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Kawasaki disease, also known as mucous skin lymph node syndrome, is a systemic vascular inflammatory disease mainly occurring in children under the age of 5 years, which has become the main cause of the acquired heart disease in children.The etiology and pathogenesis of Kawasaki disease have not been clearly defined since the initial report of Kawasaki disease in 1967.In recent years, the epidemiology of Kawasaki disease has highly suggested that the pathogenesis of Kawasaki disease is related to infection.It is reported that there are dozens of pathogens known to be associated with Kawasaki disease, including bacteria, viruses, mycoplasma pneumoniae, chlamydia, etc.This article reviews the pathogens related to Kawasaki disease reported in recent years, aiming to introduce the pathogens that cause the Kawasaki disease and its association with coronary artery lesions(CALs), the main complication of Kawasaki disease, so as to provide clinical guidance for the research of Kawasaki disease pathogenesis.
ABSTRACT
Kawasaki disease(KD) is a childhood acute self-limiting systemic vasculitis.It is an autoimmune disease.It has replaced rheumatic fever as the most common disease of children with acquired heart disease.Further study of the pathogenesis of KD,help to improve the individualized treatment and prognosis of the disease.The current study on KD,corfirmed its susceptibility to disease and clinical symptoms associated with multiple gene loci,there may be multiple signal paths involved.This paper reviews the recent advances in the genetic and immunological mechanisms of KD.
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Objective To explore the clinical manifestations, electroencephalographic characteristics and therapeutic effect of drugs in children with Jeavons syndrome. Methods The clinical and electroencephalographic characteristics and thera-peutic effect of drugs were analyzed in 4 children with Jeavons syndrome. Results Among the four children there were 3 female and 1 male. The age at the onset of the disease was from 1 to 6 years. The typical clinical manifestations of this disease were brief, fast and repeated eyelid myoclonia (EM) with or without absence seizure. The typical electroencephalography (EEG) in two patients showed 3-6 Hz generalized spike and waves and polyspikes burst, and the eye closure and intermittent photic stimu-lation helped to induce discharges and clinical events. The typictal EEG in the other two patients showed 3.0-3.5 Hz generalizedδslow wave rhythm burst. The drugs of choice for treatment was sodium valproate monotherapy in two cases, levetiracetam in one case, sodium valproate combined with levetiracetam in one case. During the follow-up, seizures were controlled in one case, decreased in frequency in two cases and were still frequent in one case. Conclusions Jeavons syndrome is one of the idiopathic and generalized epileptic syndromes and characterized by EM with or without absence seizure. Video EEG monitoring plays an important role in the diagnosis of this disease. Sodium valproate and levetiracetam were effective for this disease.
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Objective To report the clinical experience of extracorporeal membrane oxygenation (ECMO)in the emergency management of fulminant myocarditis.Methods The patient,an 11 year-old boy,had fever for 4 day and abdominal pain,vomiting for 1 day and anuria for 12 hours.Electrocardiogram showed Ⅲ atrial-ventricular block,multifocal ventricular rhythm,bundle branch block,convulsivum multifocal ventricular tachycardia,extensive low voltage,ST-T elevation at lead Ⅰ,AVR,V1,V2,V3;and there were ST-T intrusion,T wave inversion at lead Ⅱ,Ⅲ,AVR,AVF,V4～5.Blood creatine kinase was 2 161 U/L,reatine kinase isoenzyme 109 U/L,α-hydroxybutyrate dehydrogenase 612 U/L,lactate dehydrogenase 696 U/L,troponin Ⅰ 22.1 U/L.Echocardiogram showed right atrium 4.4 mm,right ventricular 2.3 mm,severe tricuspid valve regurgitation,left ejection fraction 33％,left ventricular fractional shortening 15％,ventricular wall motion dyssynchrony.Blood lactate was 4.0 mmol/L.The patient's condition was still unstable after using dobutamin,dopamine,milrinone,furosemide,large dose methylprednisolone,intravenous human immunoglobin,phosphocreatine and so on.ECMO was used for cardio-pulmonary support.It is necessary to monitoring the