Hunter syndrome


Also found in: Encyclopedia, Wikipedia.

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 ?
Hunter Syndrome is a rare and complex disorder that causes Daniel to have limited movement, breathing problems and symptoms of epilepsy.
The jury was told that after Jacob was born the couple were alerted to Hunter Syndrome and Mrs Wragg had an abortion when it emerged her unborn son, who they named Henry, was diagnosed with the disease.
One friend said: "He was a lovely lad but it was dreadful for everyone knowing he was going to die from this Hunter syndrome.
Providing health care services such as hospital treatment in the field - the program Medication - Treatment of Mucopolysaccharidosis II (Hunter syndrome), drugs in the drug package - Treatment of Mucopolysaccharidosis II (Hunter syndrome).
Ben, a pupil at Meadows Sports College, in Oldbury, was diagnosed at the age of three with Hunter Syndrome - a genetic disorder affecting around 2,000 people across the globe.
Elaine and Barbara are also urging people to get involved in the Society for Mucopolysaccharide Diseases' first awareness day on Tuesday, to highlight illnesses including Hunter syndrome.
But the judge strongly rejected a claim made during the trial by Wragg's former wife Mary that she did not know her husband was planning to kill Jacob, who was suffering with the degenerative disease Hunter Syndrome, on July 24 last year.
The boy was suffering with the rare degenerative disease Hunter Syndrome and was not expected to live past his mid teens, But the defendant admits manslaughter on the grounds of diminished responsibility, claiming he was suffering an abnormality of mind when he killed Jacob in what he later claimed was a "mercy killing".
During the 11-day trial, the court heard Jacob had a severe form of the degenerative disease Hunter Syndrome.
The youngster, who suffered from Hunter Syndrome, was treated at the address in Henty Close, Worthing, before being taken to Worthing Hospital, where he was pronounced dead.
(NASDAQ: SGMO) has presented initial safety data from the CHAMPIONS Study, the Phase 1/2 clinical trial evaluating SB-913 for the treatment of mucopolysaccharidosis type II, a rare disease also known as Hunter syndrome, at the 2018 WORLDSymposium medical congress, the company said.

Full browser ?