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

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

OBJECTIVEThe aim of the study was to review the cases of Kawasaki Disease (KD) and analyze the clinical features especially their cardiac complications.

METHODSTotally 283 patients with KD were hospitalized from 1992 to 2002. Their clinical features and factors associated with increased risk of coronary artery aneurysms were reviewed.

RESULTS(1) Among the 283 KD patients, 186 were male and 97 were female. The male-female ratio was 1.9:1. Most of them (71%) were younger than 3 years old. Seasonal peak was in spring and summer (from May to Aug). Depending on the criteria of KD, 228 (81%) were diagnosed as typical KD and 55 (19.4%) were atypical KD. All patients had fever, lasting for 6.1 days. The most common clinical features were oral mucosal changes (97.5%) and cervical lymphadenopathy (95.4%), conjunctivitis (91.2%). And changes in the extremities (89.8%) and rash (81.5%) were also noted. (2) Before the treatment, coronary artery abnormalities were seen in 103/279 (36.9%), which occurred within 4 - 30 days of fever onset. Two weeks after intravenous gamma globulin (IVIG) treatment, the new cases of coronary artery abnormalities were 28/211 (13.3%). The prevalence of coronary artery aneurysms (CAA) with KD was 4.7%. The risk factors of CAA were male cases (P < 0.05) and fever lasting longer than 9 days (P < 0.05). Other cardiac abnormalities in acute phase included left atrial and ventricular enlargement (40/279, 14.3%) and changes in ECG (57/274, 20.8%). The pericardial effusions were found in 11 cases (3.9%).

CONCLUSIONSCardiac complications of KD occurred in the early period of KD. The new cases of coronary artery abnormalities were 13.3% after IVIG treatment. The risk factors of CAA included male cases and fever lasting for longer time.

Child, Preschool ; Coronary Aneurysm ; epidemiology ; etiology ; Female ; Heart Diseases ; diagnosis ; etiology ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; drug therapy ; Prevalence ; Risk Factors

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 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

Kawasaki disease is a systemic vasculitis, mainly encountered in children. It may affect any organ. Acute cholestasis and severe obstructive jaundice is an atypical manifestation of the disease. We herein present two children with Kawasaki disease and severe direct hypebilibirunemia who also were homozygous and heterozygous respectively for the (TA)7 promoter polymorphism of Gilbert syndrome. Intravenous immunoglobulin was administered to both patients at the acute phase of the disease and the fever remitted within 24 hr following the immunoglobulin administration. Furthermore oral aspirin at a dose of 80-100 mg/kg/24 hr was also given. The first child did not develop any coronary ectasia or aneurysm, whereas dilation of the right coronary artery was identified in the second child, one month after the disease onset. We discuss the possible contribution of Gilbert syndrome to the development of jaundice in our patients.
Administration, Oral ; Aspirin/therapeutic use ; Child ; Child, Preschool ; Echocardiography ; Female ; Gilbert Disease/*complications/*diagnosis/genetics ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Jaundice/etiology ; Male ; Mucocutaneous Lymph Node Syndrome/*complications/*diagnosis/drug therapy ; Polymorphism, Single Nucleotide ; Promoter Regions, Genetic ; Sequence Analysis, DNA

Child, Preschool ; Coronary Aneurysm ; diagnosis ; etiology ; prevention & control ; Diagnosis, Differential ; Early Diagnosis ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; therapeutic use ; Immunologic Factors ; administration & dosage ; therapeutic use ; Infant ; Mucocutaneous Lymph Node Syndrome ; classification ; complications ; diagnosis ; drug therapy ; Prognosis

Aspirin ; administration & dosage ; therapeutic use ; Brain ; diagnostic imaging ; pathology ; Cerebral Infarction ; diagnosis ; drug therapy ; etiology ; Epilepsy ; diagnosis ; drug therapy ; etiology ; Humans ; Immunoglobulins, Intravenous ; administration & dosage ; therapeutic use ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; drug therapy ; Tomography, X-Ray Computed

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

Cardiac magnetic resonance (CMR) imaging is now widely used in several fields of cardiovascular disease assessment due to recent technical developments. CMR can give physicians information that cannot be found with other imaging modalities. However, there is no guideline which is suitable for Korean people for the use of CMR. Therefore, we have prepared a Korean guideline for the appropriate utilization of CMR to guide Korean physicians, imaging specialists, medical associates and patients to improve the overall medical system performances. By addressing CMR usage and creating these guidelines we hope to contribute towards the promotion of public health. This guideline is a joint report of the Korean Society of Cardiology and the Korean Society of Radiology.
Cardiomyopathies/diagnosis/radiography ; Cardiotonic Agents/therapeutic use ; Chest Pain/complications/diagnosis/radiography ; Coronary Artery Bypass ; Coronary Artery Disease/*diagnosis/drug therapy/radiography ; Dobutamine/therapeutic use ; Echocardiography ; Heart Defects, Congenital/diagnosis/radiography ; Heart Failure/diagnosis/ultrasonography ; Humans ; *Magnetic Resonance Imaging ; Mucocutaneous Lymph Node Syndrome/complications/diagnosis ; Percutaneous Coronary Intervention ; Prognosis ; Risk Assessment ; Ventricular Function, Left/physiology