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Acute focal bacterial nephritis (AFBN) is a localized bacterial infection of the kidney presenting as an inflammatory mass without frank abscess formation. In children, most patients with AFBN present with nonspecific conditions, such as fever, vomiting, and abdominal pain. A small number of reported cases are accompanied by neurological symptoms, including meningeal irritation, unconsciousness, and seizures. We experienced 2 rare cases of AFBN associated with central nervous system lesions. The first case was a 3-year-old girl who had neurological symptoms, including unconsciousness and seizures, with AFBN associated with acute reversible encephalopathy. The second case was a 5-year-old girl who had neurological symptoms, including unconsciousness, with AFBN accompanied by clinically mild encephalitis/encephalopathy with a reversible splenial lesion.

Objectives: The carotid intima-media thickness (CIMT) is a reliable screening method for vascular alterations even in a pediatric cohort; however, reference values of CIMT established recently by LMS methods for childhood and adolescence are limited when comparing patients after Kawasaki disease (KD) and controls. We tested the hypothesis that there are significant differences between the values of CIMT expressed as absolute values and z-scores in children and adolescents after KD and controls. Methods: We reviewed 12 published articles regarding CIMT on patients after KD and controls. Absolute values (Ab) of the mean±1 SD of CIMT in patients after KD and controls were transformed to z-scores (Zs) using age-specific reference values established by Jourdan et al. (J: 247 Caucasian subjects aged 10-20 years) and our own data (O: 175 Asian subjects aged 6-20 years), and the results were compared between the two references. In this study, the mean age of the study population derived from each article was designated the representative age for transformation. Results: In either reference (J) or (O), there was no significant sex difference in CIMT at any given age. The mean CIMT of (Ab) and (Zs) transformed by (J) or (O) were significantly different between patients after KD and controls, at 41.6% (Ab), 66.6% (Zs) by (J), and 83.3% (Zs) by (O) among 12 articles, respectively. Therefore, patients after KD had significantly higher (Zs) by (O) than those of controls (0.66±0.71 vs. 0.03±0.68, p=0.006, respectively). Compared with reference values, the controls of (O) were within the normal range. However, there were no significant differences in (Zs) by (J) between the two groups (1.72±0.77 vs. 1.23±0.83, p=0.116, respectively). When we assessed 9 articles dealing with Asian subjects, the difference of (Zs) between the two groups remained significant only by (O) (p=0.015). In contrast, when we assessed 3 articles dealing with mainly Caucasian subjects, there was no significant difference in (Zs) between the two groups with both (J) and (O). Conclusions: These results indicate that age and race-specific reference values for CIMT are mandatory for performing an accurate assessment of the vascular status in healthy children and adolescents and particularly in those after KD.

Background: Prognosis of Kawasaki disease and its main complications, coronary artery aneurysm (CAA) and myocardial ischemia were remarkably improved by intravenous immunoglobulin (IVIG) and other additional anti-inflammatory and anti-coagulation therapy. In contrast, rupture of coronary artery aneurysm is a rare, and still definitely fatal complication. Though approximately 10 cases were reported with sporadic occurrence in every three to four years in Japan, there is no patient whose life was saved. Case report: We experienced a 5-year-old boy who had already developed left CAA of 9 mm in diameter on the 7th day from onset of fever. Despite of repeated IVIG, left CAA was rapidly expanding day by day and finally the diameter became 18mm on the 12th day. Early in the next morning, sudden cardiac arrest was noted, but resuscitation was not effective. Autopsy revealed cardiac tamponade caused by rupture of very fragile wall of huge left CAA. Discussions: After this experience, we have tried to carry patients into the intensive care unit with deep sedation, if patient’s CAA is expanding more than 10mm within 2 weeks in acute stage of Kawasaki disease. Furthermore, we also recommend use of antihypertensive drugs such as calcium channel blocker and/or beta-blockers. We also have advised to colleagues in other hospitals to try in the same way when they asked for the management of similar cases. At least three patients with huge aneurysm were survived with this method in recent reports. If patient has strong inflammation sustaining with expanding huge aneurysm, plasma exchange and all possible anti-inflammatory agents including steroid or infliximab under the percutaneous cardiopulmonary support and surgeons' stand-by until inflammation will disappear. Following repair or platy of coronary aneurysm and coronary artery bypass surgery may be considered, but possibility and results are unknown. Conclusions: Because of its rarity, it is difficult to detect exact indication of deep sedation or intensive care, however, earlier and more cautious management will be safer for ‘super-giant’ aneurysm. Concerning to the sustaining inflammation with huge aneurysm, we would like to hear advices for this strategy from cardiac surgeons and pediatric intensivists.

