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BACKGROUND Recent studies demonstrated that low-density lipoprotein-tryglyceride (LDL-TG) may represent another marker of cardiovascular risks. We therefore measured LDL-TG including the low-density lipoprotein (LDL) subclass distribution and investigated the association between LDL-TG subclass profile and the clustering of metabolic syndrome (MetS) components and insulin resistance in Japanese children. METHODS The study included 237 schoolchildren (boys 115, girls 122). Four subclasses of low-density lipoprotein-tryglyceride (large, medium, small, and very small) was quantified using high-performance liquid chromatography. Total LDL-TG and TG levels in LDL subclasses were evaluated among four MetS component groups; non-abdominal obesity, abdominal obesity, pre-MetS, and MetS. RESULTS Total LDL-TG (P = 0.0003, P = 0.0175) and triglyceride levels in LDL subclasses were significantly different among four MetS component groups (large: P = 0.0002, P = 0.0084; medium: P = 0.0009, P = 0.0491; small: P =0.0025, P = 0.0509; very small: P = 0.0808, P = 0.0228; boys and girls, respectively). Total LDL-TG (r = 0.411, P < 0.0001, r = 0.378. P < 0.0001) and triglyceride levels in LDL subclasses correlated positively with the homeostasis model of assessment ratio (large: r = 0.396, P < 0.0001, r = 0.346, P < 0.0001; medium: r = 0.274, P = 0.0030, r = 0.228, P = 0.0115; small: r = 0.342, P = 0.0002, r = 0.292, P = 0.0011; very small: r = 0.385, P < 0.0001, r = 0.426, P < 0.0001, boys and girls, respectively). CONCLUSIONS Subclass distribution of LDL-TG was significantly associated with the clustering of MetS components in both sexes, and insulin resistance is a significant determinant of LDL-TG in all LDL subclasses. Lipoprotein-tryglyceride subclass analysis, rather than LDL-C, may provide a precise evaluation for cardiovascular disease risks in children with MetS.

In postural tachycardia syndrome (POTS), a subtype of orthostatic intolerance, the changes in hemodynamics due to postural changes are poorly understood. We speculated that inappropriate venous return, which may occur in the upright position in patients with school-aged POTS, could be detected by echocardiography. Our prospective study was conducted with 100 POTS patients (45 boys and 55 girls), aged 13.1 ± 1.5 years and 52 age- and sex-matched healthy subjects (control). Echocardiography was performed in the supine and sitting positions. Cardiac parameters [stroke volume index, cardiac index, heart rate, and the maximum inferior vena cava diameter (max IVC)] were evaluated in addition to pulse pressure. Unlike the control subjects, POTS patients demonstrated decreased stroke volume index (P = 0.02) and max IVC (P < 0.01) irrespective of posture. The rates of max IVC change did not differ between control and POTS groups. The enrolled POTS patients were divided into two subgroups [dilatation (n = 57) and contraction (n = 43)] based on whether the change rate of max IVC was less than zero or not. The contraction group showed a significantly higher heart rate than the dilatation group with respect to posture (P = 0.03), indicating the poor response of peripheral vessels in the lower limbs only in the contraction group. In conclusion, echocardiographic assessment detected decreased stroke volume and venous return in POTS. The changes in max IVC in response to postural changes may indicate an underlying pathophysiology in POTS.

Purpose The aim was to investigate the characteristics of abdominal fat distribution in Japanese adolescents with type 2 diabetes mellitus. Patients and methods Eighty-six Japanese adolescents with simple obesity or type 2 diabetes mellitus treated between 2002 and 2018 were included. The subjects were classified into the simple obesity group (SO group, n=38) and type 2 diabetes mellitus group (DM group, n=23) by matching average age and gender ratio. The metabolic parameters VFA, SFA, and V/S ratio were compared between the 2 groups. Multivariate logistic regression analysis was performed to identify clinical factors associated with type 2 diabetes mellitus. Linear regression analysis was performed between hemoglobin A1c (HbA1c) and visceral fat area (VFA), subcutaneous fat area (SFA), or VFA-to-SFA ratio (V/S ratio) among all enrolled subjects. Finally, correlation analyses were performed to determine the relationships between VFA, SFA, and V/S ratio and metabolic parameters of the DM group. For the metabolic parameters, serum lipids, alanine aminotransferase (ALT), and HbA1c were measured without fasting. The VFA and SFA at umbilical level were investigated using computed tomography. Results VFA and V/S ratio in DM group were higher than those in SO group (p=0.04 and p

We present the case of a 75-year-old man with repeated lower limb hematoma caused by consumptive coagulopathy from a type B chronic aortic dissection. His abdominal aorta was replaced with a Y-shaped graft 30 years prior to admission. As his previous aortic stent graft treatment failed, he underwent open surgical prosthetic graft replacement of the descending aorta under deep hypothermia. To reduce intra- and postoperative bleeding, we avoided cutting the ribs and intercostal arteries. The aneurysm was approached only through the 8th intercostal space; however, as the proximal descending aorta was inaccessible from this site, total endoscopic or endoscopic-assisted procedure was performed to approach the proximal descending aorta. All intercostal arterial orifices were securely closed by suture. The postoperative course was uneventful, and he was discharged home on postoperative day 11. The endoscopic surgery reduced impairment of collateral vessels during surgery and might have reduced the risk of paraplegia.

