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Triple-A syndrome (also known as Allgrove Syndrome) is a rare autosomal recessive disorder. The syndrome was discovered by Jeremy Allgrove
Jeremy Allgrove

Dr. Jeremy Allgrove is consultant paediatric endocrinologist specialising in paediatric diabetes at Barts and The London NHS Trust. He is the representative of The Royal College of Paediatrics and Child Health on the Diabetes Dataset Advisory Group ....
 and colleagues in 1978. Triple A stands for achalasia-addisonianism-alacrima syndrome.

Sufferers have adrenal insufficiency, alacrima (absence of tear secretion), and achalasia
Achalasia

Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, and esophageal aperistalsis, is an esophageal motility disorder: The smooth muscle cell layer of the esophagus loses normal peristalsis , and the lower esophageal sphincter fails to relax properly in response to swallowing....
. Achalasia is a failure of a ring of muscle fibers, such as a sphincter, to relax. Triple-A patients have achalasia of the lower oesophageal sphincter at the cardia
Cardia

The cardia is the anatomy term for the junction orifice of the stomach and the esophagus. At the cardia, the mucosa of the esophagus transitions into gastric mucosa....
 which delays food going to the stomach and causes dilation of the thoracic oesophagus.






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Triple-A syndrome (also known as Allgrove Syndrome) is a rare autosomal recessive disorder. The syndrome was discovered by Jeremy Allgrove
Jeremy Allgrove

Dr. Jeremy Allgrove is consultant paediatric endocrinologist specialising in paediatric diabetes at Barts and The London NHS Trust. He is the representative of The Royal College of Paediatrics and Child Health on the Diabetes Dataset Advisory Group ....
 and colleagues in 1978. Triple A stands for achalasia-addisonianism-alacrima syndrome.

Sufferers have adrenal insufficiency, alacrima (absence of tear secretion), and achalasia
Achalasia

Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, and esophageal aperistalsis, is an esophageal motility disorder: The smooth muscle cell layer of the esophagus loses normal peristalsis , and the lower esophageal sphincter fails to relax properly in response to swallowing....
. Achalasia is a failure of a ring of muscle fibers, such as a sphincter, to relax. Triple-A patients have achalasia of the lower oesophageal sphincter at the cardia
Cardia

The cardia is the anatomy term for the junction orifice of the stomach and the esophagus. At the cardia, the mucosa of the esophagus transitions into gastric mucosa....
 which delays food going to the stomach and causes dilation of the thoracic oesophagus. Managed effectively, patients can have a normal lifespan and bear children.

The syndrome is highly variable.In 2000, Huebner et al mapped the syndrome to a 6 cM interval on human chromosome 12q13 near the type II keratin gene cluster.

See also

  • achalasia
    Achalasia

    Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, and esophageal aperistalsis, is an esophageal motility disorder: The smooth muscle cell layer of the esophagus loses normal peristalsis , and the lower esophageal sphincter fails to relax properly in response to swallowing....
    , addisonianism
    Addison's disease

    Addison's disease is a rare endocrine disorder in which the adrenal gland doesn't produce enough steroid hormones . It may develop in children and adults, and may occur as the result many underlying causes....
    , alacrima


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