Objective:To summarize the clinical features, middle-and long-term prognosis of Kawasaki disease (KD) with giant coronary artery aneurysm (GCAA).Methods:In this retrospective cohort study, a cross-sectional analysis was conducted on 101 KD children with GCAA in the KD with GCAA database established by Beijing Children′s Hospital, Capital Medical University in 2004. GCAA was diagnosed as coronary artery absolute lumen diameter ≥8.0 mm. All patients were followed up regularly. The endpoint was the time of last follow-up or the death time. T test or χ 2 test was used for comparison between groups. Results:A total of 101 KD children with GCAA were enrolled, including 82 males (81.2%) and 19 females (18.8%). The age of disease onset was 2.5 (1.0, 4.5) years. The follow-up duration was 4.5 (2.7, 7.5) years, with a longest of 19 years. All children received routine treatment with aspirin and warfarin, and clopidogrel was added in severe cases. At the end of follow-up, 13 cases (12.9%) had cardiac enlargement, 11 cases (10.9%) developed heart failure, 13 cases (12.9%) experienced myocardial infarction, 2 cases (2.0%) underwent coronary artery bypass graft and 6 cases (5.9%) died. A total of 170 coronary arteries were involved, including 24 (14.1%) GCAAs on the main trunk of left coronary artery, 10 (5.9%) GCAAs on left circumflex, 57 (33.5%) GCAAs on left anterior descending, 78 (45.9%) GCAAs on the middle segments of right coronary artery, and 1 (0.6%) GCAA in the distal segments of right coronary artery. Eleven cases (10.9%) recovered with the coronary artery absolute lumen diameter of all GCAAs below 4.0 mm. Among 170 branches with GCAAs, 28 (16.5%) regressed below 4.0 mm. No significant difference was found in the regression rates between right and left GCAA (18.7% (17/91) vs. 13.9% (11/79), χ2=2.473, P=0.116). There was no statistically significant difference in retraction between unilateral GCAA and bilateral GCAA (16.1% (9/56) vs. 4.4% (2/45), χ 2=2.381, P=0.123). Conclusions:GCAA of KD occurred more common in the middle segments of right and left anterior descending coronary arteries. The incidence of adverse cardiac events and the mortality rate in children with GCAA complicated with KD was high. Their long-term prognosis was poor.

The incidence of incomplete Kawasaki disease(iKD) has bean increasing year by year.Significant individual differences in manifestations, insidious onset, and lack of specific symptoms and effective diagnostic biomarkers often result in delayed diagnosis or misdiagnosis, which makes iKD children at higher risk of cardiovascular complications such as coronary artery dilation or coronary aneurysm and even secondary thrombosis or sudden death.The purpose of this review was to introduce the diagnosis and treatment of iKD, and thus provide evidence for clinicians.

Cardiovascular disease(cardiomyopathy, myocardial infarction, heart failure)seriously endanger child health.Although the incidence in children is not very high, the prognosis is usually poor due to limited treatment options.With the rapid development of stem cell technology and regenerative medicine in recent years, stem cell transplantation has been proved to be able to promote cardiomyocyte regeneration, improve myocardial remodeling and left ventricular ejection fraction(LVEF), so as to alleviate cardiovascular diseases fundamentally.Mesenchymal stem cells(MSCs), a kind of pluripotent adult stem cell with profound self-renewal capacity and multi-directional differentiation potential, have been gradually applied in the treatment of children′s cardiovascular diseases, such as cardiomyopathy, myocardial infarction and end-stage heart failure, and initial success has now been achieved.This review is to summarize the advance of MSCs in the treatment of cardiovascular diseases in children.

