Morquio syndrome

pathology
verifiedCite
While every effort has been made to follow citation style rules, there may be some discrepancies. Please refer to the appropriate style manual or other sources if you have any questions.
Select Citation Style
Feedback
Corrections? Updates? Omissions? Let us know if you have suggestions to improve this article (requires login).
Thank you for your feedback

Our editors will review what you’ve submitted and determine whether to revise the article.

Also known as: MPS IV, Morquio-Brailsford disease, mucopolysaccharidosis IV
Also called:
mucopolysaccharidosis IV

Morquio syndrome, rare hereditary disorder of intracartilaginous bone development that results in severe malformation of the skeleton (particularly the spine and long bones) and dwarfing. The disease is recognized within the first two years of life and is usually progressive until bone growth ceases in late adolescence. The vertebrae of the spine are wedge-shaped and flattened, and back deformity is common; compression of the spinal cord may occur if back deformity is severe. The heads of the thighbones are small and malformed, sometimes resulting in dislocation of the hip; knock-knees and asymmetrical development of paired bones are also common. Other symptoms include clouded corneas and circulatory malformations. Individuals with Morquio syndrome have a normal life expectancy. Treatment is directed toward symptoms of the disease.

This article was most recently revised and updated by Robert Curley.