What Is Tangier Disease?
Tangier disease (TD) is also known as Tangier's disease and alpha lipoproteinemia. It is an autosomal recessive disease. Due to the disorder of alpha lipoprotein synthesis, a large amount of cholesterol esters are accumulated in the reticuloendothelial system, intestinal mucosa and skin. It is characterized by tonsil hypertrophy and orange-yellow banding. Lymph nodes, liver, and spleen can also be enlarged. Some have peripheral neuritis.
- nickname
- Tangier's disease, alpha lipoproteinemia free
- English name
- Tangier Disease
- Visiting department
- Neurology
- Common causes
- Heredity
- Common symptoms
- Hypertrophy of tonsil with orange stripes, enlarged liver, spleen, and lymph nodes with peripheral neuritis
Basic Information
Causes of Tangier disease
- Autosomal recessive disease. Due to the disorder of alpha lipoprotein synthesis, a large amount of cholesterol esters are accumulated in the reticuloendothelial system, intestinal mucosa and skin.
Clinical manifestations of Tangier disease
- Hypertrophy of tonsil with orange stripes. The liver, spleen, and lymph nodes can also be enlarged. Some patients have peripheral neuritis.
Tangier disease check
- Plasma high-density lipoprotein cholesterol content is less than 5mg / dl, triglycerides are normal or increased, chylomicrons are reduced, and apolipoprotein A1 disappears or is severely reduced.
Tangier disease diagnosis
- A preliminary diagnosis can be made based on clinical symptoms. Typical changes can be identified by histochemistry.
Tangier disease treatment
- Without special therapy, removal of upper respiratory tract obstruction caused by tonsil hypertrophy can be performed, and its tissue test can prove that there is lipid accumulation.