Peru Harun, Altun Bülent, Doğan Mustafa, Kara Fatih, Elmaci Ahmet Midhat, Oran Bülent
Department of Pediatric Nephrology, Meram Medical Faculty, Selcuk University, 42080 Konya, Turkey.
Clin Rheumatol. 2008 Jun;27(6):689-94. doi: 10.1007/s10067-007-0764-1. Epub 2007 Oct 10.
The aim is to investigate whether pediatric familial Mediterranean fever (FMF) patients have an increased risk of premature atherosclerosis and to determine the possible strength of association between atherosclerosis and Mediterranean fever (MEFV) gene mutation gene type. Demographic characteristics and MEFV mutations were defined in 49 children diagnosed with FMF (26 female, 23 male; mean age, 10.71 +/- 3.69 years). Twenty-six age-, sex-, and body-mass-index-matched healthy children constituted the control group. We evaluated the blood counts and acute-phase proteins during attack-free periods. Mean C-reactive protein (CRP), serum amyloid-A (SAA), homocysteine (Hcy), lipoprotein-a (Lp-a), and common carotid artery intima-media thickness (CCA-IMT) were 10.75 +/- 15.29 vs 4.03 +/- 1.20, 23.22 +/- 41.94 vs 3.53 +/- 1.04, 10.36 +/- 3.36 vs 8.64 +/- 3.15, 20.84 +/- 23.89 vs 8.56 +/- 7.48, and 0.038 +/- 0.007 vs 0.032 +/- 0.004, respectively, and significantly higher than the mean values of control group (p < 0.05). However, no correlation was found between CCA-IMT and CRP, SAA, Hcy, and Lp-a. Twenty-nine patients had M694V mutation, and 13 patients had other mutations. There was no correlation between CCA-IMT and MEFV mutation subgroups. In conclusion, because of the nature of the disease, FMF patients should be considered to have an increased risk of early vascular alteration and atherosclerosis. For this reason, CCA-IMT measurement can be recommended as a noninvasive and early diagnostic method.
目的是调查儿童家族性地中海热(FMF)患者过早发生动脉粥样硬化的风险是否增加,并确定动脉粥样硬化与地中海热(MEFV)基因突变基因类型之间可能的关联强度。对49例诊断为FMF的儿童(26例女性,23例男性;平均年龄10.71±3.69岁)的人口统计学特征和MEFV突变进行了定义。26例年龄、性别和体重指数匹配的健康儿童组成对照组。我们在无发作期评估了血常规和急性期蛋白。平均C反应蛋白(CRP)、血清淀粉样蛋白A(SAA)、同型半胱氨酸(Hcy)、脂蛋白a(Lp-a)和颈总动脉内膜中层厚度(CCA-IMT)分别为10.75±15.29与4.03±1.20、23.22±41.94与3.53±1.04、10.36±3.36与8.64±3.15、20.84±23.89与8.56±7.48、0.038±0.007与0.032±0.004,均显著高于对照组的平均值(p<0.05)。然而,未发现CCA-IMT与CRP、SAA、Hcy和Lp-a之间存在相关性。29例患者有M694V突变,13例患者有其他突变。CCA-IMT与MEFV突变亚组之间无相关性。总之,由于该疾病的性质,应认为FMF患者早期血管改变和动脉粥样硬化的风险增加。因此,推荐将CCA-IMT测量作为一种无创且早期的诊断方法。