consciousness,temperature,heart rate,respiration,blood pression,SaO2,urinary volume,ariterial blood gas,blood electrolytes,blood lactate,blood glucose,liver function,renal function,blood routine,activated clotting time(ACT),lower extremity blood supply and so on.ACT was maintained at 160～200 s.Heparin was used persistently[5～10 U/(kg·min)].Results ECMO system had been successfully used for 7 days.The cardiac function of the patient was improved significantly.There was no complication,such as hemorrhage,infection,and embolism.Heart arrest in the patient occurred three times,ventricular fibrillation and ventricular flutter occurred one time respectively during ECMO.The rhythm was recovered by electric defibrillation and antiarrhythmic drugs.On day 20,the patient was discharged.At the time of hospital discharge,the patient demonstrated good activity,with normal myocardial enzymes.The echocardiogram showed the size of the cardiac chambers and the contractile function of the myocardia were normal.Electrocardiogram showed Ⅰ degree atrial-ventricular block,complete right bundle branch block.Two weeks later,the electrocardiogram demonstrated complete right bundle branch block.Echocardiogram showed septal thickening(0.9 cm).Two months later,the electrocardiogram was just as that of two weeks before.Echocardiogram showed septal thickening(0.7 cm).The children had no symptom after he was discharged and acted without limitation.Conclusion ECMO is a kind of effective treatment for fulminant myocarditis.The key to desirable therapeutic effect is the timing of its application.
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Objective To evaluate the efficacy and safety of oral erythromycin in the prevention and treatment of feeding intolerance in preterm infants. Methods The Cochrane Library,PubMed,EMBASE,CBMdise,VIP,WartFang and CNKl were searched up to the year of 2008.Randomized controlled trials (RCT) of erythmmycin for feeding intolerance in preterm infants were included.According to the dosage (low-dose,3-15 mg/kg and high dose,>15 mg/kg)and gestational age(≤32 weeks vs>32 weeks),all infants were divided into several subgroups.Meta-analysis was performed with the Cochrane Collaboration's software RevMan. Results Nine RCrs involving 542 premature infants were included.(1)In preventive studies,low-dose erythromycin could significantly decrease the duration of total parenteral nutrition compared with the high-dose erythmmycin (WMD=-2.99,95%CI:-3.99--1.98).(2)Intreatment studies,highdose erythromycin could significantly decrease the duration of total parenteral nutrition (WMD=-7.06.95%CI:-7.91--6.20,P<0.01)and hospital stay (WMD=-8.10,95%CI:-14.02--2.18,P=0.007)compared with the placebo when gestational age≤32 weeks. Erythromydn could decrease the incidence of iaundice(RR=0.36,95%CI:0.21-0.63,P=0.0003)which might be the effect of bigh-dose erytromycin. Conclusions Compared with placebo,oral erythromycin of different dosage might help to achieve full enteral feeding,shorten the duration of parenteral nutrition and hospital stay,and reduce the associated cholestasis jaundice of preterm infants with different gestational age respectively.
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Objective To explore the relationship between the clinical characteristics and laboratory examinations.Methods Retrospective analysis was performed on the clinical data in 172 patients diagnosed as infectious mononucleosis(IM)in our department recently.Results The onset rate was high from 3 to7 years old.The main clinical presentations included fever,neck lymphadenopathy,pharyngitis,hepatomegaly,and splenomegaly.Some patients accompanied with obvious sonorous.The occurring rate of the complications was 74.4%,especially deteriorated liver function was the most common,next came to the lung infection.The proportion of atypical lymphocytes raised in 73.3% cases.The positive rate of EBV-VCA-IGM was 65.1%.Myocardium ferment chart went up in 27.9% cases,Erythrocyte sedimentation rate increased fast in 61.6%,CRP gently got higher in 62.8%.However,the ratio of atypical lymphocyte and EBV-VCA-IGM positive or negative had little to do with the pathologic condition and complications in IM.Conclusions The clinical progress of most IM is benign and multiple organs can be involved with atypical clinical manifestations.Examination for atypical lymphocytes is an easy and effective means to screen IM.