Abdominal bioelectrical impedance analysis (aBIA) has been in use to measure visceral fat area (VFA) in adults. Accurately measuring visceral fat using aBIA in children is challenging. Forty-six school-aged Japanese children aged 6–17 years (25 boys and 21 girls) were included in this study. All were measured, and their VFA obtained using aBIA (VFA-aBIA) and abdominal computed tomography (CT) (VFA-CT) were compared. VFA-aBIA was corrected using the Passing–Bablok method (corrected VFA-aBIA). The relationships between corrected VFA-aBIA and obesity-related clinical factors were analyzed, including non-alcoholic fatty liver disease (NAFLD) and serum leptin and adiponectin levels. Boys had higher VFA-CT than girls (p = 0.042), although no significant differences were found in their waist circumference, waist-to-height ratio, and body mass index. The corrected VFA-aBIA using y = 9.600 + 0.3825x (boys) and y = 7.607 + 0.3661x (girls) correlated with VFA-CT in both boys and girls. The corrected VFA-aBIA in patients with NAFLD was higher than that in those without NAFLD. Serum leptin and adiponectin levels were positively and negatively correlated with corrected VFA-aBIA, respectively. In conclusion, corrected VFA-aBIA was clearly correlated with VFA-CT and was related to NAFLD and serum leptin and adiponectin levels in school-aged Japanese children.

Background Helicobacter pylori (H. pylori) infection causes various extra gastric diseases. Its transmission route has still not been clarified. However, no large-scale studies have examined the extra gastric diseases caused by H. pylori in adolescents. This study aimed to examine the association of H. pylori infection with anemia, serum cholesterol levels, physique, and birth delivery method (vaginal or Cesarean), in a large number of Japanese adolescents. Methods From 2016 to 2018, we screened 2,399 adolescents (aged 13-15 years) in their second and third years of junior high school using an enzyme-linked immunosorbent assay (ELISA)-based antibody test. Red blood cell, hemoglobin, hematocrit, total cholesterol, high-density lipoprotein (HDL) cholesterol, and serum antibody levels were measured. Results Hemoglobin and hematocrit levels were significantly lower in the H. pylori antibody-positive group (H. pylori-positive group) than in the H. pylori antibody-negative group (H. pylori-negative group) in both sexes (boys: p = 0.0004 and 0.0022; girls: p = 0.0019 and 0.0005, respectively). Total cholesterol and non-HDL cholesterol levels were significantly higher in the H. pylori-positive group than in the H. pylori-negative group among boys (p = 0.0370 and 0.0293 respectively). There was no significant difference in body mass index (BMI) percentile and birth delivery method between the H. pylori-positive and H. pylori-negative groups in both boys and girls. Conclusions Among Japanese junior high school students, H. pylori antibody-positive status was associated with anemia in both sexes while total cholesterol and non-HDL cholesterol levels were associated among boys. There was no association between H. pylori antibody status, BMI percentile, and birth delivery method.

Background: Left ventricular noncompaction (LVNC) is a hereditary type of cardiomyopathy. Although it is associated with high morbidity and mortality, the related ion channel gene variants in children have not been fully investigated. This study aimed to elucidate the ion channel genetic landscape of LVNC and identify genotype-phenotype correlations in a large Japanese cohort. Methods: We enrolled 206 children with LVNC from 2002 to 2017 in Japan. LVNC was classified as follows: LVNC with congenital heart defects, arrhythmia, dilated phenotype, or normal function. In the enrolled patients, 182 genes associated with cardiomyopathy were screened using next-generation sequencing. Results: We identified 99 pathogenic variants in 40 genes in 87 patients. Of the pathogenic variants, 8.8% were in genes associated with channelopathies, 27% were in sarcomere genes, and 11.5% were in mitochondrial genes. Ion channel gene variants were mostly associated with the arrhythmia classification, whereas sarcomere and mitochondrial gene variants were associated with the dilated phenotype. Echocardiography revealed that the group with ion channel gene variants had almost normal LV ejection fraction and LV diastolic diameter Z scores. Fragmented QRS, old age, and an arrhythmia phenotype were the most significant risk factors for ventricular tachycardia ( P =0.165, 0.0428, and 0.0074, respectively). Moreover, the group with ion channel variants exhibited a greater risk of a higher prevalence of arrhythmias such as ventricular tachycardia, rather than congestive heart failure. Conclusions: This is the first study that focused on genotype-phenotype correlations in a large pediatric LVNC patient cohort with ion channel gene variants that were determined using next-generation sequencing. Ion channel gene variants were strongly correlated with arrhythmia phenotypes. Genetic testing and phenotype specification allow for appropriate medical management of specific LVNC targets.

