MPS(Mucopolysaccharidosis)

Hurler Syndrome / Hunter Syndrome / Sanfilippo Syndrome / Morquio Syndrome

Mucopolysachhrodises (MPS) is a genetic disorder of children and adult. It is basically a lysosomal storage disorder due to absence of enzyme

Types of MPS
Four types of MPS have been identified

• MPS Type-I (Hurler Syndrome)
• MPS Type-II (Hunter Syndrome)
• MPS Types-III ( Sanfilippo Syndrome)
• MPS Type-IV  (Morquio Syndrome)

MPS Type-I (Hurler Syndrome)

•  Developmental delay is seen by the end of first year and children usually stop developing between age 2 to 4 years.
• The children show hepatosplenomegaly, dwarfism, progressive mental retardation as key features.
• Enzyme replacement therapy is the main treatment available.
• Bone Marrow Transplantation and Umbilical Cord Blood Transplantation are also being used for treatment.
• Habilitation services are mainstay in the management of the child.
• Gene therapy will be the answer of this order in the long run

 
MPS Type-II (Hunter Syndrome)

• Boys are more affected
•  Aggressive behaviors
• Hyperactivity
• Mental Retardation
• Spasticity
• There is no cure available.
• Treatment is symptomatic.
•  Medicine like Idursulfane is given intravenously.
• Bone Marrow Transplantation is also being done with limited success.
• Gene therapy has been seen one of the suitable options.
• Habilitation services are required along with medication.

MPS Types-III (Sanfilippo Syndrome)

•  The brain is the primary target of the disorder.
• The onset of the disorder may be very early to fourth decades of life.
• Due to disorder the brain function decline with time.
• Breathing and hearing may be difficult for the individual.
• The incidence of all MPS III is estimated to be 1 in 25,000 live births.
•  Management requires a team approach.
• Treatment is symptomatic.
• Habilitation services are the primary options of the management.
• Haematopoetic Stem Cell transplantation and Enzyme Replacement Therapy are also available with limited success.
•  Although there is no cure but Gene therapy holds promise.

MPS Type-IV  (Morquio Syndrome)

•  It is a genetic disorder where the body lacks enough enzyme needed to break glycosaminoglycans.
•  In this condition, abnormally large amount of glycosaminoglycans is stored in the body / brain which causes problems like hyper-mobile joints, macrocephaly,  abnormal development of bones (Dwarfism) and widely spaced teeth.
• There is no cure available.
• Treatment is symptomatic.
• Management requires team effort.
• Children with this condition generally have good mental ability.
• Orthopedic procedure for the spine may help these individuals.

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Connect our expert for more information and technical support
Mail: helpicd@gmail.com
Whatsapp: +91-7838809241
Voice Call: +91-11-41012124