Mucopolysaccharidosis II: MPS II, also called Hunter syndrome, affects juveniles and includes a mild and a severe form. Signs of the severe form ... more about Mucopolysaccharidosis II.
Mucopolysaccharidosis II: Disorder of mucopolysaccharide metabolism in juveniles. More detailed information about the symptoms, causes, and treatments of Mucopolysaccharidosis II is available below.
See full list of 12 symptoms of Mucopolysaccharidosis II
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Prognosis for Mucopolysaccharidosis II: Usually death by age 15 for severe form; can have normal lifespan for mild form.
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MPS II, also called Hunter syndrome, affects juveniles and includes a mild and a severe form. Signs of the severe form are joint stiffness, mental deterioration, dwarfing, and progressive deafness. Death usually occurs by age 15. Symptoms of the mild form include short stature, limitation of motion, and features such as an enlarged forehead, lips, and tongue and misaligned teeth. (Source: excerpt from NINDS Mucopolysaccharidoses Information Page: NINDS)
An inborn mucopolysaccharide metabolism disorder with iduronate-2-sulfatase deficiency. Clinical characteristics are similar to those in MPS I, except for the absence of corneal clouding and slower progression of the course of disease and central nervous system deterioration. Retinal degeneration may occur, but the cornea usually remains clear. Appearance is normal at birth with excessive growth taking place during first two years of life. Two types are recognized: A severe form (MPS IIA) which is characterized mainly by mental retardation and progressive physical deterioration and early death, and a mild form (MPS IIB) in which patients may survive into adulthood. MPS IIA usually occurs between 2 and 4 years of age with progressive deterioration, chronic diarrhea, recurrent ear infections, hearing impairment, communicating hydrocephalus with increased intracranial pressure, and death at about 10 and 15 years. Obstructive airway disease, cardiac valvular dysfunction, myocardial thickening, pulmonary hypertension, coronary disease, and myocardial infarction may be superimposed. MPS IIB is milder with preservation of intelligence. The symptoms usually include hearing impairment, carpal tunnel syndrome, joint stiffness, discrete corneal opacities, and papilledema. Death may occur in early adulthood, usually from airway obstruction or cardiac failure. - (Source - Diseases Database)
Mucopolysaccharidosis II is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Mucopolysaccharidosis II, or a subtype of Mucopolysaccharidosis II,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Mucopolysaccharidosis II as a "rare disease".
Source - Orphanet
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