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首页> 外文期刊>International journal of rheumatology >Familial Mediterranean Fever in Iran: A Report from FMF Registration Center
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Familial Mediterranean Fever in Iran: A Report from FMF Registration Center

机译:伊朗的家族性地中海热:FMF注册中心的报告

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Background. Familial Mediterranean fever (FMF) is a periodic AR autoinflammatory disorder. This comprehensive study describes FMF in Iran as a country near Mediterranean area.Materials and Methods. From the country FMF registration center 403 patients according to Tel-Hashomer criteria enrolled this study, 239 patients had MEFV gene mutations analyses. Data, if needed, was analyzed by SPSS v20.Results. 175 patients (43.4%) were female and 228 patients (56.6%) were male. The mean age was 21.3 years. Abdominal pain was in 93.3% patients and 88.1% had fever. Abdominal pain was the main complaint of patients in (49.6%). The mean interval between attacks was36.5±29.6days and the mean duration of every episodes was43.3±34.5hours. 15.1% of patients had positive family history and 12.7% had previous surgery; in 52.3% of patients delay in diagnosis was more than three years. 12 common MEFV gene mutations were analyzed, 21.33% were without mutations, 39.7% had compound heterozygote, 25.52% showed heterozygous, and 13.38% showed homozygous results. The most common compound genotype was M694V-V726A (% 10.46) and in alleles M694V (% 20.9) and V726A (% 12.7) were the most frequent mutations, respectively.Conclusion. M694V was the most common mutation, and the most common compound genotype was M694V-V726A. Our genotype results are similar to Arabs and in some way to Armenians, erysipelas-like skin lesions are not common in this area, and clinical criteria are the preferred methods in diagnosis of FMF.
机译:背景。家族性地中海热(FMF)是一种周期性的AR自发性疾病。这项全面的研究将伊朗的FMF描述为一个靠近地中海地区的国家。材料和方法。来自国家FMF注册中心的403名患者按照Tel-Hashomer标准进行了研究,其中239名患者进行了MEFV基因突变分析。如有需要,数据将通过SPSS v20.Results进行分析。女性175例(43.4%),男性228例(56.6%)。平均年龄为21.3岁。腹部疼痛的发生率为93.3%,发烧率为88.1%。腹痛是(49.6%)患者的主要主诉。发作之间的平均间隔为36.5±29.6天,每个发作的平均持续时间为43.3±34.5小时。有15.1%的患者有积极的家族史,有12.7%的患者曾经做过手术;在52.3%的患者中,诊断延迟超过三年。分析了12个常见的MEFV基因突变,无突变的占21.33%,具有复合杂合子的占39.7%,有杂合的占25.52%,有纯合的占13.38%。最常见的化合物基因型是M694V-V726A(%10.46),在等位基因中,M694V(%20.9)和V726A(%12.7)分别是最常见的突变。 M694V是最常见的突变,最常见的化合物基因型是M694V-V726A。我们的基因型结果与阿拉伯人相似,在某种程度上与亚美尼亚人相似,丹毒样皮肤病变在该地区并不常见,临床标准是诊断FMF的首选方法。

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