Kawasaki disease (KD) is an acute multisystem vasculitic syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age. In developed countries, KD is currently the leading cause of acquired heart diseases in children. The incidence of KD is increasing globally. The first generation of patients with KD has now reached reproductive age, and the incidence of 2-generation cases may thus increase in number. Many gene polymorphisms are associated with KD in different populations. There are many individual and racial gene variation. Susceptible gene should be identified in the KD. Individuals with certain genes predisposing to occurrence and severity of KD could be detected in different ethnic populations. This might allow special treatment or other measures to prevent the disease and possibly limit the severity and complications of KD. I reviewed and summarized Korean and international articles about gene studies. Multicenter gene studies are needed to investigate the association between specific genes and coronary artery lesions in KD.
Child ; Coronary Vessels ; Developed Countries ; Heart Diseases ; Humans ; Incidence ; Infant ; Mucocutaneous Lymph Node Syndrome*

Evidence-based medicine is a medical movement combining professional experience with the best scientific research results. Treatment of Kawasaki disease in the acute phase is directed at reducing inflammation in the coronary artery wall and preventing coronary thrombosis, whereas long-term therapy in individuals who develop coronary aneurysms is aimed at preventing myocardial ischemia or infarction. I attempt to summarize the current state of knowledge of the medical management of patients with Kawasaki disease. The recommendations are evidence-based and derived from published data wherever possible.
Coronary Aneurysm ; Coronary Thrombosis ; Coronary Vessels ; Evidence-Based Medicine ; Humans ; Infarction ; Inflammation ; Mucocutaneous Lymph Node Syndrome* ; Myocardial Ischemia

Kawasaki disease (KD) is an acute systemic febrile illness, predominantly affecting children less than the age of 5 years. After its first description in 1967 by Dr Tomisaku Kawasaki, KD has become the leading cause of acquired heart diseases in children in developed nations. KD has been reported in all racial and ethnic groups but the incidence of KD is more common among Asians, and the incidence of the disease varies considerably among different Asian countries. There is a higher rate of KD reported in Asian countries such as Japan and Korea than in other countries. which suggests differences of race-specific susceptibility. In Korea, from 1990s, a nationwide epidemiologic study has been conducted every 3 years by the Korean Pediatric Heart Association to determine the epidemiologic patterns and incidence rate of KD in Korea. So the average annual incidence, 105.0/100,000 children < 5 years in recent study (2003-2005) was the second highest reported rate in the world after Japan.
Asian Continental Ancestry Group ; Child ; Developed Countries ; Epidemiologic Studies ; Epidemiology ; Ethnic Groups ; Heart ; Heart Diseases ; Humans ; Incidence ; Japan ; Korea* ; Mucocutaneous Lymph Node Syndrome* ; Prevalence

Congenital unilateral pulmonary vein atresia is a very rare cardiac malformation. In this case, the patient was brought to the hospital for recurrent upper respiratory infection without exertional dyspnea or hemoptysis, and cardiac murmurs were heard on physical examination. The volume of affected lung was lost on simple chest x-ray. Lung perfusion scan revealed no visualization of affected lung. Cardiac catheterization revealed mild pulmonary artery hypertension. Pulmonary angiography showed a small right pulmonary artery, stasis of contrast material and venous phase revealed nonvisualization of draining right pulmonary veins. In this case, the patient did not undergo any surgical treatment for no symptoms other than recurrent upper respiratory infection were reported. We report a case of congenital right pulmonary vein atresia.
Angiography ; Cardiac Catheterization ; Cardiac Catheters ; Dyspnea ; Heart Murmurs ; Hemoptysis ; Humans ; Hypertension ; Lung ; Perfusion ; Physical Examination ; Pulmonary Artery ; Pulmonary Veins* ; Thorax

Rheumatic fever with renal involvement is rarely seen. The prevalence is found to be low although it may differ by reporters. We report a case of rheumatic fever with IgA nephropathy. The patient was a 14-year-old male. The patient's chief complaint were fatigue and gross hematuria. On the physical examination, the pansystolic murmur (grade II) on the left lower sternal border was noted. Cardiomegaly was seen on chest X-ray and pulmonary vasculatures were increased. Prolonged PR intervals were found on EKG. Echocardiogram showed rheumatic carditis with grade mild to moderate MR, regurgitant shunt from LV to LA through anterior leaflet. On the laboratory findings, ESR, CRP and ASO were increased, C3, C4 and IgA were normal. On the urine analysis, gross hematuria was noted as RBC over 30/HPF and RBC dysmorphism 90%. On the immunofluoroscent microscope of renal biopsy, IgA deposit on mesangial matrix was noted.
Adolescent ; Biopsy ; Cardiomegaly ; Electrocardiography ; Fatigue ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Male ; Myocarditis ; Physical Examination ; Prevalence ; Rheumatic Fever* ; Thorax

