Three cases of triple A syndrome (Allgrove syndrome) in pediatric surgeons’ view

Abstract

Triple A syndrome, also known as Allgrove syndrome, is a rare disease, and presents mainly in children. Its cardinal symptoms are achalasia, alacrima, and adrenocorticotropic hormone (ACTH) insensitivity. We report three cases of Triple A syndrome. Our aim is to inform pediatric surgeons about the existence of this rare syndrome and to highlight the need for suspicion of alacrima and ACTH insensitivity in cases of pediatric achalasia. Triple A syndrome should be considered in patients presenting with achalasia. Alacrima should be investigated by a Schirmer test, and adrenal dysfunction should be tested in cases of suspected triple A.

Neurological Characteristics of Allgrove Syndrome: A Case Series

“…

Triple A syndrome, also known as Allgrove syndrome, was first described in 1978 within a North African family [1]. This condition is extremely rare, with fewer than 200 cases reported to date [2,3]. Allgrove syndrome is an autosomal recessive disorder resulting from a mutation in the AAAS gene, which is located on the long arm of chromosome 12 (12q13).

…”