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皮肤粘膜损害为首发症状的川崎病1例
患儿,男,4岁,因反复皮疹伴指端肿胀 10 d,发热 3 d 入院.10 d 前无明显诱因出现皮肤红色小丘疹,直径约1~2 mm,高出皮面,压之褪色,以面部、双小腿、前臂和臀部为主,疹间皮肤正常,无融合,伴轻度搔痒.同时双手指端肿胀.进食时感舌痛,口腔粘膜可见数个溃疡.于当地医院按过敏给予"扑尔敏、地塞米松"等后皮疹渐消退,双手指端肿胀减轻,从甲缘开始出现片状脱皮,但一直无发热、心慌、气短等症状.
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88例不完全川崎病的临床特点及随访分析
近年来,不完全川崎病(incomplete Kawasaki disease,iKD)似有增多趋势,由于缺乏诊断iKD的金标准,因此早期诊断很难及时作出.其继发的冠状动脉病变(coronary artery lesion,CAL)是决定病情严重程度及预后的主要因素,并且与成年后冠脉阻塞和动脉硬化有关[1-2],现将我院1984年6月至2009年6月收治的88例iKD临床特点和随访结果作一回顾性分析,旨在提高iKD的诊治水平,减少心血管并发症,改善预后.
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儿童川崎病并冠状动脉瘤冠状动脉造影及心脏超声长期随访
川崎病(Kawasaki disease,KD)基本病理改变为全身性血管炎,主要侵犯大、中血管,其中冠状动脉(简称冠脉)是常受损的血管,冠状动脉瘤(coro-nary artery aneurysm,CAA)和冠状动脉狭窄(简称冠脉狭窄)为严重,可致缺血性心脏病、心肌梗死和猝死.
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川崎病并发冠状动脉病变的临床特点
川崎病(Kawasaki disease,KD)又称皮肤黏膜淋巴结综合征(mucocutaneous lymph node syndrome,MCLS),是一种以全身血管炎为主要病变的疾病,可以导致多系统受累,冠状动脉病变(coronary artery lesion,CAL)是急性期严重的表现,并且与成年后冠脉阻塞和动脉硬化有关~([1.2]).研究发现,性别、发热时间、血白细胞数、血小板、C反应蛋白、血沉和静脉丙种球蛋白(IVIG)初治效果等因素与CAt.的发生有关~([3.4]).但临床上可以经常见到KD患者出现丙氨酸转氨酶(ALT)、天门冬氨酸转氨酶(AST)和乳酸脱氢酶(LDH)升高,这些酶的异常升高与CAI的关系的研究尚不多见.本研究对我科近5年来收治的KD临床特点进行回顾性分析,初步探讨上述指标与CAI的关系,为早期发现CAL和判断KD预后提供依据.
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静脉注射丙种球蛋白无反应型川崎病的诊治进展
川崎病(Kawasaki disease,KD)是一种病因未明的以全身性血管炎为主要病变的儿童急性发热出疹性疾病,大剂量静脉注射丙种球蛋白(intravenous immunoglobulin,IVIG)对改善临床症状、抑制冠状动脉损害(coronary artery lesions,CAL)的发生发展起着重要作用.目前标准化治疗已广泛应用,但仍有10%-15%的病例对IVIG无反应[1],且这部分患儿并发CAL的风险升高[2].因此,早期识别及有效治疗此类无反应型病例显得十分重要.
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不完全川崎病诊疗进展
川崎病(Kawasaki disease,KD)又称为皮肤黏膜淋巴结综合症,是一种病因不明的急性、自限性血管炎综合症,于1967年由日本的川崎首先报道而得名[1,2].随着对川崎病认识的不断深入,不完全川崎病(incomplete kawasaki disease,IKD)临床表现典型,误诊或漏诊的发生率较高,从而导致冠状动脉并发症发生率较高.因此日益受到儿科医生的广泛关注[3,4].在小儿不明原因的发热中要考虑到此病,以免漏诊或误诊.
