CYSTINURIA 527 



Bodtker(a) (&) reported three cases of cystinuria, one in 1892 and one 

 in 1905, on one of whom he made extensive metabolic studies. He found 

 no disturbance in nitrogen metabolism. The average daily output of 

 neutral sulphur was 23.7 per cent of the total sulphur. 



Abderhalden (&) reported the occurrence of five cases of cystinuria in 

 children of the same family. One was a boy Sl 1 /^ months old, the 

 other a girl 9^ months old and the third a boy of 17 months old when 

 they died. Two other children, boys, are alive but suffer from the same 

 disturbance. In 500 c.c. of urine of the father, 34 years of age, Abder- 

 halden found 0.046 gm. cystin. No cystin was present in the urine of 

 the mother. In the urine of the paternal grandfather 0.07 gm. cystin 

 was found in 500 c.c. of urine. In the urine of the paternal grand- 

 mother no cystin was found. Abderhalden calls attention to the fact 

 that in the paternal side of the family there is a history of tuberculosis 

 and gastrointestinal disease. 



Ackermann and Kutscher found lysin in the urine of a patient who 

 excreted cystin. Ackermann, in 1913, reported that in addition to lysin, 

 he found leucin and tyrosin in the urine. But the presence of amino 

 and diamino acids in the urine is not constant in cystinuric patients. 

 Wolff, Shaffer and Osterberg reported that they could not find any 

 diamins in the urine. Ilele found cadaver in once in the urine of his 

 patient. It has been reported by Thiele that a meat diet increases the 

 output of cadaverin. 



Simon and Campbell did not find any amino acids, other than cystin, 

 in the urine of their patient. 



Abderhalden and Samuely fed cystin per os to a dog which increased 

 the output of neutral and oxidized sulphur. With the progress of the 

 experiment more and more of the cystin sulphur appeared in the urine in 

 the oxidized state. Dialanyl cystin behaved similarly. When cystin, 

 dialanyl cystin and dileucyl cystin were administered subcutaneously, 

 the impression was obtained that the sulphur of the peptids is excreted 

 less rapidly than the sulphur of the cystin itself. 



The table on page 528 is a protocol of their results. They gave 

 their animal 8 gm. dileucyl cystin subcutaneously, but they could not 

 obtain any leucin in the urine. This shows that the dog burnt the entire 

 amount of leucin given. 



Calculating from the figures recorded for a number of cases of 

 cystinuria, Garrod shows that the cystin: nitrogen ratio is variable. (See 

 table on page 529.) 



Wolf and Shaffer carried out extensive metabolic studies on patients 

 excreting cystin. 



Their results wholly confirm the findings of Alsberg and Folin, that 

 sulphur of hair or protein-cystin fed to a cystinuric patient is normally 

 oxidized to sulphuric acid, and are directly contradictory to the conclusion 



