What is Aagenaes Syndrome?
It is defined as a syndrome in which the lymph vessels are not fully formed. It is a congenital condition. It is autosomal recessive disorder and its genetic cause is unidentified. Some suggest that the chromosome 15 q is involved. As the lymph vessels are involved it is considered to be lymphangiogenetic in origin. It is more common in the Norway. It is also found in the Europe and America. It is also known as the CLS which stands for cholestasis lymphedema syndrome. This syndrome is named after the Norway pediatrician known as Oystein Aagenaes.
What are the signs and symptoms of Aagenaes syndrome?
It is characterized by the swelling of legs due to accumulation of lymph in tissues, cholestasis in infants that is, bile cannot flow from the liver to the duodenum which leads to the hepatic cirrhosis along with the giant cell hepatitis. This is followed by the development of excess fibrous connective tissue portal tract.
What is the treatment of Aagenaes syndrome?
It includes the lymphedema therapy along with the diet therapy. It consists of the low fat intake and the use of fat soluble vitamins in the diet. One can also look for the liver transplantation.