چاپ صفحه |  صفحه نخست سامانه |  چکیده مقاله |  نویسندگان |  دانلود مقاله | دانشگاه علوم پزشکی تبریز |
| immunedeficiencyMethod We retrospectively studied the demographic, clinical and immunological character-istics of the PID patients in a single tertiary centre, from January 1989 to July 2014. Thepatients were classified according to the International Union of Immunological Societies ExpertCommittee on PID.Results: 98 patients were diagnosed with and followed-up for 15 disorders. The mean age atonset and diagnosis and the diagnostic delay were 8 ± 10, 14.2 ± 13.1 and 6.1 ± 7 years, respec-tively. Parental consanguinity rate was 57%. Predominantly Antibody Deficiency was the mostcommon diagnosis (n = 63), followed by congenital defects of phagocytes (n = 16), combinedimmunodeficiencies (n = 12), well defined syndromes (n = 4) and defects in innate immunity(n = 3). Recurrent sinopulmonary infection was the most common presentation. Active infectionswere treated appropriately, in addition to prophylactic therapy with IVIG and antimicrobials.Not all the patients were compliant with prophylactic regimens due to cost and unavailability.One SCID patient underwent successful bone marrow transplantation. The total mortality ratewas 19% during the follow-up period (7.8 ± 7.6 years). The mean age of living patients at thetime of study was 23 ± 11.7 years.Conclusions: Physicians awareness of PID has been rising dramatically in Iran, ensuring anincreasing number of patients being diagnosed and treated. More effective treatment services,including health insurance coverage and drug availability are needed to improve the outcomeof PID patients. |
| نویسنده | نفر چندم مقاله |
|---|---|
| جواد احمدیان هریس | نوزدهم |
| نام فایل | تاریخ درج فایل | اندازه فایل | دانلود |
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| Long-termfollow-upofninetyeightIranianpatientswithprimaryimmunedeficiencyinasingletertiarycentre (1).pdf | 1398/09/25 | 851396 | دانلود |