Objective To summarize the echocardiographic findings and clinical characteristics of Kawasaki disease(KD) complicated with coronary artery thrombosis (CAT).Methods Thirty-five patients with KD combined with CAT were enrolled,who were admitted to Beijing Children's Hospital,Capital Medical University between July 2005 and August 2016.The clinical characteristics and echocardiographic findings during follow-ups were retrospectively studied.According to whether the childrenhad been complicated with myocardial ischemia,the patients were divided into 2 groups:ischemic group and non-ischemic group.The duration of fever,the time when the intravenous immunoglobulin(IVIG) was first injected,the time when coronary artery aneurysms (CAA) was formed,the maximum diameter of CAA and inflammatory index inthe acute phase were compared between 2 groups.Results All of the 35 children diagnosed as KD combined with CAT suffered firom CAA,and the coronary thrombosis was detected in all the cases with aneurysms.Thirty-five patients had 99 branches of CAA,of which the maximum diameter of CAA was (9.6 ± 3.1) mm(4.0-19.0 mm).Fifty-four plots of CAT were detected in the aneurysms.The diameter of CAA that thrombosis located was larger than that of which the thrombosis was not located[(10.9 ± 2.8) mm vs.(7.9 ± 2.6) mm],and the difference was significant(P ＜0.01).During 4 months to 10 years and 8 months [(39.2 ±29.5) months] follow-ups,CAA regressed in 32 branches [32.3％ (32/99 branches)],of which 4 branches [4.0％ (4/99 branches)] completely regressed to the normal diameter.The maximum diameter of CAA regressed was smaller than the maximum diameter of CAA consistence [(7.3 ± 1.9) mm vs.(10.6 ± 3.0) mm],and the difference was significant (P ＜ 0.01).Out of 35 patients,15 cases [42.9％ (15/35 cases) had myocardial ischemia,while the other 20 cases[57.2％ (20/35 cases)] didn't have.Among 15 cases with myocardial ischemia,6 cases[17.1％ (6/35 cases)] had myocardial infarction,4 cases [11.4％ (4/35 cases)] had heart failure,and 1 case[2.9％ (1/35 cases)] died of acute heart failure complicated with severe ventricular arrhythmia.Compared with non-ischemic group,the children in the ischemic group had longer duration of fever[(19.1 ± 7.8) d vs.(12.1 ± 3.3) d],higher white blood cell account in the acute phase[(24.8 ± 13.5) × 1012/L vs.(19.7 ±4.0) × 1012/L],later treatment of IVIG [(13.9 ± 5.5) d vs.(9.8 ±3.8) d],and earlier CAA formation [(16.0 ±4.9) d vs.(20.9 ± 14.5) d],and the differences were statistically significant (all P ＜ 0.05).Conclusions CAT of children with KD commonly originates from CAA.Patients who have more serious inflammatory reaction in the acute phase,earlier formation,heavy severity and longer consistence of CAA are prone to have myocardial ischemia.Echocardiographic study plays an important role in monitoring CAA,detecting the CAT and finding the early left ventricle dysfunction,which is of clinical significance.

Endothelial Cells ; pathology ; Endothelium, Vascular ; cytology ; pathology ; Humans ; Mucocutaneous Lymph Node Syndrome ; pathology

Objective To determine the risk factors for coronary artery lesions (CALs) in patients with Kawasaki disease (KD).Methods The clinical records of 2331 patients with KD from January 2005 to December 2014 in Beijing Children's Hospital,Capital Medical University were retrospectively analyzed.The relationship between the following factors and CALs was analyzed by univariate and multivariable Logistic regression analysis:age,gender,incomplete KD,total fever duration,intravenous immunoglobulin(IVIG) treatment resistance,white blood cell count,C-reactive protein (CRP),platelet count,sodium and albumin.Results The incidence of CALs was 36.0％(840/2331 cases).Univariate Logistic regression analysis indicated that male patients,incomplete KD,total fever duration ≥10days,IVIG treatment resistance,CRP＞100mg/L,platelet count＞300×109/L and albumin＜35 g/L were associated with CALs (all P＜0.05).Multivariable Logistic regression analysis identified that male patients (OR=1.698,95％ CI 1.383-2.084,P＜0.001),incomplete KD (OR=2.730,95％ CI 2.121-3.515,P＜0.001),total fever duration ≥10 days (OR=2.556,95％ CI 1.975-3.307,P＜0.001),CRP＞100 mg/L (OR=1.556,95％ CI 1.274-1.900,P＜0.001) and albumin＜35 g/L (OR=1.665,95％ CI 1.323-2.096,P＜0.001) were the independent risk factors for CALs.Conclusions The main damage in patients with KD is CALs.The male children with KD,incomplete KD,total fever duration ≥10 days,CRP＞100 mg/L and albumin＜35 g/L were prone to CALs.

