Computed Tomography of the Liver and Kidneys in Glycogen Storage Disease

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Abstract
Glycogen, in concentrations encountered in von Gierke''s disease, has computed tomography (CT) attenuation coefficients in the 50-70 Hounsfield unit (HU: 1,000 scale) range and accounts for the increased density of the liver. However, in 8 patients with Type I glycogen storage disease, simultaneous hepatic infiltration with fat and glycogen led to a range of liver CT densities from 13-80 HU. Fatty infiltration may facilitate the demonstration of hepatic tumors in older patients with this disease. Half the patients showed increased attenuation coefficients of the renal cortex, indicating glycogen deposition in the kidneys.