i'i,-(U)i <'rs or Fh'h'\ii:\TAT/<>.\ or <• AUiioiiyiu; M-rn" 583 



putrefac'tioiu l»ut is formed in the tissues from the protein molecule, 

 and fails to he further decomposed because of some anomaly of metab- 

 olism. This view is supported by the fact that cj'stinuria often ap- 

 pears to be an hereditary disease, occurrino: in families for several 

 jivnerations; it is independent of the diet, cystine appearin<>- even if 

 proteins are withheld, and also independent of intestinal putrefac- 

 tion."'" It having been found that leucine and tyrosine may also occur 

 in the urine in cystinuria, it seems probable that this condition de- 

 pends upon a general abnormality of protein metabolism. The rela- 

 tion of the diamines to the condition is, however, very uncertain. 

 Cystine does not seem to exert any toxic effect, and patients with 

 cystinuria do not usually appear to sutfer greatly from the abnormal 

 metabolism, the chief trouble observed being due to the formation of 

 the concretions in the bladder.""'' Sometimes in children, however, 

 emaciation and early death, without other apparent cause, have been 

 observed, and may be due to the metabolic anomaly. 



The metabolic error in cystinuria is not complete, for only a por- 

 tion of the total cystine of the catabolized proteins is excreted as 

 such (Garrod). This would amount to some five grams per day, 

 whereas the average excretion is only about 0.3-0.5 gram, and sul- 

 phates and other neutral sulphur compounds are always present in 

 the urine. In no condition other than cystinuria have putrescine and 

 cadaverine been found in quantities which could be detected by ordi- 

 nary methods in 2-4-hour specimens ; they may also be found in the 

 feces of cystinurics, where cystine is never found. In the urine their 

 presence is inconstant, and the amounts are at best very small. Leu- 

 cine and tyrosine are found much less often than the diamines ; lysine, 

 has been found in one case,^* which supports the view that cadaverine 

 and putrescine come from the diamino-acids of the protein molecule 

 by metabolism rather than by putrefaction. 



B. PRODUCTS OF FERMENTATION OF CARBOHYDRATES 



These include practically all the members of the fatty acid series, 

 from formic acid to valerianic ackl; and the oxy-acids, lactic, succinic, 

 and o.ryhutijric; also, oxalic acid, acetone, ethyl alcohol, and the fol- 

 lowing gases: CO^, CH^, H,. For the most part, the various organic 

 acids are absorbed through the intestinal walls, and are oxidized 

 completely in the tissues without causing any harm whatever. The 

 possibility that acid intoxication may be produced in this way has 

 been suggested, but it is generally believed that this does not occur, 

 except possibly in infants. Lactic and butyric ''^^ acids are formed 



57 An isolated case of transient cystinuria in a patient with Raynand's disease 

 is described by Githens (Penn. ^led. Jour., 1910 (1), 507). 



57a This may be avoidetl by decreasinrr the cystine by means of a low jirotein 

 diet, and increasinof its solubility by keeping the reaction of tlie urine alkaline 

 (Smillie. Arch. Int. Med.. 1915 (IG), 50.3). 



•''•'^ Ackermann and Kutscher, Zeit. f. Biol., 1011 (57), 355. 



5sa Coleman (Ann. Inst. Pasteur, 1915 (29), 139) attempts to incriminate 



