[Diagnosis and treatment of incomplete Kawasaki disease in children]. / å„¿ç«¥ä¸å®Œå ¨æ€§å·å´Žç— çš„è¯Šæ²».

Kawasaki disease (KD) is a febrile disease mainly observed in children aged <5 years, with medium- and small-vessel vasculitis as the main lesion. Although KD has been reported for more than 50 years and great progress has been made in the etiology and pathology of KD in recent years, there is still a lack of specific indicators for the early diagnosis of KD, especially with more difficulties in the diagnosis of incomplete Kawasaki disease (IKD). At present, there are no clear diagnostic criteria for IKD, which leads to the failure of the timely identification and standardized treatment of IKD in clinical practice and even induce the development of coronary artery lesion. This article reviews the concept, epidemiological features, diagnosis, treatment, and follow-up management of IKD, in order to deepen the understanding of IKD among clinical workers and help to improve the clinical diagnosis and treatment of KD in China.

[Association of liver damage with coronary artery lesion and no response to intravenous immunoglobulin in the acute stage of Kawasaki disease]. / å·å´Žç— æ€¥æ€§æœŸè‚æŸå®³ä¸Žå† çŠ¶åŠ¨è„‰æŸä¼¤å’Œå ç–«çƒè›‹ç™½æ— ååº”çš„å ³ç³».

OBJECTIVES: To summarize the clinical features of liver damage in children in the acute stage of Kawasaki disease (KD), and to investigate the clinical value of liver damage in predicting coronary artery lesion and no response to intravenous immunoglobulin (IVIG) in children with KD. METHODS: The medical data were collected from 925 children who were diagnosed with KD for the first time in Beijing Children's Hospital from January 1, 2016 to December 31, 2017. According to the presence or absence of abnormal alanine aminotransferase (ALT) level on admission, the children were divided into a liver damage group (n=284) and a non-liver damage group (n=641). A logistic regression analysis was used to investigate the clinical value of the indicators including liver damage in predicting coronary artery lesion and no response to IVIG in children with KD. RESULTS: Compared with the non-liver damage group, the liver damage group had a significantly earlier admission time and significantly higher serum levels of inflammatory indicators (P<0.05). The liver damage group had a significantly higher incidence rate of coronary artery lesion on admission than the non-liver damage group (P=0.034). After initial IVIG therapy, the liver damage group had a significantly higher proportion of children with no response to IVIG than the non-liver damage group (P<0.001). In children with KD, coronary artery lesion was associated with the reduction in the hemoglobin level and the increases in platelet count, C-reactive protein, and ALT (P<0.05), and no response to IVIG was associated with limb changes, the reduction in the hemoglobin level, the increases in platelet count, C-reactive protein, and ALT, and coronary artery lesion (P<0.05). CONCLUSIONS: Compared with those without liver damage, the children in the early stage of KD with liver damage tend to develop clinical symptoms early and have higher levels of inflammatory indicators, and they are more likely to have coronary artery lesion and show no response to IVIG treatment.

Clinical features of recurrent Kawasaki disease and its risk factors.

The clinical features and risk factors for recurrence of Kawasaki disease (KD) remain unclear. In order to summarize clinical features of recurrent KD and identify risk factors associated with recurrence, we conducted a retrospective review of the medical records of consecutive cases of KD from January 2002 to December 2010. Demographic, clinical, laboratory, and echocardiographic data were analyzed. The maximum coronary artery Z score normalized against body surface area was assessed using coronary artery diameters. At the first onset of recurrent KD, children had longer durations of fever before intravenous immunoglobulin (IVIG) treatment and higher levels of alanine aminotransferase, serum aspartate aminotransferase (AST), and lower hemoglobin levels than those with a single episode of KD. Multivariate logistic regression analysis showed that long durations of fever before IVIG treatment, high AST levels, and reduced hemoglobin levels were significantly associated with recurrent KD. Ten of the 22 recurrent KD children had coronary artery complications during the first onset episode, and six (60 %) of these also had coronary artery complications during the recurrence. Children with longer durations of fever, lower hemoglobin levels, and higher AST levels may be at increased risk for KD and coronary artery complications are more likely to occur in children with recurrent KD if they were present during the first episode.

[Status of endothelial progenitor cell in murine model of Kawasaki disease].

