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Diseases

Genetic and Rare Diseases Information Center (GARD)

Menkes disease


Other Names for this Disease
  • Menkes syndrome
  • Steely hair disease
  • Menkea syndrome
  • Kinky hair disease
  • Copper transport disease
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Prognosis

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What is the prognosis for Menkes disease?

Since newborn screening for this disorder is not available, and early detection is infrequent because the clinical signs of Menkes disease are subtle in the beginning, the disease is rarely treated early enough to make a significant difference. In general, the prognosis for babies with Menkes disease is poor.[1] The majority of children with Menkes disease die within the first decade of life, many by age 3.[1][2][3] The major problems in Menkes disease involve the neurologic, gastrointestinal, and connective tissue (including blood vessels) systems. Pneumonia, leading to respiratory failure, is a common cause of death, although some patients with Menkes disease die suddenly in the absence of any apparent acute medical problem.[2]

If copper is administered within the first few months of life, it appears to be effective in increasing the life expectancy of some patients (from 3 to 13 years of age).[3][4] Also, when treatment is started very early, especially during the first 10 days of life, it may result in improved neurological outcomes.[2] Several investigations are ongoing to find more effective treatments.

Last updated: 4/5/2016

References
  1. NINDS Menkes Disease Information Page. National Institute of Neurological Disorders and Stroke (NINDS). September 30, 2011; http://www.ninds.nih.gov/disorders/menkes/menkes.htm.
  2. Kaler SG. Genetics of Menkes Kinky Hair Disease. Medscape Reference. September 8, 2015; http://emedicine.medscape.com/article/946985-overview.
  3. Kaler SG. ATP7A-Related Copper Transport Disorders. GeneReviews. October 14, 2010; http://www.ncbi.nlm.nih.gov/books/NBK1413/.
  4. Menkes syndrome. National Center for Biotechnology Information (NCBI). http://www.ncbi.nlm.nih.gov/books/NBK22216/. Accessed 4/5/2016.


Other Names for this Disease
  • Menkes syndrome
  • Steely hair disease
  • Menkea syndrome
  • Kinky hair disease
  • Copper transport disease
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.