- Human pathology

Home > E. Pathology by systems > Digestive system > AAA syndrome

AAA syndrome

Wednesday 16 May 2007

Allgrove syndrome associates achalasia, addisonianism, alacrima (OMIM 231550), It is an autosomal recessive disorder recently associated with the AAAS gene coding for the Aladin protein.

The pathophysiology of achalasia in Allgrove’s syndrome remains obscure.


- fibrosis of the intermuscular plane
- myenteric ganglia
- Neuronal NO synthase absent


- The triple-A syndrome is caused by mutation in the gene encoding aladin (AAAS) (MIM.605378).


- Khelif K, De Laet MH, Chaouachi B, Segers V, Vanderwinden JM. Achalasia of the cardia in Allgrove’s (triple A) syndrome: histopathologic study of 10 cases. Am J Surg Pathol. 2003 May;27(5):667-72. PMID: 12717251