OBJECTIVE: To assess whether the occurrence of coronary artery lesion was correlated with the changes of endothelial progenitor cell (EPC) number and function in murine model of Kawasaki disease (KD). METHODS: Lactobacillus casei cell wall extract (LCWE) was prepared and then C57BL/6 mice received a single intraperitoneal injection of LCWE for inducing KD. Twenty-four mice were categorized randomly into 3 groups: KD model group at Day 14 post-injection, KD model group at Day 56 post-injection and control group with an intraperitoneal injection of phosphate buffered solution (n = 8 each). The number of circulating EPC was defined as CD34(+)Flk-1(+)CD45(-) from mice. Meanwhile, bone marrow mononuclear cells were cultured in vitro to expand EPC for functional analysis. After 7 days of culturing, EPC were inoculated onto culture plate and thiazolyl blue assay was used to measure the absorbance value by enzyme labeling instrument to evaluate the proliferation. The adhesion of EPC was performed by replating cells on fibronectin coated dishes and then counting the number of adherent cells. The migration of EPC was assayed by Transwell. RESULTS: Focal inflammatory infiltrate was evident in coronary artery trunk and a series of branches at Day 14 post-injection. The inflammatory cell infiltrate consisted of mononuclear lymphocytes. The number of circulating EPC were significantly lower in the Day 14 LCWE-treating murine model versus the controls (0.017% ± 0.008% vs 0.028% ± 0.007%, P < 0.01). Disruption of elastin was consistently observed at Day 56 post-injection. And there was no apparent recovery in number of EPC (0.016% ± 0.007%, P < 0.01). When bone marrow mononuclear cells were cultured in vitro, the colony-forming ability of EPC decreased in the KD model group at Day 14 post-injection versus the controls. Test of proliferating ability showed that the absorbance was 0.39 ± 0.11 in MTT experiment and decreased than the controls (0.61 ± 0.14, P < 0.01). Adhesion and migration were also down-regulated versus the controls ((3.1 ± 0.6) and (3.2 ± 0.6) vs (6.4 ± 1.2) and (6.2 ± 0.5) cells/HPF, both P < 0.01). In the KD model group at Day 56 post-injection, the colony-forming ability of EPC was not recovered significantly. Proliferation ability, adhesion and migration were still decreased compared to the controls (0.38 ± 0.09, (3.12 ± 0.56) cells/HPF and (3.29 ± 0.63) cells/HPF, all P < 0.01). CONCLUSION: The occurrence of coronary artery lesion may be correlated with the down-regulation of EPC number and function in murine model of KD.

[Late endothelial function in children with coronary aneurysm due to Kawasaki disease].

OBJECTIVE: To evaluate the late endothelial function in children with coronary aneurysm due to Kawasaki disease (KD). METHODS: Thirty-one children with coronary aneurysms due to KD who had the disease course for more than 1 year and twenty-one age-matched healthy children were enrolled. Brachial artery endothelium-dependent and -independent flow-mediated dilation (FMD), carotid arterial stiffness index (SI) and intima-media thickness (IMT) were measured by high-frequency ultrasound. RESULTS: There were 9 cases of medium and 22 cases of giant coronary aneurysms in the KD group. Twelve KD patients had evidence of myocardial ischemia. Compared to the normal controls, the endothelium-dependent FMD decreased (P<0.05), the carotid arterial SI increased (P<0.05), and the carotid arterial intima-media thickness increased significantly (P<0.05) in children with coronary aneurysms due to KD. The endothelium-dependent FMD decreased more significantly in 12 KD patients with myocardial ischemia than in those without any evidence of myocardial ischemia (P<0.05). CONCLUSIONS: Late endothelial dysfunction exists in children with coronary aneurysms due to KD, especially in those with myocardial ischemia.

[Comparison of efficacy among early, conventional and late intravenous gamma globulin treatment of Kawasaki disease].

