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DOI: 10.4103/jdep.jdep_11_18
Allgrove syndrome (Triple-A Syndrome): A case report from north India
Triple-A syndrome, also known as Allgrove syndrome, is an uncommon disorder which is inherited as an autosomal recessive disorder. About 100 cases have been described in literature. The three AAA comprises adrenal insufficiency secondary to adrenocorticotropic hormone (ACTH) resistance, achalasia cardia, and alacramia. We are reporting a case of a 10-year-old boy diagnosed as triple-A syndrome with ACTHresistant adrenal insufficiency, achalasia cardia, and alacrimia. He has alacramia since birth, and at the age of 7 years, he was diagnosed to have achalasia cardia. He developed the symptoms of adrenal insufficiency at the age of 9 years. Allgrove syndrome might be underreported in literature as the diagnosis requires high index of suspicion. In our patient, there was a delay of 3 years after the initial diagnosis of achalasia cardia. The diagnosis of Allgrove syndrrome should be considered in every child presenting with alacremia or achalasia cardia.
Keywords
Achalasia - adrenal insufficiency - adrenocorticotropic hormone resistance - alacremia - nervous systemFinancial support and sponsorship
Nil.
Publikationsverlauf
Eingereicht: 06. Oktober 2018
Angenommen: 08. Dezember 2019
Artikel online veröffentlicht:
08. Juni 2022
© 2019. Gulf Association of Endocrinology and Diabetes (GAED). All rights reserved. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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