The efficacy and safety of intravenous immunoglobulin infusion in 12 h for the initial treatment of Kawasaki disease.

BACKGROUND: Approximately 10-20 % of individuals develop a recrudescent or persistent fever after intravenous immunoglobulin (IVIG) infusion for the initial treatment of Kawasaki disease. The aim of this study was to evaluate the efficacy and safety of the initial IVIG treatment of Kawasaki disease based on duration of infusion. METHODS: This retrospective, single-center study included 53 patients with Kawasaki disease who were initially treated with 2 g/kg of IVIG by means of a single infusion from June 2018 to August 2019. We classified patients into two groups based on the duration of the infusion: the 12-h group and the 24-h group. We compared the treatment response of the primary IVIG and its adverse events using the Mann-Whitney U test and Fisher's exact or Chi-square tests. RESULTS: There were no significant differences in the response to initial IVIG treatment between the two groups. The duration from treatment onset to defervescence was shorter in the 12-h group than the 24-h group (7 h vs. 12 h, respectively, p = 0.07); however, this was not significant. There were no significant between-group differences regarding adverse events. CONCLUSION: We concluded that the initial 12-h IVIG treatment was comparable to the 24-h treatment in terms of efficacy and safety. This will enable physicians to feel confident about pursuing a shorter course of treatment with similar results as conventional treatment and decide on administering additional therapy to their patients.

Effectiveness of inactivated influenza and COVID-19 vaccines in hospitalized children in 2022/23 season in Japan - The first season of co-circulation of influenza and COVID-19.

We have analyzed the inactivated vaccine effectiveness (VE)for preventing influenza hospitalization by test-negative design in the 2022/23 season. This is the first season of co-circulation of influenza and COVID-19, and a unique period because all inpatients received COVID-19 screening. Among 536 children hospitalized with fever, none were positive for both influenza and SARS-CoV-2. The adjusted VE for preventing influenza A for all children, the 6-12-year-old group, and those with underlying diseases was 34 % (95 ％CI, -16 %-61 %, n = 474), 76 % (95 ％ CI, 21 %-92 %, n = 81), and 92 % (95 ％ CI, 30 %-99 %, n = 86), respectively. Only 1 out of 35 hospitalized cases with COVID-19, and 42 out of 429 controls, had been immunized with COVID-19 vaccine. This is the first report showing influenza VE by age group in children in this limited season. We still recommend the inactivated influenza vaccine for children based on the significant VE in subgroup analysis.

Left bronchial compression after an arterial switch operation with the LeCompte maneuver for transposition of the great arteries: A case report and literature review.

Left bronchial compression is a rarely reported, postoperative complication of the arterial switch operation with the LeCompte maneuver for transposition of the great arteries. Postoperative neopulmonary root dilatation and the anterior-posterior, anatomical relationship of the great vessels may cause this condition. Hypoxic pulmonary vasoconstriction may mask the condition even if the left bronchus has been severely obstructed. The apparent inconsistency between the abnormally decreased pulmonary blood flow and the absence of any irregularities in the vascular structure that might account for it suggested hypoxic pulmonary vasoconstriction to be the cause. We present herein a case of left bronchial compression presenting malacia after an arterial switch operation with the LeCompte maneuver and also present a review of seven, other, reported cases. Learning objectives: Left bronchial compression is a rare complication of the arterial switch operation with the LeCompte maneuver for transposition of the great arteries and is possibly caused by root dilatation and the anatomical relationship of the great vessels. Hypoxic pulmonary vasoconstriction may mask the condition.

Analysis of Coronary Arterial Aneurysm Regression in Patients With Kawasaki Disease by Aneurysm Severity: Factors Associated With Regression.

Background Coronary arterial aneurysms (CAAs) associated with Kawasaki disease (KD) significantly affect prognosis. However, the clinical course of CAAs and factors associated with CAA regression have not been well analyzed. Methods and Results The cohort of the Z-Score 2nd Project Stage study, a multicenter, retrospective, cohort study involving 44 institutions in Japan including 1006 patients with KD, was examined. CAAs were classified by the z score of their internal diameter in the acute phase: small (z<5), medium (5≤z<10), and large (z≥10). The lower limit of small CAA was based on the Japanese Ministry of Health, Labour and Welfare criteria. In the right coronary artery, the CAA regression rates 10 years after diagnosis were 95.5% for small, 83.2% for medium, and 36.3% for large. In the proximal left anterior descending artery, the regression rates 10 years after diagnosis were 95.3% for small, 80.1% for medium, and 28.8% for large. Cox regression analysis showed that diagnosis under the age of 1 year and onset of KD in 2010 to 2012 for the right coronary artery and the left anterior descending artery, and female for the right coronary artery were significantly associated with a high regression rate, whereas large CAAs for the right coronary artery and the left anterior descending artery were significantly associated with a low regression rate. Conclusions The current study, the largest Japanese study of its kind, found that small aneurysm, recent onset, and diagnosis under the age of 1 year predict regression, and that even giant aneurysms could regress. These data may contribute to long-term management of coronary aneurysms. Registration URL: https://www.umin.ac.jp/ctr/; Unique identifier: UMIN000010606.

