Diagnostic guidelines for Kawasaki Disease was revised to meet the present situation in 2002. This issue intends to explain new guidelines and their backgrounds. Major alterations are interpretation of cases with 4 or fewer febrile days shortened by early intravenous immunoglobulin treatment, and the clinical importance of atypical (incomplete, or suspected) cases.

Objective: To investigate the clinical outcome, ECG characteristics, and optimal treatment of catecholaminergic polymorphic ventricular tachycardia (CPVT), a malignant and rare ventricular tachycardia. Patients and methods: Questionnaire responses and ECGs of 29 patients with CPVT were evaluated. Mean (SD) age of onset was 10.3 (6.1) years. Results: The initial CPVT manifestations were syncope (79%), cardiac arrest (7%), and a family history (14%). ECGs showed sinus bradycardia and a normal QTc. Mean heart rate during CPVT was 192 (30) beats/min. Most cases were non-sustained (72%), but 21% were sustained and 7% were associated with ventricular fibrillation. The morphology of CPVT was polymorphic (62%), polymorphic and bidirectional (21%), bidirectional (10%), or polymorphic with ventricular fibrillation (7%). There was 100% inducement of CPVT by exercise, 75% by catecholamine infusion, and none by programmed stimulation. No late potential was recorded. Onset was in the right ventricular outflow tract in more than half the cases. During a follow up of 6.8 (4.9) years, sudden death occurred in 24% of the patients, 7% of whom had anoxic brain damage. Autosomal dominant inheritance was seen in 8% of the patients' families. β Blockers completely controlled CPVT in only 31% of cases. Calcium antagonists partially suppressed CPVT in autosomal dominant cases. Conclusions: CPVT may arise in certain distinct areas but the prognosis is poor. The onset of CPVT may be an indication for an implanted cardioverter-defibrillator.

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Milking the umbilical cord is a safe procedure, reducing the need for RBC transfusions, and the need for circulatory and respiratory support in very preterm infants.

In adults, visceral fat accumulation is an important indicator for cardiovascular disease risk. This relationship is not fully understood in children. To determine the best predictor of cardiovascular disease risk factors among anthropometric indices such as body mass index (BMI), percent body fat (%Fat), waist-to-hip ratio (WHR), waist circumference, and waist-to-height ratio (W/Ht ratio) in Japanese schoolchildren. This study included 880 children (447 boys and 433 girls), 9-13y of age. Dependent variables were total cholesterol (TC), triglycerides (TG), high-density lipoprotein cholesterol (HDLC), low-density lipoprotein cholesterol (LDLC), atherogenic index (AI), life style related disease prevention score, and systolic (SBP) and diastolic (DBP) blood pressure. The strongest correlation was found between W/Ht ratio and the score by Pearson's correlation analysis. Multiple regression analysis showed that significant independent correlates for the score included W/Ht ratio and %Fat. Among the anthropometric indices, W/Ht ratio was the most significant predictor for TC, TG, LDLC, AI, and the score. W/Ht ratio is the best predictor of cardiovascular risk factors in Japanese schoolchildren. We propose using W/Ht ratio for detecting cardiovascular disease risk in children.

A multicenter randomized controlled study was carried out to assess the effectiveness of different, doses and kinds of γ‐globulin in Kawasaki disease. Gamma globulin lowered the incidence of coronary artery abnormalities. The effect of γ‐globulin was dose dependent. The intact type was more effective than the pepsin treated type. To establish the indications for γ‐globulin, a study was made of patients who received neither γ‐globulin nor indomethacin and who, within nine days of onset of illness, satisfied at least four of the following criteria: (1) WBC: more than 12,000/mm; (2) platelet count: less than 35×104γmm; (3) CRP: more than 3 +; (4) Hct: less than 35%; (5) albumin: less than 3.5 g/dl (6) age: 12 months or less; (7) male sex. This prospective study is continuing. Of 143 children, 73.4% received γ‐globulin, and only two demonstrated small dilatations of the coronary arteries in children who did not receive γ‐globulin. These guidelines seem satisfactory to establish the indications for γ‐globulin in Kawasaki disease.

Plasma 16:1n-7 content has a significant relation with abdominal adiposity in obese children. This change in the MUFA profile may be caused by activation of SCD that is not sufficiently suppressed by leptin. Endogenous lipogenesis may be an important factor in the pathogenesis of obesity in children.

The less distensible carotid wall in patients with KD compared with that in Cont without major alteration of the lipid profile is likely to be secondary to the changes in arterial walls after a diffuse vasculitis involving noncoronary arteries. These results indicate that the coronary arteries may be predisposed to accelerated atherosclerosis in patients with KD and CAL.