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不完全川崎病致急性肾衰竭一例
川崎病(Kawasaki disease,KD)又称皮肤黏膜淋巴结综合征,是一种免疫介导的全身性血管炎.KD致肾衰竭罕见,现报道1例不完全川崎病致急性肾衰竭.患儿,男,5岁11月.因发热7 d,阵发性腹痛伴左下肢痛、少尿3 d,于2008年12月12日入院.无传染病史,无家族史,无药物过敏及中毒史.1 d前当地查Scr 544.9μmol/L,BUN 36.4 mmol/L,拟诊急性肾衰竭,转我院.体检:BP 126/83 mm Hg,双肾叩击痛阳性,双下肢轻度水肿,左髋膝关节、左大腿压痛,左髋关节活动受限,余无特殊.
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不完全川崎病诊治进展
川崎病(Kawasaki disease,KD)因日本医生川崎富作1967年首次报道而得名,学者几十年观察、研究,现公认KD系小儿多发的急性、自限性、自身免疫性血管炎综合征.
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川崎病病因及发病机制的研究进展
川崎病(Kawasaki Disease,KD)又称皮肤粘膜淋巴结综合征(mucocutaneous lymphnode syndrome,MCLS),1967年由日本学者川崎富首次报道,是一种急性、自限性的全身性血管炎,好发于5岁以下婴幼儿[1].KD主要累及中小动脉,特别是冠状动脉病变,在发达国家或地区,已取代风湿热成为导致小儿后天性心脏病的首要原因.本文就近年来有关KD病因及发病机制的进展作一综述.
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不完全川崎病的研究进展
川崎病(kawasaki disease, KD)是一种以全身弥漫性血管炎为主要病变的急性发热出疹性疾病,80%发生于5岁以下的儿童[1].1967 年日本 Kawasaki Tomisaku 博士首先描述本病.该病目前已取代风湿热成为小儿后天性心脏病的主要原因[2].
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川崎病诊断标准的变迁
川崎病( Kawasaki disease,KD)是一种主要发生在5岁以下儿童的急性自限性血管炎综合征.它可引起全身多系统受累,尤以冠状动脉为甚,已取代风湿热成为儿童后天性心脏病常见病因[1].KD的病因不明,缺乏特异性诊断方法,诊断主要依靠临床表现,因而正确掌握临床诊断标准对KD的早期诊断有重要意义.现就不同KD诊断标准做一总结及介绍.
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川崎病的静脉注射免疫球蛋白治疗
川崎病(Kawasaki disease,KD)的病因目前尚未完全明了,尽管许多证据表明其发病可能与感染有关,但是尚未被证实.
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川崎病并发冠状动脉瘤2例双源CT表现
病例1男,11岁,诊断川崎病3年余.现心肺体检无异常,实验室检查红细胞及血小板稍偏低,肌钙蛋白正常.心脏彩超示右冠状动脉内径增宽并瘤样扩张,左冠状动脉起始段血栓形成.DSCT冠脉扫描示右冠状动脉距冠窦开口4.2 mm处见条形钙斑.管腔狭窄约40%,RCA距冠窦开口5.8 mm处长约18.7 mm管腔瘤样扩张,宽径7.4 mm,为冠状动脉瘤形成;前降支开口起始处长约8.2 mm管腔内为低密度影,以远管壁毛糙,管腔稍变窄,考虑为起始段血栓形成可能.
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成人川崎病:多发脑动脉和冠状动脉瘤样扩张1例
患者 女,30岁,以"右侧肢体麻木,言语不清15 h"入院.15 h前看电视时突感右侧肢体无力,麻木,言语不清伴有头痛,无意识不清、恶心、呕吐、视物不清等症状,即行头颅SCT扫描,未见明显异常.
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Background: Ongoing low-grade inflammation and endothelial dysfunction persist in children with coronary lesions diagnosed with Kawasaki disease (KD). Statins, frequently used in the management of high cholesterol, have also shown to improve surrogate markers of infl ammation and endothelial dysfunction. This study was undertaken to investigate the effi cacy and safety of pravastatin in children with coronary artery aneurysms due to KD.