Background: To disclose coronary artery disease (CAD) in the young is difficult by electrocardiogram screening for school students before their first events. Methods: By reviewing all reports submitted to the Mutual Aid System for Schools between 1989 and 2015, we listed all cases of sudden cardiac arrest which were considered to be caused by CAD. Kawasaki disease sequela was excluded. Epidemiology, type of CAD, symptom before onset, the intensity of the exercise at onset were investigated. Results: Thirty cases were enrolled and 26 resulted in sudden deaths, 4 were resuscitated without unfavorable sequelae. Cardioverter-defibrillator were implanted in 2 cases. Twenty-eight were male. One student was in 6th grader of primary, 11 in junior high, 15 cases in high, and 3 cases in nursing schools. Before onset, 12 cases were diagnosed as normal by ECG screening, 1 case for each was diagnosed as premature ventricular contraction, Wolff-Parkinson-White syndrome, and past history of atrial flutter. Diagnosis of 15 cases was unknown. Types based on hospital or post-mortem records: Left coronary artery (LCA); LCA of two cases were originated from the pulmonary artery, 4 cases from right coronary cuspid (RCC), and 1 from an unknown site. Two cases with LCA malformation without detail description, 3 with left ostium stenosis, and 1 with a myocardial bridge. Right coronary artery (RCA); RCA of a student was from left coronary cuspid (LCC), 1 from unknown site, 4 cases were diagnosed as hypoplastic RCA. Three cases complicated with atherosclerotic stenosis, 2 with hypoplastic CA, a case with single CA, and 4 with vasospastic angina.The school grader at onset: All of 4 cases with LCA from RCC, 2/3 with the LCA ostium stenosis and 3/4 with RCA hypoplasia were junior high school cases. Exercise intensity (level 1 to 5: most strenuous): At the onset, 19 cases were at level 5, 7 at level 4, 1 at level 3, 3 at an unknown level. Exercise at onset; soccer in 6 cases, basketball in 5, long distance running in 4, swimming, baseball, tennis, rugby, kendo, and on the way to school were in 2 for each. Conclusion: Anomalous ostium and origin of CA from the opposite side cusp tend to cause cardiac arrest in younger cases. CA evaluation by CT angiogram, MRI, or myocardial perfusion imaging is desirable.

Purpose The aim was to investigate the characteristics of abdominal fat distribution in Japanese adolescents with type 2 diabetes mellitus. Patients and methods Eighty-six Japanese adolescents with simple obesity or type 2 diabetes mellitus treated between 2002 and 2018 were included. The subjects were classified into the simple obesity group (SO group, n=38) and type 2 diabetes mellitus group (DM group, n=23) by matching average age and gender ratio. The metabolic parameters VFA, SFA, and V/S ratio were compared between the 2 groups. Multivariate logistic regression analysis was performed to identify clinical factors associated with type 2 diabetes mellitus. Linear regression analysis was performed between hemoglobin A1c (HbA1c) and visceral fat area (VFA), subcutaneous fat area (SFA), or VFA-to-SFA ratio (V/S ratio) among all enrolled subjects. Finally, correlation analyses were performed to determine the relationships between VFA, SFA, and V/S ratio and metabolic parameters of the DM group. For the metabolic parameters, serum lipids, alanine aminotransferase (ALT), and HbA1c were measured without fasting. The VFA and SFA at umbilical level were investigated using computed tomography. Results VFA and V/S ratio in DM group were higher than those in SO group (p=0.04 and p

Background: Visceral fat accumulation is strongly associated with type 2 diabetes (T2DM). In contrast to visceral fat, subcutaneous fat might even be beneficial against metabolic abnormalities. Objective: To investigate the association between simple obesity or T2DM and the ratio of visceral fat area (VFA) to subcutaneous fat area (SFA) (V/S ratio) in Japanese adolescents. Design/Methods: An age- and sex-matched retrospective study was conducted in Tokyo Metropolitan Hiroo Hospital, Nihon University Hospital, and Nihon University Itabashi Hospital with ethics approval. Sixty-one adolescents with simple obesity or T2DM aged 10 to 15 years who have been visiting in these outpatient clinics between 2002 and 2018 were enrolled in this study. Simple obesity was defined as BMI ≥95%ile but without T2DM. VFA and SFA were investigated using CT at the umbilical levels. Subjects were classified into 2 groups: simple obesity group (n=38) and T2DM group (n=23). Results: Comparisons between the 2 groups are shown in Table 1. In simple obesity group, ALT, TC, HDLC or non-HDLC levels were significantly different that those in T2DM group. SFA and V/S ratio in simple obesity group were significantly higher and lower than those in T2DM group, respectively. Conclusion: V/S ratio is different between simple obesity and T2DM in Japanese adolescents. V/S ratio is the most useful predicter of T2DM in adolescents. Disclosure Y. Abe: None. T. Urakami: None. K. Yoshida: None. M. Aoki: None. M. Hara: None. I. Morioka: None.

