Kawasaki disease patients with six principal symptoms have a high risk of being a non-responder.

BACKGROUND: A diagnosis of Kawasaki disease (KD) is established using six principal symptoms. Because the principal symptoms are deeply connected with KD, it is thus important to investigate the usefulness of the principal symptoms for evaluating the disease severity of KD. METHODS: Patients with definite KD or suspicion of KD were retrospectively examined. Blood test data and the incidence of patients who failed to respond to the initial i.v. immunoglobulin treatment (non-responders) were compared between patients with six principal symptoms, including fever of ≤ 4 days, before treatment of KD (six-symptom patients), and those with five or fewer symptoms (five-symptom patients). RESULTS: The study group of 207 patients who were treated with immunoglobulin consisted of 121 six-symptom patients and 86 five-symptom patients. The six-symptom patients were older and had higher neutrophil proportion and total bilirubin, and lower serum sodium at diagnosis than the five-symptom patients. Although the treatments did not differ between the groups, the six-symptom patients had a higher incidence of non-responders than the five-symptom patients (17% vs 5%; P= 0.008). Logistic regression analysis showed that six-symptom status was related to the risk of being a non-responder (odds ratio [OR], 5.3; 95% confidence interval [95%CI]: 1.6-17.4). This association was still significant after adjustment for the effect of age, neutrophil proportion, and total bilirubin and sodium (OR, 4.4; 95%CI: 1.4-17.3). CONCLUSIONS: The number of principal symptoms before treatment is a useful guide to KD disease severity. Six-symptom patients have a higher risk of being a non-responder than five-symptom patients.

A severe form of Kawasaki disease presenting with only fever and cervical lymphadenopathy at admission.

OBJECTIVE: To examine the characteristics of patients with Kawasaki disease (KD) presenting with only fever and cervical lymphadenopathy at admission. STUDY DESIGN: The laboratory and clinical findings of patients with definite KD presenting with only fever and cervical lymphadenopathy at admission (KDiL) were compared with those of all other patients with KD. RESULTS: Sixteen patients with KDiL (8.6%) and 171 patients without KDiL were examined. The patients with KDiL were significantly older (KDiL/non-KDiL: 4.9+/-2.5/2.2+/-1.9 years) and admitted earlier (3.0+/-1.2/3.9+/-1.3 days of illness) than the patients without KDiL. They also showed significantly elevated white blood cell counts and C-reactive protein levels. Patients with KDiL were treated with the same dose of intravenous immunoglobulin as the patients without KDiL but were treated slightly later and had significantly higher frequency of additional intravenous immunoglobulin treatment (38%/10%) and coronary artery abnormalities (25%/5%). After adjustment for age, white blood cell count, and day of illness at admission or first intravenous immunoglobulin administration, the presence of KDiL significantly increased the risk of being a nonresponder to IVIG treatment or development of a coronary artery abnormality. CONCLUSIONS: KDiL indicates a severe form of KD associated with increased risks of additional intravenous immunoglobulin treatment and coronary artery abnormalities. Patients with KDiL may require heightened surveillance and more aggressive treatment.

An elevated value of high mobility group box 1 is a potential marker for poor response to high-dose of intravenous immunoglobulin treatment in patients with Kawasaki syndrome.

We examined the serum values of high mobility group box 1 (HMGB1) in 36 patients with Kawasaki syndrome (KS) (29 responders and 7 poor-responders to initial intravenous immunoglobulin treatment). A mean value of HMGB1 of poor-responders was significantly elevated compared with those of responders (P = 0.0042). Among the 6 factors showing significant differences between responders and poor-responders including HMGB1 (admission illness day, white blood cell counts, C-reactive protein, aspartate aminotransferase, lactate dehydrogenase), values of HMGB1 showed the widest area under the receiver operating characteristic curve. In conclusion, an elevated HMGB1 value could be a potential marker for poor-responders.

Early diagnosis of Kawasaki disease in patients with cervical lymphadenopathy.

