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Background. Syncope is a common symptom in children, many of which are benign and do not require treatment. Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital malformation but can be a risk for serious cardiovascular events, including sudden death as well as cardiogenic syncope. Case Report. We describe the case of a 14-year-old boy who suffered an initial syncope and afebrile seizure during a soccer game. A detailed medical history and imaging studies led to the diagnosis of the anomalous aortic origin of the left main coronary artery with an intramural course (AAOLCA-IM). Conclusion. Symptomatic AAOLCA-IM has the highest risk of sudden death among AAOCA, and surgical repair may be performed. Onset during exercise or preceding chest symptoms are suspicious signs of cardiogenic syncope and should be considered for cardiovascular imaging evaluation. [ABSTRACT FROM AUTHOR]

Background: Delirium is common in critically ill patients. Haloperidol has long been used for the treatment of delirium. Dexmedetomidine has recently been used to treat delirium among intubated critically ill patients. However, the efficacy of dexmedetomidine for delirium in non-intubated critically ill patients remains unknown. We hypothesize that dexmedetomidine is superior to haloperidol for sedation of patients with hyperactive delirium, and would reduce the prevalence of delirium among non-intubated patients after administration. We will conduct a randomized controlled trial to compare dexmedetomidine and haloperidol for the treatment of nocturnal hyperactive delirium in non-intubated patients in high dependency units (HDUs). Methods: This is an open-label, parallel-group, randomized controlled trial to compare the efficacy and safety of dexmedetomidine and haloperidol for nocturnal hyperactive delirium in non-intubated patients at two HDUs of a tertiary hospital. We will recruit consecutive non-intubated patients who are admitted to the HDU from the emergency room, and allocate them in a 1:1 ratio to the dexmedetomidine or haloperidol group in advance. The allocated investigational drug will be administered only when participants develop hyperactive delirium (Richmond Agitation-Sedation Scale [RASS] score ≥1 and a positive score on the Confusion Assessment Method for the ICU between 19:00 and 6:00 the next day) during the night at an HDU. Dexmedetomidine is administered continuously, while haloperidol is administered intermittently. The primary outcome is the proportion of participants who achieve the targeted sedation level (RASS score of between -3 and 0) 2h after the administration of the investigational drug. Secondary outcomes include the sedation level and prevalence of delirium on the day following the administration of the investigational drugs, and safety. We plan to enroll 100 participants who develop nocturnal hyperactive delirium and receive one of the two investigational drugs. Discussion: This is the first randomized controlled trial to compare the efficacy and safety of dexmedetomidine and haloperidol for sedation of non-intubated critically ill patients with hyperactive delirium in HDUs. The results of this study may confirm whether dexmedetomidine could be another option to sedate patients with hyperactive delirium. Trial registration: Japan Registry of Clinical Trials, jRCT1051220015, registered on 21 April 2022. [ABSTRACT FROM AUTHOR]

The 2-lead Holter ECG also showed a paroxysmal second-degree atrioventricular block, with a maximal R-R interval of 1.5 s, and several episodes of sinus bradycardia (Fig. Holter electrocardiography can be helpful in preterm infants to detect and define paroxysmal atrioventricular block. The heart rate abnormalities gradually improved, and a 2-lead Holter ECG obtained at 3 months of age showed sinus arrest, a maximal R-R interval of 1.9 s, and sinus bradycardic events (maximal R-R interval, 1.0 s) without paroxysmal atrioventricular block (PAVB). [Extracted from the article]

