Von Gierke1 in 1929 described cases of hypertrophy of the liver and kidneys caused by excess accumulations of glycogen. This entity was given the name of glycogenosis. Mason and Andersen2 showed that this condition was due to abnormal metabolism of glycogen resulting from a congenital defect in that portion of the enzyme system of the liver which converts glycogen to dextrose and dextrose to glycogen.
It is not within the scope of this paper to review the literature or reports of cases of glycogenosis, as this has been excellently done by Mason and Andersen2 and by van Creveld.3 The purpose of this report is to present a case of von Gierke's disease occurring in a Negro infant in a large hospital, where investigation shows that only 2 cases have been found in more than 500,000 admissions in the past eleven years. It is also of interest