Acute Focal Bacterial Nephritis Associated With Central Nervous System Manifestations: A Report of 2 Cases and Review of the Literature.

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.

Successful control of life-threatening polymorphic ventricular tachycardia by radiofrequency catheter ablation in an infant.

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.

Characteristics of earlier atherosclerotic involvement in adolescent patients with Kawasaki disease and coronary artery lesions: significance of gray scale median on B-mode ultrasound.

OBJECTIVE: To test the hypothesis that textural changes in the carotid intima-media complex (IMC) detected by B-mode ultrasound are associated with the difference of remodeling process in earlier atherosclerotic involvement in patients with Kawasaki disease (KD) and coronary artery lesions (CALs). METHODS: Eighteen patients with KD and CALs (mean age 17.2 years), 17 patients with heterozygous familial hypercholesterolemia (FH) (mean age 16.9 years) and 15 age-matched healthy controls (Cont) were assessed and compared for carotid intima-media thickness (CIMT), elastic property (E(p)), and first- and second-order statistics. RESULTS: KD showed significantly higher gray scale median (GSM) than FH and Cont. KD and FH showed significantly higher CIMT, entropy and lower angular second moment than Cont, but no significant difference was found between KD and FH. CONCLUSION: Higher GSM in KD may indicate alteration of tissue components and heterogeneity of IMC, suggesting the development of arteriosclerotic vascular remodeling after vasculitis. This is distinct from that of atherosclerosis with lower GSM often observed in FH.

Anderson's disease/chylomicron retention disease in a Japanese patient with uniparental disomy 7 and a normal SAR1B gene protein coding sequence.

BACKGROUND: Anderson's Disease (AD)/Chylomicron Retention Disease (CMRD) is a rare hereditary hypocholesterolemic disorder characterized by a malabsorption syndrome with steatorrhea, failure to thrive and the absence of chylomicrons and apolipoprotein B48 post-prandially. All patients studied to date exhibit a mutation in the SAR1B gene, which codes for an essential component of the vesicular coat protein complex II (COPII) necessary for endoplasmic reticulum to Golgi transport. We describe here a patient with AD/CMRD, a normal SAR1B gene protein coding sequence and maternal uniparental disomy of chromosome 7 (matUPD7). METHODS AND RESULTS: The patient, one of two siblings of a Japanese family, had diarrhea and steatorrhea beginning at five months of age. There was a white duodenal mucosa upon endoscopy. Light and electron microscopy showed that the intestinal villi were normal but that they had lipid laden enterocytes containing accumulations of lipid droplets in the cytoplasm and lipoprotein-size particles in membrane bound structures. Although there were decreased amounts in plasma of total- and low-density lipoprotein cholesterol, apolipoproteins AI and B and vitamin E levels, the triglycerides were normal, typical of AD/CMRD. The presence of low density lipoproteins and apolipoprotein B in the plasma, although in decreased amounts, ruled out abetalipoproteinemia. The parents were asymptomatic with normal plasma cholesterol levels suggesting a recessive disorder and ruling out familial hypobetalipoproteinemia. Sequencing of genomic DNA showed that the 8 exons of the SAR1B gene were normal. Whole genome SNP analysis and karyotyping revealed matUPD7 with a normal karyotype. In contrast to other cases of AD/CMRD which have shown catch-up growth following vitamin supplementation and a fat restricted diet, our patient exhibits continued growth delay and other aspects of the matUPD7 and Silver-Russell Syndrome phenotypes. CONCLUSIONS: This patient with AD/CMRD has a normal SAR1B gene protein coding sequence which suggests that factors other than the SAR1B protein may be crucial for chylomicron secretion. Further, this patient exhibits matUPD7 with regions of homozygosity which might be useful for elucidating the molecular basis of the defect(s) in this individual. The results provide novel insights into the relation between phenotype and genotype in these diseases and for the mechanisms of secretion in the intestine.

