Hurler syndrome or mucopolysaccharidosis I (MPS I) is an uncommon hereditary issue ailment of digestion in which a human body can't separate long chains of sugar particles called glycosaminoglycansdue due to an insufficiency of alpha-L-iduronidase, an enzyme responsible for the degradation of mucopolysaccharides in lysosomes. Moreover, without the alpha-L-iduronidase catalyst, a development of heparan sulfate and dermatan sulfate takes place in the body. Symptoms of Hurler syndrome appear in the early stage of life (childhood) and can lead to death due to organ harm or damage. Symptoms of Hurler syndrome appear between 3 years and 8 years. Infants with severe Hurler syndrome seem normal at birth. Facial signs and symptoms become more clear and visible during the first two years of life. Hurler syndrome is marked by progressive weakening, hepatosplenomegaly, dwarfism, and unique facial features. There is progressive mental retardation, with death frequently occurring by the age of 10.
The mucopolysaccharidosis I (MPS I) or Hurler syndrome market has been segmented based on diagnosis, treatment, and region. In terms of diagnosis, the market has been categorized into blood test, urine test, amniocentesis, genetic testing, and others. The blood test segment held the largest share of the market due to easy access for outpatient department (OPD) patients to carry out tests. The genetic testing segment is likely to witness strong growth during the forecast period due to increasing awareness about mucopolysaccharidosis I (MPS I) or Hurler syndrome. In terms of treatment, the market has been categorized into enzyme replacement therapy, bone marrow transplantation, surgery, and others. The enzyme replacement therapy segment holds the largest share of the mucopolysaccharidosis I (MPS I) or Hurler syndrome market due to increasing incidence of syndrome globally. The bone marrow transplantation (BMT) segment is likely to witness significant growth in the next few years driven by effective treatment of the syndrome.
Geographically, the mucopolysaccharidosis I (MPS I) or Hurler syndrome market has been segmented into five major regions: North America (U.S. and Canada), Europe (Germany, France, U.K., etc.), Asia Pacific (Australia, China, Japan, India, etc.), Latin America (Brazil, Mexico, etc.), and Middle East &Africa (GCC Countries, South Africa, etc.). North America is the leading market for mucopolysaccharidosis I (MPS I) or Hurler syndrome in terms of revenue, followed by Europe. The U.S. holds the largest share of the market in the region. Asia Pacific and Latin America are emerging markets, which are expected to witness strong growth during the forecast period from 2017 to 2025. Strong growth in these regions is attributed to increasing incidence of syndrome, rising health care industries, and increase in government investment to develop health care infrastructure. The mucopolysaccharidosis I (MPS I) or Hurler syndrome market in countries such as China, India, and Brazil is projected to expand at high growth rate during the forecast period due to increase in awareness among the population about the syndrome and its treatment.
Other factors that drive the mucopolysaccharidosis I (MPS I) or Hurler syndrome market are rising prevalence of syndrome, increasing novel drug treatment, high adoption and acceptance in developing markets, and innovations and developments in treatment. The market is also experiencing certain restraints such as high possibilities of substance abuse and addiction, complications and severity involved in treatment, high cost of treatment, and lack of awareness among the rural population in developing and underdeveloped economies.
The treatment regime for mucopolysaccharidosis I (MPS I) or Hurler syndrome is determined depending on the severity of the symptoms and varies from patient to patient and case to case. Since there is no permanent cure for the syndrome, treatment primarily focuses on management of signs, indications, and complications associated with the disease progression. Palliative care is given to manage various conditions such as heart complications, respiratory complications, behavioral and neurological complications, sleep issues, and skeletal and connective tissue problems.
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