Plasma mid-regional pro-adrenomedullin: A biomarker of the ischemic penumbra in hyperacute stroke.

Reperfusion therapy has improved the outcomes of ischemic stroke but also emphasized the importance of ischemic penumbra. However, blood biomarkers are currently unavailable for this region. Adrenomedullin (ADM) is a neuroprotective peptide, secreted in a compensatory response to brain ischemia. We thus investigated whether an increase in mid-regional pro-ADM (MR-proADM), a stable peptide fragment of the ADM precursor, could act as a biomarker by predicting the ischemic penumbra in hyperacute ischemic stroke (HAIS). We prospectively enrolled consecutive HAIS patients (n = 119; median age, 77 years; male, 59.7%) admitted to our institutes from July 2017 to March 2019 and evaluated plasma MR-proADM levels within 4.5 h of onset. MR-proADM levels in HAIS were compared to healthy controls (n = 1298; median age, 58 years; male, 33.2%) in the Japan Multi-Institutional Collaborative Cohort Study from 2013 to 2017. Furthermore, we evaluated whether MR-proADM levels were associated with the penumbra estimated by clinical-diffusion mismatch (CDM) (National Institute of Health Stroke Scale [NIHSS] ≥8, diffusion ischemic core volume ≤25 ml), or magnetic resonance angiography-diffusion-weighted imaging mismatch (MDM) (NIHSS ≥5, a proximal vessel occlusion with core volume ≤25 ml, or a proximal vessel stenosis/distal vessel occlusion with core volume ≤15 ml). In a case-control study, multivariate logistic analysis showed a significant association between HAIS and MR-proADM ≥0.54 nmol/L (adjusted odds ratio, 7.92 [95% CI, 4.17-15.02], p < 0.001). Though MR-proADM levels in HAIS did not correlate with the ischemic core volume (rs  = 0.09, p = 0.348), they were higher in HAIS with CDM (n = 34; 0.81 vs. 0.61 nmol/L, p < 0.001) or MDM (n = 26; 0.83 vs. 0.62 nmol/L, p = 0.002). These differences remained significant after adjusting baseline factors (adjusted odds ratio, 4.06 [95% CI, 1.31-12.55], p = 0.015 and 4.65 [1.35-16.11], p = 0.015, respectively). Plasma MR-proADM is elevated in HAIS, especially in those with a substantial penumbra, suggesting potential as a blood biomarker in this region.

Intramural aortic course in anomalous origin of single coronary artery from the right pulmonary artery: A case report and review of the literature.

Preoperatively determining the intramural aortic course in a case of anomalous origin of single coronary artery from the right pulmonary artery is important for planning surgery, such as the unroofing technique. A thin wall separating the ascending aorta from the common coronary artery on transthoracic echocardiography and a crescent-shaped cross-section of the common coronary artery on computed tomography demonstrate an intramural aortic course of the common coronary artery. We present herein a case of anomalous origin of single coronary artery from the right pulmonary artery with an intramural aortic course and review eight other reported cases. .

Acute Rheumatic Fever and Kawasaki Disease Occurring in a Single Patient.

Kawasaki disease and acute rheumatic fever are two major causes of acquired heart disease in the pediatric population. Although both conditions are well-known entities, the association between them has never been described. We report herein a case of 6-year-old male patient who first presented with Kawasaki shock syndrome, followed by acute rheumatic fever 1 year later. In contrast to the prompt intervention given for atypical Kawasaki disease, in the present case the diagnosis was significantly delayed during the otherwise typical presentation of acute rheumatic fever. Our case highlights the difficulty experienced by many pediatricians in developed nations in diagnosing acute rheumatic fever due to its comparative rarity. To prevent diagnostic errors, all pediatricians should be alert to the possibility of acute rheumatic fever even if they are practicing in areas where it is not endemic.

