Triple A syndrome
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Triple-A syndrome is rare autosomal recessive disorder. Sufferers have adrenal insufficiency, alacrima (absence of tear secretion), and achalasia. Acalasia is a failure of a ring of muscle fibers, such as a sphincter, to relax. AAA patients have achalasia of the oesophagus cardia which delays food going to the stomach and causes dilation of the thoracic oesophagus.
It is also known as Allgrove syndrome because in 1978 he and others thought AAA might be inherited. Another name is achalasia-addisonianism-alacrima syndrome.
The syndrome is highly variable. Huebner and others said in 2000 they had mapped the syndrome to a 6 cM interval on human chromosome 12q13 near the type II keratin gene cluster.
Managed effectively, patients can have a normal lifespan and bear children.
External links
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
