Kawasaki disease (KD) is one of the common acquired heart diseases in under-5-year-old children and is an acute self-limiting vasculitis. After nearly 60 years of research, intravenous immunoglobulin combined with oral aspirin has become the first-line treatment for preventing coronary artery aneurysm in the acute stage of KD. However, glucocorticoid (GC), infliximab, and other immunosuppressants are options for the treatment of KD patients with a high risk of coronary artery aneurysm, no response to intravenous immunoglobulin and a confirmed diagnosis of coronary artery aneurysm. At present, there are still controversies over the use of GC in the treatment of KD. With reference to the latest research findings of KD treatment in China and overseas, this consensus invited domestic pediatric experts to fully discuss and put forward recommendations on the indications, dosage, and usage of GC in the first-line and second-line treatment of KD.
Child ; Child, Preschool ; Consensus ; Coronary Aneurysm ; Glucocorticoids/therapeutic use* ; Humans ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome/drug therapy*

OBJECTIVES: To examine the association between duration of fever before intravenous immunoglobulin (IVIG) treatment and IVIG resistance in children with Kawasaki disease (KD). METHODS: A retrospective analysis was performed on the medical data of 317 children with KD who were admitted from January 2018 to December 2020. According to the duration of fever before IVIG treatment, they were divided into two groups: short fever duration group (≤4 days) with 92 children and long fever duration group (>4 days) with 225 children. According to the presence or absence of IVIG resistance, each group was further divided into a drug-resistance group and a non-drug-resistance group. Baseline data and laboratory results were compared between groups. A multivariate logistic regression analysis was used to identify the influencing factors for IVIG resistance. RESULTS: In the short fever duration group, 19 children (20.7%) had IVIG resistance and 5 children (5.4%) had coronary artery aneurysm, and in the long fever duration group, 22 children (9.8%) had IVIG resistance and 19 children (8.4%) had coronary artery aneurysm, suggesting that the short fever duration group had a significantly higher rate of IVIG resistance than the long fever duration group (P<0.05), while there was no significant difference in the incidence rate of coronary artery aneurysm between the two groups (P>0.05). In the short fever duration group, compared with the children without drug resistance, the children with drug resistance had a significantly lower level of blood sodium and significantly higher levels of procalcitonin, C-reactive protein, and N-terminal B-type natriuretic peptide before treatment (P<0.05). In the long fever duration group, the children with drug resistance had significantly lower levels of blood sodium and creatine kinase before treatment than those without drug resistance (P<0.05). The multivariate logistic regression analysis showed that a reduction in blood sodium level was associated with IVIG resistance in the long fever duration group (P<0.05). CONCLUSIONS IVIG resistance in children with KD varies with the duration of fever before treatment. A reduction in blood sodium is associated with IVIG resistance in KD children with a duration of fever of >4 days before treatment.
Child ; Coronary Aneurysm/drug therapy* ; Fever/etiology* ; Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Infant ; Mucocutaneous Lymph Node Syndrome/drug therapy* ; Retrospective Studies ; Sodium/therapeutic use*