Kawasaki disease is an acute febrile mucocutanenous syndrome associated with multisystemic vasculitis. The etiology of Kawasaki disease is still unknown therefore this disease can be diagnosed only based upon clinician's recognition of a symptom pattern which includes high fever, oral cavity changes, polymorphous skin rash, conjuctival injections, cervical lymphadenopathy, and swelling of peripheral extremities. Early diagnosis and treatment of Kawasaki disease is critical to reduce the risk of cardiac complications such as coronary aneurysm. However there is no specific diagnostic tools for Kawasaki disease, unusual clinical manifestation resembling infectious disease involving cervical lesion often leads to delay in appropriate diagnosis and treatment of Kawasaki disease. We report a ten-year-old girl with Kawasaki disease whose initial presentation mimicking peritonsillar abscess.
Communicable Diseases ; Coronary Aneurysm ; Diagnosis ; Early Diagnosis ; Exanthema ; Extremities ; Female ; Fever ; Humans ; Lymphatic Diseases ; Mouth ; Mucocutaneous Lymph Node Syndrome* ; Peritonsillar Abscess* ; Vasculitis

PURPOSE: To standardize hospital management of patients with simple congenital heart disease (CHD), we developed a protocol called the 'clinical pathway (CP)'and evaluated quality improvement in patient outcomes. METHODS: The study included a group of 60 patients with simple CHD who had surgery between June 1 and October 31, 1998. The control group included 48 patients who had surgery for the same disease during the corresponding time in 1997. Two CPs were developed according to state and place of residence of patients. Information on patients was reviewed including: education, physical examination, hospital stay, type of care and date of discharge from the hospital. The effect of each protocol on standard procedures was reviewed. After patient discharge additional performance information was assessed including: procedures used, duration of hospital stay, medical cost, treatment outcome, complications and parent satisfaction. RESULTS: The duration of hospital stay prior to surgery (1.8 days vs. 3.0 days), the ICU stay (1.3 days vs. 3.6 days), number of ward days after surgery (5.3 days vs. 7.9 days) and total number of hospital days (8.5 days vs. 14.7 days) were significantly shortened in the study group compared to the control group. The intubation time (P=0.000) and the frequency of laboratory studies (P=0.000) during the hospitalization decreased in the study group compared to the control group. The average medical costs of cases was also significantly lower in the study group (P=0.001). There were no deaths, readmissions or serious complications in the study group. A questionnaire survey of patient family members showed that they were satisfied with care; with more than 85 percent response rate to questionnaire items. CONCLUSION: Application of our new CP protocol shortens hospital stay and reduces medical costs while improving the quality of care for patients and their families. This CP protocol should now be considered for more complex CHD.
Critical Pathways* ; Health Care Costs ; Heart Defects, Congenital ; Heart* ; Hospitalization ; Humans ; Intubation ; Length of Stay ; Parents ; Patient Discharge ; Physical Education and Training ; Quality Improvement ; Surveys and Questionnaires ; Thoracic Surgery*

PURPOSE: The optimal surgical approach for aortic arch anomaly associated complex intracardiac defects is not universally agreed upon. We reviewed our recent experience of one-stage repair for the aortic arch anomaly associated with complex intracardiac defects. METHODS: We retrospectively analyzed the medical records of 15 consecutive patients with arch anomaly associated with complex intracardiac defects underwent going a one-stage repair through median sternotomy between September 2000 and May 2004. Transposition of the great artery or Taussig-Bing was associated in 9 patients, truncus arteriosus in 2, Shone' complex in 2, aorticopulmonary window in 1, and double outlet right ventricle in 1 patient. Aortic arch anomalies were coartation in 9 patients and interruption in 6. Age at operation ranged from 3 days to 23.4 months (median 12.5 days) and body weight ranged from 2.3 to 10.3 kg (mean 3.7+/-1.9 kg). RESULTS: There were 2 early deaths (13.3%) in the patients with Taussig-Bing anomaly and Shone's complex respectively. The causes of deaths were sepsis with right heart failure and pulmonary edema respectively. There were 2 late deaths. A patient with truncus arteriosus died suddenly of unknown cause and the other patient with Taussig-Bing anomaly died of ventricular dysfunction. One of 2 patients who died lately underwent balloon aortoplasty with success. There was no residual or recurrent coarctation in the rest of the patients. A patient required reoperation for left pulmonary artery stenosis. All survivors were in NYHA functional class I. CONCLUSION: One-stage repair of aortic arch with complex intracardiac defects can be performed with acceptable operative mortality. This procedure is strongly recommended as considering their grave prognosis and adverse effects of staged repair.
Aorta, Thoracic* ; Arteries ; Body Weight ; Cause of Death ; Constriction, Pathologic ; Double Outlet Right Ventricle ; Heart Failure ; Humans ; Medical Records ; Mortality ; Prognosis ; Pulmonary Artery ; Pulmonary Edema ; Reoperation ; Retrospective Studies ; Sepsis ; Sternotomy ; Survivors ; Truncus Arteriosus ; Ventricular Dysfunction

No abstract available.
Adult* ; Ebstein Anomaly* ; Humans ; Korea*

No abstract available.
Ebstein Anomaly*