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Premature Loss of Permanent Teeth in Allgrove (4A) Syndrome in Two Related Families

Identifieur interne : 004171 ( Ncbi/Checkpoint ); précédent : 004170; suivant : 004172

Premature Loss of Permanent Teeth in Allgrove (4A) Syndrome in Two Related Families

Auteurs : Zahra Razavi ; Mohammad-Mehdi Taghdiri ; Fatemeh Eghbalian ; Nooshin Bazzazi

Source :

RBID : PMC:3446014

Abstract

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.


Url:
PubMed: 23056690
PubMed Central: 3446014


Affiliations:


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PMC:3446014

Le document en format XML

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