Objective: To investigate the efficacy and safety of infliximab (IFX) therapy for children with Kawasaki disease. Methods: Sixty-eight children with Kawasaki disease who received IFX therapy in Children's Hospital of Fudan University from January 2014 to April 2021 were enrolled. The indications for IFX administration, changes in laboratory parameters before and after IFX administration, response rate, drug adverse events and complications and outcomes of coronary artery aneurysms (CAA) were retrospectively analyzed. Comparisons between groups were performed with unpaired Student t test or Mann-Whitney U test or chi-square test. Results: Among 68 children with Kawasaki disease, 52 (76%) were males and 16 (24%) were females. The age of onset was 2.1 (0.5, 3.8) years. IFX was administered to: (1) 35 children (51%) with persistent fever who did not respond to intravenous immunoglobulin (IVIG) or steroids, 28 of the 35 children (80%) developed CAA before IFX therapy; (2) 32 children (47%) with continuous progression of CAA; (3) 1 child with persistent arthritis. In all cases, IFX was administered as an additional treatment (the time from the onset of illness to IFX therapy was 21 (15, 30) days) which consisted of second line therapy in 20 (29%), third line therapy in 20 (29%), and fourth (or more) line therapy in 28 (41%). C-reactive protein (8 (4, 15) vs. 16 (8, 43) mg/L, Z=-3.38, P=0.001), serum amyloid protein A (17 (10, 42) vs. 88 (11, 327) mg/L, Z=-2.36, P=0.018) and the percentage of neutrophils (0.39±0.20 vs. 0.49±0.21, t=2.63, P=0.010) decreased significantly after IFX administration. Fourteen children (21%) did not respond to IFX and received additional therapies mainly including steroids and cyclophosphamide. There was no significant difference in gender, age at IFX administration, time from the onset of illness to IFX administration, the maximum coronary Z value before IFX administration, and the incidence of systemic aneurysms between IFX-sensitive group and IFX-resistant group (all P>0.05). Infections occurred in 11 cases (16%) after IFX administration, including respiratory tract, digestive tract, urinary tract, skin and oral infections. One case had Calmette-Guérin bacillus-related adverse reactions 2 months after IFX administration. All of these adverse events were cured successfully. One child died of CAA rupture, 6 children were lost to follow up, the remaining 61 children were followed up for 6 (4, 15) months. No CAA occurred in 7 children before and after IFX treatment, while CAA occurred in 54 children before IFX treatment. CAA regressed in 23 (43%) children at the last follow-up, and the diameter of coronary artery recovered to normal in 10 children. Conclusion: IFX is an effective and safe therapeutic choice for children with Kawasaki disease who are refractory to IVIG or steroids therapy or with continuous progression of CAA.
Child ; Coronary Aneurysm/etiology* ; Female ; Humans ; Immunoglobulins, Intravenous/therapeutic use* ; Infant ; Infliximab/adverse effects* ; Male ; Mucocutaneous Lymph Node Syndrome/drug therapy* ; Retrospective Studies

Objective: To investigate the clinical characteristics of systemic juvenile idiopathic arthritis combined with coronary artery dilatation. Methods: A retrospective analysis was performed on the clinical data, including clinical manifestations, blood routine, inflammatory factors, echocardiography, vascular ultrasound and CT angiography, treatment and outcomes, etc, of 5 cases with systemic juvenile idiopathic arthritis combined with coronary artery dilation admitted to Department of Rheumatology in the affiliated Children's Hospital of Capital Institute of Pediatrics from May 2019 to June 2021. Results: There were 2 males and 3 females among 5 cases. The onset age ranged from 7 months to 4 years 7 months.The diagnostic time ranged from 1.5 months to 3.0 months.Four cases were diagnosed as atypical Kawasaki disease. Three cases showed unilateral coronary artery dilation.Two cases showed bilateral coronary artery dilation.Four cases developed multiple organ injuries.Three cases developed macrophage activation syndrome.Three cases developed lung injury.Two cases developed pericardial effusion.One case developed pulmonary hypertension.As for treatment, 3 cases treated with methylprednisolone pulse therapy and methotrexate combined with cyclosporine, improved after the final application of biological agents, and have stopped prednisone. The other 2 cases were treated with adequate oral prednisone and gradually reduced, and methotrexate was added at the same time, 1 case relapsed in the process of reduction. No other vascular involvement was found in 5 cases. Coronary artery dilation recovered completely after 1 to 3 months of treatment. Conclusions: Systemic juvenile idiopathic arthritis combined with coronary artery dilatation has the clinical characteristics of small onset age, long diagnostic time, prone to multiple organ injuries. Corticosteroids and conventional immunosuppressive agents are not sensitive, and biological agents should be used as soon as possible.The prognosis of coronary artery dilation is good after timely treatment.
Arthritis, Juvenile/drug therapy* ; Biological Factors/therapeutic use* ; Child ; Coronary Aneurysm/etiology* ; Coronary Artery Disease/therapy* ; Dilatation ; Dilatation, Pathologic ; Female ; Humans ; Infant ; Male ; Methotrexate ; Prednisone/therapeutic use* ; Retrospective Studies

