Incomplete Kawasaki disease with recurrent skin peeling: a case report with the review of literature

Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that has largely replaced rheumatic heart disease as a cause of acquired heart disease in children of many developed countries. We report a case of incomplete KD in a five-year-old girl. The diagnosis of incomplete KD was made after exclusion of conditions with similar presentation. She was treated with intravenous immunoglobulin following which she made an uneventful recovery but demonstrated thrombocytosis in the second week of convalescence. During the six-month follow up period, she had two episodes of recurrent skin peeling a phenomenon, which is recently reported with KD but not with atypical or incomplete KD. It is important for the treating physicians to become aware of the incomplete KD as prompt diagnosis and early treatment of these patients with intravenous immunoglobulin is vital for the prevention of lethal coronary complications. Physicians need to have a "high index of suspicion" for KD and even, higher for IKD.     

心电图与冠状动脉造影诊断冠心病的价值分析

目的 探讨心电图检查与冠状动脉造影检查在冠心病患者中的诊断价值。方法 选取2016年10月~2018年10月于九江市柴桑区人民医院住院的疑似冠心病患者110例,所有患者行常规心电图和冠状动脉造影检查,比较心电图检查结果和冠状动脉造影结果。结果 ①ST-T改变在心电图中诊断率为65.45%,高于冠状动脉造影检查的60.91%,二者比较,差异无统计学意义（P＞0.05）;②冠状动脉造影检查确诊冠心病患者67例。其中ST-T改变诊断率为58.21%,高于非ST-T改变的41.79%,ST-T改变在确诊冠心病方面优于非ST-T改变,差异具有统计学意义（P＜0.05）。结论 冠状动脉造影检查仍是临床确诊冠心病的首选方法,心电图ST-T改变能够为早期冠心病诊断方面提供依据,临床确诊还需结合冠状动脉造影等相关检查。     

儿童冠状动脉疾病32例临床分析

目的探讨儿童冠状动脉疾病的病因分布及临床特点,以提高诊断和治疗儿童冠状动脉疾病水平。方法回顾性分析2008年7月至2011年12月在首都医科大学附属北京安贞医院儿童心脏中心收治的冠状动脉疾病患儿（不包括复杂心脏畸形合并冠状动脉异常者）的临床资料,分析临床表现、辅助检查、诊断方法和治疗。结果32例冠状动脉疾病患儿进入分析,男20例,女12例,年龄1个月至18岁。经ECG、超声心动图、64排CT和心导管造影检查明确诊断。左冠状动脉异常起源于肺动脉10例（31．2％）,8例以心力衰竭起病,其中长期误诊为“心内膜弹力纤维增生症”4例,“扩张性心肌病”1例,“二尖瓣重度关闭不全”2例,均行外科手术移植左冠状动脉,1例术后死亡,余9例术后随访临床症状好转,左室缩小,二尖瓣反流减轻;冠状动脉瘘13例（40．6％）,9例以心脏杂音、4例以心力衰竭起病,外科手术行瘘缝扎或补片治疗12例,介入治疗1例,术后随访仅1例存在少量残余分流;川崎病并发冠状动脉瘤6例（18．8％）,出现左心扩大、心肌缺血征象,予长期抗凝等治疗;家族性高胆固醇血症3例（9．4％）,表现为冠状动脉粥样硬化性心脏病,予降胆固醇、改善心肌缺血等药物及饮食控制治疗,随访病情尚稳定,无明显加重。结论儿童冠状动脉疾病少见,先天性包括左冠状动脉异常起源于肺动脉和冠状动脉瘘,后天性以高胆固醇血症和川崎病累及冠状动脉多见,对于有左心扩大和心肌缺血表现者,应重视冠状动脉疾病的可能,早期诊断、合理治疗是改善预后的关键。     

