多层螺旋CT成像技术在川崎病冠状动脉病变诊断中的应用价值

目的 探讨多层螺旋CT冠状动脉成像技术在观察川崎病冠状动脉病变中的应用价值。方法 采用多层螺旋CT冠状动脉成像技术（MSCT）和二维超声心动图方法（TDE）检查34例川崎病患儿的冠状动脉,分析对比两种检查方法的价值。结果 MSCT显示冠状动脉损伤12例,累及冠状动脉16支,其中左冠状动脉扩张9支（26％）,狭窄、钙化和狭窄合并钙化各1支,右冠状动脉扩张4支（12％）。TDE显示冠状动脉扩张10例,其中左冠状动脉7支（21％）,右冠状动脉6支（18％）。MSCT显示3例狭窄和钙化,2例左前降支扩张的病变,TDE显示阴性结果。两种方法探察冠状动脉扩张的阳性率比较,差异无统计学意义;探察冠状动脉狭窄和钙化,差异有统计学意义,χ^2=24．3（P〈0．01）。两种方法测量左右冠状动脉内径有很好的相关性,相关系数分别为r=0．90,r=0．82（均P〈0．01）。结论 利用MSCT能观察冠状动脉的全貌,特别是冠状动脉的中远段,在诊断钙化和狭窄方面具有独到之处,弥补了二维超声的不足,可部分取代传统的冠状动脉造影,是一种有价值的川崎病追踪随访的检查手段。     

负荷多普勒超声技术评价川崎病患儿左心功能的临床研究

目的 探讨双嘧达莫负荷多普勒超声技术在川崎病患儿左心储备功能评价方面的临床应用价值.方法 选择超声检查均提示有冠状动脉增宽的川崎病患儿21例,根据99Tcm-甲氧基异丁基异腈心肌断层扫描检查结果 将患儿分为2组,核素分布稀疏组(A组)14例,核素分布正常组(N组)7例.分别于双嘧达莫负荷前后测量左室射血分数(LVEF)、心脏指数(CI)、射血前时间/左室射血时间(PEP/LVET)、短轴缩短率(FS)、二尖瓣环处舒张早期最大峰值流速(Emax)及舒张晚期最大峰值流速(Amax).结果 负荷试验前,A组与N组各项指标比较差异无显著性(P>0.05).负荷后,A组CI明显降低(P<0.05);PEP/LVET明显增高(P<0.05).负荷后1?h时,A组与N组CI和PEP/LVET恢复到负荷前水平.结论 心肌断层扫描核素分布异常的川崎病患儿左室收缩储备能力下降.双嘧达莫负荷超声心动图技术操作简便、安全,可连续性评价患儿的心功能,便于随访.     

High incidence of angiotensin I converting enzyme genotype II in Kawasaki disease patients with coronary aneurysm

A 287 base pair insertion/deletion polymorphism in intron 16 of the angiotensin I converting enzyme (ACE) gene was examined by polymerase chain reaction in 36 Kawasaki disease patients (16 without coronary aneurysm, 20 with coronary aneurysm). A polymorphism in the ACE gene was characterized by three genotypes: two D alleles (genotype DD), two I alleles (genotype II), and heterozygous allele (genotype DI). Genotype II was found in 65% of the patients with aneurysm and 12.5% of those without aneurysm (P < 0.01, odds ratio 13.0, 95% confidence intervals). Conclusion Patients with Kawasaki disease and coronary aneurysm more often than expected had the genotype II suggesting that reactions induced by the type of ACE polymorphism predispose to coronary aneurysm formation. Received: 15 March 1996 / Accepted: 10 August 1996     