OBJECTIVE: To compare the rates of intravenous gamma globulin (IVIG) non-responder and coronary complication among early, conventional and late IVIG treatment in children with Kawasaki disease (KD). METHODS: All children with KD and IVIG treatment were retrospectively analyzed at 45 hospitals in Beijing during the 5-year period from 2000 through 2004. The time of IVIG treatment was classified as early (Day 1 - 4), conventional (Day 5 - 9) and late treatment group (Day 10 or later). The efficacy of IVIG was judged by the rate of IVIG non-responder. Echocardiography was used to assess the coronary complication at acute (1 - 2 weeks after onset) and sub-acute (3 - 6 weeks after onset) stage. RESULTS: A total of 1052 patients (680 boys, 372 girls) aged 2 months to 13.8 years were included. They were grouped as early, conventional and late treatment in 108, 763 and 181 children respectively. The rate of IVIG non-responders was higher in early (28.7%, 31/108) as compared with conventional (11.9%, 91/763) and late treatment group (7.2%, 13/181, both P < 0.01). The incidences of coronary complications were similar in early (17.6%, 19/108 and 5.9%, 4/68) and conventional treatment group (18.3%, 140/ 763 and 5.5%, 25/452), while significantly higher in late treatment group (33.7%, 61/181 and 12.8%, 15/117) in acute and sub-acute stages (both P < 0.01). CONCLUSIONS: IVIG treatment in children with KD for a disease duration of 1 - 4 days appeared to increase the rate of IVIG non-responders. Children with IVIG given at Day 10 or later had a higher incidence of acute and sub-acute coronary complications. IVIG given at Day 5 - 9 seems to be the best time for IVIG therapy in KD.

[Diagnosis of unexplained syncope in children: a multi-center study].

OBJECTIVE: To examine the diagnostic value of head-up tilt test in children with unexplained syncope (UPS). METHODS: A total of 379 children (171 males, 208 females) aged 3-18 years, mean age (12 +/- 3) years with unexplained syncope from Beijing, Hunan, Hubei and Shanghai and undergoing baseline head-up tilt tests (BHUTT) or head-up tilt tests potentiated with nitroglycerine (SNHUTT) under a quiet circumstance were selected as the syncope group. Ten healthy children (5 males, 5 females) aged 9-15 years with a mean age of (11.4 +/- 2.1) years, were recruited as the control group. SPSS 10.0 software was used for data analysis. RESULTS: In 379 children with unexplained syncope, 67 (17.7%) were of postural orthostatic tachycardia syndrome (POTS), 157 (41.4%) of vasovagal syncope vasoinhibitory pattern, 14 (3.7%) of vasovagal syncope cardioinhibitory pattern, 47 (12.4%) of vasovagal syncope mixed pattern, 1 (0.3%) of orthostatic hypotension (OH) and 93 children (24.5%) of UPS. In syncope group and control group, the positive rate of BHUTT was 55.9% and 0 respectively and it was 75.5% and 20.0% respectively for SNHUTT. During BHUTT, the mean time of positive response occurrence was (16 +/- 12) minutes, and the posture when positive response appeared was at a tilt angle of 60 degrees. For SNHUTT, the mean time of positive response occurrence was (6 +/- 4) minutes and the posture was at a tilt angle of 60 degrees when potentiated with nitroglycerine. CONCLUSION: HUTT is an objective diagnostic tool of UPS. With a high diagnostic positive rate, SNHUTT can improve the diagnostic positive rate of BHUTT. Meanwhile the time of positive response occurrence during SNHUTT is markedly shorter than BHUTT.

New biomarkers of Kawasaki disease identified by urine proteomic analysis.

Kawasaki disease (KD) is an acute systemic vasculitis that mainly afflicts infants and young children. The symptoms of KD are similar to those of various febrile diseases. Here, we attempted to develop accurate diagnostic biomarkers of KD by performing urine proteomic analysis of samples from healthy controls, patients with KD, and patients with another febrile disease, pneumonia (two patients). We identified differentially expressed proteins (DEPs) in KD as compared to normal controls. We also constructed functional annotation and protein-protein interaction (PPI) networks of DEPs in KD and pneumonia. DEPs common to both KD and pneumonia were identified, as well as DEPs specific to KD. Compared to normal control, 43 and 62 DEPs were identified in KD and pneumonia, respectively. Serine hydroxymethyltransferase 1 is a hub protein of the KD-specific PPI network. Thirteen DEPs common to both KD and pneumonia and 30 DEPs specific to KD were identified. Of these, the expression of eight DEPs could cluster normal and pneumonia samples into one group and cluster KD samples into another group based on hierarchical clustering. Our study identified several DEPs that may play a role in KD and that may serve as diagnostic biomarkers to distinguish patients with KD from both normal control and other febrile diseases.