Pediatric patients with Kawasaki disease and a case report of Kitamura operation

Kawasaki disease (KD), also called mucocutaneous lymph node syndrome, is an acute, self-limiting, small-vessel vasculitis with an unknown cause that affects children between the ages of 6 months and 5 years. It is the most common cause of acquired coronary artery disease in childhood. Acute myocardial infarction and coronary artery aneurysm are major complications. We present a cohort of patients with KD who were followed up and treated in the Heart Center, North Rhine-Westphalia. Included is a review of important relevant items common to cases of KD, such as clinical data and management, including medical management of the acute condition and the diagnosis and management of coronary vasculitis and aneurysms as well as the application of coronary artery bypass grafting (CABG) in those conditions. Between January 2002 and January 2006, we evaluated the findings and characteristics of 18 pediatric patients with a history of KD and their long-term outcome. The acute illness occurred between the ages of 4 months and 14 years of age. Anomalies of the coronary arteries were found in 6 patients ranging in age from 5 months to 10 years. One patient had acute myocardial infarction; another underwent CABG after 5 years from disease onset at the age of 15 years. Kitamura operation was performed successfully. The other patients are still under observation.Coronary artery aneurysms and stenosis requiring surgery are rare in KD; nevertheless, CABG is the standard therapy when myocardial ischemia is detected. Kitamura operation provides good growth potential and long-term graft patency.     

Kawasaki disease seasonality in Venezuela supports an arbovirus infection trigger

Kawasaki disease (KD) is an inflammatory disease primarily affecting infants and young children, whose etiology remains uncertain. Observational studies of the overlap between KD outbreaks and seasonal peaks of arboviral infections, suggest the possible role of these pathogens as triggers of KD. In Venezuela, regions with the highest reported arboviral infections simultaneously have the highest incidence of KD. One proposed explanation for this association involves the role of proinflammatory mediators, interleukin-1 (IL-1), IL-6, tumor necrosis factor, and vascular endothelial growth factor as mediators of coronary endothelial damage. The promotion of inflammation and tissue destruction by these cytokines is thought to contribute to the coronary endothelial damage experienced in KD. The utilization of overlapping KD and arboviral infection trends contribute to the comprehension of KD etiology, with improvements in diagnosis, prognosis and treatment.     

血清缺血修饰白蛋白在冠心病患者中的临床应用价值

目的 探究血清缺血修饰白蛋白(isehemia modified albumin,IMA)在冠心病患者体内的变化及其与经皮冠状动脉介入治疗(PCI)术后再狭窄的关系,分析IMA在冠心病患者的早期诊断和PCI术后评估中的价值.方法 对疑似冠心病而收治入南京市第一医院的176例患者在入院后即行IMA检测,最终通过冠状动脉造影术确诊冠心病患者111例,非冠心病患者65例,比较两组之间血清IMA浓度有无显著差异,绘制IMA的受试者工作特征(ROC)曲线,分析IMA在ROC曲线下面积及IMA在诊断冠心病时的灵敏度和特异性.用同样的方法检测48例行PCI术治疗的患者术前IMA水平,半年后复查造影确定再狭窄患者7例,未发生再狭窄患者41例,采用t检验分析PCI术后再狭窄组与未再狭窄组患者IMA血清浓度的差别.结果 通过t检验分析发现,冠心病组IMA水平显著高于非冠心病组,差异具有统计学意义;通过绘制ROC曲线,IMA曲线下面积为0.88,其对冠心病诊断灵敏度和特异性可以分别达到88％和72％;在PCI术后再狭窄组和未再狭窄组中,两组患者术前IMA水平的差异无统计学意义(P＞0.05).结论 血清IMA作为近年来被确定的急性心肌缺血标志物,在冠心病的早期诊断中发挥着重要作用,将血清IMA作为冠心病的辅助诊断指标之一可以极大地降低冠心病患者的漏诊和误诊.血清IMA预测PCI术后患者是否发生血管内再狭窄尚需要大规模的临床研究.     

