lncRNA RP11-86H7.1在川崎病诊断中的临床意义

目的：探讨lncRNA RP11-86H7.1在川崎病（KD）患者血清中的表达及其与临床病理特征及预后的关系。方法：筛选KD特异相关的循环lncRNA，分KD治疗前患儿组、KD治疗后患儿组、普通发热患儿组及健康儿童组，采用qPCR检测各组血清lncRNA RP11-86H7.1相对表达。分析血清lncRNA RP11-86H7.1相对表达与KD临床病理特征间关系；绘制ROC曲线，分析血清lncRNA RP11-86H7.1表达水平对KD的诊断效能。结果：KD急性患儿组血清lncRNA RP11-86H7.1相对表达量高于各对照组（P＜0.05）；年龄和性别比例与低表达组比较差异无统计学意义（P＞0.05）；qPCR发现lncRNA RP11-86H7.1在KD急性期患儿血清中表达水平明显高于KD恢复期、健康儿童及发热儿童组，差异均有统计学意义（P＜0.05）。结论：血清lncRNA RP11-86H7.1在KD患者中表达上调，其可作为KD早期诊断和评估预后的潜在的生物标志物。     

糖皮质激素在川崎病治疗中作用的循证医学研究

目的 通过循证医学方法研究肾上腺皮质激素在川崎病治疗中的作用.方法 采用循证医学方法,检索国内外5种大型专业数据库,筛选出随机或半随机对照研究的文献,再人工筛查确定适宜文献.通过Meta分析,比较激素与丙种球蛋白治疗川崎病在退热时间和冠状动脉病变发生率方面的差异.结果 初筛得到443篇文献.经过人工检索,8篇文献符合要求而被纳入标准.其中,3篇文献共有49例川崎病患儿接受甲基强的松龙治疗,5篇文献共有406例患儿接受强的松治疗.对照组共有33例接受静脉丙种球蛋白治疗,435例接受阿司匹林治疗.各组间患儿性别、年龄、病程差异无显著意义.①甲基强的松龙组(3篇文献):24例发生冠状动脉病变,对照组18例发生,两组病变发生率差异无显著意义.在退热方面,1篇文献显示甲基强的松龙组发热时间明显较丙种球蛋白组缩短,差异有显著意义;2篇文献显示差异无显著意义.②强的松组(8篇文献):28例发生冠状动脉瘤,阿司匹林对照组11例发生,3篇文献提示强的松组冠状动脉瘤发生率明显高于对照组.强的松组76例发生冠状动脉扩张,对照组75例发生,3篇文献提示强的松组冠状动脉扩张发生率明显高于对照组,2篇文献提示强的松组冠状动脉扩张发生率略低于对照组,但差异无显著意义.结论 目前的研究资料显示,激素治疗与丙种球蛋白治疗川崎病在冠状动脉病变发生率方面尚无差异或增高.在缩短热程上两者差异也不显著.尚可进一步通过更大数量病例的随机对照研究来证实本结果.目前治疗川崎病不主张单独应用激素.     

Kawasaki Disease in a Father and Son

The pathogenesis and etiology of Kawasaki disease are unknown, but some studies suggest increased genetic susceptibility. The case is presented of an infant with Kawasaki disease whose father suffered from the same illness 21 years previously. The A, B and C loci of the HLA antigens were examined.     

Acute heart failure and acute renal failure in Kawasaki disease

Acute renal failure and acute heart failure are rare in Kawasaki disease. We experienced two patients with Kawasaki disease who presented acute renal failure and acute heart failure. These two patients gave us an important insight into the understanding of water balance and fluid therapy in Kawasaki disease. One patient showed acute prerenal failure due to fluid exudation from the intravascular to the extravascular space, and subsequent acute heart failure. The other patient showed acute heart failure caused by fluid infusion for the treatment of dehydration. It is suggested that acute renal failure could be caused by a fluid shift from the intravascular to the extravascular space in Kawasaki disease. It is also demonstrated that the reserve of cardiac function could be decreased in patients with Kawasaki disease due to myocarditis even with normal echocardiography and chest X-rays.     

Lower level of serum potassium and higher level of C-reactive protein as an independent risk factor for giant aneurysms in Kawasaki disease

