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

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.

journal_name

Iran J Pediatr

authors

Razavi Z,Taghdiri MM,Eghbalian F,Bazzazi N

subject

Has Abstract

pub_date

2010-03-01 00:00:00

pages

101-6

issue

1

eissn

2008-2142

issn

2008-2150

journal_volume

20

pub_type

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