Docosahexaenoic acid content in plasma phospholipids and desaturase indices in obese children.

AIMS: In obesity, fatty acid composition is altered with reduced docosahexaenoic acid (DHA) levels. Desaturating enzymes, stearoyl-CoA desaturase (SCD), delta-6 desaturase (D6D) and delta-5 desaturase (D5D) modulate fatty acid composition and are thus associated with the development of metabolic syndrome. The aim of this study was to identify the relationships among DHA content, desaturase indices and the components of metabolic syndrome in childhood obesity. METHODS: Thirty-two obese children (27 male, 5 female) aged 12.0±2.6 years (mean±SD), with a relative body weight greater than 120% of the standard weight for sex, age and height, were recruited. Fatty acid composition of plasma phospholipids was analyzed by gas chromatography, and the desaturase indices were assessed: SCD (16:1n-7/16:0 and 18:1n-9/18:0), D6D (20:3n-6/18:2n-6) and D5D (20:4n-6/20:3n-6). RESULTS: No sex difference was observed in fatty acid composition. DHA content tended to have an inverse association with body mass index (BMI) (r=-0.337, p=0.0592), and correlated significantly with very low-density lipoprotein-triglyceride (r=-0.558, p=0.0057). In addition, DHA content had significant negative relationships with SCD indices (SCD16; r=-0.373, p=0.0357 and SCD18; r=-0.580, p=0.0005), which correlated positively with BMI(r=0.439, p=0.0120, r=0.353, p=0.0473, respectively), but had no association with D5D or D6D. CONCLUSIONS: DHA content in obese children was independent of the desaturation of alpha-linolenic acid. Reduced DHA content was associated with increased SCD index, and might contribute to the development of metabolic syndrome. Dietary management including fatty acids is suggested to be important to prevent and better manage obesity.

Changes in the textural characteristics of intima-media complex in young patients with familial hypercholesterolemia: implication for visual inspection on B-mode ultrasound.

BACKGROUND: To test the hypothesis that textural changes in the carotid intima-media complex (IMC) on visual inspection by B-mode ultrasound are associated with early atherosclerotic involvement in patients with heterozygous familial hypercholesterolemia (FH). METHODS: 55 patients (mean age 13.4 years) were categorized into three groups according to the degree of thickness in IMC (intima-media thickness [IMT]) (groups I-III) and 15 healthy controls within the same age range as the patients were assessed for first- and second-order statistics and visual scoring of textural changes in IMC (1, normal; 2, proximal interface disruption; 3, granulation). RESULTS: There was no significant difference in first-order statistics among the four groups. As for second-order statistics, groups II (moderately increased IMT) and III (markedly increased IMT) had significantly higher entropy and lower angular second moment than group I (normal IMT) and control. Likewise, groups II and III received significantly higher visual scoring than group I. Visual scoring correlated with entropy (r = 0.57) and angular second moment (r = -0.50). Multiple regression analysis identified entropy (beta = 0.52) and visual scoring (beta = 0.42) as significant determinants of IMT. CONCLUSIONS: These findings demonstrate that higher visual scoring may indicate dishomogeneity of IMC, suggesting early medial infiltration. This seems to be a simple visual marker to more effectively identify high-risk young patients with FH.

The follow-up evaluation of electrocardiogram and arrhythmias in children with fulminant myocarditis.

