Circular RNA circSMAD4 regulates cardiac fibrosis by targeting miR-671-5p and in cardiac fibroblasts.

Heart failure is a leading cause of death and is often accompanied by activation of quiescent cardiac myofibroblasts, which results in cardiac fibrosis. In this study, we aimed to identify novel circular RNAs that regulate cardiac fibrosis. We applied transverse aortic constriction (TAC) for 1, 4, and 8 weeks in mice.

Effects of Dextrose Supplementation on Chloral Hydrate Sedation: A Double-Blinded, Randomized, Prospective Study.

Sedation plays a crucial role in successful pediatric imaging, and chloral hydrate is commonly used for this purpose. However, the challenges associated with chloral hydrate administration, such as its unpleasant taste and potential induction of vomiting, remain a concern. Sweet oral solutions have emerged as potential solutions for reducing distress and providing analgesia.

Platelet Indices as Diagnostic Marker for Kawasaki Disease.

Various candidate biomarkers have been investigated for the early and accurate diagnosis of Kawasaki disease (KD). We aimed to evaluate platelet activity using platelet indices (PI) in patients with KD or simple febrile illness to determine whether these indices might support a diagnosis of KD. Another objective of the study was to delineate the changes in PI from the acute to convalescent phases of KD.

Overexpression of Prohibitin 2 Protein is Associated with Adverse Prognosis in Cytogenetically Normal Acute Myeloid Leukemia.

Cytogenetically normal acute myeloid leukemia (CN-AML) accounts for 40%-50% of all AML cases. Despite advances in understanding the molecular pathophysiology of CN-AML, its clinical outcome remains unsatisfactory and unpredictable. To investigate its clinical implication in CN-AML, we measured the expression of prohibitin 2 (PHB2) using immunohistochemical staining (IHCS) of paraffin-embedded bone marrow sections from 134 CN-AML patients.

Serum Ferritin as a Diagnostic Biomarker for Kawasaki Disease.

Diagnosis of Kawasaki disease (KD) is occasionally delayed because it is solely based on clinical symptoms. Previous studies have attempted to identify diagnostic biomarkers for KD. Recently, patients with KD were reported to have elevated serum ferritin levels.

Author Correction: PP2A negatively regulates the hypertrophic response by dephosphorylating HDAC2 S394 in the heart.

After the publication of this article, the authors noticed an error in one of the grant numbers (2015R1A2A1A05001708) in the Acknowledgements section.

PP2A negatively regulates the hypertrophic response by dephosphorylating HDAC2 S394 in the heart.

Cardiac hypertrophy occurs in response to increased hemodynamic demand and can progress to heart failure. Identifying the key regulators of this process is clinically important. Though it is thought that the phosphorylation of histone deacetylase (HDAC) 2 plays a crucial role in the development of pathological cardiac hypertrophy, the detailed mechanism by which this occurs remains unclear.

Spontaneous Regression of Cardiac Rhabdomyoma Presenting as Severe Left Ventricular Inlet Obstruction in a Neonate with Tuberous Sclerosis.

Cardiac rhabdomyoma can be subclinical or have a fatal presentation according to the onset age and involved site, size, and degree of invasion. Although most cardiac rhabdomyomas become smaller with time, emergency intervention is indicated when severe obstruction has occurred. In this report, we describe the spontaneous regression of a large cardiac rhabdomyoma (20.

Serial evaluation of myocardial function using the myocardial performance index in Kawasaki disease.

Background: Kawasaki disease (KD) is known as systemic vasculitis, and more than half of the patients with KD have myocarditis, which can induce ventricular dysfunction. In this study, we evaluate left ventricular (LV) dysfunction in patients with KD based on the myocardial performance index (MPI) using pulse Doppler (PD) and tissue Doppler imaging (TDI), from the acute to convalescent phases.

Methods: We retrospectively studied 89 children diagnosed with KD from January 2010 to August 2012.

Usefulness of Age-Stratified N-Terminal Prohormone of Brain Natriuretic Peptide for Diagnosing Kawasaki Disease.

N-terminal prohormone of brain natriuretic peptide (NT-proBNP) was recently reported as a biomarker for diagnosing Kawasaki disease (KD). The basal NT-proBNP level, however, gradually decreases with age. We investigated the usefulness of an age-stratified cutoff value of NT-proBNP for diagnosing KD.

Pdgfrα functions in endothelial-derived cells to regulate neural crest cells and the development of the great arteries.

Originating as a single vessel emerging from the embryonic heart, the truncus arteriosus must septate and remodel into the aorta and pulmonary artery to support postnatal life. Defective remodeling or septation leads to abnormalities collectively known as conotruncal defects, which are associated with significant mortality and morbidity. Multiple populations of cells must interact to coordinate outflow tract remodeling, and the cardiac neural crest has emerged as particularly important during this process.

Rapid Regression of Obstructive Cardiac Rhabdomyoma in a Preterm Neonate after Sirolimus Therapy.

Cardiac rhabdomyoma can be subclinical or fatal depending on the onset age, involving site, and the size and degree of invasion. Although most rhabdomyomas become smaller with time, emergency intervention is indicated when severe obstruction induces hemodynamic instability. Mammalian target of rapamycin (mTOR) inhibitors have been used to treat neonates and children with hemodynamically obstructive cardiac rhabdomyoma.

Clinical Utility of Echocardiography for the Diagnosis and Prognosis in Children with Bronchopulmonary Dsyplasia.

