538 KAHN AND KOSENBLOOM 



Treatment of Cystinuria 



The therapy of cystinuria deserves notice. No scientist has yet been 

 able to influence the metabolism in such wise as to prevent excretion of 

 cystin. Baer recommended the exclusion of protein from the diet and 

 the ingestion of very much water in order to dilute the urine. Beale 

 advised the use of ammonium carbonate which he thought will keep the 

 cystin in solution. Simon found that on the addition of cholic acid, there 

 was no reduction in the cystin output. Klemperer and Jacobi have re- 

 cently advised alkaline treatment for cystinuria. On a test diet they 

 found that the proportion of cystin in the urine became much reduced 

 when the patient refrained from protein in the food. The amount also 

 decreased remarkedly upon the administration of an alkali. Both the 

 amount of sediment and the proportion of cystin present decreased to 

 zero under the influence of from 6 to 10 gm. daily of sodium bicarbonate. 

 This case shows that when the ordinary reaction of the blood does not 

 permit the complete cleavage of the cystin, this can be accomplished by 

 rendering the blood a little more alkaline. 



According to Smillie cystinuria is best treated by a low protein diet, 

 with the addition of sufficient alkali to keep the urine alkaline. Cystin 

 crystals will practically disappear from the urine of a cystinuric when 

 sufficient sodium bicarbonate is added to the diet to render the urine 

 alkaline. The amount of sodium bicarbonate necessary to render the 

 urine alkaline, wheii the patient is on a nitrogen intake of 10 gm. or 

 more, is greater than can be well borne by the stomach. Sodium bicar- 

 bonate does not influence body metabolism in cystinuria, but simply 

 renders the cystin soluble. 



Neumann states that 170 cases of these conditions have now been 

 reported. Of late years interest in cystin has notably increased. The 

 author has seen two cases which he reports in great detail. The first 

 case was a renal calculus. Family and personal history of the twenty- 

 four-year-old girl was good. Up to a certain day her health had been 

 perfect and the urine normal. Within two months the entire disease- 

 picture developed. Upon admission she presented a high degree of pallor. 

 Chest, abdomen, and general nutrition were without any finds. A tumor 

 was palpable at the site of the right kidney with patient in left lateral 

 decubitus. Left kidney seemed normal. Urine contained cystin crystals 

 and blood corpuscles. A part of the diagnosis was therefore readily 

 made. Under pyelography the X-ray revealed the presence of a stone in 

 the kidney pelvis. Pyelotomy then revealed a cystin calculus. This was 

 removed, everything healing cleanly. The patient, however, showed no 

 corresponding improvement. The kidney which had shown numerous 

 little points of suppuration at the operation continued to give trouble 



