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.
Basic Information
- nickname
- Tangier's disease, alpha lipoproteinemia free
- 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
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.
