Adrenal gland and paraganglia
Congenital anomalies
Storage diseases - Pompe disease

Topic Completed: 1 February 2005

Revised: 29 May 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Pompe disease adrenal

See Also: Adrenoleukodystrophy  Wolman disease

Nat Pernick, M.D.
Page views in 2018: 177
Page views in 2019 to date: 270
Table of Contents
Definition / general
Cite this page: Pernick N. Storage diseases - Pompe disease. website. Accessed October 19th, 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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