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Risk factors
By Mayo Clinic staff
There are two major risk factors for developing Hunter syndrome:
- Family history. Hunter syndrome is caused by a defective chromosome, and a child must inherit the defective chromosome to develop the disease. Hunter syndrome is what's known as an X-linked recessive disease. This means that women carry the defective disease-causing X chromosome and can pass it on, but aren't affected by the disease themselves.
- Sex. Hunter syndrome nearly always occurs in males. Girls are less at risk of developing this disease because they inherit two X chromosomes. If one of the X chromosomes is defective, their normal X chromosome can provide a functioning gene. If the X chromosome of a male is defective, however, there isn't another normal X chromosome to compensate for the problem.
References
- Scarpa, et al. Mucopolysaccharidosis type II: European recommendations for the diagnosis and multidisciplinary management of a rare disease. Orphanet Journal of Rare Diseases. 2011;6:1.
- Martin R, et al. Recognition and diagnosis of mucopolysaccharidosis II (Hunter syndrome). Pediatrics. 2008;121:e377.
- Mucopolysaccharidoses fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/mucopolysaccharidoses/detail_mucopolysaccharidoses.htm. Accessed Oct. 11, 2012.
- Kakkis E, et al. Complications and management of the mucopolysaccharidoses. http://www.uptodate.com/ index. Accessed Sept. 21, 2012.
- Muenzer J, et al. Multidisciplinary management of Hunter syndrome. Pediatrics. 2009;124:e1228.
- Da Silva EMK, et al. Enzyme replacement therapy with idursulfase for mucopolysaccharidosis type II (Hunter syndrome). Cochrane Database of Systematic Reviews. 2011; 9:CD008185. http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD008185.pub2/abstract. Accessed Oct. 1, 2012.
- Valayannopoulos V, et al. Therapy for the mucopolysaccharidoses. Rheumatology. 2011;50:v49.
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