Type 1 glycogenosis with contracted kidneys and liver cell adenoma

A 22-year-old man with type 1 glycogenosis died of renal and respiratory failure. Postmortem examination revealed deposition of glycogen in liver cells, a liver cell adenoma, bilateral contracted kidneys with scent glycogen deposition, and pulmonary edema. The development of liver cell adenoma was thought to be related to underlying metabolic disorder and contracted kidneys were considered to be the sequel of massive glycogen deposition in the renal tubular epithelium.