超声心动图和64层螺旋CT冠状动脉成像在川崎病冠状动脉瘤随访中的应用价值

目的探讨超声心动图和64层螺旋CT冠状动脉成像（64SCTCA）在川崎病（KD）冠状动脉瘤临床诊断和随访中的应用价值。方法选择2006年12 月至2007年5月在首都医科大学附属北京儿童医院门诊随访的KD并发冠状动脉瘤患儿作为研究对象。患儿观察终点随访时依据病变程度除行超声 心动图检查外,同时行64SCTCA检查,对各项检查结果进行详细描述,并分析其应用价值。结果研究期间纳入15例患儿,急性期KD超声心动图检 查显示急性期累及冠状动脉48/60支（80％）。患儿观察终点随访9个月至8.6年,平均（2.8±2.0）年,超声心动图示14/48支(29.2％)冠状动 脉瘤或扩张消退,其余进一步扩张或无明显变化,累及冠状动脉34/60支（56.7%）,同时检测出2处血栓,未发现狭窄及钙化。64SCTCA示累及 冠状动脉30/60支（50％）,主要累及右冠状动脉和左前降支,分布部位与超声心动图检查基本一致,64SCTCA与超声心动图检查对冠状动脉瘤 最大内径的测量值具有较好的相关性（r =0.837,P<0.001）,64SCTCA发现4处血栓、5处钙化及3处狭窄。结论64SCTCA和超声心动图在KD并发 冠状动脉瘤患儿的随访中具有各自的应用价值,两种方法的联合应用可增加冠状动脉病变的检出率。     

The experience with 113 patients with Kawasaki disease in Fars Province, Iran

This study was conducted to determine the epidemiology, clinical manifestations, cardiac involvements and laboratory findings of Kawasaki disease (KD) in Fars Province, Iran from January 1991 to December 2002. One hundred and thirteen patients with KD were confirmed. This syndrome occurred mostly in males (ratio=2.1:1). The most commonly affected age group was one to five years old. There was no secondary case in families, none of the patients were related and recurrence of disease was seen in only one patient. Prolonged fever and thrombocytosis were significant risk factors for developing coronary artery disease (p<0.05, respectively). Among the 10 patients with cardiac involvement, seven patients had dilated coronary arteries (>4 mm) and three patients had aneurysm of coronary artery in their first echocardiography. It has been suggested that a high index of clinical suspicion of KD could improve diagnosis and implementation of preventive treatment.     

Assessment of the Ability of Myocardial Contrast Echocardiography with Harmonic Power Doppler Imaging to Identify Perfusion Abnormalities in Patients with Kawasaki Disease at Rest and During Dipyridamole Stress

The aim of our study was to assess the ability of myocardial contrast echocardiography (MCE) with harmonic power Doppler imaging (HPDI) to identify perfusion abnormalities in patients with Kawasaki disease at rest and during pharmacological stress imaging with dipyridamole. Results were compared with those of 99mTc-tetrofosmin single-photon emission computed tomography (SPECT) imaging as the clinical reference standard. MCE with HPDI was performed on 20 patients with a history of Kawasaki disease. Images were obtained at baseline and during dipyridamole infusion (0.56 mg x kg(-1)) in the apical two- and four-chamber views. Myocardial opacification suitable for the analysis was obtained in all patients. Nine patients with stenotic lesions had a reversible defect after dipyridamole infusion detected by both MCE with HPDI and SPECT, and 3 patients with a history of myocardial infarction had a partially or completely irreversible defect detected by both methods. Three patients with coronary aneurysm without stenotic lesion, 4 patients with regressed coronary aneurysm, and 2 patients with normal coronary artery in acute phase also had normal perfusion at rest and after pharmacological stress by both methods. A 96% concordance (kappa = 0.87) was obtained when comparing the respective segmental perfusion scores using the two methods at baseline, and an 86% concordance (kappa = 0.81) was obtained at postdipyridamole infusion. After combining baseline and postdipyridamole images, each segment was labeled as having normal perfusion, irreversible defects, or reversible defects. Using these classifications, concordance for the two methods was 92% (kappa = 0.87). MCE with HPDI is a safe and feasible method by which to detect asymptomatic ischemia due to severe stenotic lesion, and it may be an important addition to the modalities used to identify patients at risk for myocardial infarction as a complication of Kawasaki disease.     

