足月危重新生儿凝血功能和D-二聚体变化的临床意义

目的 探讨危重新生儿凝血、纤维蛋白溶解（纤溶）活性变化及临床意义。方法 对19例极危重、22例危重、21例非危重疾病和10例正常新生儿作前瞻性研究，检测血浆D-二聚体（D-D）、血浆凝血酶原时间（PT）、凝血酶原时间国际正常化比率（PT-INR）、活化部分凝血活酶时间（APTT）、凝血酶时间（TT）、纤维蛋白原（Fib），计数外周血血小板（PLT），比较各组间凝血、纤溶活性变化，及其与疾病严重程度的关系。结果 血浆D-D、PT水平极危重、危重疾病组明显高于正常新生儿，极危重高于非危重疾病组，差异均有显著性（P〈0．05）；APTT极危重疾病组高于正常新生儿，差异有显著性（P〈0．05）。正常新生儿D-D超过参考值范围占70％。疾病严重程度与D．D水平呈高度正相关（r=0．828，P〈0．01），与PT、APTT水平呈中度正相关（r=0．375、0．440，P均〈0．01）。结论 新生儿D-D水平可能处于较高水平，并不一定代表异常。但危重、极危重病患儿D-D水平明显升高，存在凝血系统被激活和继发纤溶亢进，凝血、纤溶活性与疾病严重程度相关。     

川崎病的冠状动脉损害

川崎病 (ＫＤ)又称皮肤粘膜淋巴结综合征 (ＭＣＬＳ) ,临床上以持续 5ｄ以上的发热、球结膜充血、皮肤粘膜弥漫性潮红、颈部淋巴结肿大、指 (趾 )端的硬性水肿及膜样脱皮等为主要表现 ,主要危及生命的损害是冠状动脉损害 ,如冠状动脉狭窄、血栓、梗死、瘤破裂等 ,可导致猝死。此病由日本的川崎教授在 196 7年首先报道[1] ,1972年草川教授对两名有心肌梗死症状患儿进行了冠状动脉造影 ,首次发现了冠状动脉瘤的改变 ,当时其发生率为 2 4.4% ,近年来由于丙种球蛋白等药物的早期应用 ,其发病率已降低为 13% [2 ] 。一、病因与发病情况病因不详 ,…     

血清新蝶呤、D-二聚体水平与川崎病冠状动脉扩张的关系

目的检测川崎病(KD)患儿血清新蝶呤(NP)、D-二聚体水平,探讨二者与冠状动脉扩张(CAD)的关系及冠状动脉病变的危险因素。方法KD患儿45例。其中21例发生CAD,24例无CAD。分别用酶联免疫吸附法(ELISA)测定其血清NP、D-二聚体水平。冠状动脉病变与PLT、单核细胞(M)、肌酸激酶同工酶(CK-MB)、CRP、ESR、心肌肌钙蛋白I(cTnI)、NP、D-二聚体行多元回归分析。结果与恢复期比较,KD患儿急性期PLT、M、CK-MB、CRP、ESR、cTnI显著升高(Pa<0.01)。血清NP升高26例中CAD 18例;NP正常19例中CAD 3例。D-二聚体升高30例中CAD 17例;D-二聚体正常15例中CAD 4例。静脉用免疫球蛋白(IVIG)治疗25例中CAD 7例;未予IVIG治疗20例中CAD 14例。多因素多元回归分析显示NP、D-二聚体水平与冠状动脉病变密切相关。血清NP水平与M数量呈正相关(r=0.49 P<0.01);D-二聚体水平与血小板数量呈正相关(r=0.51 P<0.01)。结论血清NP、D-二聚体在KD急性期升高,可能是反映冠状动脉异常的预测标志,对评价KD预后有重要意义。     

川崎病冠状动脉损害易患基因研究进展

川崎病是一种好发于5岁以下儿童的急性全身性中、小血管炎性综合征,该病的病因和发病机制至今尚未明确.流行病学资料显示川崎病的发病存在明显的种族差异,在亚裔人群中的发病率明显高于非亚裔人群.冠状动脉损害是川崎病最为严重的并发症,可导致缺血性心肌病、心肌梗死甚至猝死,未经治疗的患儿约25％会发生冠状动脉损害,而经过治疗的患儿冠状动脉损害发生率仍为5％.近年来该病已取代风湿热成为发达国家儿童获得性心脏病最常见的病因.目前关于冠状动脉损害的研究是川崎病的研究热点,而遗传因素在川崎病及冠状动脉损害的发生过程中起重要作用.研究显示ITPCK、CASP3、TNF-α、CD40、IL-10、PELI1、GRIN3A、CTLA-4、SNX24、LRP1B等多种基因易患性与川崎病冠状动脉损害密切相关,该文就川崎病患儿冠状动脉损害易患基因研究进展作一综述.     

