چاپ صفحه |  صفحه نخست سامانه |  نویسندگان |  اطلاعات تفضیلی |  دانلود مقاله | دانشگاه علوم پزشکی تبریز |
| نویسنده | نفر چندم مقاله |
|---|---|
| شبنم اسکندرزاده | اول |
| عنوان | متن |
|---|---|
| کلمات کلیدی | New Drugs ،pediatric ،Asthma |
| خلاصه مقاله | Since 2017 IUIS report, the term “Inborn Errors of Immunity” replaced Primary Immunodeficiencies. IEI present clinically as increased susceptibility to infections, autoimmunity, autoinfammatory diseases, allergy, bone marrow failure, malignancy. The Middle East and North Africa (MENA) region has an increased prevalence of IEI because of the high rate of consanguinity with a predominance of autosomal recessive disorders. A case in point, Selective IgA deficiency 1:651 in adults from Iran. IEI are currently categorized into 10 Tables: combined immunodeficiencies, combined, mmunodefciencies with syndromic features, predominantly antibody defciencies, diseases of immune dysregulation, congenital defects of phagocytes, defects in intrinsic and innate immunity, autoinfammatory diseases, complement defciencies, bone marrow failure, and phenocopies of inborn errors of immunity. The age of onset of PID varies depending on the defect. Patients with mild genetic defects may live until adulthood asymptomatically, while those who carry severe defects may die in early life. Initial studies reported low frequencies of 5%e10% of adults among registries of patients with PID, but in recent reports of the ESID and JMF 66.37% and 60.67%, respectively, of patients were adults. In addition to prevalence, the incidence of PID is also different in each age group, with the highest incidence rate among children below 5 years. SCIDs are a group of rare, life-threatening inherited disorders that represent a paediatric emergency. These disorders are present at birth and manifest as major abnormalities of the immune system leading to extreme susceptibility to serious infections. Early diagnosis is essential to enable early intervention before infections occur. Potentially curative haematopoietic stem cell transplantation (HSCT, previously known as bone marrow transplantation) given during the first 3 months of life has a 96% success rate. Without a successful HSCT or gene therapy, patients are at constant risk of a fatal infection before 1 year of age. IMMUNOGLOBULIN REPLACEMENT THERAPY: Haematopoietic stem cell transplantation (HSCT), Antimicrobials, Granulocyte colony stimulating factor (GCSF), Gamma-interferon, Gene therapy, Targeted therapy approaches. Live vaccines (e.g. BCG, MMR, Rotavirus…) are absolutely contraindicated. Patients with SCID requiring blood or platelet transfusion should always get irradiated (CMV-negative, leukocyte-depleted) products. |
| نام فایل | تاریخ درج فایل | اندازه فایل | دانلود |
|---|---|---|---|
| Tazeh.asm.pdf | 1402/11/28 | 85303 | دانلود |
| Tazeh.Asm1.pdf | 1402/11/28 | 122985 | دانلود |
| Tabriz.Asm.2.pdf | 1402/11/28 | 876020 | دانلود |