Detection of Subclinical Anthracyclines' Cardiotoxicity in Children with Solid Tumor.

BACKGROUND: Cardiotoxicity is one of the most serious chronic complications of anthracyclines therapy. Assessment of the left ventricular ejection fraction (LVEF) fails to detect subtle cardiac dysfunction of left ventricular (LV). This study aimed to detect and evaluate new parameters of subclinical anthracyclines' cardiotoxicity in children with solid tumor. METHODS: A detailed echocardiographic examination was performed in 36 children with hepatoblastoma or rhabdomyosarcoma after receiving anthracyclines' chemotherapy and 36 healthy controls from January 2015 to December 2016. The LVEF, ratio of early diastolic peak velocity of transmitral flow (E) and septal diastolic e' mitral annular peak velocity (e'), tricuspid annular plane systolic excursion (TAPSE), and LV global longitudinal strain (GLS) were evaluated using M-mode, tissue Doppler imaging (TDI), and two-dimensional speckle tracking echocardiography (2D-STE), respectively. Echocardiographic parameters were compared between patient group and healthy controls. All patients were divided into two subgroups based on their anthracyclines' cumulative dosage (<300 mg/m2 subgroup and ≥300 mg/m2 subgroup). RESULTS: All patients had no presentation of heart failure and LVEF within normal range (65.7 ± 5.1%). Compared with healthy controls, the mean E/e' increased significantly (7.9 ± 0.7 vs. 10.2 ± 3.5, t = 3.72, P < 0.01), mean TAPSE decreased significantly (17.2 ± 1.3 mm vs. 14.2 ± 3.0 mm, t = -4.03, P < 0.01), and mean LV GLS decreased significantly (-22.2% ± 1.9% vs. -17.9% ± 2.9%, t = -5.58, P < 0.01) in patient group. Compared with subgroup with anthracyclines' cumulative dosage < 300 mg/m2, mean LV GLS decreased significantly (-18.7 ± 2.7% vs. -16.5 ± 2.1%, t = 2.15, P = 0.04), the mean E/e' increased significantly (9.1 ± 1.5 vs. 11.5 ± 4.9, t = -2.17, P = 0.04), and mean TAPSE decreased significantly (14.2 ± 2.1 mm vs. 12.5 ± 2.2 mm, t = -2.82, P = 0.02) in subgroup with anthracyclines' cumulative dosage ≥300 mg/m2. CONCLUSIONS: LV GLS is helpful in the early detection of subclinical LV dysfunction using 2D-STE. E/e' and TAPSE are other sensitive parameters in detecting subclinical cardiac dysfunction of both ventricles by TDI. These parameters show significant change with different anthracyclines' cumulative dosage, so cumulative dosage should be controlled in clinical treatment.

Epidemiologic study on Kawasaki disease in Beijing from 2000 through 2004.

A hospital-based survey of Kawasaki disease was performed in all 45 hospitals with in-patient beds in Beijing during the 5-year period from 2000 through 2004. A total of 1107 patients were enrolled, with an annual incidence varying from 40.9 to 55.1 per 100,000 children <5 years of age. The incidence of coronary complications was 20.6% in the acute stage, and 6.9% in the 1-2 month follow-up.

Congenital vascular rings: a rare cause of respiratory distress in infants and children.

BACKGROUND: Congenital vascular rings may often cause unexplained respiratory symptoms in infants and young children. Their diagnosis and treatment are often delayed. Few studies of vascular rings have been reported in China. The aim of this study was to describe the clinical presentation, diagnosis and surgical management of infants and children with congenital vascular rings. METHODS: Clinical histories, physical examinations, investigations, image studies and surgical interventions were retrospectively evaluated in 7 children (age range: 2 months-4 years, mean 7 months) with congenital vascular rings. Chest radiography was performed in all patients. Echocardiography and computed tomography (CT) with 3-dimensional (3D) reconstructions were performed in 6 patients. Esophagography, cardiac catheterization and angiography, and bronchoscopy were performed in 1, 1 and 4 children, respectively. RESULTS: Six of the 7 patients had respiratory symptoms, including recurrent cough, stridor and wheeze. Age at onset of symptoms ranged from 1 month to 11 months. Chest X-ray showed nothing important on the vascular rings, besides bronchitis and pneumonia. Contrast-enhanced CT diagnosed vascular rings in 6 patients. Four patients had double aortic arches, two had balanced arches and two were right arch dominant. One patient had a right aortic arch with left ligament and 1 patient had a pulmonary artery sling. Echocardiography failed to diagnose vascular rings in 2 patients. The esophagogram of 1 patient showed esophageal compression. Bronchoscopy of 4 patients showed compression of the distal trachea. Five of the 7 patients underwent surgical division of the vascular rings. Surgical observation confirmed the CT findings in each patient. CONCLUSIONS: Patients, especially infants or young children, with recurrent respiratory symptoms such as chronic cough, stridor and wheeze, should be examined for the possible presence of congenital vascular rings. Contrast-enhanced CT can clearly show the anatomy of vascular rings. As a noninvasive technique, echocardiography is helpful for diagnosis. Early surgical management in symptomatic patients is effective.

