CYSTINURIA 525 



The metabolic changes in cystinuria were studied by the earlier 

 chemists in this field. Stromeyer and Prout reported that cystinuric 

 individuals excreted daily less urea than normal individuals. Venables 

 in 1830 described the case of a cystinuric patient who did not excrete 

 any urea at all in the urine, a fact which appears very doubtful. Toel, 

 in 1855, on the contrary found in his case of. a woman who excreted 

 daily 1.3 to 1.5 gm. of cystin, that the output of urea and uric acid was 

 entirely normal. Lobisch, whose patient was a young physician, found 

 that the excretion of cystin in the urine had no effect on the urea, uric 

 acid and sodium chlorid output. Bartels(a) also did not find any change 

 in the urea output in such patients. But Beneke confirmed Stromeyer 

 and Prout's findings. He found less urea and uric acid in the urine of 

 the cystinuric patient, and Sir Astley Cooper and Prout reported a case 

 where no uric acid was found and the urea output was diminished. Willis 

 found similar results. 



Niemann and Cantani (1877) found the uric acid excretion dimin- 

 ished and the urea output normal. Simon observed a high uric acid 

 content of the urine in his case and Stadthagen and also Leo (a) found a 

 normal amount of uric acid in the cases they studied. Prechini and Conti, 

 on the contrary, found the uric acid increased and the urea normal 

 in the urine of their patient. These authors compare cystinuria to 

 oxaluria. 



On the other nitrogenous substances present in the urine together 

 with cystin quite a number of researches have been made: 



Von TJdranszky and Baumann found in the urine of a patient suf- 

 fering with cystin deposits in the bladder and cystinuria, certain basic 

 nitrogenous substances "ptomaines" which they identified as cada- 

 verin and putrescin. Neither the indol nor the phenol excretion was 

 above normal. This would indicate that the intestinal putrefaction is 

 not above normal. They also found diamins in the urine and feces 

 of the cystinuric. Garcia examined this case four years later and found a 

 higher diamin excretion than TJdranszky and Baumann. Borissow also 

 studied the same case one year after Garcia and obtained still higher 

 figures. In two other cases studied by Brieger and Stadthagen similar 

 results were obtained. 



These diamins were found in the urine of such patients by Lewis 

 and Simon, Simon, and other authors. The amount of these amino 

 acids is not constant, having periods of increased and lessened excretion. 

 (Garrod, Garrod and Cammidge, Schollberg.) 



Garrod(gr), in his brilliant lectures on "Inborn Errors of Metabolism," 

 gives a table showing the amount of cystin excreted by cystinuric patients. 

 We cite his table herewith: 



