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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
DOI https://doi.org/10.2147/PHMT.S173081
Checked for plagiarism Yes
Review by Single-blind
Peer reviewers approved by Dr Nicola Ludin
Peer reviewer comments 2
Editor who approved publication: Dr 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 bmb9005@nyp.org
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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