[Effects of three intravenous gamma globulin regimens on Kawasaki disease and relevant coronary complication rates in pediatric patients: a comparative study].

OBJECTIVES: To compare the effects on Kawasaki disease (KD) of 3 different intravenous gamma globulin (IVIG) regimens and coronary complication rates in children with Kawasaki disease (KD). METHODS: The clinical data of 1052 children with KD treated in 45 hospitals in Beijing from 2000 through 2004, 680 male and 372 female, aged 2 months-13.8 years, 656 (60.1%) undergoing IVIG 2 g/kg for one dose (single dose group), 292 (26.7%) undergoing 1 g.kg(-1).d(-1) for 2 days (2 d group), and 104 (9.5%) undergoing 400 - 600 mg.kg(-1).d(-1) for 4 - 5 d (4 - 5 d group) in addition of oral administration of aspirin, were analyzed retrospectively. Echocardiography was used to assess the occurrence of coronary complications 1 - 2 weeks after onset (acute stage) and 3 - 6 weeks after onset (sub-acute stage). RESULTS: The rate of IVIG non-responder of the 2 d group was 20.9%, significantly higher than those of the single dose group and 4 - 5 d group (9.9% and 8.7% respectively, both P < 0.01). There were no significant differences in rates of coronary complication, pericardial effusion, and mitral regurgitation at the acute stage among the 3 groups (all P > 0.05). However, the rates of coronary complication and of coronary aneurysm at the sub-acute stage of the single dose group were 5.1% and 1.6%, significantly lower than those of the 4 - 5 d group (11.6% and 4.7%) and 2 d group (9.8% and 5.4%, P = 0.035 - 0.047) were significantly lower in single dose group (5.1% and 1.6%) as compared to those in 4 - 5 d group and (11.6% and 4.7%) and 2 d group (9.8% and 5.4%) (P = 0.035 - 0.047). CONCLUSION: IVIG 2 g/kg in a single dose has lower rates of coronary complications and IVIG non-responders in children with KD, and is recommended for initial KD therapy.

Echocardiographic Evaluation of Coronary Abnormalities and Cardiac Function in a Murine Model of Kawasaki Disease Using High-frequency Ultrasound.

BACKGROUND: Murine model of coronary arterial inflammation has been widely accepted as an animal model of and used in Kawasaki disease (KD). This study sought to evaluate the developmental changes of coronary arteries and cardiac function in a murine model of KD with a high-frequency ultrasound system and to provide evidence for the preparation of the model of KD. METHODS: Lactobacillus casei cell wall extract was prepared and injected into C57BL/6 mice intraperitoneally (i.p.) to induce KD. A total of 120 mice were grouped into three groups. The intravenous immunoglobulin (IVIG) treatment group was i.p. injected with IVIG (2 g/kg), while the KD model and normal control groups were i.p. injected with 0.5 ml of phosphate buffered solution on day 5. All high-resolution echocardiography detection of mouse heart was performed by the same senior technician. Animal echocardiography was performed by measuring the coronary artery dimensions and cardiac function on days 0, 7, 14, 28, and 56 (high-resolution small animal ultrasound [Vevo770 pattern; VisualSonic, Canada] with broadband probe [RMVTM707B; frequency, 30 mHz; depth of focus, 1.2 cm]) which were measured and analyzed with Vevo770 software. RESULTS: Pathological studies revealed focal inflammatory infiltrate asymmetrically distributed around the coronary artery trunk in the KD model group. Echocardiographic study including coronary dimension and cardiac function measurements was successfully performed in all subjects. The KD model and IVIG treatment groups showed left coronary artery dilation on days 7, 14, 28, and 56. The diameter of left coronary artery in the KD model group (0.53 ± 0.09 mm; 0.36 ± 0.07 mm; 0.34 ± 0.05 mm; 0.34 ± 0.04 mm) was significantly larger than those of IVIG treatment group (0.22 ± 0.02 mm; 0.28 ± 0.03 mm; 0.26 ± 0.03 mm; 0.27 ± 0.05 mm; 0.26 ± 0.03 mm; all P < 0.01) and the normal control group (0.21 ± 0.02 mm; 0.22 ± 0.03 mm; 0.22 ± 0.02 mm; 0.23 ± 0.02 mm; 0.27 ± 0.04 mm; all P< 0.01) on days 7, 14, 28, and 56. No significant differences were observed in the measurements of cardiac function among the groups on days 0, 7, 14, 28, and 56 (all P > 0.05). CONCLUSIONS: Echocardiography could identify the consecutive changes of coronary artery in KD mice. Echocardiography is more convenient and direct in evaluating the coronary abnormalities in this animal model.

