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Triple A syndrome (Allgrove syndrome): improving outcomes with a multidisciplinary approach

Authors Flokas ME, Tomani M, Agdere L, Brown B

Received 14 February 2019

Accepted for publication 11 July 2019

Published 29 August 2019 Volume 2019:10 Pages 99—106


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Roosy Aulakh

Myrto Eleni Flokas, Michael Tomani, Levon Agdere, Brande Brown

Department of Pediatrics, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, USA

Correspondence: Brande Brown
Department of Pediatrics, NewYork-Presbyterian Brooklyn Methodist Hospital, 506, 6th Street, Brooklyn, NY 11215, USA
Tel +1 718 780 5260
Fax +1 718 780 3266
Email [email protected]

Abstract: Allgrove syndrome or triple A (3A) syndrome is a multisystem disorder which classically involves the triad of esophageal achalasia, alacrima, and adrenal insufficiency due to adrenocorticotropin hormone insensitivity. It follows an autosomal recessive pattern of inheritance and is associated with mutations in the AAAS (achalasia–addisonianism–alacrima syndrome) gene. Since its first description in 1978, the knowledge on clinical and genetic characteristics has been expanding; however, the current literature is limited to case reports and case reviews. Early recognition of the syndrome is challenging, given the rarity of the condition and high phenotypic heterogeneity even among members of kin. The coordination of care for these patients requires a multidisciplinary team of specialists, including endocrinologists, neurologists, gastroenterologists, ophthalmologists, developmental specialists, dentists, geneticists, and surgeons. In this review, we aim to summarize the current recommendations for the diagnosis, management, and follow-up of patients with 3A syndrome.

Keywords: AAA, guidelines, alacrima, achalasia, adrenal failure

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