Marrinesco-Sjogren Syndrome

Marrinesco-Sjogren syndrome is name given to Mucopolysaccharidosis Type VI. This is also known as Maroteaux-Lamy syndrome. It is a congenital (inherited disease) caused by a deficiency in the enzyme ASRB (arylsulfatase) which is responsible for the breakdown of large sugar molecules called glycosaminoglycans. The physical symptoms become obvious in early childhood may include: Macrocephaly, Hydrocephalus, coarse facial features, heart valve disease, enlarged liver and spleen and umbilical hernia.