[Mean values of brain natriuretic peptide in 190 healthy children].

OBJECTIVE: Overseas research has shown the value of brain natriuretic peptide (BNP) in the diagnosis and treatment of heart failure in children. However, a reference range of BNP values is lacking, limiting its clinical application. This study was designed to determine a reference range for children aged 1 to 16 years. METHODS: Plasma BNP (BNP32, NT-proBNP) concentrations were measured in 190 healthy children (95 boys and 95 girls) using an enzyme immunoassay. Their age ranged from 1 to 16 years (mean=10.6 +/- 4.2 years). RESULTS: The mean plasma concentration of BNP32 in 190 children was 51.89 +/- 48.36 pg/mL, with the 10th and the 90th percentile rank of 27.00 pg/mL and 75.00 pg/mL respectively. The mean plasma concentration of BNP32 in girls was 60.33 +/- 62.91 pg/mL, and 44.22 +/- 27.14 pg/mL in boys, but no statistical differences were found. The mean plasma concentration of NT-proBNP in 190 children was 246.04 +/- 67.27 fmol/mL. The girls had slightly higher plasma NT-proBNP levels than the boys, but there were no statistical differences between them. Neither plasma BNP32 concentration nor NT-proBNP concentration was related to age. CONCLUSIONS: This study first reported a reference range of BNP values for healthy Chinese children aged 1-16 years. Both age and gender are not related to BNP values.

Corticosteroid Therapy Might be Associated with the Development of Coronary Aneurysm in Children with Kawasaki Disease.

BACKGROUND: Coronary artery lesions (CALs) are known to be the main complication in children with Kawasaki disease (KD). Instead of intravenous immunoglobulin (IVIG), corticosteroid therapy has been accepted to be used for children with KD who are unresponsive to IVIG. This study aimed to evaluate risk factors for CALs in children with KD. METHODS: We retrospectively reviewed the clinical records of 2331 children with KD from January 2005 to December 2014. To identify the independent risk factors for CALs, multivariable logistic regression models were constructed using significant variables identified from univariate logistic regression analysis. RESULTS: The incidence of CALs was 36.0% (840 of 2331), including 625 (26.8%) coronary artery dilations and 215 (9.2%) coronary artery aneurysms (CAAs). Multivariable logistic regression analysis identified that male, incomplete KD, longer fever duration, and C-reactive protein (CRP) >100 mg/L were independent risk factors for coronary artery dilatations. On the other hand, male, incomplete KD, longer fever duration, prolonged days of illness at the initial treatment, corticosteroid therapy, sodium ≤133 mmol/L, and albumin <35 g/L were the independent risk factors for CAAs. In addition, corticosteroid therapy, prolonged days of illness at the initial treatment, and albumin <35 g/L were the independent risk factors for giant CAAs. CONCLUSIONS: CALs might be associated with male sex, incomplete KD, longer fever duration, prolonged days of illness at the initial treatment, albumin <35 g/L, sodium ≤133 mmol/L, CRP >100 mg/L, and corticosteroid therapy. Corticosteroid therapy was an independent risk factor for CAAs and giant CAAs. Thus, corticosteroids should be used with caution in the treatment of KD with the risk for CALs.