Impaired endothelial function in the brachial artery after Kawasaki disease and the effects of intravenous administration of vitamin C

BACKGROUND: Previous studies in patients with a history of Kawasaki disease have focused on vascular endothelial function in coronary arteries, and the endothelial function of systemic arteries is not fully understood. Furthermore the effect of vitamin C on systemic endothelial function after Kawasaki disease has not been elucidated. OBJECTIVES: We attempted to analyze endothelium-dependent vasodilatation in the brachial artery after Kawasaki disease by using high resolution ultrasonography and to investigate whether the acute administration of vitamin C could restore such systemic endothelial dysfunction. METHODS: We compared 39 patients (7.1 +/- 2.7 years) 1.0 to 9.6 years after acute Kawasaki disease with 17 matched healthy subjects (7.0 +/- 3.1 years) as controls. Using high resolution vascular ultrasound, we measured brachial artery responses to reactive hyperemia (with increased flow causing endothelium-dependent dilatation) and sublingual nitroglycerin (causing endothelium-independent dilatation). RESULTS: The percent change in diameter of the brachial artery induced by reactive hyperemia in the patients with a history of Kawasaki disease (6.2 +/- 3.9%) was significantly lower than that in the control group (14.1 +/- 6.8%; P < 0.0001). No significant difference could be found in percent change in diameter induced by sublingual administration of nitroglycerin between the control (33.2 +/- 13.7%) and the patients with a history of Kawasaki disease (30.6 +/- 9.2%; P = 0.49). There was no significant difference in percent change in diameter of the brachial artery induced by reactive hyperemia between the patients who received gamma-globulin (6.0 +/- 4.0%) and those who did not receive gamma-globulin (7.9 +/- 3.3%; P = 0.33). Intravenous infusion of vitamin C significantly increased the percent change in diameter of brachial artery induced by reactive hyperemia in 19 patients with history of Kawasaki disease (6.6 +/- 3.5 to 13.0 +/- 5.5%; P < 0.0001), whereas no significant increase was seen in the percent change in diameter of brachial artery induced by reactive hyperemia in 20 patients with history of Kawasaki disease after placebo administration (6.5 +/- 4.5 to 7.3 +/- 4.9%; P = 0.20). CONCLUSIONS: Our study showed decreased percent change in diameter of the brachial artery induced by reactive hyperemia in patients with history of Kawasaki disease compared with the healthy children, indicating that systemic endothelial dysfunction exits after Kawasaki disease. Although such systemic endothelial dysfunction after Kawasaki disease is not influenced by early treatment with high dose gamma-globulin in the acute stage of Kawasaki disease, it can be restored by the acute intravenous administration of vitamin C.     

Evaluation of Preclinical Atherosclerosis by Flow-Mediated Dilatation of the Brachial Artery and Carotid Artery Analysis in Patients with a History of Kawasaki Disease

Cardiac sequelae of Kawasaki disease are an important cause of ischemic heart disease in young adults. The possibility of early progression of atherosclerosis following Kawasaki disease is therefore of great concern. We examined whether preclinical atherosclerotic changes are seen in patients with a history of Kawasaki disease, and whether these changes appear in all or in only a proportion of patients. Sixty-five patients with a history of Kawasaki disease, aged 13.1 ± 2.1 years, and 20 aged-matched controls participated in the study. All subjects underwent flow-mediated dilatation (FMD) of the brachial artery and analysis of carotid artery size and pulse-wave transmission. Patients were classified into four groups depending on the severity of the maximum coronary artery lesion: group 0 (normal), group 1 (mild), group 2 (moderate), and group 3 (severe). There was no statistical difference in the carotid artery analyses between the four groups. FMD (mean ± SD) was significantly lower in groups 2 and 3 than in groups 0 and 1 and the control group (group 0, 19.4 ± 3.9%; group 1, 19.5 ±4.1%; group 2, 8.9 ± 2.8%; group 3, 4.2 ± 1.5%; control group, 18.8 ± 2.8%; p < 0.0001). There was a significantly negative correlation between the severity of the coronary artery lesion and FMD (p < 0.0001 for both). Endothelial dysfunction was revealed by FMD in patients with persistent coronary artery lesions subsequent to Kawasaki disease. Preclinical atherosclerosis may be present only in patients with coronary aneurysms.     

