Definition:
Tangier disease is a rare inherited disorder marked by a severe reduction in the amount of high density lipoprotein (HDL), often referred to as "good cholesterol," in the bloodstream.
Symptoms and Signs:
People affected by this condition also have slightly increased amounts of fat in the blood (mild hypertriglyceridemia) and disturbances in nerve function (neuropathy). The tonsils are visibly affected by this disorder; they usually appear orange or yellow and are extremely enlarged. Affected people often develop premature atherosclerosis, which is marked by fatty deposits and scar-like tissue lining the arteries. Other symptoms of this condition may include an enlarged spleen (splenomegaly), an enlarged liver (hepatomegaly), clouding of the cornea, and early-onset cardiovascular disease.
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