Chediak-Higashi syndrome


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Treatment

There is no specific treatment for Chediak-Higashi syndrome. Bone marrow transplants appear to have been successful in several patients. Infections are treated with antibiotics and abscesses are surgically drained when appropriate. Antiviral drugs such as acyclovir have been tried during the terminal phase of the disease. Cyclophosphamide and prednisone have been tried. Vitamin C therapy has improved immune function and clotting in some patients.


Support Groups

Chediak-Higashi Syndrome Association -- www.chediak-higashi.org


Expectations (prognosis)

The frequent infections of Chediak-Higashi syndrome cannot be prevented. The terminal phase of the illness is not treatable.




Complications
  • Frequent infections especially with Epstein-Barr virus
  • Lymphoma-like cancer
  • Early death

Calling your health care provider

Call your health care provider if you have a family history of this disorder and you are planning to have children.

Call for an appointment with your health care provider if your child shows symptoms of Chediak-Higashi syndrome.



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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