Flecainide improves cardiac synchronization in an early infant with Wolff-Parkinson-White syndrome with left ventricular dyssynchrony.

Recently, cases of pharmacological resynchronization for Wolff-Parkinson-White syndrome (WPWS) in children with left ventricular dyssynchrony (LVD) were reported, but an appropriate pharmacological therapy has not yet been established. A 3-month-old, previously healthy female patient was referred to our hospital due to supraventricular tachycardia (SVT). After resolution of the SVT, 12-lead electrocardiography (ECG) showed ventricular pre-excitation. Transthoracic echocardiography showed LVD with no findings of congenital heart disease or cardiomyopathy. To prevent SVT recurrence, oral propranolol administration was started, but the SVT recurred one month later. To prevent further recurrences, oral flecainide administration was started, as the patient's body weight was insufficient for catheter ablation to be performed safely. When the flecainide dosage was increased to 50 mg/m2/day, the pre-excitation resolved, and the LVD improved. Holter ECG showed that the resolution of pre-excitation depended on the serum concentration of flecainide. There are only few reports on pharmacological resynchronization in WPWS patients with LVD (LVD-WPWS). The present report is the first to examine the efficacy of flecainide in patients with recurrent SVT. Flecainide may be a safe and effective alternative resynchronization therapy for LVD-WPWS patients, especially for children in whom catheter ablation cannot be performed safely due to insufficient body weight. .

Clinical and structural aspects of tracheal stenosis and a novel embryological hypothesis of left pulmonary artery sling.

OBJECTIVES: To identify the imaging features peculiar to congenital tracheal stenosis (CTS) complicated with left pulmonary artery sling (LPAS) with the aim of presenting a hypothesis of tracheal stenosis embryology in LPAS. METHODS: We retrospectively reviewed CTS patients (with complete cartilaginous rings) admitted between April 2010 and July 2018. All the patients were classified into the LPAS or non-LPAS group, and their clinical characteristics and qualitative variables on computed tomography (CT) imaging were compared. RESULTS: Of the 72 patients enrolled, 61 had bilateral lungs. Among the bilateral lung patients, 26 (43%) had LPAS. The tracheal bifurcation was significantly deeper, the stenotic region was longer, and the bronchial angle (especially in the right) was wider, in the LPAS group. The cut-off values for the thoracic vertebral level at the tracheal bifurcation (>4.8), subcarinal angle (>118.1), and right bronchial angle (>61.9) were useful for diagnosing suspected cases of LPAS. In the time-dependent course, LPAS complicated with a congenital heart defect was a statistically significant risk factor of respiratory symptoms (hazard ratio, 3.01; 95% confidence interval, 1.23-7.37; P = .02). CONCLUSIONS: The CT findings described here should immediately raise suspicion of LPAS on chest X-ray and also suggest tracheal "squeezing and milking" by the surrounding vessels in the embryo. Patients with LPAS complicated with a heart defect should be followed carefully to determine the optimal timing of intervention.

[Primary Pulmonary Vein Stenosis Developed after Bidirectional Glenn Procedure;Report of Two Cases].

We experienced 2 cases of primary pulmonary vein stenosis(PVS),which developed after a bidirectional Glenn procedure was performed for complex heart defects with normal pulmonary venous return. Although the patients successfully underwent primary sutureless repair for left PVS, restenosis of the affected pulmonary veins occurred several months after surgery in both patients. Stent implantation followed by balloon angioplasty was performed for stent stenosis in 1 patient without effect. However, the patient later underwent a successful fenestrated Fontan procedure. Catheter intervention was contraindicated in the 2nd patient due to almost complete obstruction of the left pulmonary veins with upstream hypoplasia. To improve the results of PVS treatment, earlier diagnosis by quantitative lung perfusion scintigraphy, magnetic resonance imaging, and close echocardiographic observation together with earlier, aggressive treatment combining surgery and catheter interventions are recommended.

Association of Severity of Coronary Artery Aneurysms in Patients With Kawasaki Disease and Risk of Later Coronary Events.

