Omenn syndrome
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| Omenn syndrome Classification and external resources | |
| ICD-10 | D81.2 (ILDS D81.210) |
|---|---|
| OMIM | 603554 |
| DiseasesDB | 32676 |
| eMedicine | ped/1640 |
Omenn syndrome is a rare immunodeficiency disease associated with recombination activating gene.
Symptoms
Symptoms include:
- Desquamation (shedding the outer layers of skin)
- Chronic diarrhea
- Erythroderma (widespread reddening of the skin)
- Hepatosplenomegaly (simultaneous enlargement of both the liver and the spleen)
- Leukocytosis (elevation of the white blood cell count)
- Lymphadenopathy (swelling of one or more lymph nodes)
- Repeated bacterial infections
Treatment
Omenn syndrome is among the diseases treated with bone marrow transplantation and cord blood stem cells.
External links
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
