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Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
ISSN: 1018-4406
EISSN: 1018-4406
Vol. 20, No. 1, 2010, pp. 101-106
Bioline Code: pe10014
Full paper language: English
Document type: Research Article
Document available free of charge

Iranian Journal of Pediatrics, Vol. 20, No. 1, 2010, pp. 101-106

 en Premature Loss of Permanent Teeth in Allgrove (4A) Syndrome in Two Related Families
Razavi, Zahra; Taghdiri, Mohammad-Mehdi; Eghbalian, Fatemeh & Bazzazi, Nooshin


Background: Allgrove syndrome is a rare autosomal recessive condition characterized by adrenal insufficiency, achalasia, alacrima and occasionally autonomic disturbances. Mutations in the AAAS gene, on chromosome 12q13 have been implicated as a cause of this disorder.
Case(s) Presentation: We present various manifestations of this syndrome in two related families each with two affected siblings in which several members had symptoms including reduced tear production, mild developmental delay, achalasia, neurological disturbances and also premature loss of permanent teeth in two of them,
Conclusion: The importance of this report is dental involvement (loss of permanent teeth) in Allgrove syndrome that has not been reported in literature.

Achalasia, Adrenocortical Insufficiency, Alacrimia (Allgrove, triple-A) Protein, Human; AAAS Protein, Human; Teeth; Allgrove Syndrome; Triple A Syndrome Protein, Human

© © 2010 by Center of Excellence for Pediatrics, Children’s Medical Center, Tehran University of Medical Sciences,All rights reserved.
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