Background The aim of this study was to investigate the clinical significance and factors that affect N-terminal pro-brain natriuretic peptide (NT-proBNP) elevation in the acute phase of Kawasaki disease (KD) despite the absence of apparent cardiac complications. Methods The laboratory and echocardiography results of 44 KD patients in the acute and subacute phases were reviewed. Results With preserved cardiac function, median NT-proBNP was significantly elevated in the acute phase compared with the subacute phase (343 pg/mL, IQR, 162–1182 pg/mL vs 98 pg/mL, IQR, 61–205 pg/mL, respectively; P < 0.0001). The respective levels of tumor necrosis factor (TNF)-α, soluble TNF receptor (sTNFR)1, and sTNFR2 were also significantly elevated in the acute phase compared with the subacute phase: TNF-α, 3.3 pg/mL (IQR, 2.6–4.8 pg/mL) versus 2.4 pg/mL (IQR 1.9–4.0 pg/mL; P < 0.01), sTNFR1, 2741 pg/mL (IQR, 2080–3183 pg/mL) versus 976 pg/mL (IQR, 814–1247 pg/mL; P < 0.0001), sTNFR2, 5644 pg/mL (IQR, 4693–7520 pg/mL) versus 3169 pg/mL (IQR, 2132–3878 pg/mL; P < 0.0001). Log-transformed NT-proBNP was correlated with TNF-α (r = 0.29, P = 0.056), sTNFR1 (r = 0.60, P < 0.0001), and sTNFR2 (r = 0.65, P < 0.0001). TNF-α was correlated with sTNFR1 (r = 0.35, P = 0.02) and sTNFR2 (r = 0.51, P < 0.001). Conclusion Tumor necrosis factor-α may cause NT-proBNP elevation in the acute phase of KD, and NT-proBNP level may be an indicator of TNF-α activity.

Kawasaki disease (KD) is a form of acute multisystem vasculitis that presents with various complications, including coronary artery aneurysm. Heart failure and brain damage are rare, but life-threatening complications are associated with KD. Here, we describe a 4-year-old girl who developed intravenous immunoglobulin-resistant KD with both left ventricular failure and acute encephalopathy. On day 8 of the illness, the low left ventricular ejection fraction, mitral regurgitation, and low blood pressure, which required continuous administration of dobutamine, were observed during the treatments for KD, including intravenous immunoglobulin. She also appeared unconscious, where the electroencephalogram showed slow waves of activity in all regions of the brain. The cardiac performance improved after she received plasma exchange for three days. However, her unconsciousness with slow waves of activity on electroencephalogram and fever continued after the plasma exchange. Therefore, she was treated with methylprednisolone pulse, followed by prednisolone, as well as intravenous immunoglobulin. Finally, she recovered without any cardiac or neurological sequelae not only at the time she was discharged, but also throughout the follow-up period. The combination therapy using plasma exchange and methylprednisolone pulse may be a treatment option for severe KD with left ventricular failure and acute encephalopathy complications.

BackgroundThere is no standard dose or protocol for beta-blocker administration as preconditioning in children undergoing coronary CT angiography.MethodsA total of 63 consecutive patients, with a mean age of 10.0±3.1 years, who underwent coronary CT angiography to assess possible coronary complications were enrolled in a single-centre, retrospective study. All patients were given an oral beta-blocker 1 hour before coronary CT angiography. Additional oral beta-blocker or intravenous beta-blocker was given to those with a high heart rate. We compared image quality, radiation exposure, and adverse events among the patients without additional beta-blocker, with additional oral beta-blocker, and with additional intravenous beta-blocker.ResultsThere were no significant differences in image quality or radiation exposure among the groups. The heart rate just before scanning was significantly correlated with image quality (pConclusionInitial oral beta-blocker administration (0.8 mg/kg/dose) should be administered to all children undergoing coronary CT angiography. Additional intravenous beta-blocker should be given to those with poor heart rate control to improve image quality without increasing radiation exposure or allowing adverse events.

We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH.

Yuriko Abe1), Mamoru Ayusawa1), Masataka Kato1), Hirofumi Watanabe1), Ami Cho1), Akiko Komori1), Hiromi Okuma1), Rie Ichikawa1), Hiroshi Kamiyama1), Naokata Sumitomo2), Sango Ito1), and Shori Takahashi1) 1) Department of Pediatrics and Child Health, Nihon University School of Medicine, Tokyo, Japan 2) Department of Pediatric Cardiology, Saitama Medical University International Medical Center, Saitama, Japan

AIM The aim of this study was to identify the age and sex-specific reference ranges for the non-high-density lipoprotein cholesterol (non-HDLC) levels in Japanese children. METHODS The subjects included 441,431 schoolchildren (207,015 boys, 234,416 girls) 9-16 years of age who participated in a screening and care program for lifestyle-related diseases from 2006 to 2011. The serum total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C) and triglyceride (TG) levels were measured, and the non-HDL-C levels were calculated. The serum lipid levels were analyzed according to age and sex. RESULTS The overall mean non-HDL-C level was 105.7±24.0 mg/dL, with a sex difference: boys= 103.0±24.0 mg/dL and girls=108.2±23.8 mg/dL. In boys, the median non-HDL-C level decreased gradually from 104 mg/dL in the 9-year-old age group to 96 mg/dL in the 15-year-old age group. The 75th percentile level was approximately 120 mg/dL in the 9- to 11-year-old groups and decreased at approximately 113 mg/dL in the 12- to 15-year-old groups, whereas the 95th percentile level was approximately 150 mg/dL in the 9- to 11-year-old groups and decreased at approximately 140 mg/dL in the 13- to 15-year-old groups. In girls, the median non-HDL-C level remained unchanged at approximately 105 mg/dL, with 75th and 95th percentile levels of approximately 122 and 150 mg/dL, respectively. CONCLUSIONS The non-HDL-C levels vary by age and sex. The age- and sex-specific reference ranges for the non-HDL-C levels may be a valuable tool for management with respect to preventing the development of atherosclerosis in childhood.

