Alpha-Mannosidosis Market - Global Industry Analysis and Forecast, 2014 – 2020

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Date Submitted: 07/01/2014 02:10 AM

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Alpha-mannosidosis is a rare inherited disorder that affects organs and tissues of the body. This may cause an intellectual disability, distinguishing facial features, and skeletal abnormalities in an individual. Characteristic facial features can include a large head, low hairline, large ears, and rounded eyebrows, protruding jaw, flattened bridge of the nose, overgrown gums, widely spaced teeth, and large tongue. The skeletal abnormalities caused by alpha-mannosidosis disorder include thickening of the bones at the top of the skull (calvaria), reduced bone density (osteopenia), deformations of the spinal bones (vertebrae), weakening of the bones and joints and bowed legs or knock knees.

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Some individuals may also experience ataxia i.e. difficulty in movement co-ordination, myopathy i.e. muscle weakness; poor development of motor skills such as speech impairments; sitting and walking; enlargement of the liver and spleen, increased risk of infections; a buildup of fluid in the brain also known as hydrocephalus; hearing loss; and cataract. In some cases psychiatric symptoms are also observed which include depression, hallucinations, or anxiety.

Individuals suffering from alpha-mannosidosis disorder may experience signs and symptoms which can range from mild to severe. The onset of this disorder in infants occurs with severe neurological deterioration coupled with rapid progression. In such cases, the chances of survival for the individual past childhood are very low. Moreover, an affected fetus may die before birth in the most severe cases. Few individuals with alpha-mannosidosis may experience milder signs and symptoms that are known to appear later and progress more slowly. Individuals with later-onset of alpha-mannosidosis are known to survive into their fifties. Laboratory testing is required for detection of even the mildest cases.

A diagnosis is...