Importance: Few studies with sufficient statistical power have shown the association of the z score of the coronary arterial internal diameter with coronary events (CE) in patients with Kawasaki disease (KD) with coronary artery aneurysms (CAA). Objective: To clarify the association of the z score with time-dependent CE occurrence in patients with KD with CAA. Design, Setting, and Participants: This multicenter, collaborative retrospective cohort study of 44 participating institutions included 1006 patients with KD younger than 19 years who received a coronary angiography between 1992 and 2011. Main Outcomes and Measures: The time-dependent occurrence of CE, including thrombosis, stenosis, obstruction, acute ischemic events, and coronary interventions, was analyzed for small (z score, <5), medium (z score, ≥5 to <10; actual internal diameter, <8 mm), and large (z score, ≥10 or ≥8 mm) CAA by the Kaplan-Meier method. The Cox proportional hazard regression model was used to identify risk factors for CE after adjusting for age, sex, size, morphology, number of CAA, resistance to initial intravenous immunoglobulin (IVIG) therapy, and antithrombotic medications. Results: Of 1006 patients, 714 (71%) were male, 341 (34%) received a diagnosis before age 1 year, 501 (50%) received a diagnosis between age 1 and 5 years, and 157 (16%) received a diagnosis at age 5 years or older. The 10-year event-free survival rate for CE was 100%, 94%, and 52% in men (P < .001) and 100%, 100%, and 75% in women (P < .001) for small, medium, and large CAA, respectively. The CE-free rate was 100%, 96%, and 79% in patients who were not resistant to IVIG therapy (P < .001) and 100%, 96%, and 51% in patients who were resistant to IVIG therapy (P < .001), respectively. Cox regression analysis revealed that large CAA (hazard ratio, 8.9; 95% CI, 5.1-15.4), male sex (hazard ratio, 2.8; 95% CI, 1.7-4.8), and resistance to IVIG therapy (hazard ratio, 2.2; 95% CI, 1.4-3.6) were significantly associated with CE. Conclusions and Relevance: Classification using the internal diameter z score is useful for assessing the severity of CAA in relation to the time-dependent occurrence of CE and associated factors in patients with KD. Careful management of CE is necessary for all patients with KD with CAA, especially men and IVIG-resistant patients with a large CAA.

Effect of catheter positions on hemodynamics and coil formation after coil embolization.

Coil embolization using a micro catheter and coils is one of the most popular surgical methods used for treating intracranial aneurysms. Surgeons need to better understand the effects of changing catheter position because this is under their control during operations. In this study, we simulate coil embolization for a basic bifurcation-type aneurysm using finite element method and computational fluid dynamics. We evaluated the reduction in velocity after embolization and the distance of the embolized coil's center of gravity from the center of the aneurysm (rCG) while changing the catheter tip position. The results show there is a high correlation between velocity reduction and neck volume embolization ratio (NVER) with a correlation coefficient of 0.955, and between the catheter tip position and rCG with a correlation coefficient of 0.866. These results indicate the NVER is also important for effective velocity reduction in both bifurcation-type and sidewall-type aneurysms. In addition, it is useful to set the catheter tip at the bottom of the aneurysms to deploy the first coil as a framing coil.

[A case of anti-MOG antibody-positive multiphasic disseminated encephalomyelitis co-occurring with unilateral cerebral cortical encephalitis].

A 20-year-old woman first developed acute disseminated encephalomyelitis (ADEM) at 11 years of age. At 17 years of age, she was hospitalized due to generalized seizure and diagnosed with encephalitis. Brain MRI revealed a FLAIR-hyperintense lesion in the unilateral cerebral cortex. At 18 years of age, serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody was detected. At 20 years of age, she was admitted to our hospital, diagnosed with multifocal disseminated encephalomyelitis (MDEM). MDEM has been observed in patients that are seropositive for the anti-MOG antibody. More recently, unilateral cerebral cortex encephalitis with epilepsy has also been reported in such patients. The co-occurrence of MDEM and cortical encephalitis in the same patient has important implications for the pathogenesis of anti-MOG antibody-associated autoimmune diseases.

Relationships between geometrical parameters and mechanical properties for a helical braided flow diverter stent.

BACKGROUND: Although flow diversion is a promising procedure for aneurysm treatment, the safety and efficacy of this strategy have not been sufficiently characterized. Both mechanical properties and flow reduction effects are important factors in the design of an optimal stent. OBJECTIVE: We aimed to clarify the contributions of strut size and pitch to the mechanical properties (radial stiffness and longitudinal flexibility) and geometric characteristics (porosity and pore density) related to flow reduction effects. METHODS: Crimping and bending behaviors of the stents were simulated with the finite element method. The relationships between the mechanical properties and geometric characteristics were investigated by changing the strut size and pitch. RESULTS: Within the porosity range of 79-82%, the radial stiffness of the stent was similarly influenced by either the strut size or pitch. However, the longitudinal flexibility tended to be influenced more by strut size than by pitch. CONCLUSIONS: Adjusting the strut size rather than the pitch can change the mechanical properties while minimizing the change in porosity or pore density related to flow reduction effects.