Methods: The study enrolled 14 healthy children and 13 male children, aged 2-10 years, with medium-to-giant coronary aneurysms for at least 12 months after the onset of KD. Pravastatin was given orally to the KD group at a dose of 5 mg/day for children under 5 and 10 mg/day for children older than 5 years. To determine the effects of pravastatin on endothelial function, high-frequency ultrasound was performed before the start of the study and 6 months after pravastatin therapy. The parameters measured were brachial artery flow-mediated dilation (FMD), non-flow mediated dilation (NMD), and carotid artery stiffness index (SI). High sensitive C-reactive protein (hs-CRP) levels, the circulating endothelial progenitor cells (EPCs) number, and serum lipid profiles were also determined at baseline and after 6 months of pravastatin treatment.
Results: Before treatment, the KD group had significantly decreased FMD (P<0.05) and increased SI and hs-CRP levels (P<0.05) compared with controls. After 6 months of pravastatin therapy, FMD improved significantly compared to the baseline KD group (3.16±6.49 to 10.05±7.74, P<0.05), but remained significantly less than that in the control group with no signifi cant changes in NMD and SI. There were signifi cant decreases in markers of inflammation after treatment. The hs-CRP levels decreased signifi cantly from 2.93±0.81 mmol/L to 2.14±0.82 mmol/L (P<0.05) and the serum apo-B and apo-B/apo-A1 ratio were also reduced (P<0.05) in the KD group. However, the circulating EPC number was not signifi cantly different between baseline and that following pravastatin treatment in the KD group and the control group (P>0.05). No signifi cant complications were noted with paravastatin therapy.
Conclusions: Pravastatin improves endothelial function and reduces low-grade chronic infl ammation in patients with coronary aneurysms due to KD. Children with coronary aneurysms due to KD may benefit from statin therapy. -
Background: The comparative effectiveness of intravenous immunoglobulin (IVIG) for Kawasaki disease was regarded as inconclusive in the international guidelines. However, several new evidences have been published in recent years.
Data sources: A literature search of PubMed was conducted using key words of "Kawasaki disease or mucocutaneous lymph node syndrome" and "immunoglobulin" in combination. Only original articles published after 2004 were selected. A total of 813 papers were found in PubMed. These papers were screened manually by their titles and abstracts.
Results: Patients treated with IVIG prepared by betapropiolactonation might have worse outcome (a higher non-responsive rate in one report and a higher rate of coronary aneurysm in two reports). Storage of IVIG in acidic solution might be correlated with a higher rate of coronary aneurysm (two reports).
Conclusions: Different processes of preparation and conditions of preservation of IVIG may have profound effects on its clinical effectiveness. Randomized controlled studies are needed to further elucidate this issue. -
Nursing care of25children with Kawasaki disease
Objective The aim of this study is to estimate the incidence and describe the diagnosis,treatment and nursing care of Kawasaki Disease among children in Guilin city,Guangxi Province,China.The study is also designed to assess clinical aspects of retrospection,management and outcome,specifically in relation to cardiac disease.Methods To retrospectively collected the clinical data of pediatric patients with Kawasaki's disease at the Affiliated Hospital of Guilin Medical University in China,which covered a two-year period from 2015 to 2016.Results During the two-year study period,a total of 25 cases of Kawasaki disease were reported in the hospitals,comprising of I 1 boys and 14 girls (male-female ratio was 1:1.27).The seasonal distribution showed a slightly higher incidence in spring and summer.The patients' mean age of onset was 2.78 years old.The average hospitalization days of patients were 8.08 (range from 5 to18 days).16 Kawasaki patients were diagnosed as bronchial pneumonia.Coronary arterial abnormalities were detected during follow up by UCG in 5 cases,but no coronary artery aneurysm was found.All 25 patients recovered and were discharged from the hospital.Conclusion Early treatment and proper nursing management are key issues to prevent the occurrence of coronary artery aneurysm in Kawasaki Disease patients.