The oldest person with Morquio syndrome was a man hat lived to age 81 and was diagnosed with Morquio syndrome at 2 and half years old.
Morquio syndrome, also known as mucopolysaccharidosis type IV, is a rare metabolic disorder in which the body cannot process certain types of sugar molecules called glycosaminoglycans.
When a person has Morquio syndrome, the specific GAG which builds up in the body is called keratan sulfate
People with untreated severe Morquio A syndrome die of respiratory obstruction, cervical spinal cord compression, or heart valve disease in their second or third decade of life.
The lifespan of someone with the attenuated form of Morquio A syndrome is as long as 70 years.
Medication has been developed to help in the treatment of Morquio, but currently there is no cure.
Based on the persons symptoms, the care team may recommend careful monitoring or non-surgical treatment.
Symptoms of Morquio syndrome include.
Short stature (height less than other people the same age)
A curved spine (scoliosis or kyphosis)
“Hypermobile” joints (joints that are more flexible than normal)
Knock-knees (knees curve in)
A chest that juts out (pectus carinatum)
Misshapen arms and legs.
Abnormal lysosomal storage of keratan sulfate selectively affects the skeletal system and connective tissues.
Both types are characterized by short-trunk dwarfism with kyphosis and scoliosis, genu valgum, corneal opacity, and severe skeletal dysplasia that is distinct from other MPS disorders.
Intelligence is intact.
Most children with Morquio syndrome have normal intelligence.
Children with spine and bone conditions can have difficulty walking and may require a wheelchair; others may be more mobile.
Some children have ongoing breathing, vision or cardiac issues that may become more serious in adulthood.
Problems with a person's chest due to Morquio syndrome can put a strain on their heart and lungs.
This may lead to respiratory failure.
Evidence notes that spinal cord compression and airway obstruction are the two main causes of death for a person with Morquio syndrome.