Acute Coronary Artery Dilation Due to Kawasaki Disease and Subsequent Late Calcification as Detected by Electron Beam Computed Tomography

We wanted to clarify the relationships between the degree of acute coronary artery dilation caused by Kawasaki disease and subsequent late calcification. Electron beam computed tomography (EBCT) was used to study 79 patients who had previously undergone selective coronary angiograms less than 100 days after the onset of Kawasaki disease. The EBCT was performed using an Imatron C-150 with a 100-ms exposure time and consecutive images at 6-mm intervals. The interval from the onset of Kawasaki disease to EBCT ranged from 2 to 242 months (median, 103 months). The maximum diameters of the right coronary, the left anterior descending, and the left circumflex arteries, as well as the bifurcation of the left coronary artery were measured in the initial coronary angiograms. A total of 250 branches, including 53 left coronary arteries, were measured, and the relationship between the degree of the initial coronary artery dilation and subsequent calcification in the branches and left coronary artery was analyzed. The coronary arterial diameter of all branches that eventually calcified was 6 mm or greater. The incidence of calcification in branches measuring 6 mm or greater on the initial coronary angiogram was 12% at 5 years, 44% at 10 years, and 94% at 20 years (n = 141). Dilation greater than 6 mm is associated with a high probability of late calcification.     

Coronary Artery Dilatation and Aortic Outflow Tract Enlargement in Children with Unicommissural Aortic Valves

We evaluated the aortic outflow tract (AOT) and coronary artery dimensions in pediatric patients with unicommissural aortic valves. A retrospective review of an echocardiographic database identified 37 patients with unicommissural aortic valves. A total of 115 echocardiograms were reviewed, and the right coronary artery (RCA), left main coronary artery (LM), left anterior descending coronary artery aortic valve annulus, aortic root, sinotubular junction (STJ), and ascending aorta were measured and z scores determined. The aortic stenosis peak gradient and the amount of aortic regurgitation (AR) were also measured. The RCA diameter (z score, 1.85 ± 1.8, p = 0.03) and LM diameter (z score, 1.74 ± 1.47, p = 0.04) are significantly dilated, as are all the AOT measurements: aortic annulus (2.02 ± 1.9, p = 0.02), aortic root (2.25 ± 1.9, p = 0.02), STJ (2.22 ± 1.74, p = 0.01), and ascending aorta (4.38 ± 2.03, p < 0.001). Longitudinal follow-up showed that there was no significant variation over time in any variable. The AOT measurements were significantly correlated with each other. A trend was found in which an increasing amount of AR gave an increase in AOT measurements. The aortic gradient was not significantly associated with any measurement. Our study population demonstrated significant dilatation of the RCA and LM as well as the AOT. The dilatation of the AOT structures is likely caused by the same mechanism that accounts for the AOT dilatation in patients with bicommissural aortic valves. Dilatation of the coronary arteries may represent an intrinsic abnormality in the vessel wall. Further studies are needed to define possible changes. Work was performed at Johns Hopkins Hospital, Baltimore, MD 21287, USA.     

Ischémie de la main révélant une maladie de KawasakiDistal arm ischemia revealing Kawasaki disease.

An aneurysm of the medium arteries is one of the major complications of Kawasaki syndrome. Eleven cases of giant coronary aneurysm have been reported. Case report. – We report a new case in a nine-month-old child. Ischemia of the left hand was the first symptom. Diagnosis was made 30 days later. Anticoagulant and fibrinolytic treatments did not prevent necrosis of four fingers. Conclusion. –The prognosis of Kawasaki disease depends on early diagnosis and management. Infants younger than seven months of age are more susceptible to developing coronary and mid-artery aneurysms, which therefore justifies a close observation during the acute phase and later on. In the case of a coronary artery aneurysm, Doppler echography of the upper and lower limbs is mandatorily recommended. Anticoagulant therapy should be started once the diagnosis of mid-artery aneurysms is made. Ischemia of the extremities can lead to necrosis and amputation. Until now, anticoagulants, vasodilators and fibrinolytic agents were not proven to be effective.     