冠状动脉造影在川崎病冠状动脉损害检查中的作用

目的　通过对疑有冠状动脉损害川崎病 (KD) 1 2例患儿行经皮冠状动脉造影 ,旨在准确了解冠状动脉损害情况 ,以指导临床对KD患儿的后期治疗及管理。方法　在静脉麻醉下采用Sedinger法穿刺右股动脉 ,采用Judkin法将冠状动脉造影管送达冠状动脉口 ,推注非离子造影剂 ,同时行电影摄影记录。结果　接受冠状动脉造影检查 1 2例患儿中 9例冠状动脉异常 ,其中左冠状动脉和 (或 )其分支受累 5例 ,右冠状动受累、左右冠状动脉同时受累各 2例。与超声心动图结果不尽一致。结论　冠状动脉造影可明确显示冠状动脉损害位置、形状、数目及病变程度 ,是一种安全、有效的检查方法。     

过敏性紫癜患儿不同时期血清D-二聚体与病程的关系

目的探讨儿童过敏性紫癜血清D-二聚体数值与病程之间的关系。方法检测诊断且未经治疗的过敏性紫癜单纯型的47例患儿及39例混合型患儿病程初期与发病两周后的血清D-二聚体,并进行对比、判断。结果入院时诊断单纯型和混合型的患儿初期血清D-二聚体对比,差异有显著性;两组患儿发病两周后血清D-二聚体对比,差异无显著性;将病程初期与病程超过两周后两组血清D-二聚体增高患儿的例数分别与最终病程超过3月的例数进行对比,有显著性差异。结论过敏性紫癜患儿初期血清D-二聚体增高不能提示病程迁延;而病程超过两周后血清D-二聚体增高多提示病程迁延。     

Harada评分预测川崎病冠状动脉病变的临床意义

目的 探讨Harada评分对中国人群样本中川崎病（KD）合并冠状动脉病变高危因素判断的临床价值。方法 参照KD诊断标准及Harada评分标准,回顾性分析187例KD息儿发病10d内白细胞（WBC）计数、红细胞压积（HCT）、C反应蛋白（CRP）、发热天数、心电图及超声心动图等各项指标;采用χ^2检验和多元回归分析,分别检验上述因素与KD合并冠脉损害的相关性;比较冠脉损害组与非冠脉损害组Harada评分的差异。结果发病10d内单因素分析发病年龄、WBC计数、HCT、CRP、心电图改变及发热天数与KD合并冠脉损害有密切相关性。多因素分析结果显示,发病10d内CRP、发热天数与KD合并冠脉损害的发生独立相关。发生冠脉损害患儿Harada评分的平均分高于无冠脉损害者。结论 Harada评分可作为川崎病冠状动脉病变的一项预见性指标,也可作为中国人群川崎病患者早期是否应用静脉丙球的筛选指标。     

