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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, most by age 3.[1][2][3] The major problems in Menkes disease involves the neurologic, gastrointestinal, and connective tissue (including blood vases) 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 process.[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 no neurological problems.[2] Several investigations are ongoing to find more effective treatments.

Last updated: 3/8/2011

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. Accessed 12/2/2015.
  2. Kaler SG. Genetics of Menkes Kinky Hair Disease. Medscape Reference. September 8, 2015; http://emedicine.medscape.com/article/946985-overview. Accessed 12/2/2015.
  3. Kaler SG. ATP7A-Related Copper Transport Disorders. GeneReviews. October 14, 2010; http://www.ncbi.nlm.nih.gov/books/NBK1413/. Accessed 12/2/2015.
  4. Menkes syndrome. National Center for Biotechnology Information (NCBI). http://www.ncbi.nlm.nih.gov/books/NBK22216/. Accessed 12/2/2015.


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.