运动平板心电图与24小时动态心电图在诊断冠心病中的应用价值对比

目的分析运动平板心电图和24小时动态心电图应用于冠状动脉粥样硬化性心脏病诊断的临床价值。方法选取通过冠脉造影进行确诊冠心病患者56例,分别采取运动平板心电图及24小时动态心电图检查,对比两组检查措施诊断冠状动脉粥样性心脏的特异度及敏感度。结果运动平板心电图诊断冠心病的特异度为46.2%,敏感度为86%,24小时动态心电图诊断冠心病的特异度为62.5%,敏感度为75%。两组者比较差异无统计学意义(P0.05)。结论运动平板心电图及24小时动态心电图应用于冠心病的诊断均具有较好的临床价值,为提高冠心病的临床诊断准确度,应当联合上述两种诊断手段。     

静息心电图、胸痛症状与冠脉CTA筛查冠心病的临床价值

目的 探讨静息心电图、胸痛症状与256层螺旋CT冠脉成像筛查冠心病的诊断价值。方法 收集入院后接受静息心电图、256层螺旋CTA检查共计68例非急症胸痛疑似冠心病患者的相关资料,所有患者30 d内均进行CAG检查。以CAG诊断为标准,采用ROC曲线分析静息心电图、胸痛症状与冠脉CTA筛查冠心病的诊断价值。结果 256层螺旋CTA诊断冠心病的ROC曲线下面积为0.73（P＜0.05）,其诊断敏感性为96.55%,特异性为50.00%,诊断价值大于静息心电图（AUC=0.50,P＞0.05）和胸痛症状（AUC=0.51,P＞0.05）。结论 256层螺旋CTA筛查冠心病有较高的诊断价值;静息心电图、胸痛症状对冠心病的筛查存在明显的局限性。     

Kawasaki disease: current aspects on aetiopathogenesis and therapeutic management

Kawasaki disease (KD) is a vasculitis that affects mainly children of 6 months to 4 years old. It is important to be early recognised so as to limit the inflammatory cascade that may lead to aneurysmatic dilatations of coronary arteries. The causative agent of KD has not been still indentified and the aetiopathogenetic theories are based on epidemiologic, laboratory and histological data. The management of the disease is divided according to the clinical stage and patients' follow up should be continued for years after the disease onset. The exact period is determined by the risk level of the KD.     

单中心9年不完全川崎病回顾性分析

目的探讨不完全川崎病（KD）的临床特征,以提高临床诊治水平。方法回顾性分析2002年1月至2010年12月KD住院患儿的临床资料,比较不完全KD与典型KD在发病年龄、性别、临床表现、实验室检查、治疗及冠状动脉损害等方面的差异。结果 1484例KD患儿进入分析,其中不完全KD262例（17.6%）,典型KD1222例;〈1岁患儿中不完全KD占24.9%。不完全KD和典型KD患儿的平均发热时间分别为（7.8±5.0）和（6.7±3.6）d,差异有统计学意义。不完全KD四肢改变、多形皮疹、眼结膜充血、口唇改变、颈部淋巴结肿大和肛周改变的发生率显著高于典型KD;卡疤改变、扁桃体肿大、阴囊或外阴改变、呕吐和腹泻的发生率与典型KD差异无统计学意义。两组CRP、ESR、Hb、WBC、PLT、ALT、AST、CK-MB和LDH等实验室指标差异无统计学意义。不完全KD与典型KD患儿对IVIG无反应的发生率差异无统计学意义（14.1%vs17.5%）;不完全KD患儿冠状动脉扩张、冠状动脉瘤和巨大冠状动脉瘤的发生率分别为57.5%、14.1%和1.9%,典型KD患儿分别为31.5%、5.9%和0.6%,两组差异有统计学意义。结论不完全KD较典型KD发热时间长,且冠状动脉损害发生率高,但实验室指标差别不大。     

Kawasaki disease: characteristics,diagnosis, and unusual presentations

ABSTRACT

Introduction: Kawasaki disease (KD) is one of the commonest pediatric vasculitides and is associated with a significant risk of development of coronary artery abnormalities if left untreated.

Areas covered: In this review, we have highlighted the incomplete and unusual presentations of KD and also emphasize the controversies pertaining to 2D echocardiography in KD. A PubMed search was performed regarding diagnosis and unusual presentations of KD.