OBJECTIVE: To study the clinical effect and safety of clopidogrel combined with aspirin in antithrombotic therapy for children with Kawasaki disease (KD) complicated by coronary artery aneurysm (CAA). METHODS: A total of 77 KD children who were diagnosed with multiple small/medium-sized CAAs by echocardiography between January 2013 and June 2018 were enrolled. They were randomly divided into observation group with 38 children (treated with clopidogrel and aspirin) and control group with 39 children (treated with low-molecular-weight heparin and aspirin). All children were followed up regularly, and the first 3 months of the course of the disease was the observation period. The children were observed in terms of the change of the coronary artery and the incidence of complications. RESULTS: At month 3 of follow-up, among the children in the observation group, 6 had normal coronary artery, 11 had coronary artery retraction, 19 had stable coronary artery, and 2 progressed to giant coronary aneurysm; among the children in the control group, 7 had normal coronary artery, 12 had coronary artery retraction, 19 had stable coronary artery, and 1 progressed to giant coronary aneurysm; there was no significant difference in the change of the coronary artery between the two groups (P>0.05). There were 2 cases of epistaxis and 6 cases of skin ecchymosis in the observation group, and 1 case of epistaxis and 7 cases of petechiae and ecchymosis at the injection site in the control group, and no other serious bleeding events were observed in either group. CONCLUSIONS Clopidogrel combined with low-dose aspirin is safe and effective in antithrombotic therapy for children with KD complicated by CAA.
Aspirin ; therapeutic use ; Child ; Clopidogrel ; Coronary Aneurysm ; drug therapy ; etiology ; Coronary Vessels ; Fibrinolytic Agents ; Humans ; Mucocutaneous Lymph Node Syndrome ; complications

The ultimate objective of dyslipidemia management is to prevent and treat atherosclerotic cardiovascular diseases. It is important to combine lifestyle modification together with medication. To determine whether to start drug therapy, comprehensive consideration should be given to both the risk status for atherosclerotic cardiovascular disease and low density lipoprotein cholesterol levels. Drug treatment can be initiated according to whether the patients have coronary artery disease, ischemic cerebral infarction, peripheral artery disease, atherosclerotic artery diseases (abdominal aortic aneurysm, carotid artery stenosis), or diabetes, as well as the number of atherosclerotic risk factors (smoking, hypertension, low high density lipoprotein cholesterol level, family history of premature coronary artery disease, and age). Statin is the first-choice drug for the treatment of hypercholesterolemia. The first goal for drug therapy is to lower the low density lipoprotein cholesterol and the secondary goal is to lower the non-high density lipoprotein cholesterol level. Secondary causes of dyslipidemia should be considered and corrected before starting any medication. These recommendations are based on the new treatment guideline 2015 of Korean Society of Lipidology and Atherosclereosis and Korean Medical Association.
Aortic Aneurysm ; Arteries ; Cardiovascular Diseases ; Carotid Arteries ; Cerebral Infarction ; Cholesterol ; Cholesterol, HDL ; Cholesterol, LDL ; Coronary Artery Disease ; Drug Therapy ; Dyslipidemias* ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Hypercholesterolemia ; Hypertension ; Life Style ; Lipoproteins ; Peripheral Arterial Disease ; Risk Factors

BACKGROUNDCoronary 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.

METHODSWe 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.

RESULTSThe 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.

CONCLUSIONSCALs 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.

Adolescent ; Adrenal Cortex Hormones ; adverse effects ; Child, Preschool ; Coronary Aneurysm ; chemically induced ; Female ; Humans ; Infant ; Logistic Models ; Male ; Mucocutaneous Lymph Node Syndrome ; drug therapy ; Retrospective Studies

A 44-year-old man, who had a history of myocardial infarction (MI) due to thrombotic occlusion of right coronary artery (RCA) aneurysm, visited emergency department presenting with ST-segment elevation myocardial infarction (STEMI). The patient had been on oral anticoagulant therapy (warfarin) from the first thrombotic event, but the medication had been recently changed to aspirin 4 months before the second event. Emergent coronary angiography revealed thrombotic total occlusion of RCA with heavy thrombotic burden from middle RCA to the ostium of the posterior descending branch. Combination pharmacotherapy was performed with anticoagulants (heparin), fibrinolytics (urokinase), and Glycoprotein IIb/IIIa antagonists (abciximab), in addition to mechanical thrombosuction. However, on hospital day 2, the patient complained recurrent chest pain and again underwent coronary angiography, which revealed distal embolization of large thrombus to the posterior lateral branch. Coronary flow was recovered after repeated mechanical thrombosuction was performed. This case has shown the importance of aggressive combination drug therapy, accompanied by mechanical thrombosuction in patient with myocardial infarction due to thrombotic occlusion of coronary artery aneurysm and the importance of unceasing life-long anticoagulant therapy in those particular patients.
Adult ; Aneurysm* ; Anticoagulants ; Aspirin ; Chest Pain ; Coronary Aneurysm ; Coronary Angiography ; Coronary Occlusion* ; Coronary Vessels* ; Drug Therapy ; Drug Therapy, Combination ; Emergency Service, Hospital ; Glycoproteins ; Humans ; Myocardial Infarction* ; Thrombectomy ; Thrombosis ; Warfarin*

OBJECTIVETo improve the awareness of acute coronary artery thrombosis in Kawasaki disease (KD).

