Kawasaki disease, an acute febrile vasculitis of unknown etiology, is usually treated with high doses of immunoglobulin (IVIG) and aspirin. However, 20% of children show persistent or recurrent fever despite IVIG, and coronary artery aneurysm progression. In such cases of resistance to IVIG treatment, repeated IVIG administration or the initiation of steroid therapy, and the use of cyclophosphamide have been reported. We aimed to show in this study that methotrexate (MTX) may be used as a treatment for Kawasaki disease resistant to IVIG treatment. We report the case of a 6-year old boy who was admitted at another hospital with an initial complaint of a fever for 5 days and skin rashes for 3 days. The patients fever persisted despite three courses of IVIG (2 gm/kg, 1 gm/kg, 1 gm/kg, respectively) over a 14-day period. On day 14 of his illness he showed a dilated right coronary artery, and on day 19 dexamethasone, at a daily dose of 0.3 mg/kg, was given but this resulted in defervescence. However, upon stopping the dexamethasone treatment, his fever recurred and he was transferred to our hospital. On days 31 and 38 of his illness, IVIG (400 mg/kg for 5 days, twice) was administered and from day 38 onwards the patient was given dexamethasone (0.6 mg/kg, daily) and MTX (10 mg/BSA, once weekly) whereupon his fever subsided and did not recur. On day 48 dexamethasone was replaced with prednisolone, which was subsequently tapered. The patient is now taking MTX and being observed on an outpatient basis. We report the case of a boy with IV-globulin resistant Kawasaki disease, who after repeated infusions of IVIG and steroid therapy showed fever recruuence, which that subsided after MTX treatment.
Case Report ; Child ; Human ; Immunoglobulins, Intravenous/*therapeutic use ; Male ; Methotrexate/*therapeutic use ; Mucocutaneous Lymph Node Syndrome/complications/*drug therapy

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; Pneumonia, Mycoplasma ; diagnosis ; drug therapy ; etiology

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

Kawasaki disease (KD) is an acute self-limiting vasculitis, and it is the most common cause of acquired heart disease in children under 5 years old. One of the improvement goals in pediatric quality control work for the year 2023, as announced by the National Health Commission, is to reduce the incidence of cardiac events and KD-related mortality in children with KD. In order to standardize the diagnosis, treatment, and long-term management practices of KD in China, and effectively prevent and reduce the incidence of coronary artery lesions and long-term adverse effects, the guideline working group followed the principles and methods outlined by the World Health Organization and referenced existing evidence and experiences to develop the "Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023)". The guidelines address the clinical questions regarding the classification and definition of KD, diagnosis of different types of KD, treatment during the acute phase of KD, application of echocardiography in identifying complications of KD, and management of KD combined with macrophage activation syndrome. Based on the best evidence and expert consensus, 20 recommendations were formulated, aiming to provide guidance and decision-making basis for healthcare professionals in the diagnosis and treatment of KD in children.
Child ; Humans ; Child, Preschool ; Mucocutaneous Lymph Node Syndrome/complications* ; Vasculitis/drug therapy* ; Heart ; Heart Diseases ; China ; Immunoglobulins, Intravenous/therapeutic use*

OBJECTIVES: To investigate the clinical features and treatment strategies of multisystemic inflammatory syndrome in children (MIS-C) after severe acute respiratory syndrome coronavirus 2 infection. METHODS: A retrospective analysis was performed on the medical data of four children with MIS-C who were admitted to the Department of Cardiology, Xuzhou Children's Hospital, Xuzhou Medical Universityfrom January to February 2023. RESULTS: All four children had multiple organ involvements and elevated inflammatory markers, with a poor response to standard therapy for Kawasaki disease after admission. Two children were treated with intravenous immunoglobulin therapy pulse therapy twice, and all four children were treated with glucocorticoids. The children had a good prognosis after the treatment. CONCLUSIONS MIS-C often appears within 4-6 weeks or a longer time after severe acute respiratory syndrome coronavirus 2 infection, and anti-inflammatory therapy in addition to the standard treatment regimen for Kawasaki disease can help to achieve a favorable treatment outcome.
Child ; Humans ; COVID-19/complications* ; SARS-CoV-2 ; Mucocutaneous Lymph Node Syndrome/drug therapy* ; Retrospective Studies ; Systemic Inflammatory Response Syndrome/therapy*

Kawasaki disease (KD) is a self-limited systemic inflammatory illness, and coronary artery lesions (CALs) are a major complication determining the prognosis of the disease. Epidemiologic studies in Asian children suggest that the etiologic agent(s) of KD may be associated with environmental changes. Laboratory findings are useful for the diagnosis of incomplete KD, and they can guide the next-step in treatment of initial intravenous immunoglobulin non-responders. CALs seem to develop in the early stages of the disease before a peak in inflammation. Therefore early treatment, before the peak in inflammation, is mandatory to reduce the risk of CAL progression and severity of CALs. The immunopathogenesis of KD is more likely that of acute rheumatic fever than scarlet fever. A hypothetical pathogenesis of KD is proposed under the premise of a "protein homeostasis system"; where innate and adaptive immune cells control pathogenic proteins that are toxic to host cells at a molecular level. After an infection of unknown KD pathogen(s), the pathogenic proteins produced from an unknown focus, spread and bind to endothelial cells of coronary arteries as main target cells. To control the action of pathogenic proteins and/or substances from the injured cells, immune cells are activated. Initially, non-specific T cells and non-specific antibodies are involved in this reaction, while hyperactivated immune cells produce various cytokines, leading to a cytokine imbalance associated with further endothelial cell injury. After the emergence of specific T cells and specific antibodies against the pathogenic proteins, tissue injury ceases and a repair reaction begins with the immune cells.
Animals ; Coronary Artery Disease/drug therapy/etiology/metabolism ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Mucocutaneous Lymph Node Syndrome/complications/*diagnosis/drug therapy/metabolism

