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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Voice Call: +91-11-41012124

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