x.1955.tb03486.x
Subject(s) - cystinuria , urine , cystine , fluid intake , supersaturation , medicine , chemistry , aminoaciduria , biochemistry , cysteine , organic chemistry , enzyme
SUMMARY1 The treatment of cystinuria has been investigated bearing in mind present concepts of the pathogenesis of the disease and using biochemical criteria of success or otherwise. 2 Treatments recommended in the literature and a number of further therapeutic possibilities that we ourselves thought of were uniformly unsuccessful. 3 The solubility of cystine in urine at various physiological p H's has been determined experimentally. The results show that under normal conditions the daily output of cystine of a cystinuric subject can be held in solution by about 3 litres of urine at pH 6. 4 While an alkaline urine has an increased solvent power for cystine the pH must be over about 7.6 before appreciably increased quantities are held in solution. Since this pH cannot be maintained during the long‐term management of a cystinuric subject, alkalis are of doubtful value as a prophylactic measure. 5 The day urine of a cystinuric subject is usually undersaturated with cystine while the night urine is supersaturated. It is concluded that stones probably form as a result of precipitation from the concentrated night urine. 6 By promoting a high fluid intake at regular intervals one may be able to ensure the passage of a urine that is constantly undersaturated with cystine. Under these circumstances stone formation is impossible, and if stones are already present dissolution should be initiated. 7 A long‐term clinical follow‐up of the effects of this regime of fluid intake is in progress.