We herein describe the first pediatric case of an internal mammary artery (IMA) aneurysm caused by a median sternotomy. He was a 2-year-old with tricuspid atresia who underwent an extracardiac conduit Fontan procedure. On the 36th postoperative day, an asymptomatic left IMA aneurysm was detected via contrast computed tomography, which was successfully treated with coil embolization. The patient had no underlying disease such as vasculitis, connective tissue disease, or other hereditary diseases, and there were no episodes of infection or hypertension before or after the onset of the IMA aneurysm. Because the left IMA ran medially to the periphery and was in a vulnerable position during median sternotomy, we considered the IMA aneurysm was caused by the median sternotomy. We pediatric cardiologists should be aware that IMA aneurysms can occur in pediatric cardiac surgery, and we should be proactive in performing postoperative imaging studies in cases where the preoperative internal thoracic artery runs medially toward the periphery. Internal mammary artery (IMA) aneurysm is a rare vascular disease, especially in children. One-third of adult IMA aneurysms have been reported to be caused by sternotomy, but not in children. We report the first pediatric case of an IMA aneurysm caused by sternotomy. We should recognize that there is a potential risk of IMA aneurysms in pediatric cardiac surgery as well. [ABSTRACT FROM AUTHOR]

The article presents a case study of a neonate with a rare condition of neonatal complicated right ventricular diverticulum (RVD) with effective contraction. It is reported that the baby, prenatally diagnosed with double chamber right ventricle and pulmonary valve stenosis, exhibited cyanosis at birth. It is further reported that echocardiography revealed an abnormal muscle bundle dividing the right ventricle, diagnosed as RVD.

Objectives: It is important to clarify the relationship between irreversible organ damage and the quality of life (QOL) by considering the unique factors of patients with systemic lupus erythematosus (SLE). We aimed to clarify their correlation using SLE-specific QOL assessment tools. We also aimed to identify which type of organ damage is adversely correlated with the QOL. Methods: We conducted a questionnaire-based survey of outpatients with SLE at Kyoto University Hospital and evaluated irreversible organ damage using the SLICC/ACR damage index (SDI). LupusPRO and the SLE symptom checklist (SSC) were employed as SLE-specific QOL tools, and the SF-36v2 was used as a conventional QOL tool. Multiple linear regression analyses were performed to examine the correlations between the total SDI score and each QOL score, and between each SDI item/system score and each QOL score. Results: We analyzed the data of 265 patients. The total SDI score was significantly correlated with physical (PCS) and role/social component summary (RCS) of the SF-36v2, health-related QOL (HRQOL) of LupusPRO, and SSC (p < 0.001). Among the SDI items, atrophy/weakness and osteoporosis with fracture/vertebral collapse were negatively correlated with PCS (β = −0.40, p < 0.001/β = −0.28, p < 0.001), RCS (β = −0.30, p < 0.001/β = −0.35, p < 0.001), and HRQOL (β = −0.34, p < 0.001/β = −0.31, p < 0.001), respectively. Among the SDI systems, musculoskeletal damage had higher negative correlations with PCS (β = −0.51, p < 0.001), RCS (β = −0.29, p < 0.001), and HRQOL (β = −0.40, p < 0.001). Conclusion: We demonstrated the QOL of patients with SLE is negatively correlated with irreversible organ damage. We also revealed musculoskeletal damage is adversely correlated with the health-related QOL, especially the physical and role/social QOL. [ABSTRACT FROM AUTHOR]

Innate immune activity plays an essential role in the development of Kawasaki disease (KD) vasculitis. Extracellular release of high mobility group box-1 (HMGB-1), an endogenous damage-associated molecular pattern protein that can activate the innate immune system and drive host inflammatory responses, may contribute to the development of coronary artery abnormalities in KD. Prednisolone (PSL) added to intravenous immunoglobulin treatment for acute KD may reduce such abnormalities. Here, we evaluate the dynamics of HMGB-1 and therapeutic effects of PSL on HMGB-1-mediated inflammatory pathways on KD vasculitis in vitro. Serum samples were collected prior to initial treatment from patients with KD, systemic juvenile idiopathic arthritis (sJIA), and from healthy controls (VH), then incubated with human coronary artery endothelial cells (HCAECs). Following treatment of KD serum-activated HCAECs with PSL or PBS as a control, effects on the HMGB-1 signaling pathway were evaluated. Compared to that from VH and sJIA, KD serum activation induced HCAEC cytotoxicity and triggered extracellular release of HMGB-1. KD serum-activated HCAECs up-regulated extracellular signal-regulated kinase (ERK)1/2, c-Jun N-terminal kinase (JNK) and, p38 phosphorylation in the cytoplasm and nuclear factor kappa B (NF-κB) phosphorylation in the nucleus and increased interleukin (IL)-1β and tumor necrosis factor (TNF)-α production. PSL treatment of KD serum-activated HCAECs inhibited extracellular release of HMGB-1, down-regulated ERK1/2, JNK, p38, and NF-κB signaling pathways, and decreased IL-1β and TNF-α production. Our findings suggest that extracellular HMGB-1 plays an important role in mediating KD pathogenesis and that PSL treatment during the acute phase of KD may ameliorate HMGB-1-mediated inflammatory responses in KD vasculitis. [ABSTRACT FROM AUTHOR]

