Adrenal gland & paraganglia
Adrenal insufficiency
Adrenal hypoplasia
Author: Nat Pernick, M.D.
Last author update: 1 February 2005
Last staff update: 15 January 2020
Copyright: 2002-2024, PathologyOutlines.com, Inc.
PubMed Search: Adrenal hypoplasia[TI]
Cite this page: Pernick N. Adrenal hypoplasia. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalhypoplasia.html. Accessed April 24th, 2024.
Definition / general
- Congenital aplasia is very rare; found in 10% with unilateral renal agenesis
- Bilateral hypoplasia is associated with anencephaly due to lack of ACTH cells; causes adrenal insufficiency
- Unilateral absence occurs in 1 per 10,000 live births
- May be associated with sudden infant death syndrome (Arch Pathol Lab Med 1977;101:168)
- Infant symptoms: weight loss, vomiting, dehydration and severe electrolyte disturbances - due to adrenal insufficiency
- Primary hypoplasia: X linked, associated with mutations or deletions of DAX-1 gene at Xp21; cortex is hypoplastic, but fetal zone is intact and often has cytomegalic features; associated with hypogonadotrophic hypogonadism in young men
- Miniature adult type of hypoplasia: sporadic or autosomal recessive; small glands but normal architecture
Gross description
- Small for age adrenal glands, decreased fetal zone in newborns, scattered cytomegalic cells, cells have decreased lipid
Differential diagnosis
- Chronic exogenous glucocorticoids: cause acquired hypoplasia