BACKGROUND: Fulminant myocarditis involves various serious arrhythmias that sometimes have lethal consequences. The purpose of the present study was to investigate the electrocardiogram findings, arrhythmogenicity and abnormalities of the cardiac conduction system in children with fulminant myocarditis. METHODS AND RESULTS: Between 1999 and 2008, 7 consecutive patients (mean age: 7 years) who suffered from fulminant myocarditis were included in the study. A 12-lead electrocardiogram, Holter monitoring and signal-averaged electrocardiograms were performed and compared between the acute, convalescent, and recovery phases in the 4 surviving patients. Also, electrophysiologic assessment was carried out during the convalescent phase. Five out of 7 patients developed complete atrioventricular block, 3 developed ventricular tachycardia, 2 had cardiac arrest, 2 developed sinus tachycardia, 1 developed ventricular fibrillation, 1 had advanced atrioventricular block, and 1 developed sick sinus syndrome. Among the surviving patients, all arrhythmias resolved during the convalescent and remote phases. No atrial or ventricular arrhythmias were induced in any patients during the programmed stimulation study. In the convalescent phase, no arrhythmias could be induced and there were no signs of any conduction abnormalities on electrophysiological assessment. CONCLUSIONS: Close follow-up should be performed to observe for the occurrence of any new arrhythmias and/or a decrease in cardiac function in children with fulminant myocarditis.

Electrophysiological characteristics of idiopathic ventricular tachycardia in children.

BACKGROUND: Idiopathic ventricular tachycardia (VT) has been reported to have a good prognosis, but there still might be the potential risk of sudden death. METHODS AND RESULTS: The 46 consecutive children (mean age 11.7 ± 3.4 years) with idiopathic VT were enrolled in this study. Monomorphic VT was detected in 39 patients and polymorphic VT in 7 patients. The VT originated from the right ventricle (RV) in 22 patients, and left ventricle (LV) in 17 patients. The VT was induced by exercise in 68% of the RVVT, 41% of the LVVT, and 100% of the polymorphic VT. The VT was induced by programmed ventricular stimulation in 41% of the RVVT, 35% of the LVVT, and none of the polymorphic VT. Adenosine tri-phosphate terminated the VT in 9 of 15 patients (60%). The mechanism of the VT was suspected to be triggered by activity in 36.4%, automaticity in 40.9%, and re-entry in 22.7% of the RVVT, whereas it was 52.9%, 5.9%, and 41.2% of the LVVT, respectively. CONCLUSIONS: The exercise inducibility was higher in polymorphic VT than the RVVT and LVVT, but no difference in the programmed stimulation. The sensitivity to adenosine tri-phosphate was not different between the RVVT and LVVT. In some patients with idiopathic VT, a non-verapamil sensitive re-entry was documented, which was more common in patients with ischemic heart disease or cardiomyopathy.

Quantitative evaluation of coronary artery wall echogenicity by integrated backscatter analysis in Kawasaki disease.

BACKGROUND: Coronary artery wall echogenicity increases on echocardiograms during the acute phase of Kawasaki disease (KD). According to this background, echogenicity of the coronary artery wall in patients with KD is quantified by using integrated backscatter (IB) analysis. METHODS: IB analysis is a quantitative method for evaluating echogenicity. We examined the value of IB in the wall of the left anterior descending coronary artery and compared it with that in adjacent intracardiac blood as a measure of background. The difference between these values is represented as corrected IB for the coronary artery wall. RESULTS: Corrected IB for the coronary artery wall was higher in patients with KD than in controls (KD with pre-immunoglobulin therapy vs. controls: 27.4 +/- 5.3 dB vs. 22.0 +/- 3.5 dB, P < .05) and in patients with coronary enlargement after intravenous immunoglobulin (with vs. without coronary enlargement, 29.2 +/- 5.2 dB vs. 24.1 +/- 5.5 dB, P < .05). CONCLUSION: The magnitude of IB from the coronary artery wall reflects the effectiveness of immunoglobulin therapy. Furthermore, this method and its value might be useful to predict the occurrence of coronary enlargement in patients with KD.

Replacing dietary glucose with fructose increases ChREBP activity and SREBP-1 protein in rat liver nucleus.