To investigate the effects of n-3 polyunsaturated fatty acids on stearoyl-CoA desaturase (SCD) activity, we treated 10 obese children (mean age: 12.9 years) with cod liver oil once daily for 12 weeks. The effects of cod liver oil supplementation on SCD activity, as estimated by the palmitoleate/palmitate ratio, depended on the docosahexaenoic acid (DHA) contents at baseline. Baseline DHA contents were negatively correlated with baseline SCD activity. After the treatment, baseline DHA contents were found to be significantly associated with the reduction of SCD activity. Cod liver oil supplementation may be a complementary treatment for obese children with low baseline contents of DHA.

BACKGROUND Serum N-terminal pro-brain natriuretic peptide (NT-proBNP) tends to rise in acute phase Kawasaki disease (KD), but the cause of NT-proBNP elevation has not been clarified. In a previous study, cardiac function evaluated on 2-D echocardiography (2D-E) such as ejection fraction was normal, but this does not reflect subtle changes in cardiac dysfunction, and hence the association between cardiac function and NT-proBNP elevation is still controversial. The aim of this study was therefore to elucidate the influence of cardiac function on NT-proBNP elevation, by evaluating cardiac function via strain on 3-D speckle tracking imaging (3D-STI), in acute and subacute KD patients. Given that cytokines are also thought to induce NT-proBNP in acute phase KD, serum cytokines and cytokine receptors were measured at the same time. METHODS Laboratory data and echocardiography in 52 KD patients in the acute and subacute phases were reviewed. RESULTS Median NT-proBNP was significantly elevated in the acute phase compared with the subacute phase (356.5 pg/mL; IQR, 145-904 pg/mL vs 103.5 pg/mL; IQR, 59-150 pg/mL, P < 0.01). All cytokines were also significantly elevated in the acute phase compared with the subacute phase. Tumor necrosis factor (TNF)-α, soluble TNF receptor (sTNFR)1, and sTNFR2 concentration were all significantly higher in the acute phase. Indices of cardiac function were not significant different between phases. NT-proBNP in the acute and subacute phases correlated with sTNFR1 (r = 0.63/0.43, P < 0.01), sTNFR2 (r = 0.50/0.31, P < 0.05), and interleukin-6 (r = 0.58/0.43, P < 0.01). NT-proBNP did not correlate with global longitudinal strain (GLS) on 3D-STI. CONCLUSION Although no correlation was seen between NT-proBNP and GLS on 3D-STI, correlations between NT-proBNP and cytokines were clear. NT-proBNP might be a marker of inflammation in KD, but is not a marker of cardiac function.

Summary Aim In a mice study, insulin suppressed apolipoprotein A-V (apoA-V) gene expression in a dose dependent manner. Thus, we investigated the association between apoA-V levels and dyslipidemias in obese children with hyperinsulinemia. Methods The subjects were 17 obese children (15 male, 2 female) aged 11.8±2.4 years. Total cholesterol (TC), high-density lipoprotein cholesterol (HDLC), triglyceride (TG), apoA-V and insulin levels were determined. Results Obese children with hyperinsulinemia had greater percent overweight, higher TG level, lower HDLC level and lower apoA-V level than those without hyperinsulinemia. In simple regression analysis, apoA-V level correlated negatively with TG ( r =−0.613, p =0.0152) and insulin levels ( r =−0.566, p =0.0279), and positively correlated with HDLC ( r =0.811, p =0.0002). In stepwise regression analysis, insulin level emerged as the independent determinant of TG level after apoA-V level was taken into account, whereas apoA-V emerged as the independent determinant of HDLC level after adjusting for insulin level. Conclusions Insulin may be a potent regulator of serum apoA-V level in obesity, and apoA-V level may partly contribute to the development of obesity-associated dyslipidemia.

We present a case of a 9-month-old girl in whom malignant polymorphic ventricular tachycardia (VT) was successfully controlled by radiofrequency catheter ablation under guidance with a three-dimensional mapping system. The VTs originated from the left ventricular lateral wall, left ventricular anterior wall, and left ventricular apex. At least six types of VTs were documented during the electrophysiology study. All VTs were successfully controlled after two sessions of radiofrequency catheter ablation, and she was discharged from our hospital on propranolol, mexiletine, flecainide, and aprindine.

