What is another name for Tangier disease?
What is another name for Tangier disease?
Tangier disease, also known as familial alpha lipoprotein deficiency, is an extremely rare, inherited condition that was first described in a child on Tangier Island, an island located off of the coast of Virginia.
Who discovered Tangier disease?
In 1959, a five-year-old patient named Teddy Laird from Tangier Island, Virginia, presented with strikingly large and yellow-orange tonsils which were removed by armed forces physicians.
When was Tangier disease discovered?
Tangier disease was first recognized in 1960 in a sibling pair living on Tangier Island in the Chesapeake Bay in Virginia. Enlarged, yellow-orange tonsils and little or no circulating HDL cholesterol are the classic findings in the disorder.
How is Tangier disease diagnosis?
Diagnosis of Tangier disease is achieved through clinical evaluation and can be confirmed through genetic testing involving the sequencing of the ABCA1 gene. HDL-C deficiency and an extremely low apolipoprotein A1 (ApoA1) level are typical diagnostic criteria.
Where is Tangier Island located?
Tangier Island is on the Chesapeake Bayside of the Eastern Shore of Virginia and is located 12 miles into the Chesapeake Bay. The Chesapeake Bay, its people, nature and wildlife at its best.
How is Tangier disease treated?
Treatment of Tangier disease is supportive and based on specific disease manifestations in a given individual. There are no known specific treatments for Tangier disease. Surgical removal of the spleen, tonsils, or other enlarged tissues may become necessary in some patients.
Is there alcohol on Tangier Island?
Yes, you can bring alcohol on board the boat; however, Tangier is a “dry” island, so no alcohol can be purchased on the island. That does not mean alcohol is not consumed on the island, but it cannot be purchased. We suggest you leave your alcohol on the boat while touring the island.
What language is spoken on Tangier Island?
Language. Many who live on Tangier speak a distinctive dialect of American English, which scholars have disputed as derived from 17th and 18th-century British English (Early Modern and Modern English) lexicon and phonetics.
What gene causes Abetalipoproteinemia?
Abetalipoproteinemia is caused by mutations in the MTTP gene, which provides instructions for making a protein called microsomal triglyceride transfer protein. This protein is essential for creating molecules called beta-lipoproteins in the liver and intestine .
How do spur cells arise?
[2] When remodeled by the spleen in liver disease patients, acanthocytes develop more blunt spicules and become relatively more spherocytic; these are known as spheroacanthocytes or ‘spur cells.
What are the symptoms of Tangier disease?
Signs & Symptoms. Symptoms of Tangier disease are variable and depend on which organs are involved and the severity of those manifestations. Tangier disease is most often characterized by enlarged orange- or yellow-colored tonsils. This discoloration is due to fatty deposits accumulating in the tonsils.
What does Tangier disease mean?
What does Tangier Disease mean? Here are all the possible meanings and translations of the word Tangier Disease. Tangier disease or Hypoalphalipoproteinemia is a rare inherited disorder characterized by a severe reduction in the amount of high density lipoprotein, often referred to as “good cholesterol,” in the bloodstream.
What does Tangier mean?
Tangier, Tangiers(noun) a city of northern Morocco at the west end of the Strait of Gibraltar . “the first tangerines were shipped from Tangier to Europe in 1841”.
