Edentulous child with allgrove syndrome: A rare case report

Vahedi, M. and Fathi, S. and Allahbakhshi, H. (2016) Edentulous child with allgrove syndrome: A rare case report. Korean Journal of Pediatrics, 59 (11). pp. 456-459.

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Abstract

Triple-A syndrome, also known as Allgrove syndrome, is a rare autosomal recessive disorder. The 3 features of this syndrome are achalasia, adrenal insufficiency, and alacrima. Achalasia could be the first manifestation of the triple-A syndrome; however, its etiology is unclear. Alacrima is generally asymptomatic but can be detected by obtaining patient history. Although adrenal insufficiency could have manifestations such as asthenia, it might be wrongly diagnosed as muscle fatigue. Vitamin D and calcium supplements are usually prescribed for the prevention of osteoporosis. Neurologic manifestations could be present in adults. In some individuals with this disorder, genetic examination indicates mutations in both alleles of the AAAS gene, which encodes a special 546-amino-acid protein designated ALADIN, and in chromosome 12q13. The genetic cause of the triple A syndrome in some patients who do not have an identified mutation is unknown. While very few such cases have been reported till date, one such case was presented to us as an edentulous child. © 2016 by The Korean Pediatric Society.

Item Type: Article
Additional Information: cited By 0
Subjects: Dentistry
Divisions: Faculty of Dentistry > Department of Dental and Oral Surgery
Depositing User: editor . 2
Date Deposited: 03 Mar 2017 17:50
Last Modified: 03 Mar 2017 17:50
URI: http://eprints.kaums.ac.ir/id/eprint/55

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