چاپ صفحه |  صفحه نخست سامانه |  نویسندگان |  اطلاعات تفضیلی |  دانلود مقاله | دانشگاه علوم پزشکی تبریز |
| نویسنده | نفر چندم مقاله |
|---|---|
| مهناز صادقی شبستری | اول |
| عنوان | متن |
|---|---|
| خلاصه مقاله | A Case of LI10R Deficiency Mahnaz Sadeghi-SHabestari Clinical Immunologist and Allergist, Immunology research center of Tabriz, Children Hospital,Tabriz university of Medical Science, Tabriz, Iran IL-10 and IL-10 receptor (IL-10R) deficiencies, caused by loss-of-function mutations in the genes encoding IL-10 or IL-10R, are primary immunodeficiency. IL-10 is an important anti-inflammatory cytokine secreted by various cells, including monocytes, macrophages, T and B lymphocytes, dendritic cells, epithelial cells, and mast cells. IL-10 also inhibits the release of tumor necrosis factor α and thus is critical to the maintenance of immune homeostasis in gastrointestinal tract. Mutant IL-10 did not inhibit LPS-mediated TNF-a release in PBMCs. IL-10 and IL-10R deficiencies are rare, with only a few cases reported. The most clinical manifestation of disease is inflammatory bowel disease (IBD) that is presents early in life. Other presentations of disease are recurrent infections, including chronic folliculitis, arthritis, multiple abscesses, anal fissures, and fistulae, repeated bloody diarrhea, marked weight loss, FTT, pulmonary infections and complications, such as EBV-positive lymphoma. Usually near all of patients had a similarly severe course of disease and also are from consanguineous families. The immunologic work-up of IL-10– and IL-10R–deficient patients reveal various minor abnormalities without any genotype-phenotype correlation. Increased, normal or slightly decreased serum immunoglobulin levels; and variations in T-cell, B-cell, and NK cell numbers occurred. Treatment: IL-10 and IL-10R– deficient patients are typically unresponsive to immunosuppressive therapies. However, some of them have later onset with more response to anti-inflammatory drugs. Because of life-threatening nature of the IBD and its resistance to conventional Immunosuppressive therapies, allogeneic hematopoietic stem cell transplantation (HSCT) must be done that leading to sustained remission. Here we report a case of IL10R deficiency at the age of 6month with recurrent life-threatening gastroenteritis. |
| کلمات کلیدی | IL10R deficiency ,Case report N |
| نام فایل | تاریخ درج فایل | اندازه فایل | دانلود |
|---|---|---|---|
| IL 10 def 3 m.docx | 1398/12/12 | 14145 | دانلود |
| Certificate m id t.pdf | 1398/12/12 | 139194 | دانلود |