Dipyridamole-provoked chest pain implies severe coronary artery disease in children

The diagnostic significance of dipyridamole-provoked chest pain was studied in 17 children with severe coronary arterial stenotic lesions (CAL) complicated with Kawasaki disease. Although dipyridamole induced chest pain in seven patients (symptomatic group), 10 reported no pain (asymptomatic group). In the asymptomatic group, seven children had one vessel disease (1VD) of right coronary artery (RCA) and the other three had two vessel disease (2VD) involving the RCA and left anterior descending artery (LAD). Four multivessel disease patients, one three vessel disease (3VD) and three 2VD of LAD and RCA, and three 1VD of LAD, were symptomatic. In the thallium scans, all patients, except two of the asymptomatic group, showed perfusion abnormalities. In addition, the extent score of the symptomatic group was significantly worse than that of the asymptomatic group (P = 0.01). While only one in six of the asymptomatic group showed abnormal ST depression on treadmill exercise electrocardiography, all patients in the symptomatic group (P = 0.02) showed ischemic ST depression. These findings suggest that the occurrence of chest pain after medication with dipyridamole closely correlates with the severity of CAL in children.     

Exercise myocardial perfusion stress testing in children with Kawasaki disease

AIM: Kawasaki disease is the commonest cause of acquired coronary artery disease in children. Although echocardiography and treadmill stress testing have been the traditional methods of assessing coronary artery abnormalities and detecting myocardial ischaemia, respectively, these are inadequate for assessing perfusion and performance. We studied the safety and utility of exercise myocardial perfusion stress testing in children with previous Kawasaki disease. METHODS: Eleven subjects (median age 12 years; seven male and four female adolescents) with a history of childhood Kawasaki disease underwent 99mTechnetium-Tetrofosmin myocardial perfusion scan using a modified protocol. All had New York Heart Association effort tolerance class 1 and were asymptomatic at time of testing. Eight of 11 subjects had been treated with intravenous immunoglobulin during the acute phase. Six of 11 subjects had transient coronary artery dilatation. One subject with persistently dilated coronary arteries suffered an acute myocardial infarction 6 months after onset of Kawasaki disease but recovered well. Results were compared with clinical and echocardiographic findings. RESULTS: All subjects were able to complete the exercise test. There were no side effects associated with radioisotope injection. Ten of 11 patients, including the one who suffered a myocardial infarction had normal tests. The single subject with an abnormal scan showed a minimal (2%) fixed defect in the left ventricular wall. CONCLUSION: Exercise myocardial perfusion stress is a safe and useful method for the assessment of myocardial perfusion in co-operative children with a history of Kawasaki disease and is a useful addition to conventional methods for coronary risk stratification in such patients.     

双嘧达莫负荷超声背散积分评价川崎病心肌缺血价值的研究

目的探讨双嘧达莫负荷超声背向散射积分(简称背散积分,IBS)技术诊断川崎病心肌缺血和损伤的价值。方法2007～2008年利用超声背散积分的方法观察31例有冠状动脉损伤的川崎病患儿,全部病例在静息和潘生丁负荷后都经历了超声检查,测量前间壁(AS)、左室后壁(PW)和下壁(INF)3个节段的IBS周期变化(CV),测值与铊-201(Thallium-201,Tl-201)核素心肌扫描结果比较,评价IBS诊断川崎病心肌缺血和损伤的价值。结果静息状态下,Tl-201心肌扫描正常和异常节段的CVIBS均无明显变化。负荷后,核素异常分布节段CV明显的下降。负荷后延迟的研究表明全部病例CV都恢复到静息状态水平。与Tl-201心肌扫描比较,在PW和INF节段,CVIBS的敏感性分别是75%和91%,特异性分别是91%和90%。结论双嘧达莫负荷CVIBS方法可定量评估川崎病患儿心肌缺血或损伤的严重性,为评价预后提供了一个敏感的检查方法。     

Retrograde holodiastolic flow in the abdominal aorta detected by pulsed Doppler echocardiography in patients with Kawasaki disease

