Under the title ' chronic hepatogenic hypoglyeaemia in childhood ' we described1', some time ago a number of investigations in a boy who had had a very large liver since birth. Clinically he gave the impression of adiposogenital dystrophy, and, in addition, he showed a very marked disturbance of carbohydrate metabolism. The predominant features of this disturbance were chronic hypoglyeaemia in the fasting state, without the usual symptoms, and accompanied by ketosis; little or no change in the blood-sugar value after subcutaneous injection of adrenalin, and hypersensitiveness to small quantities of insulin. As a cause of this anomalous disturbance we conceived a continuation of the foetal condition, and we sought in faulty glycogenolysis an explanation of the condition. Thus in our boy the ketosis and the absence of hyperglycaemic response to adrenalin might not depend upon a poor glycogen content of the liver.We found support for our ideas especially in the publications of von Gierke3'4 and Sch6nheimer5. We thought we had to deal in our boy with a case of hepatomegalia glycogenica as described by von Gierke, and that the disturbances in metabolism in our case were characteristic for this form of liver hypertrophy. The features of this hypertrophy were the accumulation of glycogen, which during life and after death could be mobilized only with difficulty. Since then the interest in ' glycogen disease has-greatly increased and different authors6-12a have published definite cases of ' hepatomegalia glycogenica,' which have been studied clinically. The deviations in metabolism found by these authors as a whole agree with those found in our case. In our opinion our case is therefore one of hepatomegalia glycogenica and in this we are supported by the recent findings of other investigators. It is important to note that this interesting glycogen disease must be regarded as a general disease of metabolism, in which the glycogen can only be mobilized with difficulty, and may accumulate in various organs, not only in the liver. In this way it may give rise to hypertrophy of different organs. This accumulation of glycogen plays a big role in the occurrence of a number of congenital deviations and in those arising shortly after birth. Thus, it is a disturbance which merits the consideration of paediatricians, and the study of this disturbance will increase our insight into several diseases of childhood. Further, glycogen disease and on 12 May 2018 by guest. Protected by copyright.
