Echogenic kidneys and medullary calcium deposition in a young child with glycogen storage disease type 1a.

We report the case of a young child with Glycogen Storage Disease (GSD) type-Ia who developed echogenic kidneys, medullary calcium deposition and disturbance of renal function. These severe renal abnormalities are seen in young adults whose GSD-I has been ineffectively treated. Renal disease can be considered a major problem in GSD-I.