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

ERGINEL B., Gün F., KOCAMAN H., ÇELIK A., Salman T.

Acta chirurgica Belgica, vol.116, pp.119-21, 2016 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 116
  • Publication Date: 2016
  • Doi Number: 10.1080/00015458.2016.1139835
  • Journal Name: Acta chirurgica Belgica
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.119-21
  • Istanbul University Affiliated: Yes

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.