Preferred Label : Triple A syndrome;
ICD-11 definition : Triple A syndrome is an autosomal recessive disorder characterized by the triad of
adrenocorticotropic hormone (ACTH) resistant adrenal insufficiency, achalasia (hypertrophy
of the gastroesophageal cardia, preventing food from entering the stomach), and alacrima
(absence of tears).;
ICD-11 synonym : Allgrove syndrome; Achalasia - addisonianism - alacrima syndrome;
Origin ID : 180128195;
Currated CISMeF NLP mapping
Triple A syndrome is an autosomal recessive disorder characterized by the triad of
adrenocorticotropic hormone (ACTH) resistant adrenal insufficiency, achalasia (hypertrophy
of the gastroesophageal cardia, preventing food from entering the stomach), and alacrima
(absence of tears).