Expert opinion: Diagnosis of KD is essentially clinical and based on recognition of typical clinical features that may appear sequentially and all signs and symptoms may not be present at one point of time. There is no confirmatory laboratory test for diagnosis of this condition. Further complicating the picture is the fact that incomplete and atypical forms KD may be seen in up to 50% patients. Although 2D echocardiography continues to be the preferred imaging modality for cardiac assessment in patients with KD, it has its limitations.     

IL-1-dependent electrophysiological changes and cardiac neural remodeling in a mouse model of Kawasaki disease vasculitis

Kawasaki disease (KD) is the leading cause of acquired heart disease in children. In addition to coronary artery abnormalities, aneurysms and myocarditis, acute KD is also associated with echocardiogram (ECG) abnormalities in 40–80% of patients. Here, we show that these ECG changes are recapitulated in the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model. LCWE-injected mice developed elevated heart rate and decreased R wave amplitude, with significant differences in prolonged ventricular repolarization. LCWE-injected mice developed cardiac ganglion inflammation, that may affect the impulse-conducting system in the myocardium. Furthermore, serum nerve growth factor (NGF) was significantly elevated in LCWE-injected mice, similar to children with KD vasculitis, associated with increased neural remodeling of the myocardium. ECG abnormalities were prevented by blocking interleukin (IL)-1 signaling with anakinra, and the increase in serum NGF and cardiac neural remodeling were similarly blocked in Il1r1^−/− mice and in wild-type mice treated with anakinra. Thus, similar to clinical KD, the LCWE-induced KD vasculitis mouse model also exhibits electrophysiological abnormalities and cardiac neuronal remodeling, and these changes can be prevented by blocking IL-1 signaling. These data support the acceleration of anti-IL-1 therapy trials to benefit KD patients.     

Histo-blood group gene polymorphisms as potential genetic modifiers of the development of coronary artery lesions in patients with Kawasaki disease

Abnormal immunological responses to certain microbial agents may play a crucial role in the pathogenesis of Kawasaki disease (KD). The association studies between histo-blood group genes (Lewis and ABO blood types) and various types of infectious diseases or vasculopathy have been carried out based on the fact that glycosylated antigens could directly mediate microbial infections. We attempted to clarify the role of blood type antigens in the development of KD and coronary artery lesions in KD patients. The subjects included 164 KD patients enrolled from 1998 to 2003 (1st group), 232 patients from 2004 to 2009 (2nd group), and 223 healthy children and 118 patients with growth hormone deficiency as controls. The genotyping of the FUT2 and FUT3 genes, and ABO genotypes, was determined with the TaqMan SNP assay and allele-specific polymerase chain reaction. No significant differences were observed in the genotypes and allele frequencies of the FUT2 and FUT3 genes between the groups. The frequency of the BB blood genotype was significantly higher in KD patients with coronary artery lesions in the 1st and 2nd groups than in the controls (17% and 14% vs. 5%, P = 0.0020). This is the first report to investigate the roles of ABO and Lewis blood types in the development of KD, and in the formation of coronary artery lesions in KD patients. These data suggest that the ABO blood type may play a role in the development of coronary artery lesions in KD patients.     

Pathology of Kawasaki disease in the healed stage. Relationships between typical and atypical cases of Kawasaki disease

To define the pathological features of Kawasaki disease (KD) in the healed stage (over 40 days of illness), 69 autopsied infants with clinically typical KD (25 in the healed stage and 38 in the acute stage) and atypical KD (four in the healed stage and two in the acute stage) were examined. In the medium-sized arteries with coronary aneurysm in KD, panvasculitis was evident in the acute stages, while scar formation was noted in the healed stage. Thrombi, marked intimal thickening, marked stenosis, and recanalization were seen in the coronary arteries. In KD without aneurysm, acute inflammation was localized in the intima and the perivascular area of the coronary arteries in the acute stages, but was absent in the healed stage. Although intimal thickening was noted, marked stenosis and thrombi were not evident in KD without aneurysm. In KD with and without aneurysm, acute angiitis in small arteries and micro-vessels and acute inflammation of various organs were noted in the acute stages, but disappeared in the healed stage. Fibrinoid necrosis of the artery was rare in the acute stages and was not noted in the healed stage. Atypical cases of KD showed the same pathological features as those in cases with clinically typical KD. The common cause of death in the healed stage was ischemic heart disease. Old myocardial infarction was noted in 80% of the cases, and acute myocardial infarction was seen in 35%. KD is an acute systemic inflammatory disease and KD without aneurysm is a disease with mild angiitis.     