BACKGROUND: Among typical patients with Kawasaki disease (KD), a few KD patients present with only fever and cervical lymphadenopathy at admission (KDL). These patients have a significant risk for misdiagnosis, delay in treatment for KD, and development of coronary artery abnormalities. Therefore, the development of an easy tool for early diagnosis in these patients is desirable. METHODS AND RESULTS: Patients who presented with only fever and cervical lymphadenopathy at admission were studied. Of these, 14 patients were eventually diagnosed with KD (KDL) and 24 patients were successfully treated using antibiotics (control). KDL patients were significantly older than control patients (P > 0.022). Among the laboratory findings, neutrophil counts (P > 0.003), C-reactive protein (CRP; P < 0.001), and aspartate aminotransferase (AST; P > 0.018) were significantly different between the groups. To discriminate KDL patients from controls, cut-off points of the aforementioned parameters (KDL indices) were determined using the receiver operating characteristic curves in order to maximize sensitivity and accuracy (age, 5.0 years; neutrophil counts, 10,000 /microL; CRP, 7.0 mg/dL; AST, 30 IU/L). One point was assigned if a subject exceeded the cut-off point in a KDL index. If a patient with three or four KDL indices was considered to have KD, the sensitivity was 78% and the specificity 100%. None of the patients with one or zero KDL index developed KD. CONCLUSIONS: KDL indices may be helpful in discriminating KDL from lymphadenitis at admission. It is important to monitor the symptoms of KD in a patient with three or four KDL indices at admission.

Electrocatalytic formal [2+2] cycloaddition reactions between anodically activated enyloxy benzene and alkenes.

Electrocatalytic formal [2+2] cycloadditions between anodically activated enyloxy benzene and alkenes have been accomplished in a lithium perchlorate/nitromethane electrolyte solution. The enyloxy benzene moiety of these electrolytic substrates played an important role in the formation of a radical cation that could accept nucleophilic alkenes, followed by intramolecular electron transfer between the cyclobutane and phenyl ether moieties of the intermediates.

Serum procalcitonin value is useful for predicting severity of Kawasaki disease.

We measured serum procalcitonin concentrations in 160 patients suffering from Kawasaki disease. Serum procalcitonin was significantly higher in nonresponders to an initial intravenous immunoglobulin treatment than in responders. A cutoff value of procalcitonin (0.5 ng/mL) for nonresponders showed that the sensitivity was 85% and the accuracy was 64%. Multivariate logistic regression analysis revealed that procalcitonin-positive cases showed the highest risk for nonresponders.

Association between the number of cardiovascular risk factors and each risk factor level in elementary school children.

BACKGROUND: Little is known regarding the association between numbers of cardiovascular (CV) risk factors and the level of each risk factor in elementary school children based on a longitudinal study. METHODS AND RESULTS: A descriptive study of 319 obese children aged 6-11 years who participated in a screening program for comorbidity of obesity between 2003 and 2005, and who participated in consecutive years thereafter, was performed. Abdominal obesity, hypertension, dyslipidemia (low high-density lipoprotein-cholesterol levels and/or high triglyceride levels), and raised fasting glucose levels were used as the CV risk factors. Metabolic syndrome and each CV risk factor were defined using the criteria newly established by a Task Force financed by the Health and Labour Science Research in Japan. An increase in the total number of CV risk factors implied a worsening of each CV risk factor level over a 1-year interval, and vice versa. Abdominal obesity in males and insulin resistance in females were prevalent in children who were at elementary school level. CONCLUSIONS: We should assess not only obesity but all CV risk factor levels, because a cluster of risk factors implies a worsening of the individual risk factor levels in children as young as those in elementary school.

Four cases of Kawasaki syndrome complicated with myocarditis.

BACKGROUND: Myocarditis frequently occurs in the acute phase of Kawasaki syndrome (KS), and a few severe cases have been reported. Four cases of myocarditis in KS required additional catecholamine treatment because of severe left ventricular dysfunction (LVD). CASE REPORTS: Three cases were relatively older children and 2 cases were complicated with encephalopathy. All 4 developed coronary artery abnormalities during convalescence. There was 1 case of LVD because of prolonged severe inflammation prior to administration of intravenous immunoglobulin (IVIG). The remaining 3 patients had normal values for ejection fraction before the administration of IVIG but decreased values (42-51%) and increased C-reactive protein levels after IVIG administration. These cases demonstrate an association between myocarditis in KS and severe or worsened inflammation. CONCLUSIONS: Even with prior normal echocardiography, careful observation of cardiac function may be necessary for patients with KS, especially older children, when inflammation deteriorates after administration of IVIG.