Carotid intima-media thickness,lipid profile,serum amyloid A and vitamin D status in children with familial Mediterranean fever

Aim of the workIt was to estimate the carotid intima-media thickness (CIMT), lipid profile, serum amyloid A and vitamin D in Familial Mediterranean Fever (FMF) patients and to explore the relationship between CIMT and FMF.Patients and methodsThe study comprised forty-five FMF patients diagnosed during the attack free period and 40 healthy children with similar demographic features as control. The diagnosis of the FMF cases was confirmed by clinical, laboratory assessments and confirmed by the molecular diagnosis. The CIMT, lipid profile, complete blood picture, serum amyloid A (SAA) levels, vitamin D and the growth pattern were investigated.ResultsThe study showed no significant difference of CIMT among patients and controls, significant decrease of vitamin D levels, while lipid profile parameters, triglyceride (TG) to HDL-Cholesterol (HDL-C) ratio, serum amyloid A were significantly increased. A significant correlation was present between the CIMT with the serum cholesterol, low density lipoprotein and triglycerides as well as between SAA and the number of attacks. In addition, vitamin D levels showed significant negative correlation with colchicine. M694I mutation was the most prevalent among FMF patients. Growth parameters were normal in FMF cases.ConclusionThis study sheds light that the normal CIMT in the FMF patients makes it difficult to describe the children as having subclinical atherosclerosis although the higher TG/HDL ratio reflects their risk of atherosclerosis. Moreover, significant decrease of vitamin D in FMF patients was observed. The growth parameters of the FMF patients on regular treatment of colchicine were not affected.     

慢性便秘继发肠系膜上动脉压迫综合征(李氏三联征)的诊断与治疗

目的归纳按“李氏三联征”概念指导诊断和治疗慢性便秘继发肠系膜上动脉压迫综合征(SMAS)的经验。方法“李氏三联征”的概念包括:(1)临床症状:便秘、营养不良、上消化道梗阻(呕吐、进食困难)三联症状;(2)解剖学表现:具有横结肠下垂、脾曲升高和肠系膜上动脉压迫三联解剖异常;(3)治疗:均给予肠内营养支持、胸膝位、菌群移植三联治疗。根据“李氏三联征”概念,采用描述性病例系列研究方法,前瞻性纳入2014年6月至2018年11月期间,同济大学附属第十人民医院和东部战区总医院收治的78例慢性便秘继发SMAS患者的病例资料,包括基本信息、症状体征、影像学资料、营养指标、胃肠生活质量指数和Wexner排粪评分,按上述“李氏三联征”的标准进行评估和治疗,对临床症状和解剖学特征指标进行随访,记录治疗后1、3、6和12个月的变化。结果治疗前所有患者均有“李氏三联征”特征,均存在严重便秘、营养不良及上消化道梗阻的临床表现,均具有肠系膜上动脉压迫征象和明显的脾曲升高的解剖学表现。经肠内营养支持、胸膝位及菌群移植三联治疗后,69例(88.5%)症状明显改善,9例治疗无效行手术治疗。69例非手术患者经12个月随访,结果显示,所有患者均恢复正常进食,1个月后便秘相关指标改善,至12个月后时,每周自主排粪次数从治疗前(1.0±0.8)次增加至(5.0±1.6)次,胃肠生活质量指数由治疗前(52.7±8.5)分增加至(93.2±7.5)分,Wexner排粪评分由治疗前(19.1±2.5)分下降至(6.2±2.1)分,差异均有统计学意义(均P<0.001)。随访1个月后营养指标改善,至12个月后时,体质指数从治疗前(17.9±1.8)kg/m2增加至(21.0±1.3)kg/m2,总蛋白由(65.2±5.7)g/L增加至(68.3±4.2)g/L,白蛋白从(32.1±5.1)g/L增加至(40.4±3.0)g/L,纤维蛋白原从(1.9±0.5)g/L增加至(2.4±0.5)g/L,前白蛋白从(163.2±5.3)mg/L增加至(259.1±45.6)mg/L;差异均有统计学意义(均P<0.001)。上消化道造影及肠系膜上动脉成像均显示十二指肠受压征象解除,肠系膜上动脉与腹主动脉夹角从治疗前(17.4±3.8)°增加至(37.8±5.8)°(t=-22.26,P<0.001)。结论慢性便秘继发SMAS患者具有“李氏三联征”的异常三联临床症状和解剖学表现时,应给予肠内营养支持、胸膝位及菌群移植三联治疗。