Background: Alterations in the very low-density lipoprotein (VLDL) subclass profile have been demonstrated in obese and insulin resistant adults; however, little information on VLDL subclasses is available in children. We determined the association between abdominal adiposity, insulin resistance, and triglyceride (TG) levels in each VLDL subclass in Japanese school children. Methods: One hundred sixty-four children (79 boys and 85 girls), 10.9 ± 1.6 years of age (mean ± SD), were recruited. We obtained the waist-to-height ratio (WHtR), lipid profile, and HOMA-R data from all children. The VLDL subclass profile was analyzed by HPLC. Results: Children of either gender with abdominal obesity (WHtR ≥ 0.5) had higher concentrations of large, medium, and small VLDL-TG in conjunction with the shift to larger size and elevated HOMA-R. The WHtR was positively related to concentrations of large (boys: r = 0.5800, p < 0.0001; girls: r = 0.3841, p = 0.0003) and medium (r = 0.5265, p < 0.0001; r = 0.3320, p = 0.0019) VLDL-TG. The small VLDL-TG concentration was positively correlated to the WHtR in boys (r = 0.4902, p < 0.0001), but not girls. In multiple regression analyses, the WHtR was an independent determinant of large, medium, and small VLDL-TG concentrations in boys, but not in girls, taking HOMA-R into account. Conclusions: Abdominal obesity was associated with the VLDL subclass profile in Japanese school children; however, the impact of abdominal adiposity on the VLDL subclass alteration demonstrated a gender difference.

A series of square planar platinum(II) salen complexes containing 4-substituted alkoxy chains of aromatic rings, [Pt((4-CnH2n+1O)2salen)] (n = 3 (1), 4 (2), 6 (3), 8 (4), 10 (5), 12 (6), 14 (7), 16 (8), and 18 (9)) has been prepared, and photophysical and mesomorphic properties have been investigated. Complexes 1–9 emitted intense phosphorescence from the mixed exited-state between the MLCT (MLCT = metal-to-ligand charge transfer) and LLCT (LLCT = ligand-to-ligand charge transfer) states both in dichloromethane solution and in solid states at room temperature. The red-shifted solid-state emission spectra from monomers in solution are caused by the supramolecular contact with one-dimensional stacking between neighboring chromophores (salen moieties) through the weak C–H⋯O type hydrogen bonding and van der Waals interactions in the solid state revealed by an X-ray crystallographic analysis for 2. Though 1–4 did not exhibit any mesophases (liquid–crystalline phases), 5–9 showed a lamello-columnar (ColL) mesophase with the high thermal stability, leading to the different phosphorescence spectra from both in solution and in solid states. This is affiliated with the appearance of the intermolecular d z 2 Pt ( II ) - d z 2 Pt ( II ) orbital interaction in a one-dimensional stacking distance with ca. 3.0 A due to self-assemblies in the liquid crystal which gives rise to the MMLCT (MMLCT = metal–metal-to-ligand charge transfer) transition. This is supported by absorption and emission measurements in the wide range from room temperature in the solid state to the clearing points (around 280 °C). The relationship between molecular assemblies and photophysical properties is discussed.

Aims Plasma fatty acid composition can change with age, reflecting diet and levels of desaturating enzymes such as stearoyl-CoA desaturase (SCD), delta-6 desaturase (D6D) and delta-5 desaturase (D5D), which contribute to the development of insulin resistance. This study analyzed longitudinal changes in fatty acid composition in Japanese children during early puberty and the association between changes in desaturase indices and changes in body fatness and insulin resistance. Methods The study included 77 children (38 boys and 39 girls) aged 9.6±0.5 years. Relative weight (RW) and waist-to-height ratio (WHtR) were determined. The fatty acid composition of plasma phospholipids was analyzed by gas chromatography, and the desaturase indices were calculated: SCD (16:1n-7/16:0: SCD16 and 18:1n-9/18:0: SCD18), D6D (20:3n-6/18:2n-6) and D5D (20:4n-6/20:3n-6) in 2006 and 2009. Results Obese children showed higher dihomo-gamma linolenic acid (DGLA; 20:3n-6), a higher D6D index and lower D5D index than non-obese children. Longitudinal changes in fatty acid com-position were generally similar in both sexes. Increased D6D index and DGLA and decreased D5D index were significantly associated with increased WHtR in boys and girls. In addition, increased D6D index was associated with an increased homeostasis model of assessment ratio (HOMA-R) only in girls. Conclusion The change in abdominal adiposity is a determinant of longitudinal changes in D6D and D5D indices and DGLA during early puberty.

一般学童を対象とした従来の生活習慣病予防健診に，腹囲の測定を加えたメタボリックシンドローム (MetS) 健診を追加し健診後の指導を希望した学童を対象に，小児期における MetS や関連する事象について検討を行った．その結果，第二度近親者における 2 型糖尿病の家族歴を有する学童は，その家族歴のない者と比較して腹囲 (p = 0.0136)， 腹囲 /身長比 (p = 0.017)， ALT (p = 0.0019)， 中性脂肪 (TG) 値 (p = 0.0683) のいずれも有意に高値かその傾向を示した．また，MetS を構成するリスクファクターの数が増加するにつれ内臓脂肪蓄積の指標となる腹囲が増大していた．休日における屋外 (体育館を含む) での運動時間が長い群は短い群と比較して腹囲の平均が有意に小さいことも示された (運動時間 1 時間未満の群：5 時間以上の群，72.9 ± 11.6 v.s. 60.3 ± 11.7 cm, p = 0.0322)．MetS の予防や改善について運動の習慣が重要であることが示唆された．