Objective To study thegenetic and molecula repid emiological characteristics of ECHO29 virusVP1 region coding in Tibet under 5-year-old children in 1999-2000.Method 7 strains of ECHO29 viruses isolated from 659 stool specimens of AFP cases and under 5-year-old patients and some healthy children in Tibet in 1999-2002 were used to extract ribonucleic acid (RNA) while VP1 coding area were amplified by reverse transcription-polymerase chain reaction (RT-PCR) and the nucleotide sequence of PCR products were determined and analyzed.Result A total of 7 strains ECHO29 viruses were isolated from the stool specimens and identified.5 strains were isolated from specimens in 1999 while the other 2 strains isolated from samples in 2000.The complete nucleotides sequence of VP1 region of 7 strains ECHO29 viruses were 876nt coding 292 amino acids.The sequence homology of 7 strains detected ECHO29 viruses was between 75.41％-99.3％ while amino acid homology was between 71.26％-79.69％.The phylogenetic tree of ECHO29 viruses showed 4 genotypes which the isolates from Tibet were belong to Genotype A and C.Conclusion It was first reported that the popular ECHO29 viruses in children in Tibet were proposed to 2 different genotypes in 1999 and 2000.The popular ECHO29 virus epidemic in 1999 was belong to Genotype C,while popular ECHO29 virus in 2000 belong to Genotype A.ECHO29 virus increasingly popular was not found in 2001 and 2002.

BACKGROUNDCoronary artery damage from Kawasaki disease (KD) is closely linked to the dysfunction of the endothelial progenitor cells (EPCs). The aim of the present study was to evaluate the modulatory effect of granulocyte colony-stimulating factor (G-CSF) on EPCs and elastin breakdown of coronary arteries in a KD mouse model.

METHODSA Lactobacillus casei cell wall extract (LCWE)-induced KD model was established in C57BL/6 mice that were subsequently administrated with recombinant human G-CSF (rhG-CSF). Nω-nitro-L-arginine methyl ester (L-NAME) was administrated for the negative intervention. Evaluations included coronary artery lesions, EPC number and functions, and the plasma concentration of nitric oxide (NO).

RESULTSElastin breakdown was found in the coronary arteries of model mice 56 days after injection of LCWE. The number of circulating EPCs, plasma concentration of NO, and functions of bone marrow EPCs, including proliferation, adhesion, and migration abilities, were all lower in the KD model group compared with those in the control group. After administration of rhG-CSF, the number of circulating EPCs and plasma concentration of NO were increased significantly compared with those in the KD model group. There were also increases in the functional indexes of EPCs. Furthermore, rhG-CSF administration improved the elastin breakdown effectively. However, these protective effects of rhG-CSF on coronary arteries were attenuated by L-NAME.

CONCLUSIONThe present study indicated that the administration of G-CSF prevents elastin breakdown of the coronary arteries by enhancing the number and functions of EPCs via the NO system, and then accelerates the repair of coronary artery lesions in the KD.

Animals ; Coronary Vessels ; cytology ; drug effects ; metabolism ; Disease Models, Animal ; Elastin ; metabolism ; Endothelial Progenitor Cells ; cytology ; Granulocyte Colony-Stimulating Factor ; therapeutic use ; Male ; Mice ; Mice, Inbred C57BL ; Mucocutaneous Lymph Node Syndrome ; blood ; drug therapy ; metabolism ; NG-Nitroarginine Methyl Ester ; pharmacology ; Nitrogen Oxides ; blood

Background Coronary artery damage from Kawasaki disease (KD) is closely linked to the dysfunction of the endothelial progenitor cells (EPCs).The aim of the present study was to evaluate the modulatory effect of granulocyte colony stimulating factor (G-CSF) on EPCs and elastin breakdown of coronary arteries in a KD mouse model.Methods A Lactobacillus casei cell wall extract (LCWE)-induced KD model was established in C57BL/6 mice that were subsequently administrated with recombinant human G-CSF (rhG-CSF).Nω-nitro-L-arginine methyl ester (L-NAME) was administrated for the negative intervention.Evaluations included coronary artery lesions,EPC number and functions,and the plasma concentration of nitric oxide (NO).Results Elastin breakdown was found in the coronary arteries of model mice 56 days after injection of LCWE.The number of circulating EPCs,plasma concentration of NO,and functions of bone marrow EPCs,including proliferation,adhesion,and migration abilities,were all lower in the KD model group compared with those in the control group.After administration of rhG-CSF,the number of circulating EPCs and plasma concentration of NO were increased significantly compared with those in the KD model group.There were also increases in the functional indexes of EPCs.Furthermore,rhG-CSF administration improved the elastin breakdown effectively.However,these protective effects of rhG-CSF on coronary arteries were attenuated by L-NAME.Conclusion The present study indicated that the administration of G-CSF prevents elastin breakdown of the coronary arteries by enhancing the number and functions of EPCs via the NO system,and then accelerates the repair of coronary artery lesions in the KD.