Allgrove Syndrome

Said Azzoug; Faiza Boutekdjiret; Farida Chentli

Allgrove Syndrome

Said Azzoug

, Faiza Boutekdjiret

, Farida Chentli

Endocrinological department Bab El Oued Hospital Algiers, Algeria

Author

Correspondence author
International Journal of Clinical Case Reports, 2015, Vol. 5, No. 16 doi: 10.5376/ijccr.2015.05.0016
Received: 15 Feb., 2015 Accepted: 27 Mar., 2015 Published: 17 Apr., 2015

This is an open access article published under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Preferred citation for this article:

Azzoug et al., 2015, Allgrove Syndrome, International Journal of Clinical Case Reports, Vol.5, No.16 1-2 (doi: 10.5376/ijccr.2015.05.0016)

Abstract

Background Allgrove syndrome is a rare autosomal recessive disorder characterized by the association of the triad adrenal insufficiency, alacrima and achalasia.

Objective The aim of our study is to analyze the clinical characteristics of this rare syndrome.

Methods The medical records of eight patients (6M/2F) recorded over thirty three years period were reviewed.

Results In this report we demonstrate that the mean age at diagnosis is 10.75 years, in one patient the diagnosis was delayed at 27 years although the clinical symptoms began many years before. Alacrima was present in all patients, adrenal insufficiency was present in 75%, achalasia was present in 87.5% of cases, growth retardation and/or pubertal delay were reported in 75%, 62.5 % of patients have neurological dysfunction mainly pyramidal syndrome. Deficiency anemia was noted in 75%.

Conclusion: Allgrove syndrome is a rare hereditary disease which may have fatal consequences if undiagnosed, an earlier diagnosis and adequate treatment of the patient and genetic counseling for the family improve the prognosis of this disease.

Keywords

Allgrove syndrome; Adrenal insufficiency; Achalasia; Alacrima

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. Said Azzoug

. Faiza Boutekdjiret

. Farida Chentli