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How are patients with a severe form of MPS VI affected?
Individuals with a more severe form of MPS VI can develop airway obstruction, hydrocephalus (extra fluid accumulating in the brain), and abnormal growth and formation of the bones
What are common heart problems of patients with MPS disorders?
Common problems include abnormal heart valves, narrowing of the blood vessels in the heart, and weak heart muscles (cardiomyopathy). Patients with MPS I H and the severe form of MPS II usually have damage to the mitral valve
What is notable about Scheie syndrome?
Scheie syndrome is considered the mild form of MPS I. Individuals with Scheie syndrome usually have normal intelligence
What treatment is effective for patients with seizures?
Seizures are a problem found in severe forms of MPS and especially in MPS III (Sanfilippo syndrome). Patients with seizures are given a type of prescription medication known as an anticonvulsant.
What causes MPS IV B?
MPS IV B is considered the milder form of the condition. The enzyme, beta-galactosidase, is deficient in MPS IV B. The gene involved with MPS IV B (GLB1) is located on chromosome 3.
Which MPS are inherited in an autosomal recessive manner?
Some examples of metabolic disorders inherited in an autosomal recessive manner include phenylketonuria (PKU), cystic fibrosis, and Tay-Sachs disease. In autosomal recessive inheritance, two copies of the abnormal gene are needed to manifest the disorder.
When do symptoms of MPS III first appear?
MPS III is a variable condition with symptoms beginning to appear between ages two and six years of age. The condition is characterized by developmental delay, behavioral problems, and mild physical problems
What is significant about Hurler Syndrome?
Individuals with Hurler syndrome tend to have the most severe form of MPS I. Hurler syndrome may also be referred to as severe MPS I.
What causes MPS II?
Mutations in the iduronate-2-sulphatase (IDS) gene cause both forms of MPS II (mild and severe). Nearly all individuals with Hunter syndrome are male, because the gene that causes the condition is located on the X chromosome.
What are the 7 types of mucopolysaccharidoses?
Hurler (MPS I H), Hurler-Scheie (MPS I H/S), Scheie (MPS I S), Hunter (MPS II), Sanfilippo (MPS III), Morquio (MPS IV), Maroteaux-Lamy (MPS VI),
Number of English MPs at Westminster?
528In total there are 646 Members of Parliament (MPs) in the UK Parliament. Excluding the 59 Scottish MPs, 18 Northern Irish MPs and 41 Welsh MPs, there are 528 English MPs.
Are Westminster MPs also Scottish MPs?
No. Only MPs from Scotland are Scottish MPs. There are MPs in Westminster from Scotland, England, Wales and Northern Ireland. There are also MSPs, which are Members of the Scottish Parliament. They are different to the Scottish MPs. MPs attend Westminster in London. MSPs attend the Scottish Parliament in Edinburgh.
