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Allgrove syndrome: case report of 7 years old boy from Bahawalpur.

Authors :
Akram S
Khan MA
Rehman A
Source :
JPMA. The Journal of the Pakistan Medical Association [J Pak Med Assoc] 2018 Aug; Vol. 68 (8), pp. 1260-1262.
Publication Year :
2018

Abstract

Allgrove syndrome is a rare autosomal recessive syndrome of unknown prevalence. The first case of Allgrove syndrome was reported in 1978 by Allgrove. It is characterized by triad of achalasia, alacrima and adrenal hypoplasia. There are also associated autonomic and neurological manifestations. We report the case of a 7 years old boy being treated for achalasia cardia, presented with fits and altered sensorium which on further investigations was found to be due to adrenal insensitivity (Raised ACTH level, low Cortisol level, and normal Aldosterone and Renin ratio). He also had undiagnosed alacrima since birth, mild degree of hearing loss and autonomic instability in the form of episodic hypertension.

Details

Language :
English
ISSN :
0030-9982
Volume :
68
Issue :
8
Database :
MEDLINE
Journal :
JPMA. The Journal of the Pakistan Medical Association
Publication Type :
Academic Journal
Accession number :
30108399