METABOLISM IN NEUROMUSCULAR DISEASES 723 



venoiisly to throe dogs showing hypoglycemia as a result of hydrazin 

 poisoning, and the animals all died. 



Hypoglycemia is not the only evidence of disturbed carbohydrate 

 metabolism that has been observed in progressive muscular dystrophy. 

 Besides hypoglycemia there is : 



(1) Creatinurea. 



(2) A hypocholesterinemia. 



(3) A delayed glucose utilization. 



Creatinuria. Large quantities of creatin are constantly present in the 

 urine of these patients with progressive muscular dystrophy. Table 6 

 shows the amounts found per day in sixteen cases (the first five reported 

 by Levene and Kristeller(a), the next two by McCrudden and Sargent(a) 

 (6), the last nine by Janney, Goodhart, and Isaacson). 2 



TABLE 6 

 AMOUNT OF URINARY CREATIN PER DAY IN PROGRESSIVE MUSCULAR DYSTROPHY 



Traces of creatin have been reported occasionally in the urine of 

 apparently normal women, and creatin occurs in the urine of normal chil- 

 dren. But creatin does not occur in the urine of normal men. It has 

 been found in the urine in man in diabetes, in starvation (Benedict(fr)) 

 and when carbohydrate is withheld from the diet. That there is a rela- 

 tionship of some sort between carbohydrate metabolism and creatinuria 

 was first pointed out by Cathcart(6). This feature has been further 

 studied by Mendel and Rose, who showed that creatin disappears from the 

 urine in these cases when carbohydrate is administered; neither protein 

 nor fat can replace the carbohydrate in this respect. In dogs, creatinuria 

 accompanies hydrazin poisoning (Underbill and Kleiner) and phlorizin 

 poisoning (Underbill and Bauman(a)), both of these conditions accom- 

 panied by hypoglycemia. In the rabbit, an animal in which hypoglycemia 

 does not always follow hydrazin poisoning, creatinuria occurs only in 

 those cases in which hypoglycemia does follow (Macadam (6)). The con- 



2 As this book goes to press Brock and Kay report one more case, and Gibson, 

 Martin, and Buell nine cases, all of them showing creatinuria. Gibson and Martin 

 find that ingested creatin is promptly and completely eliminated in this disease. 