Comparison between transcatheter and surgical closure of secundum atrial septal defect in children and adults: results of a multicenter nonrandomized trial.

OBJECTIVES: This study sought to compare the safety, efficacy and clinical utility of the Amplatzer septal occluder (ASO) for closure of secundum atrial septal defect (ASD) with surgical closure. BACKGROUND: The clinical utility of a device such as the ASO can only be judged against the results of contemporaneous surgery. METHODS: A multicenter, nonrandomized concurrent study was performed in 29 pediatric cardiology centers from March 1998 to March 2000. The patients were assigned to either the device or surgical closure group according to the patients' option. Baseline physical exams and echocardiography were performed preprocedure and at follow-up (6 and 12 months for device group, 12 months for surgical group). RESULTS: A total of 442 patients were in the group undergoing device closure, whereas 154 patients were in the surgical group. The median age was 9.8 years for the device group and 4.1 years for the surgical group (p < 0.001). In the device group, 395 (89.4%) patients had a single ASD; in the surgical group, 124 (80.5%) (p = 0.008) had a single ASD. The size of the primary ASD was 13.3 +/- 5.4 mm for the device group and 14.2 +/- 6.3 mm for the surgery group (p = 0.099). The procedural attempt success rate was 95.7% for the device group and 100% for the surgical group (p = 0.006). CONCLUSIONS: The early, primary and secondary efficacy success rates were 94.8%, 98.5% and 91.6%, respectively, for the device group, and 96.1%, 100% and 89.0% for the surgical group (all p > 0.05). The complication rate was 7.2% for the device group and 24.0% for the surgical group (p < 0.001). The mean length of hospital stay was 1.0 +/- 0.3 day for the device group and 3.4 +/- 1.2 days for the surgical group (p < 0.001). Mortality was 0% for both groups. The early, primary and secondary efficacy success rates for surgical versus. device closure of ASD were not statistically different; however, the complication rate was lower and the length of hospital stay was shorter for device closure than for surgical repair. Appropriate patient selection is an important factor for successful device closure. Transcatheter closure of secundum ASD using the ASO is a safe and effective alternative to surgical repair.

Salvianolic Acid B Down-regulates Matrix Metalloproteinase-9 Activity and Expression in Tumor Necrosis Factor-α-induced Human Coronary Artery Endothelial Cells.

BACKGROUND: Salvianolic acid B (Sal B) is a bioactive water-soluble compound of Salviae miltiorrhizae, a traditional herbal medicine that has been used clinically for the treatment of cardiovascular diseases. This study sought to evaluate the effect of Sal B on matrix metalloproteinase-9 (MMP-9) and on the underlying mechanisms in tumor necrosis factor-α± (TNF-α±)-activated human coronary artery endothelial cells (HCAECs), a cell model of Kawasaki disease. METHODS: HCAECs were pretreated with 1-10 αµmol/L of Sal B, and then stimulated by TNF-α± at different time points. The protein expression and activity of MMP-9 were determined by Western blot assay and gelatin zymogram assay, respectively. Nuclear factor-κB (NF-κB) activation was detected with immunofluorescence, electrophoretic mobility shift assay, and Western blot assay. Protein expression levels of mitogen-activated protein kinase (c-Jun N-terminal kinase [JNK], extra-cellular signal-regulated kinase [ERK], and p38) were determined by Western blot assay. RESULTS: After HCAECs were exposed to TNF-α±, 1-10 αµmol/L Sal B significantly inhibited TNF-α±-induced MMP-9 expression and activity. Furthermore, Sal B significantly decreased IκBα± phosphorylation and p65 nuclear translocation in HCAECs stimulated with TNF-α± for 30 min. In addition, Sal B decreased the phosphorylation of JNK and ERK1/2 proteins in cells treated with TNF-α± for 10 min. CONCLUSIONS: The data suggested that Sal B suppressed TNF-α±-induced MMP-9 expression and activity by blocking the activation of NF-κB, JNK, and ERK1/2 signaling pathways.

Qing Re Liang Xue Decoction Alleviates Hypercoagulability in Kawasaki Disease.