A new l -tartrato manganese(III) complex was synthesized and characterized as the dinuclear dimanganese(III) structure with a stereospefically formed [Λ–Λ] absolute configuration around Mn(III) ions. The thermal and photo decomposition gave the first example of dihydrogen gas evolution besides CO and CO2 gas associated with cis-[MnII(ox)(bpy)(H2O)2]. A proposed redox reaction proceeds from Mn(III) to Mn(II) via intermediate Mn(IV) and Mn(II) with CO anion radical species followed by oxidation of tartrate ligands.

Schiff base VO2V and VOV complexes with long 4-substituted alkoxy chains have been synthesised from the corresponding VOIV complexes, [VO{(4-CnH2n+1O)xsalen}] [x = 1, 2, n = 3–16 and salen = N,N′-ethylenebis(salicylideneiminato)], in solutions in the absence or presence of HClO4 under aerobic conditions. The green single-crystals of the VOIV complexes (x = 2, n = 3, 4, 6) coexisting with solutions without HClO4 turned slowly to the yellow single-crystals of the species with n = 3 (1) and 4 (2), a process which took several months. An X-ray crystallographic analysis revealed that the VOIV complexes are transformed into the dimeric VO2V complexes with the tridentate sal-en (sal-en = N-salicylideneethylenediamine) ligand from the tetradentate salen moieties. However, the single crystals of the VOIV complexes with longer alkoxy chains of n ≥ 8 did not yield the corresponding yellow crystals of the VO2V complexes. This crystal-to-crystal transformation is discussed in relation to the crystal packing structures of the VOIV complexes. In contrast, addition of HClO4 solution to two series with one and two alkoxy chains in the 4-positions immediately afforded the corresponding blue VOV perchlorate complexes, [VO{(4-CnH2n+1O)xsalen}]ClO4 (x = 1, 2) without transformation of the salen moieties. The structure of 8·MeOH·0.5H2O determined from an X-ray crystallographic analysis was a unique dimeric structure supported by electrostatic stacking and CH–π interactions in an octahedral environment. The DFT calculations were studied to obtain the stabilisation energy for the dimerisation. Furthermore, in methanol solution, the effects of the alkoxy chain lengths on the ion association were investigated by conductivity measurements. The ion-association state in methanol solution is discussed with the solid-state structure.(© Wiley-VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2008)

This is a preprint of an article whose final and definitive form has been published in the MOLECULAR CRYSTALS AND LIQUID CRYSTALS © 2007 copyright Taylor & Francis; MOLECULAR CRYSTALS AND LIQUID CRYSTALS is available online at : http://www.informaworld.com/openurl?genre=article&issn=1542-1406&volume=466&issue=1&spage=129, Article, MOLECULAR CRYSTALS AND LIQUID CRYSTALS. 466(1): 129-147 (2007)

Aim To investigate the relationship between the clustering of metabolic syndrome (MetS) components and non-high-density lipoprotein cholesterol (non-HDL-C) levels in Japanese obese boys. Methods Subjects were 58 obese boys aged 12.0±2.6 years, which were categorized into three subgroups: abdominal obesity, pre-MetS (abdominal obesity＋1 component), and MetS (abdominal obesity＋2 or more components). Results Sixteen (27.6%) and 32 (55.2%) of the obese boys were diagnosed as pre-MetS and MetS, respectively. The mean non-HDL-C level in total subjects was 139.0±36.4 mg/dl and that in boys with abdominal obesity, pre-MetS, and MetS were 112.9±34.4, 135.4±37.9, and 149.0±32.6 mg/dl, respectively (p=0.0183, ANOVA). Conclusions Japanese obese boys with MetS exhibited elevated non-HDL-C levels, suggesting that they may have a higher risk for the development of atherosclerotic diseases.

Previous studies that used carotid ultrasound have been largely conflicting in regards to whether or not patients after Kawasaki disease (KD) have a greater carotid intima-media thickness (CIMT) than controls. To test the hypothesis that there are significant differences between the values of CIMT expressed as absolute values and standard deviation scores (SDS) in children and adolescents after KD and controls, we reviewed 12 published articles regarding CIMT on KD patients and controls. The mean ± SD of absolute CIMT (mm) in the KD patients and controls obtained from each article was transformed to SDS (CIMT-SDS) using age-specific reference values established by Jourdan et al. (J: n = 247) and our own data (N: n = 175), and the results among these 12 articles were compared between the two groups and the references for comparison of racial disparities. There were no significant differences in mean absolute CIMT and mean CIMT-SDS for J between KD patients and controls (0.46 ± 0.06 mm vs. 0.44 ± 0.04 mm, p = 0.133, and 1.80 ± 0.84 vs. 1.25 ± 0.12, p = 0.159, respectively). However, there were significant differences in mean CIMT-SDS for N between KD patients and controls (0.60 ± 0.71 vs. 0.01 ± 0.65, p = 0.042). When we assessed the nine articles on Asian subjects, the difference of CIMT-SDS between the two groups was invariably significant only for N (p = 0.015). Compared with the reference values, CIMT-SDS of controls was within the normal range at a rate of 41.6 % for J and 91.6 % for N. These results indicate that age- and race-specific reference values for CIMT are mandatory for performing accurate assessment of the vascular status in healthy children and adolescents, particularly in those after KD considered at increased long-term cardiovascular risk.

