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Doctors for Wolman disease in Agaramthen, Kanchipuram
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Wolman disease is the most severe expression of LAL deficiency, a milder form of LAL deficiency is known as cholesteryl ester storage disease. The symptoms of Wolman disease usually become apparent shortly after birth, usually during the first few weeks of life. Affected infants may develop bloating or swelling of the stomach (abdominal distention) and may have significant enlargement of the liver and spleen (hepatosplenomegaly). Scarring (fibrosis) of the liver may also occur. In some cases, fluid may accumulate in the abdominal cavity (ascites).
HOW IS WOLMAN DISEASE DIAGNOSED?
A diagnosis of Wolman disease may be suspected in newborn infants based upon identification of characteristic symptoms such as abnormally enlarged liver and gastrointestinal problems. A diagnosis may be confirmed by a thorough clinical evaluation, a detailed patient history (including family history) and specialized tests that reveal absence or deficient activity of the enzyme lysosomal lipase acid (LIPA) in certain cells and tissues of the body. Molecular genetic testing for mutations in the LIPA gene is also done for diagnosis.
HOW IS WOLMAN DISEASE TREATED?
Till date, there are no treatments available for this disease but there is management options available. The medicines used in this reference are sebelipase alfa, which is a recombinant form of LAL and is medically approved in many countries.
DID YOU KNOW?
The disease affects less than .2 percentage of people out of 10,000 and it is a very aggressive disease that requires effective management or it can be fatal.