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Alternative names
Aase-Smith syndromeDefinition Return to top
Aase syndrome is a rare, inherited disorder characterized by anemia with some joint and skeletal deformities.Causes, incidence, and risk factors Return to top
Aase syndrome is thought to be an autosomal dominant inherited disorder. The genetic basis of the disease is not known. The anemia is caused by underdevelopment of the bone marrow, which is where blood cells are formed.Symptoms Return to top
Signs and tests Return to top
Treatment Return to top
Frequent blood transfusions are given in the first year of life to treat anemia. Prednisone may be given, although this should be avoided in infancy because of side effects on growth and brain development. A bone marrow transplant may be necessary if other treatment fails.Expectations (prognosis) Return to top
Anemia usually resolves over the years.Complications Return to top
Calling your health care provider Return to top
Call your health care provider if you notice possible signs of Aase syndrome in your child. Genetic counseling is recommended if there is a family history of Aase syndrome.Prevention Return to top
As with most genetic diseases there is no way to prevent the entire disease. With prompt recognition and treatment of infections in childhood, the complications of low white blood cell counts may be limited. Update Date: 8/6/2003 Updated by: Douglas R. Stewart, M.D., Division of Medical Genetics, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
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Page last updated: 28 October 2004 |