| Morquio syndrome is a mucopolysaccharide | | | | knock-knees and irregular development of paired |
| storage disease. Morquio syndrome is a genetic | | | | bones are also common. Other symptoms involve |
| disease of metabolism in which the body is | | | | clouded corneas and circulatory malformations. |
| missing or doesn't have enough of a substance | | | | Symptoms usually start between ages 1 and 3. |
| needed to break down long chains of sugar | | | | There are two forms of Morquio syndrome: Type |
| molecules called glycosaminoglycans. Lysosomes | | | | A and Type B. Persons with Type A do not have |
| function as the primary digestive units within cells. | | | | a substance (enzyme) called |
| Enzymes within lysosomes break down or digest | | | | galactosamine-6-sulfatase. Persons with Type B |
| particular nutrients. In individuals with MPS | | | | do not produce enough of an enzyme called beta |
| disorders, deficiency or malfunction of specific | | | | galactosidase. The syndrome is estimated to |
| lysosomal enzymes leads to an abnormal | | | | occur in 1 of every 200,000 births. A family |
| accumulation of certain complex carbohydrates in | | | | history of the syndrome raises one's risk for the |
| the arteries, skeleton, eyes, joints, ears, skin, and | | | | condition. |
| or teeth. | | | | There are many complication is associated with |
| These accumulations may also be predicate in the | | | | this syndrome. Cognitive function is usually normal |
| respiratory system, liver, spleen, central nervous | | | | in patients with Morquio syndrome. Cardiac |
| system, blood, and bone marrow. This compilation | | | | complications may lead to death. Bone problems |
| eventually causes progressive damage to cells, | | | | can lead to expressive complications. For example, |
| tissues, and various organ systems of the body. | | | | the small bones at the top of the neck may slip |
| The disease is recognized within the first two | | | | and damage the spinal cord, causing paralysis. |
| years of life and is usually progressive until bone | | | | There is no specific treatment for Morquio |
| growth ceases in late adolescence. The vertebrae | | | | syndrome. Treatment is currently only palliative. |
| of the spine are wedge-shaped and flattened, and | | | | Genetic counseling is recommended for |
| back deformity is common; compression of the | | | | prospective parents with a family history of |
| spinal cord may occur if back deformity is severe. | | | | Morquio syndrome. A spinal fusion may prevent |
| The heads of the thighbones are small and | | | | irreversible spinal cord injury in persons whose |
| malformed. | | | | neck bones are underdeveloped. |
| Sometimes resulting in dislocation of the hip; | | | | |