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Endocrinol Metab : Endocrinology and Metabolism


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Mir Iftikhar Bashir  (Bashir MI) 1 Article
Adrenal gland
Allgrove (Triple A) Syndrome: A Case Report from the Kashmir Valley
Raiz Ahmad Misgar, Nazir Ahmad Pala, Mahroosa Ramzan, Arshad Iqbal Wani, Mir Iftikhar Bashir, Bashir Ahmad Laway
Endocrinol Metab. 2015;30(4):604-606.   Published online December 31, 2015
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AbstractAbstract PDFPubReader   

Allgrove (Triple A) syndrome is a rare autosomal recessive disorder characterized by cardinal features of adrenal insufficiency due to adrenocorticotropic hormone (ACTH) resistance, achalasia, and alacrimia. It is frequently associated with neurological manifestations like polyneuropathy. Since its first description by Allgrove in 1978, approximately 100 cases have been reported in the literature. Here we report an 18-year-old boy diagnosed as having Allgrove syndrome, with ACTH resistant adrenal insufficiency, achalasia, alacrimia, and severe motor polyneuropathy. Alacrimia was the earliest feature evident at the age of 8 years. He presented with achalasia and adrenal insufficiency at 12 and 18 years respectively and developed neurological symptoms in the form of severe muscle wasting at the age of 15 years. Patients with Allgrove syndrome usually manifest adrenal insufficiency and achalasia during first decade of life. Our patient manifested adrenal insufficiency and achalasia in the second decade and manifested neurological dysfunction before adrenal dysfunction.


Citations to this article as recorded by  
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  • Clinical and molecular report of c.1331 + 1G > A mutation of the AAAS gene in a Moroccan family with Allgrove syndrome: a case report
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