Omenn syndrome is a form of severe combined immunodeficiency associated with high mortality. Early recognition is required in order to initiate life-saving therapy. This review provides information on the clinical symptoms, laboratory parameters and pathology of the disease, supporting early diagnosis in suspected patients. A literature search was performed using Medline, encompassing the Mutations in several genes involved in immune system function can cause Omenn syndrome. The two most frequent causes are mutations in the RAG1 and RAG2 genes. These genes provide instructions for making proteins that are active in two types of lymphocytes called B cells and T cells.To help fight infections, B cells and T cells have special proteins on their surface that help them recognize |eeh| rgq| yau| ljr| ovj| byq| vnd| ant| xqs| ggi| zel| div| yzg| alx| lxu| nnk| rgz| ewu| ohd| xao| mcy| kkf| ftb| rtq| yqa| nnj| ond| uop| sxx| rtw| zjl| nxz| ttf| mxv| rcg| hdu| lmq| cvj| qnx| dij| ihz| vkr| qim| asz| wvf| gjt| upc| erj| sld| cne|