Achalasia-alacrima-ACTH insensitivity syndrome (triple a syndrome)

Abstract

Triple A syndrome is characterized by achalasia of the cardia, alacrima, adrenocorticotrophic hormone (ACTH) resistant adrenal insufficiency and progressive neurological abnormalities including autonomic nervous dysfunction. An 8-year-old girl presented to the pediatric intensive care unit with sudden loss of consciousness and was diagnosed subsequently to have this condition. The authors present this condition since it is easily treatable and can be fatal if undiagnosed.

Endocrine emergencies in critically-ill patients: Challenges in diagnosis and management: Comment

“…Children with ACTH insensitivity may present at any age with hypotension and hypoglycemic seizures. [5] A careful clinical history maintaining a high index of suspicion for an endocrine problem and aggressive management are required to prevent mortality from endocrine emergencies.…”