Diets high in fructose cause hypertriglyceridemia and insulin resistance in part due to simultaneous induction of gluconeogenic and lipogenic genes in liver. We investigated the mechanism underlying the unique pattern of gene induction by dietary fructose. Male Sprague-Dawley rats (n=6 per group) were meal-fed (4h/d) either 63% (w/w) glucose or 63% fructose diet. After two weeks, animals were killed at the end of the last meal. Nuclear SREBP-1 was 2.2 times higher in fructose-fed rats than glucose-fed rats. Nuclear FoxO1 was elevated 1.7 times in fructose group, but did not reach significance (P=0.08). Unexpectedly, no difference was observed in nuclear ChREBP between two groups. However, ChREBP DNA binding was 3.9x higher in fructose-fed animals without an increase in xylulose-5-phospate, a proposed ChREBP activator. In conclusion, the gene induction by dietary fructose is likely to be mediated in part by simultaneously increased ChREBP activity, SREBP-1 and possibly FoxO1 protein in nucleus.

Concentrations of very long-chain fatty acid in whole blood are associated with cardiovascular risk factors in children.

BACKGROUND: Fatty acid metabolism has a close relationship with metabolic syndrome. Saturated very long-chain fatty acid (hexacosanoic acid; C26:0) was recently reported to be associated with cardiovascular risk factors in adults. METHODS: Eighty-eight children (47 male, 41 female; average age, 10.9 y) participated in this study. Concentrations of C26:0 in whole blood were measured. RESULTS: Compared with reported concentrations in the whole blood of adults, children had lower C26:0 concentrations, which had a close relationship with abdominal obesity, increased concentrations of low-density lipoprotein cholesterol and high blood pressure. C26:0 concentrations increased with increasing number of risk factors. CONCLUSIONS: Elevation of C26:0 concentrations may be one of the metabolic features of children with cardiovascular risk factors.

Dual myocardial scintigraphy mismatch in an infant with Bland-White-Garland syndrome.

Bland-White-Garland syndrome (BWG) is a rare disorder that includes abnormalities of the coronary arteries that cause severe myocardial ischemia or infarction in infancy. In this case report, we describe an infant with BWG evaluated by dual single photon emission computed tomography using thallium-201 and I-123 beta-methyl-p-iodophenyl-pentadecanoic acid before and after surgery.

Association of sinus node dysfunction, atrioventricular node conduction abnormality and ventricular arrhythmia in patients with Kawasaki disease and coronary involvement.

BACKGROUND: This study was performed to investigate the incidence of arrhythmias in patients with Kawasaki disease (KD). METHODS AND RESULTS: Electrophysiologic studies (EPS) were performed in 40 patients (mean age: 10.3+/-5.1 years; 30 males, 10 females) with KD who had severe to moderate coronary artery disease. Clinical arrhythmias were documented in 4 patients (premature ventricular contractions, ventricular tachycardia, atrioventricular block, and ventricular fibrillation). Dual atrioventricular nodal pathways were demonstrated in 3 patients. Nonsustained atrial fibrillation was induced in 1 patient. The AH interval was prolonged in 2 patients. The Wenckebach rate was 164+/-37 beats/min, and 4 of the patients had a decreased Wenckebach rate. The maximum and corrected sinus node recovery times were 997+/-257 ms and 281+/-130 ms, respectively, and 7 patients were thought to be abnormal. The sino-atrial conduction time was 108+/-64 ms, and 2 patients had prolonged conduction times. CONCLUSIONS: Although there was no relationship between coronary stenosis or obstruction and the EPS parameters, the incidence of abnormal sinus node and atrioventricular node function is apparently higher in KD patients than in the normal population. These functional abnormalities may possibly be caused by myocarditis or an abnormal microcirculation in the sinus node and atrioventricular node artery. In some patients, myocardial ischemia may provoke malignant ventricular arrhythmia.

Clinical importance of Koch's triangle size in children: a study using 3-dimensional electroanatomical mapping.

