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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Omenn syndrome


Other Names for this Disease
  • Reticuloendotheliosis familial with eosinophilia
  • Severe combined immunodeficiency with hypereosinophilia
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Treatment


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How might Omenn syndrome be treated?

The standard treatment for Omenn syndrome is bone marrow transplantation or cord blood stem cell transplantation.[1][2] General care for any patient with severe combined immunodeficiency (SCID), including Omenn syndrome, includes isolation to prevent infection and meticulous skin and mucosal hygienic practices while the patient is awaiting stem cell reconstitution. Broad-spectrum antibiotics may be administered parenterally while cultures and body fluid analyses are in progress. Parenteral nutrition may also be provided as therapy for diarrhea and failure to thrive.[1] A detailed description of therapeutic options is provided in the referenced eMedicine article.
Last updated: 3/21/2012

References
  1. Schwartz RA, Lin RY. Omenn Syndrome. eMedicine. May 9, 2011; http://emedicine.medscape.com/article/887687-overview. Accessed 3/21/2012.
  2. Aleman K, Noordzij JG, de Groot R, van Dongen JJ, Hartwig NG. Reviewing Omenn syndrome. Eur J Pediatr. 2001; http://www.ncbi.nlm.nih.gov/pubmed/11795679. Accessed 3/21/2012.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Omenn syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.