Selection of helical braided flow diverter stents based on hemodynamic performance and mechanical properties.

BACKGROUND: Although flow diversion is a promising procedure for the treatment of aneurysms, complications have been reported and it remains poorly understood. The occurrence of adverse outcomes is known to depend on both the mechanical properties and flow reduction effects of the flow diverter stent. OBJECTIVE: To clarify the possibility of designing a flow diverter stent considering both hemodynamic performance and mechanical properties. MATERIALS AND METHODS: Computational fluid dynamics (CFD) simulations were conducted based on an ideal aneurysm model with flow diverters. Structural analyses of two flow diverter models exhibiting similar flow reduction effects were performed, and the radial stiffness and longitudinal flexibility were compared. RESULTS: In CFD simulations, two stents-Pore2-d35 (26.77° weave angle when fully expanded, 35 µm wire thickness) and Pore3-d50 (36.65°, 50 µm respectively)-demonstrated similar flow reduction rates (68.5% spatial-averaged velocity reduction rate, 85.0% area-averaged wall shear stress reduction rate for Pore2-d35, and 68.6%, 85.4%, respectively, for Pore3-d50). However, Pore3-d50 exhibited greater radial stiffness than Pore2-d35 (40.0 vs 21.0 mN/m at a 3.5 mm outer diameter) and less longitudinal flexibility (0.903 vs 0.104 N·mm bending moments at 90°). These measurements indicate that changing the wire thickness and weave angle allows adjustment of the mechanical properties while maintaining the same degree of flow reduction effects. CONCLUSIONS: The combination of CFD and structural analysis can provide promising solutions for an optimized stent. Stents exhibiting different mechanical properties but the same flow reduction effects could be designed by varying both the weave angle and wire thickness.

A neonatal prolonged QT syndrome due to maternal use of oral tricyclic antidepressants.

UNLABELLED: It is known that tricyclic antidepressants induce long QT intervals associated with forms of life-threatening arrhythmia such as torsades de pointes (TdP), and these adverse effects may also occur in neonates whose mothers take tricyclic antidepressants. We report a neonatal case of prolonged QT interval and TdP caused by clomipramine that was transferred transplacentally from the mother. Administration of magnesium sulfate was effective to abolish TdP. CONCLUSION: When mothers take tricyclic antidepressants during pregnancy, their newborns should be watched carefully for drug-induced long QT syndrome and TdP. WHAT IS KNOWN: •Tricyclic antidepressant can prolong the QT interval. It may be used for depression in pregnancy. What is New: •This is the first neonatal case report of prolonged QT interval and TdP caused by clomipramine transferred transplacentally from the mother.

Bioelectrochemical analysis of a hyperthermophilic microbial fuel cell generating electricity at temperatures above 80 °C.

We examined whether a hyperthermophilic microbial fuel cell (MFC) would be technically feasible. Two-chamber MFC reactors were inoculated with subsurface microorganisms indigenous to formation water from a petroleum reservoir and were started up at operating temperature 80 °C. The MFC generated a maximum current of 1.3 mA 45 h after the inoculation. Performance of the MFC improved with an increase in the operating temperature; the best performance was achieved at 95 °C with the maximum power density of 165 mWm(-2), which was approximately fourfold higher than that at 75 °C. Thus, to our knowledge, our study is the first to demonstrate generation of electricity in a hyperthermophilic MFC (operating temperature as high as 95 °C). Scanning electron microscopy showed that filamentous microbial cells were attached on the anode surface. The anodic microbial consortium showed limited phylogenetic diversity and primarily consisted of hyperthermophilic bacteria closely related to Caldanaerobacter subterraneus and Thermodesulfobacterium commune.

Idiopathic mitral valve chordae rupture in an infant: importance of rapid diagnosis and surgery.

Mitral valve chordae rupture in infancy is a rare, but life-threatening disease. The progression of acute cardiac failure has been reported, with emergency surgery being required in most cases. Mitral valve chordae rupture typically occurs at the age of 4-6 months. Echocardiography is needed to diagnose this disease, and accurate diagnosis is difficult for general pediatricians. We herein describe the case of an acutely ill 4-month-old infant, who was saved and discharged without neurological sequelae due to the early diagnosis of mitral valve chordae rupture, life support, and surgery. We confirm the importance of acute pre-surgery treatment and immediate surgery for the survival and good outcome of infants.