Studies in Muscular Dystrophies the Presence of Simple Gtjanidine Derivatives in the Urine

In studies on muscular dystrophy in young individuals investigations of the urine were made along three lines, as follows: (a) the estimation of creatinine and creatine, according to Folin (1) and Benedict (2) respectively; (b) the study as to whether the creatine findings include other substances than creatine; (c) the possibility that in muscular dystrophy guanidine intoxication plays a prominent r61e. The present paper deals primarily with the guanidine question with some attention to the creatine to creatinine ratio. Unsubstituted guanidine was early shown by Gergens and Baumann (3) and by Putzeys and Swaen (4) to have an injurious action, especially on muscle function, findings later corroborated by Fiihner (5); while Kawakita (6) found guanidine toxic for plants, a finding verified by unpublished work from this laboratory. To facilitate the study of guanidine in relation to health and disease Sullivan (7) devised a new test with a high degree of specificity for unsubstituted guanidine, NH : C(NH&. This test is not given by any substituted guanidine. Accordingly, it is not given by methylguanidine which might be formed from creatine and creatinine by oxidation, as early shown by Dessaignes (8) and corroborated by Ewins (9), Baumann and Ingvaldsen (lo), and by Greenwald (11). When the guanidine reaction was applied to urines, little evidence was obtained of the presence of unsubstituted guanidine in normal or pathological urine. A search was then made for simple derivatives comparable to glycocyamine or glycocyamidine, which