PATHOLOGY OF KAWASAKI DISEASE IN THE HEALED STAGE Relationships between Typical and Atypical Cases of Kawasaki Disease

To define the pathological features of Kawasaki disease (KD) in the healed stage (over 40 days of illness), 69 autopsied infants with clinically typical KD (25 in the healed stage and 38 in the acute stage) and atypical KD (four in the healed stage and two in the acute stage) were examined.
In the medium-sized arteries with coronary aneurysm in KD, panvasculitis was evident in the acute stages, while scar formation was noted in the healed stage. Thrombi, marked intimal thickening, marked stenosis, and recanalization were seen in the coronary arteries. In KD without aneurysm, acute inflammation was localized in the intima and the perivascular area of the coronary arteries in the acute stages, but was absent in the healed stage. Although intimal thickening was noted, marked stenosis and thrombi were not evident in KD without aneurysm. In KD with and without aneurysm, acute angiltis in small arteries and micro-vessels and acute inflammation of various organs were noted in the acute stages, but disappeared in the healed stage. Fibrinoid necrosis of the artery was rare in the acute stages and was not noted in the healed stage. Atypical cases of KD showed the same pathological features as those in cases with clinically typical KD. The common cause of death in the healed stage was ischemic heart disease. Old myocardial infarction was noted in 80% of the cases, and acute myocardial infarction was seen In 35%. KD is an acute systemic inflammatory disease and KD without aneurysm is a disease with mild angiitis.     

Multisystem inflammatory syndrome in children: A dysregulated autoimmune disorder following COVID-19

Multisystem inflammatory syndrome in children (MIS-C) is a dysregulated autoimmune-mediated illness in genetically susceptible patients following COVID-19 with an interval of 2–6 weeks. The median age of patients with MIS-C is 6–11 years. Most common manifestations are involvement of gastrointestinal tract, cardiovascular system, hematological system, and mucocutaneous system. Respiratory tract, neurological system, musculoskeletal system, and kidney are less frequently affected. Mucocutaneous manifestations and coronary artery abnormalities characteristic for Kawasaki disease (KD) may be observed in a significant proportion of MIS-C patients that may make the differential diagnosis be difficult for some patients, especially in the post-pandemic era. The mortality rate is 1–3%. Management and prognosis of MIS-C are similar to that of KD. MIS-C and KD may share a common pathogenic process. Based on the observation of MIS-C-like illness in uninfected neonates, i.e. multisystem inflammatory syndrome in neonates, both MIS-C and KD may be a consequence of dysregulated, over-exaggerated humoral immune responses triggered by a specific infectious agent.     

CTA在冠状动脉钙化斑块管腔狭窄诊断中的研究进展

随着我国人口老龄化趋势不断上升,冠心病患病率随之提升,好发于60周岁以上老年患者。冠心病又称为冠状动脉粥样硬化性心脏病,具有较高的致残率、致死率。随着医学影像技术不断成熟,CT因无创、扫描快的优势已经在临床中得到广泛推广应用,成为了诊断疾病的主要方法之一。CT可分为64排螺旋CT、DSCT、320DVCT,使得正确诊断冠状动脉疾病率得到很大的提升,为无创性检查和评估冠心病提供有效的依据。据有关研究报道,冠状动脉血管成像（CTA）假阳性、假阴性提升的主要原因为患者心率不齐、心率过速造成为心脏运动轨迹伪影;冠状动脉壁管存在钙化伪影均是其主要原因。因此临床常重点关注钙化伪影、心脏运动轨迹伪影对诊断准确性的影响。本文主要就CTA在冠状动脉钙化斑块管腔狭窄诊断中的价值进行综述。     