METHODSix KD patients with acute coronary artery thrombosis (Jan. 2004 to Jan. 2013) were studied retrospectively. The basic information, clinical manifestations, laboratory data, echocardiography and electrocardiography (ECG), method and consequence of thrombolytic therapy were analyzed.

RESULTThe mean age of patients with coronary artery thrombosis (5 males and 1 female) was (17.2 ± 11.3) months.Five cases had thrombosis in left coronary artery (LCA), and four cases had thrombosis in aneurysm of left anterior descending artery (LAD). One case had thrombosis in both left and right coronary artery (RCA).One case died. Maximum thrombus was about 1.60 cm × 0.80 cm, locating in LAD. The diameter of LCA and RCA was (0.44 ± 0.07) cm and (0.45 ± 0.07) cm. Two patients showed abnormal ECG. Case 3 showed ST segment depression in lead V5. Case 6 showed myocardial infarction.In acute phase of KD, three patients received treatment with intravenous immunoglobin (IVIG), five patients were treated with aspirin.In sub-acute and convalescent phase of KD, all patients were treated with low-dose aspirin.Warfarin and dipyridamole were applied in 5 patients. All cases were treated with thrombolytic therapy using urokinase and/or heparin. After thrombolytic therapy, echocardiography showed thrombolysis in four cases and no change in one.One patient died of myocardial infarction.

CONCLUSIONMost of acute coronary thrombosis in KD occurred in LAD. KD patients with coronary artery thrombosis are at risk of sudden death due to myocardial infarction.

Acute Disease ; Anticoagulants ; administration & dosage ; therapeutic use ; Aspirin ; administration & dosage ; therapeutic use ; Child, Preschool ; Coronary Aneurysm ; diagnosis ; drug therapy ; etiology ; Coronary Thrombosis ; diagnosis ; drug therapy ; etiology ; Echocardiography ; Electrocardiography ; Female ; Fibrinolytic Agents ; administration & dosage ; therapeutic use ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; therapeutic use ; Infant ; Infant, Newborn ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; drug therapy ; Myocardial Infarction ; diagnosis ; etiology ; mortality ; Retrospective Studies

INTRODUCTIONPrior studies have demonstrated evidence of a disparity in the treatment and outcome of male compared to female patients undergoing percutaneous coronary intervention (PCI).

MATERIALS AND METHODSFrom a dedicated database, we retrospectively analysed all consecutive patients with acute coronary syndrome (ACS) admitted to our institution for PCI in 2008. Baseline and procedural characteristics as well as complications were then evaluated for male patients (n = 331) as compared with female patients (n = 137).

RESULTSWomen were noted to be older at the time of presentation (66.1 +/- 10.0 vs 60.7 +/- 11.6 years, P <0.00001), the groups were otherwise well matched in terms of baseline characteristics. Female patients were treated with significantly smaller diameter stents (2.86 +/- 0.44 vs 2.96 +/- 0.50 mm, P = 0.04), though the proportion of drug-eluting stents was similar (53.7% vs 50.5%, P = 0.5). Female patients were significantly less likely to receive optimal medical therapy with lesser use of glycoprotein IIb/IIIa inhibitor (26.3% vs 55.3%, P <0.0000001), and beta-blockers (83.9% vs 90.9%, P = 0.04). At 30 days, there were no differences in the rate of major adverse cerebrovascular or cardiac events (2.9% vs 3.9%, P = 0.8), though females had a significantly higher rate of femoral access site pseudoaneurysm (4.4% vs 0.9%, P = 0.02).

CONCLUSIONSThere remains evidence for continued gender disparity in contemporary practice; despite evidence for efficacy in ACS patients, females received a notably lower use of glycoprotein IIb/IIIa inhibitors and beta-blockers. Women are also significantly more likely to develop femoral access site complications with pseudoaneurysm development; it is important therefore to optimise procedures for sheath removal in female patients or give strong consideration to the use of radial access site.

Acute Coronary Syndrome ; drug therapy ; surgery ; Age Factors ; Aged ; Aneurysm, False ; epidemiology ; etiology ; Angioplasty, Balloon, Coronary ; adverse effects ; Drug-Eluting Stents ; utilization ; Electrocardiography ; Female ; Humans ; Incidence ; Male ; Middle Aged ; Platelet Aggregation Inhibitors ; therapeutic use ; Postoperative Complications ; epidemiology ; Sex Factors ; United Kingdom ; epidemiology