Salehzadeh Farhad, Emami Dina, Zolfegari Ali Asgar, Yazdanbod Abas, Habibzadeh Sharam, Bashardost Bahman, Barak Manuchehr, Feizi Iraj, Azimi Hormoz, Jastan Marina, Khalafi Jafar
Department of Pediatrics, Ardabil University of Medical Science (ARUMS), Ardabil, Iran.
Turk J Pediatr. 2008 Jan-Feb;50(1):40-4.
Familial Mediterranean fever (FMF), which is the prototype of the hereditary periodic fever syndromes, is common in the countries around the Mediterranean Sea. Considering its geographical position in the northwest of Iran, with its population of Turkish origin and its vicinity to the Mediterranean Sea, th incidence of FMF should be high in Ardabil. The goal of this study was to introduce FMF as a disease with significant outbreak in this area. Based on the Tel-Hashomer criteria, patients suffering from FMF were collected from private clinics together with the medical records of adult and pediatric rheumatology clinics. Of 112 total patients determined, 74 were studied. All of the patients were interviewed and completed a questionnaire. Familial Mediterranean fever was common among children under 18 years (76%), and it was more common in males than females (M/F 1.17). Abdominal pain was the most common complaint (74%) and abdominal pain and fever (95% and 84%, respectively) were the main clinical symptoms. The average duration of pain was 12-72 hours and the average recovery (attack-free period) was from one week to one month (63.5%). The majority of the patients had hospital admission for diagnostic work-up (85%) and some (32%) ha undergone surgical operation erroneously; 92% of the patients had taken medications with incorrect diagnosis; and 20% had positive familial history of FMF. Fifty percent of the patients' parents were first-degree relatives an in 59.5% delay in diagnosis was more than three years. It seems that FMF is more common in the Northwest of Iran than previousl thought, although physicians are not familiar with it. The common age for manifestation of this disease is under 18 years and its presentation after the age of 40 years is very rare.
家族性地中海热(FMF)是遗传性周期性发热综合征的典型代表,在地中海周边国家较为常见。考虑到伊朗西北部的地理位置、当地土耳其裔人口以及与地中海的临近程度,阿尔达比勒的FMF发病率应该较高。本研究的目的是介绍FMF在该地区是一种有显著暴发情况的疾病。根据泰尔-哈绍默标准,从私人诊所收集了患有FMF的患者以及成人和儿科风湿病诊所的病历。在确定的112名患者中,对74名进行了研究。所有患者均接受了访谈并填写了问卷。家族性地中海热在18岁以下儿童中很常见(76%),男性比女性更常见(男/女比例为1.17)。腹痛是最常见的主诉(74%),腹痛和发热(分别为95%和84%)是主要临床症状。疼痛的平均持续时间为12至72小时,平均恢复时间(无发作期)为一周至一个月(63.5%)。大多数患者因诊断检查而住院(85%),一些患者(32%)曾错误地接受过手术;92%的患者在诊断错误的情况下服用过药物;20%的患者有FMF的阳性家族史。50%患者的父母是一级亲属,59.5%的患者诊断延迟超过三年。尽管医生对其并不熟悉,但似乎FMF在伊朗西北部比以前认为的更为常见。这种疾病的常见发病年龄在18岁以下,40岁以后发病非常罕见。