Background: Bronchopulmonary dysplasia (BPD) may result in chronic pulmonary artery hypertension and right ventricular (RV) dysfunction. Various echocardiographic assessments of RV dysfunction have been used to determine whether echocardiographic measurements of premature infants with BPD could provide sensitive measures of RV function that correlates with BPD severity.

Methods: Twenty-eight control subjects without BPD (non BPD group), 28 patients with mild BPD, 11 patients with moderate BPD, and six patients with severe BPD underwent echocardiograms with standard measurement such as ejection fraction by M-mode, tricuspid regurgitation pressure gradient, myocardial performance index (MPI) derived from pulse Doppler, and tissue Doppler imaging (TDI) measurements.

Coronary vasculature patterning requires a novel endothelial ErbB2 holoreceptor.

Organogenesis and regeneration require coordination of cellular proliferation, regulated in part by secreted growth factors and cognate receptors, with tissue nutrient supply provided by expansion and patterning of blood vessels. Here we reveal unexpected combinatorial integration of a growth factor co-receptor with a heterodimeric partner and ligand known to regulate angiogenesis and vascular patterning. We show that ErbB2, which can mediate epidermal growth factor (EGF) and neuregulin signalling in multiple tissues, is unexpectedly expressed by endothelial cells where it partners with neuropilin 1 (Nrp1) to form a functional receptor for the vascular guidance molecule semaphorin 3d (Sema3d).

Radiodensity on serial chest X-rays for the diagnosis of foreign body aspiration in children.

Objective: To evaluate the utility of measuring lung radiodensity from chest X-ray for the diagnosis of foreign body aspiration.

Methods: Records of 59 children with foreign body aspiration were retrospectively reviewed. Lung radiodensity and radiodensity ratio (right/left lung radio density) before and after foreign body removal were measured.

Total anomalous pulmonary venous return in siblings.

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur.

Ret finger protein mediates Pax7-induced ubiquitination of MyoD in skeletal muscle atrophy.

Skeletal muscle atrophy results from the net loss of muscular proteins and organelles and is caused by pathologic conditions such as nerve injury, immobilization, cancer, and other metabolic diseases. Recently, ubiquitination-mediated degradation of skeletal-muscle-specific transcription factors was shown to be involved in muscle atrophy, although the mechanisms have yet to be defined. Here we report that ret finger protein (RFP), also known as TRIM27, works as an E3 ligase in Pax7-induced degradation of MyoD.

Small heterodimer partner blocks cardiac hypertrophy by interfering with GATA6 signaling.

Rationale: Small heterodimer partner (SHP; NR0B2) is an atypical orphan nuclear receptor that lacks a conventional DNA-binding domain. Through interactions with other transcription factors, SHP regulates diverse biological events, including glucose metabolism in liver. However, the role of SHP in adult heart diseases has not yet been demonstrated.

Transient complete atrioventricular block in a preterm neonate with congenital myotonic dystrophy: case report.

Congenital myotonic dystrophy (CMD) is an inherited neuromuscular disorder with cardiac rhythm abnormalities that may occur as a child grows. No report has described complete atrioventricular (AV) block detected in a neonate with CMD. We report a floppy infant of 31(+4) weeks gestation with complete AV block at birth, who was diagnosed with CMD by Southern analysis.

Procalcitonin levels in patients with complete and incomplete Kawasaki disease.

Incomplete Kawasaki disease (iKD) is considered to be a less complete form of Kawasaki disease (cKD), and several differences in the laboratory presentations of iKD and cKD have been noted. We investigated serum procalcitonin levels in patients with iKD, cKD, and other febrile diseases (a control group). Seventy-seven patients with cKD, 24 with iKD, and 41 controls admitted to our hospital from November 2009 to November 2011 were enrolled in the present study.

Random urinary calcium/creatinine ratio for screening hypercalciuria in children with hematuria.

Background: Hypercalciuria is one of the most common causes of unexplained isolated hematuria. The diagnostic methods for hypercalciuria have not yet been standardized. The aim of this study was to assess whether random urinary calcium/creatinine ratio could be used as a screening tool for hypercalciuria in children with hematuria.

Timing in resolution of left heart dilation according to the degree of mitral regurgitation in children with ventricular septal defect after surgical closure.

Objective: Children with ventricular septal defects (VSD) can have chronic volume overload, which can result in changes of left heart echocardiographic parameters. To evaluate the changes before and after surgical closure, the children were divided into three groups according to the degree of mitral regurgitation (MR), and their echocardiographic characteristics were reviewed at serial follow-up after surgical closure.

Methods: The preoperative, and one-, three-, and 12-month postoperative echocardiographic data of 40 children who underwent surgical closure of VSD were retrospectively reviewed.

Estrogen-related receptor gamma induces cardiac hypertrophy by activating GATA4.

Estrogen-related receptor gamma (ERRγ) is an orphan nuclear receptor that has biological roles mainly in metabolism and that controls metabolic switching in perinatal heart. In adult heart diseases, however, the functional roles of ERRγ have not yet been elucidated. In the present study, we aimed to characterize the role of ERRγ in cardiac hypertrophy.

Right ventricular failure in congenital heart disease.

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression, and ventricular remodeling.

Transvenous proximal closure of large congenital coronary arteriovenous fistula using the single Amplatzer vascular plug in a 3-year-old girl.

Congenital coronary arteriovenous fistulas (CCAFs) are rare coronary artery abnormalities in which blood is shunted into a cardiac chamber or great vessel. If the fistula itself is large and tortuous, it is generally recommended to occlude the fistula to prevent several complications. In approaches of transcatheter occlusion, the transvenous approach is preferred over the transarterial approach.