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.

Basic Information

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

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.

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