Retrograde holodiastolic flow in the abdominal aorta (retrograde flow) detected by pulsed Doppler echocardiography is usually noted in patients with aortic regurgitation or patent ductus arteriosus. Similar abnormal flow often is present in patients with acute phase Kawasaki Disease (KD). In 21 patients with acute phase KD, 15 had a retrograde flow. Retrograde flow was recognised in only 3 of patients with acute infection (n=31) and in no healthy controls (n=10). The ratio of the time velocity integral for diastolic retrograde flow to that for antegrade flow (regurgitant fraction) was significantly greater in patients with KD (median value 23%) than in patients with acute infection (1%) or healthy individuals (1%) (P < 0.001 for both). Four patients with coronary arterial involvement (one with an aneurysm and three with transient dilation of the coronary arteries) had a greater regurgitant fraction than the 17 patients without coronary arterial involvement (median values: 31% versus 18%, P < 0.05). The C-reactive protein was increased for a longer period of time in patients with KD with a greater regurgitant fraction (P < 0.01). Plasma nitric oxide (NO) metabolite concentrations were significantly greater in patients with KD than in those with acute infection or in healthy controls (P < 0.001 for both). There was a positive correlation between plasma NO metabolite concentrations and the regurgitant fraction in patients with KD (r=0.69). Conclusion Retrograde flow in the abdominal aorta is increased in patients with Kawasaki disease. Further studies are needed to clarify the causal relationship between the abnormal flow and the overproduction of nitric oxide. Received: 16 March 1999 / Accepted: 12 January 2000     

Pathological Study of Postcoronary Arteritis in Adolescents and Young Adults: With Reference to the Relationship Between Sequelae of Kawasaki Disease and Atherosclerosis

To clarify whether the cardiac sequelae of Kawasaki disease (KD), postcoronary arteritis lesions can become a risk factor for atherosclerosis of the coronary arteries, six autopsy cases of patients older than 15 years of age with coronary arterial lesions caused by arteritis in childhood were examined histologically. Twenty-four arteries were inspected: 10 had no evidence of aneurysm formation, 7 arteries manifested simple dilatation of the lumen, and in 7 arteries there were aneurysms with recanalization. In the group in which there were no aneurysms, ``new intimal thickening' was observed in addition to the preexisting intimal thickening which had been caused by arteritis in the acute phase of KD. In the second group with aneurysmal arteries whose lumen remained dilated, thrombotic occlusion occurred in 4 of 6 aneurysms. In addition, advanced atherosclerotic changes (i.e., complicated lesions) were found in a 39-year-old patient. Finally, in the third group of arteries which manifested recanalized lumens after thrombotic occlusion of the aneurysms, new intimal thickening was seen on the internal side and some of them were occluded. The findings in this study suggest that aneurysms present in coronary arteries in individuals with a history of KD constitute a risk factor for atherosclerosis later in life.     

Follow-Up Assessment of the Collateral Circulation in Patients with Kawasaki Disease Who Underwent Dipyridamole Stress Technetium-99m Tetrofosmin Scintigraphy

To assess the alteration of myocardial ischemic findings and the role of collateral vessels in patients with Kawasaki disease (KD), we used dipyridamole stress technetium-99m tetrofosmin (Tf) single photon emission computed tomography (SPECT). A comparison study of coronary angiography and dipyridamole stress (0.70 mg/kg) Tf-SPECT was repeated at least twice in 20 patients. The subjects included 7 patients with coronary stenosis, 1 with pre- and post-coronary artery bypass grafting (CABG) due to coronary stenosis, 1 with progression to coronary stenosis, and 11 with persistent coronary aneurysms. In the stenosis group, Tf-SPECT revealed that 6 of the 7 patients had some degree of ischemic findings, and 5 of these 6 did not show any change in their ischemic findings during follow-up. In 1 patient, the ischemic findings changed according to the collateral circulation changes. The patient who underwent CABG had pre-CABG ischemic changes that disappeared after CABG. In the patient whose coronary arteries progressed to stenosis, the ischemic findings progressed as the coronary stenosis progressed. In the persistent aneurysm group, there were no ischemic findings. In the future Tf-SPECT may become one of the most useful methods for monitoring the progressive changes of myocardial ischemia in KD.     

