Autosomal recessive syndrome consisting of achalasia, alacrima and isolated adrenal insufficiency. Alacrima is theearliest and most consistent clinical sign. All affected children shows esophageal dysmotility even in the absence ofsymptomatic dysphagia.

The adrenal insufficiency is due to adrenocorticotropic hormone (ACTH) resistance. Otherassociated neurological abnormalities include autonomic, sensory, and upper- and lower-motor neuropathy, deafness, andmental retardation. Genetically the syndrome has been mapped to mutations in chromosome 12q13 region near the type Ikeratin gene cluster.

Though most cases arise in children between the ages of two and eight years, a few cases originateduring early adult years. Syn: triple-A syndrome..

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Allgrove syndrome .-::11769::-.