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CASE REPORT
Year : 2019  |  Volume : 2  |  Issue : 1  |  Page : 13-15

Allgrove syndrome (Triple-A Syndrome): A case report from north India


1 Rajiv Gandhi Centre for Diabetes and Endocrinology, JN Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India
2 Department of Medicine, JN Medical College, Aligarh Muslim University, Aligarh, Uttar Pradesh, India

Correspondence Address:
Dr. Hamid Ashraf
Rajiv Gandhi Centre for Diabetes and Endocrinology, JN Medical College, Aligarh Muslim University, Aligarh - 202 002, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdep.jdep_11_18

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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.


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