Objectives: Natriuretic peptides are an important prognostic marker in patients with heart failure (HF). However, little is known regarding their prognostic significance in patients with atrial fibrillation (AF) without HF and natriuretic peptides levels are underused in these patients in daily practice.Methods: The Fushimi AF Registry is a community-based prospective survey of patients with AF in Fushimi-ku, Kyoto, Japan. We investigated patients with AF without HF (defined as prior HF hospitalisation, New York Heart Association functional class≥2 or left ventricular ejection fraction<40%) using the data of B-type natriuretic peptide (BNP, n=388) or N-terminal pro-B-type natriuretic peptide (NT-proBNP, n=771) at enrolment. BNPs were converted to NT-proBNP using a conversion formula. We divided the patients according to quartiles of NT-proBNP levels and compared the backgrounds and outcomes.Results: Of 1159 patients (mean age: 72.1±10.2 years, median CHA2DS2-VASc score: 3 and oral anticoagulant (OAC) prescription: 671 (56%)), the median NT-proBNP level was 488 (IQR 169-1015) ng/L. Patients with high NT-proBNP levels were older, had higher CHA2DS2-VASc scores and had more OAC prescription (all p<0.001). Kaplan-Meier curves demonstrated that NT-proBNP levels were significantly associated with higher incidences of stroke/systemic embolism, all-cause death and HF hospitalisation during a median follow-up period of 5.0 years (log rank, all p<0.001). Multivariable Cox regression analyses revealed that NT-proBNP levels were an independent predictor of adverse outcomes even after adjustment by various confounders.Conclusion: NT-proBNP levels are a significant prognostic marker for adverse outcomes in patients with AF without HF and may have clinical value.Trial Registration Number: UMIN000005834. [ABSTRACT FROM AUTHOR]

The article presents case study of patient was a 14-day-old female neonate with no specific abnormalities, who had growth restriction during her fetal period. It mentions diagnosis with neonatal cerebral sinovenous thrombosis (CSVT) because of its lower risk of bleeding compared with unfractionated heparin.

Transitive inference (TI) is the ability to infer unknown relationships from previous information. To test TI in non-human animals, transitive responding has been examined in a TI task where non-adjacent pairs were presented after premise pair training. Some mammals, birds and paper wasps can pass TI tasks. Although previous studies showed that some fish are capable of TI in the social context, it remains unclear whether fish can pass TI task. Here, we conducted a TI task in cleaner wrasses (Labroides dimidiatus), which interact with various client fishes and conspecifics. Because they make decisions based on previous direct and indirect interactions in the context of cleaning interactions, we predicted that the ability of TI is beneficial for cleaner fish. Four tested fish were trained with four pairs of visual stimuli in a 5-term series: A-B+, B-C+, C-D+, and D-E+ (plus and minus denote rewards and non-rewards, respectively). After training, a novel pair, BD (BD test), was presented wherein the fish chose D more frequently than B. In contrast, reinforcement history did not predict the choice D. Our results suggest that cleaner fish passed the TI task, similar to mammals and birds. Although the mechanism underlying transitive responding in cleaner fish remains unclear, this work contributes to understanding cognitive abilities in fish. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 23-year-old woman with no significant medical history was diagnosed with Wolff–Parkinson–White (WPW) syndrome during pregnancy and her fetus was diagnosed with tetralogy of Fallot (TOF). Topics include the electrocardiogram has showed right axis deviation and short PR interval with a narrow QRS complex, the transthoracic echocardiogram has showed a ventricular septal defect, and the medications were not required during the neonatal period.