BACKGROUND: Catheter ablation inside the Koch's triangle has a risk for complete atrioventricular block. METHODS AND RESULTS: The anatomic size of the coronary sinus (CS) and His bundle (HB) in children and the distance between them was studied using a 3-dimensional electroanatomical mapping system (CARTO). Fifty-three children (mean age, 11.8+/-3.7 years) without congenital heart disease (ie, 24 with atrioventricular re-entrant tachycardia, 18 with atrioventricular nodal re-entrant tachycardia, 7 with atrial tachycardia, 2 with ventricular tachycardia and 2 with atrial flutter) were studied. The size of the HB recording area was 148+/-97 mm2 and the size of the CS was 66+/-44 mm2. The size of the CS and the distance between the HB and CS (18+/-7 mm) were proportional to body weight, body length and body surface area. All patients underwent catheter ablation, including 25 ablations inside Koch's triangle. Catheter ablation was successful in 52 patients without any atrioventricular nodal injury. CONCLUSIONS: The CS size and the distance between the HB and CS increased proportionally with children's growth. To know the distance from the HB to the ablation point is useful in avoiding atrioventricular node injury, and information about the length of Koch's triangle may provide supportive information when applying radiofrequency energy inside Koch's triangle without needing to use the CARTO system in children, but this merits further investigation.

Recommendations for weaning off cardiopulmonary support in children with fulminant myocarditis.

BACKGROUND: The purpose of this study was to determine the appropriate recommendations for weaning off cardiopulmonary support (CPS) in children with fulminant myocarditis. METHODS AND RESULTS: Four consecutive patients diagnosed with fulminant myocarditis who were treated with CPS were enrolled (mean age: 9 years). The relationships between timing of weaning from CPS and various factors, including bleeding episodes, platelet count, serum concentration of the MB isoform of creatine kinase (CK-MB), echocardiographic findings, and the mixed venous blood oxygen saturation (SvO2), were retrospectively analyzed. All patients had CPS performed safely for a mean duration of 53.1 h without exchange of the circuit. Three of the 4 patients had a bleeding episode before discontinuation. The minimum platelet count occurred during weaning in all 4 patients. The peak serum CK-MB concentration when initiating CPS was higher than the peak value on the day of weaning. Echocardiographic findings before stopping CPS were similar to those after weaning. The SvO2 was 62.5% when initiating CPS and 71.3% before weaning. CONCLUSIONS: In children with fulminant myocarditis CPS can be used without exchanging the circuit and can be discontinued before bleeding episodes become symptomatic, with improvement in the biomarkers and SvO2 on weaning off CPS.

Association of atrial arrhythmia and sinus node dysfunction in patients with catecholaminergic polymorphic ventricular tachycardia.

BACKGROUND: This study was performed to investigate the frequency and importance of supraventricular arrhythmia and sinus node (SN) dysfunction in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT). METHODS AND RESULTS: Eight patients with CPVT (mean age: 16.8+/-8.1 years) underwent an electrophysiological study. SN recovery time (1,389+/-394 ms) was slightly prolonged, and 4 of 8 patients had abnormal values. Atrial flutter (AF) was induced by low-rate atrial pacing in 2 patients and by isoproterenol infusion in 1 patient. Atrial fibrillation (Af) was induced by isoproterenol infusion in 2 patients. One patient presented with Af during the follow-up period, and 2 of 4 patients with AF/Af presented with increased SN recovery time. CONCLUSIONS: Patients with CPVT frequently have associated with SN dysfunction, and inducible atrial tachyarrhythmias, which indicate that the pathogenesis of CPVT is limited not only to the ventricular myocardium, but also to broad regions of the heart, including the SN and atrial muscle.

[Advantages of multislice spiral computed tomography for evaluation of serious coronary complications after Kawasaki disease].

