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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Triple A syndrome


Other Names for this Disease
  • AAA
  • AAA syndrome
  • Achalasia Addisonianism Alacrimia syndrome
  • Achalasia alacrima syndrome
  • Addisonian achalasia syndrome
More Names
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Treatment


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How might triple A syndrome be treated?

There is no cure for triple A syndrome at this time; treatment typically focuses on managing individual signs and symptoms of the condition.

Glucocorticoid deficiency in individuals with known adrenal insufficiency (present with Addison disease) is typically treated by replacement of glucocorticoids. This may be important for avoiding an adrenal crisis and allowing for normal growth in children. In adult individuals, as well as those who have difficulty with compliance, replacing hydrocortisone with prednisone or dexamethasone is sometimes recommended. It is usually recommended that affected individuals wear a medical alert bracelet or necklace and carry the emergency medical information card supplied with it.

Achalasia is typically managed with surgical correction. Individuals may be monitored for pulmonary complications (due to reflux and aspiration). Gastric acid reduction therapy in individuals with reflux after surgical intervention is usually recommended. The symptoms in individuals with achalasia may be improved partially with pneumatic dilatation (also called balloon dilation). For those who remain symptomatic after this, other surgeries may be recommended.

Alacrima is typically managed by applying topical lubricants (such as artificial tears or ointments), and with punctal occlusion (a procedure used to close the tear ducts that drain tears from the eye). The symptoms of alacrima typically improve with punctal occlusion. However, this procedure is usually only done when therapy with topical lubricants is unsuccessful.[1]
Last updated: 2/9/2011

References
  1. Bruce A Boston, Daniel L Marks. Allgrove (AAA) Syndrome. eMedicine. February 20, 2009; http://emedicine.medscape.com/article/919360-treatment. Accessed 2/7/2011.


Clinical Trials & Research for this Disease

  • Orphanet lists clinical trials, research studies, and patient registries enrolling people with this condition. Click on Orphanet to view the list.