Allgrove syndrome (triple A syndrome) is an autosomal recessive disorder characterized by achalasia, alacrima and adrenocorticotropic hor-mone (ACTH) resistant adrenal insufficiency. It is a multisystem disease and in addition to cardinal manifestations, associated features es-pecially neurologic problems, must be detected and treated. In this case report, we report a 17 year-old boy diagnosed as having Allgrove syndrome with predominant symptoms of achalasia and additional features consisting of short neck, long eye lashes, unexplained fever and chills, reduced visual acuity because of amblyopia, thenar and hypothenar atrophy and abnormal opposition of fingers. If necessary screening with stimulatory tests in patients with unexplained features such as long eye lashes, short neck, muscle atrophy, na-sal speech, skin and neurologic abnormalities and hyperkeratosis, should be recommended.

"/> Allgrove syndrome (triple A syndrome) is an autosomal recessive disorder characterized by achalasia, alacrima and adrenocorticotropic hor-mone (ACTH) resistant adrenal insufficiency. It is a multisystem disease and in addition to cardinal manifestations, associated features es-pecially neurologic problems, must be detected and treated. In this case report, we report a 17 year-old boy diagnosed as having Allgrove syndrome with predominant symptoms of achalasia and additional features consisting of short neck, long eye lashes, unexplained fever and chills, reduced visual acuity because of amblyopia, thenar and hypothenar atrophy and abnormal opposition of fingers. If necessary screening with stimulatory tests in patients with unexplained features such as long eye lashes, short neck, muscle atrophy, na-sal speech, skin and neurologic abnormalities and hyperkeratosis, should be recommended.

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Allgrove Syndrome: A Case Report

AUTHORS

A Soltani 1 , * , M Arab Ameri 2 , SH Hasani Ranjbar 2

1 Endocrinology and Metabolism Research Center, Tehran University of Medical Sciences, [email protected], I.R Iran

2 Endocrinology and Metabolism Research Center, Tehran University of Medical Sciences, I.R Iran

How to Cite: Soltani A, Arab Ameri M, Hasani Ranjbar S. Allgrove Syndrome: A Case Report, Int J Endocrinol Metab. Online ahead of Print ; 5(4):160-163.

ARTICLE INFORMATION

International Journal of Endocrinology and Metabolism: 5 (4); 160-163
Article Type: Case Report
Received: June 9, 2007
Accepted: July 17, 2007
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Abstract

Allgrove syndrome (triple A syndrome) is an autosomal recessive disorder characterized by achalasia, alacrima and adrenocorticotropic hor-mone (ACTH) resistant adrenal insufficiency. It is a multisystem disease and in addition to cardinal manifestations, associated features es-pecially neurologic problems, must be detected and treated. In this case report, we report a 17 year-old boy diagnosed as having Allgrove syndrome with predominant symptoms of achalasia and additional features consisting of short neck, long eye lashes, unexplained fever and chills, reduced visual acuity because of amblyopia, thenar and hypothenar atrophy and abnormal opposition of fingers. If necessary screening with stimulatory tests in patients with unexplained features such as long eye lashes, short neck, muscle atrophy, na-sal speech, skin and neurologic abnormalities and hyperkeratosis, should be recommended.

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