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.

A series of nickel(II) salen complexes containing 4-substituted alkoxy chains of aromatic rings, [Ni((4-CnH2n + 1O)2salen)] (n = 3 (1), 4 (2), 6 (3), 8 (4), 10 (5), 12 (6), 14 (7), 16 (8), 18 (9), and 20 (10)), and their parent complex, [Ni((4-HO)2salen)] (11) (salen = N,N′-ethylenebis(salicylideneiminato)), have been prepared and mesomorphic properties have been investigated. An X-ray crystallographic analysis revealed that complex 11 · 2DMF has one-dimensional stacking structure supported by the π–π interaction between the six-membered chelate and aromatic rings with the Ni Ni distances of alternatively 3.3957 and 3.7224 A and that complex 3 is formed by one-dimensional stacking by weak C H⋯O type hydrogen bonded interaction between the five-membered chelate ring and phenoxo atoms of the dramatically distorted salen moieties with the Ni Ni distance of 5.994 A. Complexes 1–6 did not exhibit any mesophases. On the other hand, complexes 7–10 with longer alkoxy chains of n = 14–20 showed an unusual metallomesogen of a lamello-columnar mesophase within the smectic layers with an interlamellar distance of 31.1 A (7), 33.6 A (8), 37.1 A (9), and 39.5 A (10) and nearly constant stacking distance of 6.19–6.24 A between the inter-dimers, irrespective of the variation of the alkoxy chain lengths by the X-ray diffraction measurements of the liquid crystal. The relationship between molecular assemblies and mesomorphic properties is discussed.

X-ray crystallographic analysis of Na5[Mn( l -tart)2] · 12H2O demonstrated that Mn(III) ion has two tridentate l -tartrates of which two deprotonated hydroxo groups and one carboxylate are coordinated in contrast to the previously proposed structure with two didentate tartrates and two coordinated water. The tetragonally distorted octahedron due to the Jahn–Teller effect was discussed in relation with the large splitting components for the UV–Vis and circular dichroism (CD) spectra in the d–d transition in the solid. The significant difference in the UV–Vis and CD spectra between the solid and the solution suggests that the tridentate coordination of the tartrate in the solid complex changes to the didentate one in the solution.

A series of V(IV) complexes with 4-substituted salen ligands, formulated as [VO((4-C n H 2 n +1 O) 2 salen)] ( n =12 ( 1 ), 14 ( 2 ), 16 ( 3 ), 18 ( 4 ), and 20 ( 5 )), which formed a novel biomembrane-like bilayer crystal structure, exhibited an unprecedented bilayer mesophase having symmetry Pa2 1 in the layers.

A novel solid surface is created by being modified with a monolayer of a bi-functional Ru(II) complex. Modification is performed by the combination of Langmuir–Blodgett (LB) and self-assembly methods. At the first step of the method, an ITO electrode is modified with a single layered clay film by the LB method. Thereafter a water-soluble Ru(II) complex, [Ru(bpy)2(m-PhOH)]2+ (m-PhOH=4-methyl-4′(CONH-phenol)-2,2′-bipyridyl), was self-assembled onto the electrode simply by being immersed in its aqueous solution. Cyclic voltammogram in a 0.1 M NaClO4 solution showed two centered oxidation waves at 710 and 1200 mV (vs Ag/AgCl) due to a –CONH-phenol group and a central Ru(II) ion, respectively. Keywords: Bi-functional Ru(II) complex, Modified electrode, Clay minerals

The chiral and racemic oxovanadium(IV) salmmen complexes, [VO( X )-(C16-salmmen)] ( X = R ( 1 ), S ( 2 ), and rac ( 3 ); C16-salmmen = N , N ′-monomethylenebis(5-hexadecyloxysalicylideneimine)) containing 5-substituted alkoxy chains on aromatic rings were synthesized, and their mesomorphic behaviors and ferroelectric properties were also investigated. All complexes exhibited both smectic A and chiral smectic C mesophases above 360 K, and they showed ferroelectric properties in the chiral smectic C mesophase.

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.

We report the case of a 9-year-old girl with Down syndrome (DS) diagnosed with idiopathic pulmonary hemosiderosis (IPH). Although acute pneumonia complicated by hemolytic anemia was suspected, IPH was finally diagnosed on bronchoscopy. Treatment with prednisolone achieved good clinical response. An association between IPH and DS was not able to be identified, but immunological issues in DS may contribute to the onset of IPH. Recurrent and intractable respiratory symptoms with marked infiltrative shadows in the bilateral lungs and complicated by severe anemia in patients with DS should suggest IPH.