Kawasaki disease is a vasculitis syndrome of unknown cause, first reported by Tomisaku Kawasaki in 1967. It is the leading cause of acquired heart disease in developed countries. Because the causes of Kawasaki disease have not been identified, there are no specific therapies for Kawasaki disease patients. Now, intravenous immunoglobulin (2 g/kg single infusion) plus aspirin (30 mg/kg/day) therapy is considered to be the standard therapy. However, 20% of the patients do not become afebrile despite completion of the intravenous immunoglobulin therapy; these intravenous immunoglobulin non-responders are considered to be high risk for coronary artery lesion. Recently, new models to predict intravenous immunoglobulin non-responders were developed in Japan. Risk stratification of initial therapy for Kawasaki disease patients became not a vision, but a fact. In this review, I explain the history of acute therapy for Kawasaki disease patients, the most recent therapeutic strategy, and future perspectives.
Background: Since the Amplatzer Septal Occluder (ASO) was approved in Japan on March 2005, more than 1000 procedures have been attempted as of July 2009. Meanwhile severe complications had occurred, including 2 erosion cases and 6 migrations. Methods: To investigate the efficacy and the safety of percutaneous ASD closure, 115 cases of ASD closure with the ASO device between August 2006 and July 2009 were compared with 55 surgical closure cases retrospectively. Results: The age was 9 ± 4 years for the device group and 6 ± 3 years for the surgical group (p < 0.0001). The body weight was 29 ± 15 kg for the device group and 19 ± 9 kg for the surgery group (p < 0.0001). The procedural attempt success rate of device closure was 98%. The defect size of ASD was 15 ± 4 mm measured with the sizing balloon and 18 ± 4 mm for the surgery group (p < 0.001). The success rate was 100% for both groups. The mean length of hospital stay was 5 days for the device group and 6 days for the surgery group (p < 0.001). The mean estimated cost per case was 1.4 ± 0.4 × 106 Japanese yen for the device closure group and 1.6 ± 0.3 × 106 Japanese yen for the surgery group. There was no erosion or severe complications among the device group. Mortality was 0% for both groups. Conclusions: A total of 2/3 of the ASD cases that received transcatheter closure depended on the size of the defect and the rims. Transcatheter closure of secundum ASD using ASO is a safe and effective alternative therapy.
Purpose: (1) The purpose of this paper is to identify the relationship between social independence and psychological profiles in adult congenital heart disease patients with physically disabled patients certificate and (2) to identify patients' difficulty, anxiety, and demands regarding social life. Method: A total of 143 patients with physically disabled patients certificates older than 15 years of age were selected for this study from data obtained by a questionnaire survey conducted by the patients association. In the questionnaire, each participant was asked about employment status, income, receipt status of disability pension as an index of social independence, and financial and psychological distress as an index of psychological status. Furthermore, each participant was asked to freely describe any difficulty, anxiety, and demands with regard to social life. Result: The ages of the subjects ranged from 15-73 years. Of the total, 71 patients (50%) were females, and 94 patients (66%) possessed the first grade physically disabled person's certificate. Fifty-nine patients (41%) were employed, and of the employed, the annual income of 34 patients (58%) was lower than two million Japanese yen per year. The frequency of hospital visits, low total household income, low individual income of the patient, dissatisfaction toward work, and receipt of the disability pension were related to the psychological profiles. In the free descriptions, the participants desired better pension systems, support for medical fees, and employment support systems. Conclusion: Financial issues can affect the patient's psychological profiles. It is a pressing need to enhance the social welfare and employment support systems.
Background: Along with cell infiltration, exacerbated damage to the extracellular matrix (ECM) plays a major role in the development of coronary artery lesions in Kawasaki disease. Known for their antihypertensive effects, angiotensin receptor blockers (ARB) also act to suppress ECM damage; however, there are no reports at present regarding the efficacy of ARB for Kawasaki disease. Methods: Four-week-old male C57/BL6 mice were intraperitoneally injected with 0.5 mg Lactobacillus casei cell wall extract (LCWE group; n = 10) or Phosphate-Buffered Saline (PBS group; n = 10). As a treatment group (LCWE + ARB group, n = 10), mice with intraperitoneal injection of LCWE were fed ARB-containing water (100 mg/l). Four weeks after the administration of LCWE, the degree of vasculitis was histologically evaluated. Matrix metalloproteinase-2 (MMP-2), matrix metalloproteinase-9 (MMP-9), and interleukin-6 (IL-6) mRNA expressions at the aortic root were also studied using real-time polymerase chain reactions (PCR; n = 5). Results: Histological examination revealed that LCWE-induced coronary arteritis was ameliorated by ARB as shown by decreased mononuclear cell infiltration and activated macrophage infiltration. Consistent with that result, real-time PCR demonstrated that increased mRNA expressions of MMP-9 and IL-6 in the LCWE group were slightly ameliorated by ARB administration. Conclusion: ARB inhibits coronary arteritis via a mechanism that includes suppression of activated macrophages in a murine model of Kawasaki disease. Future therapeutic intervention with ARB as a rational drug is anticipated for Kawasaki disease.
Background: Recently, hypertriglyceridemia, especially in the non-fasting state, has been recognized as one of the most important atherosclerotic risk factors, in addition to hypercholesterolemia, in adults. The purpose of this study was to evaluate the usefulness of measuring non-fasting triglyceride levels and low-density lipoprotein cholesterol levels in schoolchildren. Subjects and Methods: The subjects were 2,961 fouth and seventh grade schoolchildren in Takaoka City. They received the screening for preventing life-style related disease in 2010. Along with anthropometric measurements, blood samples obtained immediately after the school lunch were tested for total cholesterol, high-density lipoprotein cholesterol (HDLC), triglyceride (TG), and low-density lipoprotein cholesterol (LDLC) levels. Some children were re-examined later during fasting, and their data were compared to those in the non-fasting state. Results: There were no differences in the mean values of non-fasting TG between children in either grade or between the different sexes. The 50th percentile levels were 86 to 90 mg/dl, which are 20 to 30 mg/dl higher than the fasting levels in the nationwide reports. Non-fasting TG correlated with the values of body mass index, percentage overweight, atherosclerotic index, non-HDLC (TC-HDLC), and LDLC positively and with HDLC negatively. However, the subjects with high levels of non-fasting TG did not always have high levels of fasting TG. Directly measured LDLC highly correlated with non-HDLC (r = 0.98, p < 0.001). Conclusions: Non-fasting TG levels are considered useful predictors of metabolic syndrome in schoolchildren. LDLC levels can be estimated from non-HDLC levels instead of measuring directly.
We report a case of a fetus with premature constriction of the ductus arteriosus (PCDA) diagnosed by prenatal fetal echocardiography. The mother was administered indomethacin for tocolysis and acetaminophen at week 31 of gestation. Fetal echocardiography showed enlargement of the right atrium (RA) and right ventricle (RV) with hypertrophic RV, moderate tricuspid valve regurgitation (TR), and a narrowed ductus arteriosus (DA). We continued to perform fetal echocardiography until the fetus matured to assess fetal cardiac function after the medication was discontinued. Right heart failure of the fetus was not noticed during the pregnancy. A male infant was delivered at 37 weeks and 5 days of gestation by cesarean section. Although echocardiography just after birth revealed marked RV pressure overload, the infant was in good condition without any treatment; subsequently, the RV pressure normalized after 1 month. Typically, urgent delivery is thought to be required in cases of PCDA. However, in some patients, constriction of the DA can be reversed by discontinuation of medication. Echocardiographic examinations of the fetus can be used to determine the appropriate timing of delivery.