The Analysis Of The Clinical Features And Prognosis Of Children With Giant Coronary Artery Aneurysms Caused By Kawasaki Disease

PARTⅠThe clinical features of children with giant coronary artery aneurysms caused by Kawasaki disease in acute stage and the analysis of the risk factorObjectiveTo analyze the clinical features of children with GCAA caused by KD in acute stage and investigate the risk factors of GCAA.Methods1. From May 2001 to May 2009, inpatients diagnosed as KD in Children's Hospital Guangzhou Women and Children Medical Care Centre were enrolled into the study. Inpatients diagnosed as KD with CAD or without CAL were excluded, but inpatients with delayed diagnosis as KD with CAA were included. Patients with incomplete information were also excluded.2. Sex,age,clinical manifestations,laboratory examination and treatment of patients were collected.Sex(male,female),fever(≤14d,>14d),delayed diagnosis, WBC[<12, -20,≥20 (×109·L-1)], PLT max[<400, -800,≥800 (×109·L-1)], PLT min[<100,-400,≥400(×109·L-1)],Hb[ < 90,-110,≥110(g·L-1)],ESR[ < 100,≥100 (mm·h-1)], CRP[<3, -50,≥50(mg·L-1)],ALB[<30,≥30 (mg·L-1)], days of using IVIG(≤10d,>10d) and the use of corticosteroid before diagnosis were chosen as risk factors of GCAA to be analyzed. The risk factors of GCAA caused by KD were estimated by Chi-square test and Logistic regression analysis. Results1. According to the inclusion and exclusion criteria,22 patients with GCAA were enrolled into GCAA group.The average age was (2.9±2.8) years(3 months-10 years). 65 patients with small or medium CAA were enrolled into control group. The average age was (1.5±1.2) years(3 months to 6 years).2. There was significant difference in delayed diagnosis(P<0.05), but no difference in other clinical manifestations.The results of Chi-square test showed that the age≤6 months or≥5 years, fever lasting over 14 days, delayed diagnosis, use of corticosteroid before KD was diagnosed, higher ESR, lower Hb and ALB were associated with GCAA significantly(P<0. 05).3. The results of Logistic regression analysis indicated that the odds ratio (OR) of delayed diagnosis was 2.998(95%CI:1.004-8.950,P=0.047), the OR of the usage of corticosteroid before diagnosis was 6.556(95%CI:1.561-28.542,P=0.010), and the OR of ESR≥100 mm·h-1 was 3.591(95% CI: 1.164-11.079, P=0.026).All of them were the independent risk factors of GCAA.Conclusions1. The age≤6 months or≥5 years,long-lasting fever(>14d),delayed diagnosis,use of corticosteroid before diagnosed, higher ESR(≥100 mm·h-1), lower Hb(<90 g·L-1) and lower ALB(<30 g·L-1) were associated significantly with the development of GCAA(P<0.05).2. Delayed diagnosis, the usage of corticosteroid before diagnosis and ESR≥100 mm·h-1 were the independent risk factors for GCAA. PARTⅠThe prognosis of children with giant coronary artery aneurysms caused by Kawasaki diseaseObjectiveTo analyze the prognosis of Kawasaki disease with GCAA.Methods1. From May 2001 to May 2006, inpatients diagnosed as KD with CAL in Children's Hospital Guangzhou Women and Children Medical Care Centre were enrolled into the study, which were divided into CAD group, small or medium CAA group and GCAA group.The follow-up starting point was discharging after acute stage and the ending point was May,2009.2. The patients enrolled were followed-up in 6 month,1 years,2 years,3 years after discharging by echocardiography (Echo); The patient whose coronary artery(CA) didn't return to normal by Echo or whose EKG presented suspect myocardial ischemia in 3 months after acute stage would be underwent ATP stress Echo and coronary angiography. The patient died would be taken autopsy and pathological examinations.Results1. 15 patients with GCAA were enrolled into GCAA group. The average age was (2.7±2. 6) years(3 months-10 years).166 patients with CAD and 59 patients with small or medium CAA were enrolled into control group.2. In the 3 years follow-up time point, in GCAA group 2/15 cases(13.3%)were showed normal coronary artery by Echo,and it was significantly different from CAD group and small or medium CAA group (P<0.01);1/15 case(6.7%) showed dilation,10/15 cases(66.7%) prestened CAA, including 3 cases with GCAA and 3 cases with stenosis or oclussion in CA.3. The rate of abnormalities of wall motion in GCAA group by ATP stress Echo was significant different from the rate in CAD group(χ2=12.3,P<0.01), but there was no difference with small or medium CAA group (χ2=0.4,P>0.05). Parametric test showed that there was significant difference among the rates of abnormalities of wall motion in three groups and the rate of GCAA group was higher(Z score=-4.2,P<0.01).4. In GCAA group, 6/15 cases were underwent coronary angiography. 4/6 cases (66.7%) were still presenting CAA,1/6 case was regressed to CAD.5/6 cases were found stenosis and(or) occlusions ,including 1 cases with coronary recanalization after occlusion and 1 case with branch vessels.5. 2 cases were died in GCAA group. Autopsy findings: LCA and RCA were markedly and continuously beaded dilated; The aneurysms contained dark red thrombus, which almost occluded the entire lumen and mural thickening was also noted. Pathological findings:inflammatory cell infiltrations was shown and intimal was thickening.Conclusions1. The prognosis of children with GCAA caused by KD was not good.The CAL will be lasting for a long time or even casing death.The GCAA will develop to stenosis and occlusions which would lead to ischemic heart disease in late stage.2. It is important and useful to apply the echocardiography combined with ATP stress echocardiography and coronary angiography in the long-term follow-up of KD with GCAA.