Hunter syndrome

(redirected from Mucopolysaccharidosis Type II)
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Hun·ter syn·drome

(hŭn'tĕr), [MIM*309900]
an error of mucopolysaccharide metabolism characterized by deficiency of iduronate sulfatase, with excretion of dermatan sulfate and heparan sulfate in the urine; clinically similar to Hurler syndrome but distinguished by less severe skeletal changes, no corneal clouding, and X-linked recessive inheritance; caused by mutation in the iduronate sulfatase gene (IDS) on chromosome Xq.

Hunter syndrome

(hŭn′tər)
n.
A type of mucopolysaccharidosis (MPS II) that occurs almost exclusively in males, characterized by the presence of dermatan sulfate and heparan sulfate in the urine, skin lesions, airway obstruction, enlargement of the liver and spleen, short stature due to skeletal abnormalities, hearing loss, and, in some forms, intellectual disability.
An X-linked recessive lysosomal storage disease type of inborn error of metabolism [MIM 309900] due to a deficit of iduronate-2-sulfatase, resulting in an accumulation of glycosaminoglycans
Management Nothing has proven effective

Hunter syndrome

Mucopolysaccharidosis type II Molecular medicine An X-R inborn error of metabolism caused by a deficit of sulfoiduronate or iduronosulfate sulfatase Clinical Type A–early onset is associated with a large skull, coarse facial features, profound mental retardation, spasticity, stiffness, aggressive behavior; type B is milder

Hunt·er syn·drome

(hŭn'tĕr sin'drōm)
An error of mucopolysaccharide metabolism characterized by deficiency of iduronate sulfatase, with excretion of dermatan sulfate and heparan sulfate in the urine; clinically similar to Hurler syndrome but distinguished by less severe skeletal changes, an absence of corneal clouding, and X-linked recessive inheritance; caused by mutation in the iduronate sulfatase gene (IDS) on chromosome Xq.

Hunter,

Charles H., Canadian physician, 1872-1955.
Hunter syndrome - an error of mucopolysaccharide metabolism. Synonym(s): type II mucopolysaccharidosis
References in periodicals archive ?
Mucopolysaccharidosis Type II. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Mefford HC, Stephens K, Amemiya A, Ledbetter N (eds) GeneReviews[R] [Internet].
Fognani, "Failure of the laryngeal mask to secure the airway in a patient with Hunter's syndrome (mucopolysaccharidosis type II)," Pediatric Anesthesia, vol.
Rothera et al., "Tracheostomy in mucopolysaccharidosis type II (Hunter's Syndrome)," International Journal of Pediatric Otorhinolaryngology, vol.
Cervical myelopathy in mucopolysaccharidosis type II (Hunter's Syndrome).
Joe has Mucopolysaccharidosis type II, which causes hearing loss, joint stiffness and difficulty in breathing.
He has a condition known as Mucopolysaccharidosis type II, which causes hearing loss, joint stiffness and difficulty in breathing.
Hunter Syndrome (mucopolysaccharidosis type II) is a rare, disabling and often fatal genetic disease that occurs more frequently in Asian than in Western countries1-4.
announced that the Company will host a webcast to review mucopolysaccharidosis Type II (MPS II, or Hunter syndrome) disease biology, standard of care treatment options, and scientific rationale for the development of SB-913, Sangamo's investigational in vivo genome editing treatment for MPS II.
Mortality and cause of death in mucopolysaccharidosis type II: a historical review based on data from the Hunter Outcome Survey (HOS).
(NASDAQ : SGMO) reported 16 week reductions in urinary glycosaminoglycans, a key biomarker of Mucopolysaccharidosis Type II (MPS II) disease pathophysiology, in Cohort 2 of the Phase 1/2 CHAMPIONS Study evaluating SB-913.

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