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

Zahra Razavi, Mohammad-Mehdi Taghdiri, Fatemeh Eghbalian, Nooshin Bazzazi
Iranian Journal of Pediatrics 2010, 20 (1): 101-6

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.

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