OBJECTIVES: Novel multislice spiral computed tomography (MSCT) findings were identified in patients after Kawasaki disease that could not be detected by coronary angiography (CAG). METHODS: Eighteen patients had suffered from serious coronary arterial lesions after Kawasaki disease (mean age 21.7 years, range 13-34 years). Seventeen patients had stenotic lesions, and all of them had coronary aneurysms. MSCT was performed using a Siemens SOMATOM Volume Zoom (4-detector row) or a Toshiba Aquillion 16 (16-detector row). Findings of coronary calcification, stenotic lesion, and intimal hypertrophy in all coronary arteries were compared to those of CAG. RESULTS: Eleven of the 18 patients (61%) had novel findings detected by MSCT. Coronary calcifications were found in 11 of the 18 patients (61%). Five patients had concentric calcified aneurysms, four had eccentric calcified aneurysms, and two had mixed calcified aneurysms. Coronary stenotic lesions were present in 6 of the 18 patients (33%) with calcified aneurysms. Two patients had intimal hypertrophy (11%). One patient had intimal hypertrophy along the left main trunk with a giant calcified aneurysm along the left anterior descending artery. Two patients had severe stenoses just distal to giant calcified aneurysms that were regarded as false positive findings, and were identified as mild stenoses by CAG. CONCLUSIONS: MSCT offers advantages over CAG in the evaluation of calcified aneurysms and intimal hypertrophy, and is a potential diagnostic modality for coronary intervention in patients after Kawasaki disease.

Characteristics of obese children with low content of arachidonic acid in plasma lipids.

BACKGROUND: Although there have been many studies on the relationship between obesity and long-chain polyunsaturated fatty acid (LCPUFA), the results and their interpretation are controversial, especially in children. Arachidonic acid (AA), the product of n-6 LCPUFA, is reported to be related to insulin resistance. The purpose of the present paper was to investigate the LCPUFA profile in obese children and mechanisms that contribute to reduced AA content. METHOD: An age- and sex-matched control study was performed. The study subjects were 59 obese children (mean age, 11.8 years) and 53 healthy non-obese children (mean age, 12.5 years). The study parameters included anthropometric measurements, serum lipids, leptin and fatty acid composition in plasma. RESULTS: Plasma fatty acids in obese children had lower linoleic acid (P < 0.0001) and higher dihomo-gamma-linolenic acid (P = 0.0004) than those in non-obese children. In all subjects combined, delta-6 desaturase (D6D) index (ratios of [C 18:3n-6+C 20:2n-6]/C 20:4n-6 or C 20:4n-6/C 18: 2n-6) correlated with leptin (P < 0.0001). There was no significant difference in AA content between obese and non-obese. However, the AA content was low (

Usefulness of real-time 3-dimensional echocardiography for the evaluation of coronary artery morphology in patients with Kawasaki disease.

OBJECTIVES: This study was designed to evaluate the usefulness of real-time 3-dimensional echocardiography (RT-3DE) for evaluating coronary artery morphology in patients with Kawasaki disease. BACKGROUND: The diagnosis of coronary artery morphology in the acute phase of the disease is of prime importance for evaluating the likelihood of cardiovascular sequelae. Occasionally, visualization of the right coronary artery and bifurcated regions, including the circumflex artery, has proved challenging with traditional echocardiographic methods. METHODS: A total of 111 patients with Kawasaki disease were studied. Coronary aneurysms were detected in 8 patients (4 had giant aneurysms), and coronary dilation was found in 11 patients. Coronary artery visualization was evaluated and scored as 1 of 4 grades, 0 to 3 points, for both 2-dimensional echocardiography (2DE) and RT-3DE. Evaluation criteria for each coronary artery were defined according to the American Heart Association classification of coronary angiographic features. Total scores for each patient and for individual coronary branches were compared between 2DE and RT-3DE. RESULTS: The total scores for coronary artery visualization were, respectively, showing a significantly higher score for RT-3DE than for 2DE (P < .01). A mural thrombus could be clearly delineated in the giant coronary aneurysms by RT-3DE. CONCLUSIONS: RT-3DE is superior to 2DE for coronary artery visualization. This diagnostic system is expected to improve the screening of coronary artery abnormalities in Kawasaki disease.