N端脑利钠肽前体在川崎病急性期的变化及临床价值

目的：监测川崎病( KD)患儿急性期血浆N端脑利钠肽前体( NT-proBNP)变化,研究其与冠状动脉损害( CAL)高危因素的相关性,探讨血NT-proBNP诊断KD及早期预测CAL的临床意义。方法选取2014年11月至2015年11月我院儿科及PICU收治的64例KD患儿为研究对象,典型川崎病(TKD)43例(占67．2%),不完全型川崎病(IKD)21例(占32．8%);选取同期在本院儿科住院伴有发热的呼吸道感染患儿45例作为对照组。64例KD患儿根据冠状动脉有无损害分为冠状动脉损害( CAL)组22例和无冠状动脉损害( nCAL)组42例,测定血浆NT-proBNP水平并行常规检查,KD急性期患儿行心脏超声检查。比较各组患儿血浆NT-proBNP水平的变化,并对NT-proBNP值进行受试者工作特征曲线分析,获得预测CAL的最佳临界值。采用Pearson相关分析,探讨NT-proBNP与其他高危生化指标间的相关性。结果对照组患儿血 NT-proBNP 为(559．7±202．1) ng/L, TKD 组为(982．1±335．4) ng/L,IKD组为(1103．4±357．4) ng/L,三组间差异有统计学意义(F=32．646,P=0．000), TKD组与IKD组比较差异无统计学意义(P=0．127);TKD组、IKD组明显高于对照组(P=0．000)。 nCAL组血NT-proBNP为(914．7±257．6) ng/L;CAL组为(1237．3±428．4) ng/L,与对照组比较三组间差异有统计学意义(F=45．816,P=0．000);nCAL组明显高于CAL组(P=0．000)。 CAL组患儿血谷丙转氨酶高于nCAL组,白蛋白、血钠明显低于nCAL组,差异有统计学意义( P<0．05)。CAL组血NT-proBNP与白细胞计数、CRP、ALT均呈正相关(r=0．251,P=0．003;r=0．410,P=0．002;r=0．465,P=0．001),与白蛋白、血钠水平均呈负相关(r=-0．387,P=0．012;r=-0．346,P=0．018)。以NT-proBNP 925．95 g/L为截断值预测CAL,曲线下面积为0．780,敏感度81．8%,特异度76．2%。结论急性期血浆NT-proBNP水平不仅有助于KD的诊断,而且具有良好的预测CAL的价值。     

川崎病冠状动脉病变危险因素及其预测北大核心CSCD

冠状动脉损害(CAL)、尤其是冠状动脉瘤是川崎病最严重的并发症。为了预测可能发生CAL的风险,尽早采取措施,使CAL发生率和损伤最大程度减小,文章逐一评述了年龄和性别、发热持续时间、C反应蛋白、血浆N-端脑钠肽前体(NT-pro BNP)、外周血白细胞计数、红细胞沉降率、血浆白蛋白水平、静脉注射丙种球蛋白(IVIG)的时间和剂量、难治性川崎病复发等单一临床特征及实验室指标与CAL发生率的关系。还介绍了原田评分、小林评分评价系统评估CAL风险的应用价值。此外,腱糖蛋白C(TN-C)、micro RNAs(mi RNAs)、基因多态性等实验室指标也在文中有所介绍。     

Thrombocytopenia: a complication of Kawasaki disease

Thrombocytopenia was observed in 10 (2.0%) of 486 children with Kawasaki disease. In nine of the ten, the minimal paltelet count of 94000±38000 (SD)/mm³ was seen on day 6.8±2.2 (SD) of illness and the platelet counts were elevated to the normal level in 1–2 weeks. Thrombocytopenia in the nine appeared to be caused via coagulation-mediated platelet consumption, while the remaining child was diagnosed as having idiopathic thrombocytopenic purpura. One of the two who had severe coagulation-mediated thrombocytopenia of less than 50000/mm³ developed coronary aneurysms persisting over 1 year.Abbreviations APTT Activated partial thromboplastin time - ESR Erythrocyte sedimentation rate - FDP Fibrinogen/fibrin degradation products - ITP Idiopathic thrombocytopenic purpura - PT Prothrombin time - SD Standard deviation     

Small bowel obstruction as a complication of Kawasaki disease

A case of small bowel obstruction in an 8-month-old infant with Kawasaki disease is described. At laparotomy a discrete area of jejunal stricture with adhesions was noted. Microscopic examination revealed evidence of small artery thrombosis. Kawasaki disease results in a diffuse vasculitis, which may produce significant abnormalities in multiple organ systems. Serious abdominal complications can occur and should be considered when gastrointestinal symptoms develop in a patient with Kawasaki disease.     

