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Definition

An autosomal recessive disorder characterized by adreno-corticotropic hormone (ACTH)-resistant adrenal failure, achalasia of the esophageal cardia and alacrima. The syndrome is associated with variable and progressive neurological impairment involving the central, peripheral, and autonomic nervous system. Other features such as palmoplantar hyperkeratosis, short stature, facial dysmorphy and osteoporosis may also be present.

Acronym

AAAS

Synonyms

ACTH-resistant adrenal insufficiency with achalasia and alacrima
Addisonian-achalasia syndrome
Alacrima-achalasia-addisonianism
Alacrima-achalasia-adrenal insufficiency neurologic disorder
Allgrove's syndrome
Allgrove syndrome
Glucocorticoid deficiency and achalasia
Hypoadrenalism with achalasia
Triple-A syndrome

 
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