Menkes syndrome


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Hypotonia
Hypotonia
Alternative Names

Steely hair disease; Menkes kinky hair syndrome; Kinky hair disease


Treatment

Injection of copper has been used with mixed results. Present therapy consists of:

  • Copper acetate administered into a vein (intravenously)
  • Subcutaneous (injected under the skin) copper histidine

Support Groups

Corporation for Menkes Disease -- 219-436-0137


Expectations (prognosis)

Menkes is a serious disorder, with early death being common.


Complications

Seizures, abnormally light pigmentation of the skin and hair. Death usually occurs in the first few years of life.


Calling your health care provider

Consult with your health care provider if you have a family history of Menkes syndrome and you plan to have children. A baby with this condition will often show symptoms early in infancy.



Review Date: 04/20/2005
Reviewed By: Neal Sondheimer, M.D., PhD., Division of Genetics and Metabolism, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.


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