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تب مديترانه اي فاميليال در کودکان ايرانی ،اولين گزارش جامع

صالح زاده, فرهاد ، جهانگیری, سپیده ، امامی, دینا ، شیاری, رضا (1389) تب مديترانه اي فاميليال در کودکان ايرانی ،اولين گزارش جامع. Clinical and experimental rheumatology ــ 28 (2). ص. 287. شاپا 0392-856X

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عنوان انگليسي

Familial Mediterranean fever in Iranian children. First report from Iran.

خلاصه انگلیسی

Familial Mediterranean fever which is the prototype of the hereditary periodic fever syndromes is common in the countries around the Mediterranean Sea. "Familial Mediterranean fever" is the common form of the "Hereditary Periodic Fever Syndromes" which has autosomal recessive pattern and presents with self-limited periodic fever and serositis in its classic form. Jewish, Armenian, Arab and Turkish people or in other words people who have Mediterranean originality are affected by disease. MEFV is the responsible gene in this disease and it is located on the short arm of chromosome 16. The mutation of this gene leads to defect in synthesis of a protein known as pyrin-Marenostrin.. Northwest of Iran is near to turkey and people who live there has Turkish origin. This is the first report of FMF in children who live in northwest of Iran. Although it is a common disorder in North West of Iran, interestingly it was not a familiar syndrome to physicians in this area. Methods: This research is a descriptive study from October 2004 to October 2007 at pediatric rheumatology clinic. According to the Tel-Hashomer criteria, FMF had been diagnosed. . All of the patients had been examined, interviewed (and their parents when it needed) and have filled out a questionnaire in pediatric rheumatology clinic. Findings:43 patients inter to this study, all were under 18 years old and minimum age was 1.5years.25 patients were female and 18 were male(M/F ratio was0.7) Abdominal pain (60.5% ) and fever (11.6%) were the main clinical symptoms in this group. The most common symptoms in these patients were systemic symptoms (97.7%) GI (95.3%) and musculoskeletal (55.8%) respectively. The most common period of pain was 12-72 hours. Majority of the patients had hospital admission for diagnostic work up (85%) and some of them (32%) had surgical operation erroneously. More than 90% of the patients had taken different drugs before diagnosis and 20% had positive family history of FMF. The parents of patients were first degree relatives in more than 50% and in 59.5% delay in diagnosis was more than 3 years. All took Colchicine as a first choice therapy, and more than 95% had good response to colchicine Result: Not surprisingly, FMF is a common chronic and periodic auto inflammatory disorder in northwest of Iran, although it is not a familiar disease to physicians in Iran.

نوع سند :مقاله
زبان سند : انگلیسی
نویسنده مسئول :فرهاد صالح زاده
اطلاعات اضافی :Impact Factor: 2.148 Indexed In: ISI-MEDLINE /PubMed -Index medicus
کلید واژه ها (انگلیسی):FMF, periodic fever syndrome, children, colchicine
موضوعات :WB پزشکی عملی
WE سیستم عضلانی اسکلتی
بخش های دانشگاهی :دانشكده پزشكي > گروه اطفال ، پزشکی اجتماعی
کد شناسایی :2948
ارائه شده توسط : خانم صغری گلمغانی
ارائه شده در تاریخ :06 دی 1390 10:37
آخرین تغییر :31 تیر 1394 06:22

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