We reported a pediatric case of Kawasaki disease complicated with mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) from Beijing Tsinghua Changgung Hospital. The clinical data were retrospectively analyzed and the related literature was reviewed. The clinical features, treatment and prognosis of the disease were summarized to improve recognition of Kawasaki disease complicated with MERS. A 7-year-old boy was diagnosed with Kawasaki disease due to continuous high fever for 6 d, accompanied by strawberry tongue, conjunctival congestion, erythema-like hyperemia rash, and cervical enlarged lymph nodes. And treatment was started with intravenous immunoglobulin (IVIG: 2 g/kg) and oral aspirin [40 mg/(kg·d)]. Twenty-four hours after the treatment of IVIG, the patient' s fever persisted and in addition he developed headache and drowsiness. His cranial magnetic resonance imaging (MRI) demonstrated a localized lesion in the splenium of the corpus callosum with high intensity signal on diffusion-weighted images (DWI) and T2-weighted, and low intensity signal on apparent diffusion coefficient (ADC) and T1-weighted. Based on these findings, he was diagnosed with MERS-complicated Kawasaki disease. Methylprednisolone [2 mg/(kg·d)] treatment was started intravenously, and within several hours he was afebrile and the neurological symptoms disappeared. A follow-up MRI was conducted after 1 week was normal. He was discharged without any neurological sequelae and coronary artery lesions. A total of 12 qualified foreign literature were retrieved, with no Chinese literature searched. Seventeen children were reported, the median age was 6.5 years (range: 1-14 years), among them 11 cases were children over 5 years old, and 4 cases were complicated with coronary artery lesions. All children had neurological symptoms, such as consciousness disorder, visual hallucination or convulsion. MRI conformed to MERS imaging changes. After active treatment, the neurological manifestations and radiological abnormalities completely disappeared, leaving no neurological sequelae. Kawasaki disease complicated with MERS had not been reported in China by now. Literature that identified Kawasaki disease complicated with MERS mostly occurred in children over 5 years old. Cranial MRI examination is helpful for early diagnosis. Timely treatment can reverse MERS in a short time, without neurological sequelae left.
Brain Diseases/diagnosis* ; Child ; Child, Preschool ; Encephalitis/pathology* ; Fever ; Humans ; Hyperplasia/complications* ; Immunoglobulins, Intravenous/therapeutic use* ; Magnetic Resonance Imaging ; Male ; Mucocutaneous Lymph Node Syndrome/diagnosis* ; Retrospective Studies

INTRODUCTION: Kawasaki disease (KD) is a challenging diagnosis. Erythema and induration of the Bacillus Calmette-Guérin (BCG) site is increasingly recognised as a significant clinical clue. However, there is little data to support its specificity for KD as compared to other febrile illnesses. We aimed to evaluate BCG reaction or induration as a diagnostic tool for KD. METHODS: A retrospective case-controlled study of patients discharged with a diagnosis of KD from 2007 to 2010 was conducted. Another group of patients admitted over the same period for possible KD, but later found not to have KD, served as control. RESULTS: Significantly more infants with KD (69.7%) had BCG site changes than older children (27.8%; p < 0.001). It also presented earlier in the course of KD; < 5 days (53.3%) compared to ≥ 5 days of fever (30.0%; p < 0.001). Positive predictive value of BCG site reaction or induration for KD was 90.8% (95% confidence interval [CI] 0.819-0.962) for infants and 96.2% (95% CI 0.868-0.995) for older children. The prevalence rate of changes at the BCG site was 9.9% among patients with non-KD febrile illnesses and 42.6% among patients with KD. CONCLUSION BCG site reaction or induration is a useful clinical clue for the diagnosis of KD in both infants and older children, with a higher prevalence in infants. Physicians should consider KD in children with febrile illness and redness or crust formation at the BCG site, especially in view of low rates of BCG reaction or induration in non-KD febrile illnesses.
BCG Vaccine ; administration & dosage ; adverse effects ; Case-Control Studies ; Child, Preschool ; Erythema ; complications ; epidemiology ; Female ; Fever ; complications ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; epidemiology ; Risk Factors ; Singapore ; epidemiology

Kawasaki disease (KD) is characterized with acute systemic vasculitis, occurs predominantly in children between 6 months to 5 years of age. Patients with this disease recover well and the disease is self-limited in most cases. Since it can lead to devastating cardiovascular complications, KD needs special attention. Recent reports show steady increases in the prevalence of KD in both Japan and Korea. However, specific pathogens have yet to be found. Recent advances in research on KD include searches for genetic susceptibility related to KD and research on immunopathogenesis based on innate and acquired immunity. Also, search for etiopathogenesis and treatment of KD has been actively sought after using animal models. In this paper, the recent progress of research on KD was discussed.
*Genetic Predisposition to Disease ; Heart Diseases/*complications ; Humans ; Mucocutaneous Lymph Node Syndrome/*diagnosis/etiology/physiopathology/therapy

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

Child ; Coronary Aneurysm ; diagnosis ; etiology ; surgery ; Humans ; Male ; Mucocutaneous Lymph Node Syndrome ; complications

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 Surgical Procedures ; methods ; Child ; Child, Preschool ; Combined Modality Therapy ; Coronary Aneurysm ; diagnosis ; etiology ; therapy ; Coronary Artery Disease ; diagnosis ; etiology ; therapy ; Coronary Thrombosis ; prevention & control ; Echocardiography ; Humans ; Mucocutaneous Lymph Node Syndrome ; complications ; Platelet Aggregation Inhibitors ; therapeutic use ; Thrombolytic Therapy ; methods ; Troponin I ; analysis

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

We report a 38-year-old man with previous Kawasaki disease who presented with exertional dyspnoea and was found to have multivessel coronary aneurysm and stenoses on coronary angiography. Coronary artery bypass surgery was subsequently performed. This case highlights the angiographic and intravascular ultrasonographic imaging features of this relatively uncommon condition presenting to an adult general cardiology service in Singapore.
Adult ; Coronary Aneurysm ; diagnosis ; etiology ; Coronary Angiography ; methods ; Coronary Stenosis ; diagnosis ; etiology ; Coronary Vessels ; diagnostic imaging ; Diagnosis, Differential ; Humans ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; Ultrasonography, Interventional ; methods

Kawasaki disease is a common paediatric vasculitide. It is usually diagnosed by its classical constellation of mucocutaneous signs. Recurrent Kawasaki disease is a rare phenomenon that occurs in approximately 3% of all patients diagnosed with Kawasaki disease. Its presentation is usually similar to the first episode of Kawasaki disease, and early diagnosis with prompt treatment is key in preventing associated cardiovascular morbidities. Recurrent Kawasaki disease is not well reported, and atypical presentations have not been previously reported in medical literature. Here, we report the case of a young girl with recurrent Kawasaki disease who presented atypically with acute airway obstruction secondary to retropharyngeal phlegmon.
Acute Disease ; Airway Obstruction ; diagnosis ; etiology ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Lymph Nodes ; diagnostic imaging ; pathology ; Mucocutaneous Lymph Node Syndrome ; complications ; diagnosis ; Neck ; Recurrence ; Tomography, X-Ray Computed