Giant aneurysms are the most serious issue of patients with Kawasaki disease (KD). To clarify risk factors for these giant aneurysms, we conducted a matched case-control study. Among the patients reported in nationwide surveys, 117 patients with giant aneurysms had an unequivocal new diagnosis and presented at the treatment center within 9 d of illness. We obtained clinical information on admission of about 69 patients (case) from the treatment centers. One control was selected for each case, an age- and sex-matched patient without coronary involvement, reported from the same treatment center at about the same time as the case, and we obtained the same clinical information about controls. Fourteen variables were analysed with a conditional logistic regression model: body temperature, hematocrit, hemoglobin, numbers of leukocyte and platelets, concentrations of serum albumin, globulin, total cholesterol, sodium, potassium and chloride, erythrocyte sedimentation rate, C-reactive protein and alanine aminotransferase activity. After adjustment for age, duration of illness before admission and use of intravenous gamma globulin therapy, C-reactive protein [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.054-1.237], alanine aminotransferase activity (OR = 1.008, 95% CI 1.002-1.014), serum sodium concentration (OR = 0.877, 95% CI 0.770-0.999) and serum potassium concentration (OR = 0.319, 95% CI 0.124-0.822) were significantly related to the risk for giant aneurysms. Further analyses with these four explanatory variables revealed that C-reactive protein (OR = 1.159, 95% CI 1.022-1.315) and serum potassium concentration (OR = 0.222, 95% CI 0.052-0.948) met the significant level. Thus, the values for serum C-reactive protein and potassium are independent risk factors for the development of the giant aneurysms of Kawasaki disease.     

Mediastinal lymphadenopathy: a variant of incomplete Kawasaki disease

A 14-month-old girl presented with a 4-d history of fever and generalized exanthema. Four characteristic symptoms of incomplete Kawasaki disease (KD) were present on admission (fever, rash, non-purulent conjunctival injection, oropharyngeal changes) and then followed by oedema of the hands and feet and mild plantar desquamation. The typical laboratory features of KD, such as elevated erythrocyte sedimentation rate, leukocytosis, thrombocytosis, and positive C-reactive protein were also seen. Ultrasound examination of the mediastinum revealed the presence of a lymph node, 30 mm in diameter, below the tracheal carina. Thoracic CT scan confirmed the mediastinal lymph node. The patient was treated with aspirin and intravenous γ -globulin. Ultrasound study of the mediastinum, which was carried out 6 weeks after hospital discharge, showed that the lymph node had disappeared. This case illustrates that lymph nodes other than cervical lymphadenopathy should be sought when the diagnosis of classical or atypical KD is suspected.     

冠状动脉超声显像研究川崎病冠状动脉瘤：十二年回顾分析

1984年～1996年间应用冠状动脉超声显像研究川崎病502例，检出冠状动脉瘤（CAA）70例（13．9％），冠状动脉内径＞4mm（CA／AO＞0．30），呈校形扩张，51例（72．8％）1年好转。14例巨大CAA（BCAA）内径＞8mm（CA／AO＞0．60），回缩困难，经2～5年稍好转。7例并发冠状动脉血栓，2例并发心肌梗塞，4例并发腋动脉瘤，10例以心肌断层显像99mTC－MIBI对照研究，显示心肌灌注异常。1例CAA患儿因急性心肌梗塞死亡。冠状动脉超声显像用于诊断和随访川崎病冠状动脉瘤具有方便、迅速、直观、定位准确的优点，值得临床推广使用。     

川崎病患儿丙种球蛋白耐药列线图模型的构建与验证

目的 基于Lasso回归和列线图构建并验证川崎病患儿对丙种球蛋白耐药的预测模型,以期为临床诊疗提供帮助。方法 回顾性收集2014年7月—2020年7月西南医科大学附属医院收治的474例川崎病患儿的临床资料,采用Lasso回归分析筛选重要的临床因素构建Nomogram模型,通过绘制受试者工作特征（ROC）曲线、Calibration校准曲线及DCA曲线验证模型的区分度、校准度及临床有效性。结果 共纳入474例患儿资料,其中339例作为训练集,135例作为验证集。Lasso回归分析显示,心脏表现、心外并发症、首剂静脉注射免疫球蛋白使用时间、中性粒细胞比例、红细胞分布宽度-标准差、血小板压积、白蛋白、系统性免疫-炎症指数及C反应蛋白/白蛋白是川崎病患儿丙种球蛋白耐药的预测因素。基于上述预测因素构建Nomogram模型,并分别在训练集与验证集人群中进行验证。训练集ROC曲线下面积（AUC）为0.784(95%CI:0.701,0.867),当最佳阈值取0.045时,相应的特异性和敏感性分别为0.490(95%CI:0.434,0.546)和0.935(95%CI:0.849,1.000);验证...     

Soluble cell adhesion molecules and von Willebrand factor in children with Kawasaki disease.

Fifty-nine children with acute Kawasaki disease (KD), a childhood vasculitis, were compared with 35 children with fever due to infection and 48 healthy children. Levels of soluble E-selectin (sE-selectin), soluble intercellular adhesion molecule-1 (sICAM-1), and soluble vascular cell adhesion molecule-1 (sVCAM-1) in the healthy children were double those found in adults. All three soluble cell adhesion molecules and von Willebrand factor (vWF) were higher in the children with KD than in the healthy children, but only sE-selectin, a marker for activated endothelial cells, and sICAM-1 were higher than in the febrile children. The high levels of vWF in KD appear to reflect the prominent acute-phase reaction. This information can help us to understand further the complex interactions between cytokines, circulating inflammatory cells and the vascular endothelium, and may lead to new therapeutic avenues in KD and other inflammatory diseases and vasculitides.