胆红素与冠心病及炎性标志物hs-CRP的相关性

目的:分析总胆红素水平(total bilirubin level,TBIL)是否为冠心病的保护性因素及其预测价值,同时通过分析TBIL与超敏C反应蛋白(hs-CRP)的相关关系,探讨其冠心病保护机制.方法:连续选取2014年10月至2016年10月安徽医科大学第二附属医院心血管内科住院疑诊冠心病心绞痛并接受冠状动脉造影检查,且临床资料完整的180例患者作为研究对象,根据造影结果分为冠心病组(n=90)与非冠心病组(n=90),采用logistic回归分析探讨TBIL与冠心病的相关性;双变量Pearson 法分析TBIL与hs-CRP的关系;通过绘制受试者工作曲线(receiver operating characteristic curve,ROC),探讨TBIL对冠心病的诊断价值.结果:与非冠心病组相比,冠心病组TBIL水平较低,差异有统计学意义(P＜0.05);二元logistic回归分析显示TBIL为冠心病的独立保护性因素(95％CI 0.738～0.922,P=0.001);Pearson双变量分析显示TBIL与hs-CRP呈负相关(r=-0.367,P＜0.001);ROC曲线显示TBil正确预测冠心病的界点值为10.35μmol/L,最大ROC曲线下面积0.739(95％CI 0.668～0.811,P＜0.01),对应的敏感度为0.633,特异度为0.700.结论:TBIL可能为冠心病的独立保护性因素,低水平TBIL可以作为冠心病的预测因素;TBIL与hs-CRP的负相关关系提示其抗炎作用可能是其对冠心病的保护机制之一;TBIL ＜10.35μtmol/L可以为作为诊断冠心病的界定指标.     

淋巴结Kikuchi病与结核病的病理鉴别诊断

目的 探讨Kikuchi病(KD)病理诊断和鉴别诊断的重要意义.方法 复习31例原病理诊断KD的HE切片,并对其中的20例进行结核病相关的病原学榆测.结果 31例原病理诊断为KD的病例中,13例(41.94%)改诊为结核病,12例仍诊断为KD.结论 淋巴结碎屑性坏死并非KD特有病变,诊断KD需先除外有明显碎屑性坏死的淋巴结结核病,后者主要表现:①碎屑性坏死虽明显,但趋于干酪样坏死;②坏死区内或同时在淋巴窦(主要边缘窦)内,组织细胞、巨噬细胞和泡沫细胞增生,并演变为上皮样细胞和趋于肉芽肿形成;③坏死灶内、外可有数量不等的中性粒细胞浸润;④抗酸杆菌/结核杆菌病原学检测阳性;⑤缺乏KD的典型临床过程.     

Genetic Polymorphisms in the CD40 Ligand Gene and Kawasaki Disease

BACKGROUND: Although some previous studies have reported that genetic and immunological factors play important roles in the pathogenesis of Kawasaki disease (KD), the etiological factors of this enigmatical pediatric disease are still poorly understood. PURPOSE: This study aims to investigate whether polymorphisms of the CD40 ligand (CD40L) gene are associated with KD and the development of coronary artery lesions (CAL) in the Taiwanese children. MATERIALS AND METHODS: The CD40L -3459 A/G and IVS4+121 A/G single nucleotide polymorphisms (SNPs) were genotyped in 167 children with KD and 1,010 ethnically matched healthy controls by TaqMan assay. RESULTS: None of the CD40L polymorphisms was associated with susceptibility or CAL development of KD, and this finding was supported by the haplotype analysis. CONCLUSION: In summary, these results provide little support for specific CD40L SNPs in the susceptibility or CAL development of KD in Taiwanese children. However, it will be necessary to validate or replicate this association in other independent large-size ethnic groups.