Table of ContentsDefinition / general
Cite this page: Storage diseases, Pompes disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenalstoragediseases_pompes.html. Accessed November 12th, 2018.
Definition / general
- Also called type II glycogenosis
- Infantile form of generalized glycogenosis, due to deficiency of lysosomal acid alpha-1,4-glucosidase
- Intralysosomal glycogen storage in CNS, heart, liver, skeletal muscle, thyroid, parathyroid, pituitary and pancreatic islets
- Also in adrenal cortex and medulla with severe accumulation in zona fasciculata (Arch Pathol Lab Med 1985;109:921)