Evaluation of the Efficacy of Treatment of Kawasaki Disease before Day 5 of Illness

We evaluated the efficacy of treating Kawasaki disease earlier than Day 5 of illness with a standard dose of immunoglobulin and aspirin. We performed a case–control study of patients with Kawasaki disease admitted to Princess Margaret Hospital from 1994 to 1999. Patients with pretreatment coronary aneurysm or those treated after day 10 of illness were excluded. All patients received immunoglobulin (2 g/kg) and aspirin (80–100 mg/kg/day) until fever subsided for 48 hours. Immunoglobulin retreatment was given for persistent fever 48 hours after the first dose of immunoglobulin or recrudescent fever. The case group consisted of 15 patients who received treatment earlier than day 5 of illness, and the control group consisted of 66 patients who were treated on or after day 5. Patients sex, age, duration of posttreatment fever, need for additional immunoglobulin, and coronary artery status were noted. Treatment efficacy was assessed by the duration of posttreatment fever and the prevalence of coronary artery aneurysms. Eighty-one patients were included in this study. There were 15 patients in the case group and 66 in the control group. No significant difference was noted in age and sex between the case and control groups. Thirty-three percent (5/15) and 8% (5/66) of the case and control groups, respectively, had persistent/ recrudescent fever 48 hours after the first dose of immunoglobulin that required retreatment (p = 0.017). Thirteen percent (2/15) and 5% (3/66) of the case and control groups, respectively, had coronary aneurysms (p = 0.158). Treatment of Kawasaki disease before day 5 of illness was associated with persistent/recrudescent fever that required retreatment. However, there was no significant increase in the prevalence of coronary aneurysm if retreatment was given. Poster presented at the third World Congress of Pediatric Cardiology and Cardiac Surgery, Toronto, Ontario, Canada, 2001     

Prediction of non-responsiveness to standard high-dose gamma-globulin therapy in patients with acute Kawasaki disease before starting initial treatment

Clinical, laboratory, and echocardiographic data were retrospectively analyzed in 112 patients with acute Kawasaki disease who received high-dose (2 g/kg) intravenous gamma-globulin (IVIG) treatment within 2 days and were compared for those who were responsive and non-responsive to initial IVIG treatment. Coronary arteries adjusted for body surface area (BSA) were evaluated quantitatively by comparison with the mean dimensions for 85 normal control subjects. The incidence of coronary abnormalities was higher in IVIG-non-responsive patients as compared to IVIG-responsive patients (71% versus 5%, p<0.0001). Univariate analysis of pre-IVIG data showed that the neutrophil count and serum levels of C-reactive protein (CRP), total bilirubin (TB), aspartate aminotransferase (AST), alanine aminotransferase, and lactate dehydrogenase (LDH) were significantly higher in IVIG-non-responsive versus responsive patients. Multivariate analysis selected CRP (p=0.009), TB (p<0.001), and AST (p=0.002) as independent predictors of non-responsiveness to initial IVIG treatment. By defining predictive values, patients with at least two of three predictors (CRP≥7.0 mg, TB≥0.9 mg, or AST≥200 IU/L) are considered to be non-responsive to IVIG for acute Kawasaki disease. Alternatively, more intense initial therapy may be a promising therapeutic strategy for patients who are predicted to be IVIG-non-responsive.     

肾上腺糖皮质激素联合乌司他丁治疗儿童川崎病的非随机对照临床研究

目的 探讨肾上腺糖皮质激素联合乌司他丁治疗儿童川崎病的临床疗效。方法 根据患儿病情和家长意愿,将2011年1月至2013年12月入院确诊为典型川崎病(KD)的104例患儿分为乌司他丁组(甲基强的松龙+乌司他丁,n=46)和静脉注射丙种球蛋白(IVIG)组(n=58)。观察两组患儿治疗前、治疗后1周、3个月及6个月时的冠状动脉内径的变化,热退时间,再次治疗情况,治疗前、治疗后1周及3周时白细胞(WBC)、血小板(PLT)、血红蛋白(HB)、C反应蛋白(CRP)、血沉的变化以及住院总费用。结果 两组患儿在治疗前、治疗后1周、3个月及6个月时冠状动脉内径比较差异均无统计学意义(P> 0.05)。治疗48 h乌司他丁组患儿体温均正常(100%),正常率高于IVIG组(83%)(PPP结论 甲基强的松龙联合乌司他丁治疗儿童KD没有增加冠状动脉瘤的发生风险;并能大大降低住院费用;与IVIG治疗相比,在KD急性期能更好的控制患儿的实验室指标,缩短发热时间。