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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 boy is defective, however, there isn't another normal X chromosome to compensate for the problem.
References
- Martin R, et al. Recognition and diagnosis of mucopolysaccharidosis II (Hunter syndrome). Pediatrics. 2008;121:e377.
- MPS II (Hunter syndrome). National MPS Society. http://www.mpssociety.org/index.php?option=com_content&view=article&id=129&Itemid=18. Accessed May 6, 2010.
- Mucopolysaccharidoses fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.nih.gov/disorders/mucopolysaccharidoses/detail_mucopolysaccharidoses.htm. Accessed May 8, 2010.
- Kakkis E, et al. Clinical features and diagnosis of the mucopolysaccharidoses. http://www.uptodate.com/home/index.html. Accessed May 5, 2010.
- Kakkis E, et al. Complications and management of the mucopolysaccharidoses. http://www.uptodate.com/home/index.html. Accessed May 5, 2010.
- Muenzer J, et al. Multidisciplinary management of Hunter syndrome. Pediatrics. 2009;124:e1228.
- Newborn screening. Centers for Disease Control and Prevention. http://www.cdc.gov/nbslabbulletin/bulletin.html. Accessed May 6, 2010.
- Guffon N, et al. Bone marrow transplantation in children with Hunter syndrome: Outcome after 7 to 17 years. Journal of Pediatrics. 2009;154:733.
- Elaprase (prescribing information). Cambridge, Mass.: Shire Human Genetic Therapies; 2007. http://www.elaprase.com/pdf/ElaprasePI40-0120_REV_0_GT4.pdf. Accessed May 5, 2010.
- Safety and clinical outcomes in Hunter syndrome patients 5 years of age and younger receiving idursulfase therapy. ClinicalTrials.gov. http://clinicaltrials.gov/ct2/show/NCT00607386. Accessed May 6, 2010.
- Burrow TA, et al. Review of the use of idursulfase in the treatment of mucopolysaccharidosis II. Biologics: Targets & Therapy. 2008;2:311.
- Friso A, et al. Genistein reduces glycosaminoglycan levels in a mouse model of mucopolysaccharidosis type II. British Journal of Pharmacology. 2010;159:1082.
- Piotrowska E, et al. Genistein-mediated inhibition of glycosaminoglycan synthesis as a basis for gene expression-targeted isoflavone therapy for mucopolysaccharidoses. European Journal of Human Genetics. 2006;14:846.
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