Kawasaki disease is a multisystem disorder with varying clinical expression. This is a report on one case of Kawasaki disease which during its clinical course developed facial nerve palsy and spontaneous recovery without specific treatment. It is hoped that this report will serve to remind physicians of the association of facial nerve paralysis with Kawasaki disease.
Aspirin/administration & dosage ; Case Report ; Facial Paralysis/*etiology ; Female ; Gamma-Globulins/administration & dosage ; Human ; Infant ; Mucocutaneous Lymph Node Syndrome/*complications/diagnosis/drug therapy

OBJECTIVETo investigate the clinical features of recurrent Kawasaki disease (KD) and the relationship of recurrent KD with coronary artery lesions.

METHODSThe medical data of 20 children with recurrent KD who were admitted to the Children's Hospital from January 1998 to May 2007 were retrospectively studied. Their clinical features were compared with those of children with initial KD.

RESULTSThe incidence of recurrent KD was 1.34% (20/1489). KD relapsed 2 months to 4.6 years (average: 1.2 years) after the first episode in the 20 children. Compared with the initial KD group, the clinical symptoms in the recurrent KD group were incomplete, complicated and less severe. The period of fever, platelet count, C-reactive protein and ESR were remarkably reduced in the recurrent KD group, but the incidence of coronary artery lesions increased significantly compared with the initial KD group (40% vs 25%; P<0.05).

CONCLUSIONSThe clinical symptoms of recurrent KD were incomplete in children. Recurrent KD was associated with an increased incidence of coronary artery lesions.

Child ; Child, Preschool ; Coronary Artery Disease ; etiology ; Female ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; drug therapy ; Recurrence ; Retrospective Studies

OBJECTIVETo investigate the expression of soluble intercellular adhesion molecule-1 (sICAM-1) and itd significance in children with intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD).

METHODSA total of 271 children with KD who received IVIG treatment (including 252 IVIG-sensitive cases and 19 IVIG-resistant cases) were selected in the study; 78 of the 271 children had coronary artery dilation. Thirty-six age-matched healthy children were selected as the control group. Plasma sICAM-1 levels were measured using enzyme-linked immunosorbent assay. White blood cell count (WBC), neutrophil count, C-relative protein (CRP), aspartate aminotransferase(AST), serum sodium, and serum potassium were measured by laboratory tests.

RESULTSBefore IVIG treatment, the IVIG-sensitive cases and IVIG-resistant cases had significantly higher sICAM-1 levels than the control group (P<0.05), and the IVIG-resistant cases had significantly higher sICAM-1 levels than the IVIG-sensitive cases (P<0.05). After 24-48 hours of IVIG treatment, the IVIG-resistant cases had significantly higher sICAM-1 levels than the IVIG-sensitive cases (P<0.05). Before IVIG treatment, among the IVIG-sensitive cases, the sICAM-1 level was significantly higher in those with coronary artery dilation than in those without coronary artery dilation (P<0.05); among the IVIG-resistant cases, the sICAM-1 level was significantly higher in those with coronary artery dilation than in those without coronary artery dilation (P<0.05). In the IVIG-resistant cases, sICAM-1 level was positively correlated with WBC (before and after treatment) (r=0.7562, P<0.01; r=0.8435, P<0.01) and CRP (after treatment) (r=0.8936, P<0.01).

CONCLUSIONSHigh sICAM-1 level may be used as a risk factor for resistance to IVIG and coronary artery dilation in children with KD.

Child ; Child, Preschool ; Coronary Artery Disease ; blood ; Female ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Infant ; Intercellular Adhesion Molecule-1 ; blood ; Male ; Mucocutaneous Lymph Node Syndrome ; blood ; complications ; drug therapy

OBJECTIVETo study the clinical characteristics of pediatric Kawasaki disease complicating mycoplasma pneumoniae pneumonia.

METHODSRetrospective analysis was conducted on 11 children who had been diagnosed with Kawasaki disease with Mycoplasma pneumoniae pneumonia.

RESULTSThe 11 cases presented with varying degrees of fever, conjunctival congestion, skin rashes, lymphadenectasis, distal extremities lesions, heart and lung lesions. 8 of them were standartly treated with azithromycin, while 3 of them were treatad with azithromycin and erythromycin. 2 patients with pleural effusion complicated by lobar pneumonia consolidation were treated with gamma globulin combined aspirin. All of the 11 patients were healed.

CONCLUSIONInfections are common at the diagonosis of KD. Reasonable examination and antibiotics is useful to cure KD with MPP.

Child ; Child, Preschool ; Female ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; Pneumonia, Mycoplasma ; diagnosis ; drug therapy ; etiology ; Retrospective Studies