Objective. Kawasaki disease (KD) is a multisystemic autoimmune vasculitis. Intravenous immunoglobulin (IVIG) is the first-line treatment for KD. It is unclear whether traditional Chinese medicine (TCM) has an effect on KD. We aimed to observe the clinical efficacy of TCM on acute KD via serum interleukin-33 (IL-33) and tumor necrosis factor alpha (TNF-α) measurements. Methods. Thirty-one KD patients were treated with Qing Re Liang Xue decoction and Western medicine (integrative medicine treatment group), while 28 KD patients were treated with Western medicine only (Western medicine treatment group). Thirty patients were included in a febrile group and 28 healthy children were included in the control group. Clinical characteristics and laboratory findings were gathered and compared. Serum IL-33 and TNF-α levels were measured by multiplex Luminex assay. Results. The platelet count in the integrative medicine treatment group was significantly lower than that in the Western medicine treatment group. The integrative medicine group had a shorter fever duration and lower IL-33 and TNF-α levels than those in the Western medicine group, but there were no significant differences between the two KD groups after treatment. Conclusion. Qing Re Liang Xue decoction improved the hypercoagulable state of KD patients. Potential myocardial protective effects require further research.

Comparison of transcatheter closure of secundum atrial septal defect using the Amplatzer septal occluder associated with deficient versus sufficient rims.

To evaluate the feasibility of transcatheter closure of secundum atrial septal defects (ASDs) associated with deficient rims (<5 mm) using the Amplatzer septal occluder (ASO), 23 patients (median age 10.7 years) underwent an attempted transcatheter closure. The patients had a deficient anterior rim of 0 to 4 mm (n = 20), an inferior rim of 2 mm (n = 2), or a posterior rim of 4 mm (n = 1) as assessed by transesophageal echocardiography (TEE) or intracardiac echocardiography (ICE). Forty-eight patients with sufficient rims (>5 mm) who underwent closure served as controls. There were no differences between the 2 groups in ASD stretched diameter and device size (p >0.05). Of 23 patients with deficient rims, 17 (74%) had immediate complete closure compared with 44 of 48 patients (92%) with sufficient rims (p <0.05). At 24-hour and 6-month follow-up, the complete closure rates were not significantly different between the 2 groups (91% for patients with deficient rims vs 94% for patients with sufficient rims at 24 hours and 100% vs 93% at 6 months, respectively). The fluoroscopic time and procedure time were longer in patients with deficient rims (13 +/- 7 and 72 +/- 26 minutes, respectively) compared with those with sufficient rims (10 +/- 4 and 61 +/- 22 minutes, respectively). No major complications were encountered either during or after the closure procedure in both groups. Thus, transcatheter closure of ASDs associated with small anterior, inferior, or posterior rims is feasible using an ASO. Long-term follow-up data are still needed to assess long-term safety and efficacy.

Epidemiologic picture of Kawasaki disease in Beijing from 1995 through 1999.

OBJECTIVE: Kawasaki disease (KD) is supposed to be more common in the Asian race. The incidence in Japan is 10-fold higher than rates reported from western countries. This study sought to evaluate the epidemiologic picture of KD in Beijing and its suburbs. METHOD: A questionnaire form and diagnostic guidelines for KD were sent to all hospitals with pediatric inpatient beds throughout Beijing and its suburbs. Pediatricians were asked to review the medical records and report all patients with KD diagnosed during the 5-year period from January, 1995, through December, 1999. RESULTS: A total of 710 patients with KD were reported from 37 (95%) of 39 hospitals with pediatric inpatient beds. The incidences of KD for each year of the study were 18.2 (1995), 21.1 (1996), 18.6 (1997), 30.6 (1998) and 27.8 (1999) per 100,000 children <5 years of age. The male:female ratio was 1.7:1. The age at onset ranged from 1 month to 13.4 years (median, 2.3 years), with 85.2% <5 years old. The disease occurred more frequently in spring and summer and less frequently in autumn and early winter. Lymph node enlargement was the least common clinical sign, and its incidence decreased from 1995 to 1999. Cardiac abnormalities were found in 21.5% of patients and were more prevalent in patients diagnosed 10 days or longer after the onset. No patients died in the acute stage. CONCLUSION: The incidence of KD in Beijing is lower than that reported in Japan, similar to the incidence in the United States and higher than in other western countries. The age and gender distribution and increasing trend in incidence are similar to those in previous reports, but seasonal distribution is unique.