Allgrove (AAA) Syndrome Addison, Achalasia, Alacrimia

Abstract

Triple A syndrome is an extremely rare syndrome. It is an autosomal recessive disorder characterized by adrenal
insufficiency, alacrima and achalasia. 16 patients from different parts of Libya were selected and were studied for
the disorder in our Department of Pediatric Endocrinology, Tripoli Medical Centre, Libya. The patients presented
with classical signs and symptoms of adrenal insufficiency, ocular symptoms and achalasia which developed
subsequently. The diagnosis was based on clinical grounds and some laboratory studies like electrolyte disbalance
due to mineralocorticoid deficiency (Adrenal crisis), high base ACTH, low cortisol level and aldosterone levels.
Barium swallows demonstrated achalasia of esophagus. Careful replacement therapy with glucocorticoid, topical
lubricant for the eyes (artificial tears) and dilatation of the esophagus was performed for dysphagia.