OBJECTIVES: Hypertrophic cardiomyopathy (HCM) has been one of the leading causes of sudden cardiac death in school students. However, recent spread of basic life support combined with public access defibrillation and implantable cardioverter-defibrillator (ICD) has a possibility of a paradigm shift for prognosis of HCM in young. METHODS: In Japan, more than 98% of all students in primary, middle, and high schools subscribe in the mutual aid system, and a school that experienced event relating sudden death is obliged to submit all health checking documents to refund for the affected student. Submitted reports were investigated for 8 years between 2004 and 2011. All victims or survivors diagnosed as HCM were extracted from those reports. Demographic data, presence of diagnosis before event, and intensity of physical activity at event were compared between them. RESULTS: Though data in 2011 is still tentative, annual numbers of victims with HCM from 2004 to 2011 were 4, 4, 5, 6, 3, 2, 4, 1, respectively. The first resuscitated survivor with HCM was reported in 2007, thereafter from 2008 until 2011, those were 6, 2, 4, 2, respectively. Therefore, totals of 29 victims and 15 survivors were enrolled in this study. All survivors were middle or high school students. Three primary school students were all victims. The male-to-female ratio was 4.8 and 2.0, respectively (NS). Ratio of students who were previously diagnosed as HCM was 48% and 20%, respectively (p=0.06). Concerning exercise intensity at cardiac event, 24% and 6.7% were occurred without exercise, respectively (p=0.08). ICD insertion after successful resuscitation was reported in 80% of survivors. CONCLUSIONS: Induction of basic life support and defibrillator has a certain impact for increasing survivors with HCM in school students. After 2008, survivors become equal or more than victims. Majority of survivors were disclosed by initial cardiac event by strenuous exercise, and cardioverter-defibrillator was implanted after resuscitation. Strategy for survival of HCM students with previously diagnosed should be reviewed. School caregivers including general physicians should acquire the management of students with ICD.

BACKGROUND: Considerable numbers of cardiac emergencies have been resuscitated without neurological sequel by public action of resuscitation and defibrillation. Since resuscitated cases are increasing also in Japanese schools, it is necessary to investigate factors for successful resuscitation. METHODS: Students resuscitated with AED were picked up from all issues in Mutual Aid Insurance System for Schools from 2005 to 2011. A total of 128 students were listed and classified to victims and survivors. Victims consisted of 62 cases that died or had brain sequel, and survivors consisted of 66 cases that recovered without sequel. Demography, causative disease, AED user, and the location of resuscitation in both groups were compared. RESULTS: Number of enrolled cases increased from 4 to 35 annually. School grade distribution is similar between two groups. Male to female ratio was 4.6 in victims and 2.1 in survivors (p=0.07). The most causative disease was cardiomyopathy (HCM) (22.6%) in victims, and ventricular fibrillation (VF) (28.8%) in survivors. They are the second in each other group. The third was congenital disease (8.1%) in victims, and long QT syndrome (LQT) in survivors (10.6%). In survivors, 6 cases were previously implanted ICD and 11 cases were implanted ICD after resuscitation and returned to school. Ambulance crew operated AED in 35.8% and 12.2% cases, and schoolteachers did in 64.2% and 87.8% of victims and survivors, respectively. Rate of successful resuscitation was significantly higher in cases operated by schoolteachers than that by ambulance crew (p CONCLUSIONS: AED operation by teachers improve prognosis of cardiac emergencies in Japanese schools. AED should be placed near athletic field, gymnasium, pool, and ideally on the way to school for possible student.