Doppler detection of tricuspid regurgitation following Kawasaki disease

Between June 1984 and April 1985, five patients (4.2%) had tricuspid regurgitation detected on pulsed Doppler echocardiography among 119 patients with Kawasaki disease. The grade of tricuspid regurgitation was moderate in three and mild in two patients; conventional cardiac examinations revealed no evidence of this complication. All patients had associated coronary artery aneurysms except one, but no patients developed subsequent myocardial infarction. The occurrence of tricuspid regurgitation following Kawasaki disease has been considered to be causally related to the carditis, a frequent complication during the acute febrile period of the illness. The present data indicate that tricuspid regurgitation is an additional cardiovascular complication of Kawasaki disease and that Doppler echocardiography is useful in detecting this disorder.     

Kawasaki disease and sensorineural hearing loss: an (un)expected complication

Kawasaki disease (KD) is an acute, self-limiting, idiopathic form of vasculitis. The preventive effect of early therapy on coronary artery aneurysms, the hallmark of the disease, is well established. The spectrum of complication includes not only cardiac involvement but also central nervous system lesions. We report a 4-year-old boy with a clinical presentation suggestive of KD treated with intravenous immunoglobulin and acetylsalicylic acid. Clinical manifestations regressed within 24 hours and cardiac anomalies were not seen. Two weeks later, the parents noticed a sudden absence of response to sound stimuli. Investigations confirmed bilateral severe sensorineural hearing loss for which oral steroid therapy was given. This resulted in an improvement only on the right side, with severe hearing loss persisting on the left. Conclusion: Sensorineural hearing loss is an uncommonly reported complication of KD. Pediatricians should be aware of this potential complication to allow for early intervention.     

Fatal Kawasaki disease with incomplete criteria: Correlation between optical coherence tomography and pathology

Coronary artery aneurysm is a serious complication of Kawasaki disease (KD). A 3‐month‐old infant presented with severe KD 27 days after onset of fever. The patient presented with shock, inferolateral ischemia on electrocardiogram and high troponin. Echocardiography showed severe myocardial dysfunction with diffuse coronary dilation and right coronary artery aneurysm. Arterial Doppler demonstrated thrombosis of aneurysmal axillary and iliac arteries. Withdrawal of support was implemented due to multi‐organ failure. Post‐mortem optical coherence tomography correlated with pathology. The pulmonary artery was normal on OCT and histology. Coronary arteries showed aneurysmal dilatation, with intimal hyperplasia and preserved media on OCT. Pathology confirmed these findings, with destruction of the internal elastic lamina, luminal myofibroblastic proliferation, neovascularization, and partial disappearance of the media. This is the first report of pathologic correlation in KD with OCT at the subacute stage, which adequately identified structural wall changes.     

心率变异性、心率减速力与静脉注射免疫球蛋白无反应川崎病的相关性

目的:探讨川崎病(KD)患儿急性期心率变异性(HRV)、心率减速力(DC)与静脉注射免疫球蛋白(IVIG)无反应KD的关系。方法:纳入2015年8月至2019年5月成都市妇女儿童中心医院收治的临床资料完整的KD患儿679例进行前瞻性队列研究,选择同期进行健康体检儿童150例为健康对照组。根据接受初始IVIG治疗14 d...     

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目的观察尿白三烯(LTs)E4(LTE4)在儿童典型川崎病(KD)中的变化,探讨是否可以将LTs作为KD后的监测指标。方法东南大学附属盐城医院儿科2007年10月至2010年9月收治68例完全性KD患儿,采用竞争性酶联免疫吸附技术检测其在治疗前后尿LTE4水平,55名正常儿童尿LTE4作为对照组,同时对KD急性期患儿行心脏彩超检查,分为有冠状动脉损害组(8例)和无冠状动脉损害组(60例)。结果 KD患儿治疗前尿LTE4均高于正常对照组,差异有统计学意义(P<0.01);恢复期与正常对照组比较,差异无统计学意义(P>0.05);KD合并冠状动脉损害组尿LTE4明显高于无冠状动脉损害组,差异有统计学意义(P<0.01);所有KD患儿治疗前后比较,LTE4均明显降低(P<0.01)。结论 KD尤其是合并冠状动脉损害的患儿LTs水平明显增高,尿LTE4可作为KD后临床监测指标,可以考虑通过早期应用LTs受体拮抗剂干预来达到预防合并冠状动脉病变发生、促进冠状动脉损伤修复的目的。