Aim: We aimed to validate the incidence of, risk factors for, and postoperative outcomes of acute kidney injury (AKI) according to the modified Kidney Disease Improving Global Outcomes (m‐KDIGO) criteria and compare this criteria with both the paediatric Risk, Injury, Failure, Loss, End‐stage disease (pRIFLE) and Acute Kidney Injury Network (AKIN) criteria in infants after cardiac surgery. Methods: We retrospectively enrolled 145 consecutive infants who underwent open‐heart surgery at Kagoshima University Hospital. Results: Acute kidney injury was present in 55 (37.9%), 111 (75.9%), and 95 (65.5%) patients according to the m‐KDIGO, pRIFLE, and AKIN criteria, respectively. Among these, 71.9% of patients pRIFLE Risk patients and 60.5% of AKIN 1 patients were categorized in the 'no‐AKI' group according to the m‐KDIGO criteria. Low body weight (m‐KDIGO odds ratio [OR], 0.73; P = 0.015; pRIFLE OR, 0.66; P = 0.001; AKIN OR 0.69, P = 0.002) and prolonged cross‐clamp time (m‐KDIGO OR, 1.02;

A 14-year-old boy was diagnosed with an anomalous left coronary artery coursing between the ascending aorta and the main pulmonary artery and associated with a single coronary ostium. Owing to the high risk of sudden cardiac death, surgery was performed although he was asymptomatic with no sign of myocardial ischemia. Reimplantation of an anomalous left coronary artery is generally considered difficult because an aortic cuff is unavailable for coronary anastomosis; however, we accomplished a successful direct reimplantation in this patient. This procedure offers another choice in the surgical treatment of anomalous left coronary artery. [ABSTRACT FROM AUTHOR]

Background: Low levels of serum immunoglobulin G (IgG) before intravenous immunoglobulin (IVIG) therapy for Kawasaki disease (KD) have been reported as one of the risk factors for coronary artery abnormalities (CAAs). This risk factor needs to be re-evaluated because the dosage of IVIG has changed from 0.2-0.4 g/kg/day for 5 days to a single high dose of 2 g/kg. Methods: We reviewed the clinical records of KD patients admitted to our hospital from January 2001 to August 2011. Patients who were given a single high dose of IVIG within 7 days of illness, and who had blood collected for serum immunoglobulin values before treatment, were selected. The serum immunoglobulin levels and coronary artery diameters measured by echocardiogram were transformed to z-scores. Results: The subjects were 197 KD patients, including 22 IVIG nonresponders and 16 patients with CAAs. Of these, 150 (76%) had a z-score for IgG (IgGz) of ≤0. There were no differences in IgGz values between patients with CAAs and those without CAAs. However, nonresponders had higher IgGz values than responders (median, 25th percentile and 75th percentile: −0.26, −0.83 and 0.34 vs. −0.79, −1.40 and −0.03; p = 0.020). Logistic regression analysis showed that the IgGz value was an independent risk factor for resistance to IVIG (OR 1.36, 95% CI 1.002-1.849; p = 0.048). Conclusions: Low IgGz values were not a risk factor for CAAs in this study. However, KD patients with relatively high IgGz values before treatment may have an increased risk of resistance to initial IVIG therapy. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The article discusses case study of 5-year-old girl with no past medical history had dyspnea and fatigue during exercise. It mentions She developed a cough and was presented to a pediatric department. A chest radiograph showed a rightward deviation of the trachea and mediastinum with volume loss over the right lung but she underwent no further medical examination.

The article presents a case study of an infant girl diagnosed with Interrupted aortic arch underwent the Norwood procedure with the RV-PA conduit (8 mm GORETEX) at 3 months of age. It mentions that She had left hemiplegia after surgery; computed tomography confirmed cerebral infarction in the right middle cerebral artery region.