Adrenal gland and paraganglia
Congenital anomalies
Storage diseases - Pompe disease


Topic Completed: 1 February 2005

Revised: 19 December 2018

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Pompe disease adrenal

See Also: Adrenoleukodystrophy  Wolman disease
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Table of Contents
Definition / general
Cite this page: Pernick N. Storage diseases - Pompe disease. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/adrenalstoragediseases_pompes.html. Accessed March 20th, 2019.
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)
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