Abstract：
:Numerous RNA-binding proteins (RBPs) are expressed in the heart, and mutations in several RBPs have been implicated in cardiovascular disease through genetic associations, animal modeling, and mechanistic studies. However, the functions of many more cardiac RBPs, and their relevance to disease states, remain to be elucidated. Recently, we have initiated studies to characterize the functions of the RBPs RBPMS and RBPMS2 in regulating myocardial biology in zebrafish and higher vertebrate species. These studies began when we learned, using an unbiased gene discovery approach, that rbpms2a and rbpms2b in zebrafish are robust markers of embryonic myocardium. This observation, which is consistent with published data, suggests that the encoded proteins are likely to be performing critical functions in regulating one or more aspects of cardiomyocyte differentiation, proliferation, survival, and/or contractility. This notion is supported by recent reports demonstrating that zebrafish embryos with disrupted Rbpms2 function exhibit gross signs of cardiac distress. Interestingly, a 20-year-old study determined that myocardial tissue from the frog, chick, and mouse also express high levels of Rbpms and/or Rbpms2, which is suggestive of evolutionary conservation of function. In this review, we will provide a historical account of how RBPMS and RBPMS2 genes were discovered, attempt to clarify some potentially confusing nomenclature, and summarize published observations that inform our ongoing studies.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Akerberg AA,Burns CE,Burns CGdoi
10.1007/s00246-019-02180-6subject
Has Abstractpub_date
2019-10-01 00:00:00pages
1410-1418issue
7eissn
0172-0643issn
1432-1971pii
10.1007/s00246-019-02180-6journal_volume
40pub_type
杂志文章,评审abstract：:A blood pump specifically designed to augment flow from the great veins through the lungs would ameliorate the poor physiology of the failing univentricular circulation and result in a paradigm shift in the treatment strategy for Fontan patients. This study is the first to examine mechanical cavopulmonary assistance w...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0649-9
更新日期：2013-08-01 00:00:00
abstract：:Postoperative arrhythmia is a common complication after open heart surgery in children. JET is the most common and dangerous arrhythmia. We aimed to assess safety and efficacy of prophylactic amiodarone in preventing JET in children underwent cardiac surgery and to assess risk factors for JET among our patients. In to...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-016-1343-5
更新日期：2016-04-01 00:00:00
abstract：:In August 2010, the Nit-Occlud® Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud® Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 E...
journal_title：Pediatric cardiology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00246-016-1502-8
更新日期：2017-02-01 00:00:00
abstract：:The authors present an unusual complication of the balloon atrial septostomy procedure performed in a neonate with D-transposition of the great arteries. Cardiac tamponade developed shortly after the balloon atrial septostomy procedure and the infant was found to have a tear in the superior aspect of the left atrium, ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02076340
更新日期：1983-04-01 00:00:00
abstract：:Prior to initiation of a formal screening program, 30 % of patients referred for device closure of an atrial septal defect (ASD) at our institution had atrial septal anatomy which precluded ASD device closure. This resulted in inefficiencies in workflow, staff utilization, and inconvenience for families. Our objective...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-015-1135-3
更新日期：2015-08-01 00:00:00
abstract：:To describe great-vessel dimensions in patients with D-loop transposition of the great arteries (TGA) who have undergone atrial switch operation (ATSO). Patients who have undergone arterial switch operation for TGA have a high incidence of dilation of the neoaortic root. The incidence and degree of great artery dilati...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0800-7
更新日期：2014-03-01 00:00:00
abstract：:Circulating progenitor cells have been extensively studied in the context of heart disease in adults. In these patients, they have been demonstrated to be markers of myocardial injury and recovery as well as potential therapeutic agents. However, studies in children are much more limited. Here we review current knowle...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-015-1300-8
更新日期：2016-02-01 00:00:00
abstract：:Controversy exists as to whether a hemodynamically significant left-to-right shunt due to a patent ductus arteriosus (PDA) affects ventricular contractility. Load-dependent indices such as ejection fraction and shortening fraction have traditionally been used to assess contractility, but the relationship between the r...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-003-0452-0
更新日期：2004-03-01 00:00:00
abstract：:Kawasaki disease (KD) is an acute febrile disease of unknown etiology that develops in children and is sometimes accompanied by myocardial dysfunction and systemic vasculitis. However, myocardial repolarization lability has not yet been fully investigated. Thus, the objective of this study was to evaluate myocardial r...
journal_title：Pediatric cardiology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00246-011-9908-9
更新日期：2011-04-01 00:00:00
abstract：:The outcome in a series of 23 cases of tetralogy of Fallot diagnosed prenatally was compared to published figures for this condition when (a) identified postnatally or (b) treated surgically. There was a marked difference in the survival between these groups, with the highest mortality (75%) occurring when the diagnos...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF00788220
更新日期：1992-01-01 00:00:00
abstract：:Right ventricular (RV) pacing can be associated with impairment of left ventricular (LV) function due to electrical dyssynchrony and myocardial remodeling (Janousek et al. in J Cardiovasc Electrophysiol 15:470-474, 2004). RV-pacing induced ventricular dysfunction is reversible by techniques such as biventricular pacin...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-017-1629-2
更新日期：2017-08-01 00:00:00
abstract：:Crossed pulmonary arteries (CPAs) are a rare abnormality in which the ostium of the left pulmonary artery originates superior to the right pulmonary artery and to its right. Recognition of this rare pathology is important because it generally is accompanied by other congenital heart defects, extracardiac anomalies, an...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0714-4
更新日期：2013-01-01 00:00:00
abstract：:Right ventricular (RV) function as assessed by deformation has been evaluated prenatally and after palliation in hypoplastic left heart syndrome (HLHS). However, limited data exist about the immediate postnatal cardiac adaptation and RV function in HLHS. We compared echocardiographic measures of cardiac performance in...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-1974-9
更新日期：2019-01-01 00:00:00
abstract：:Kearns-Sayre syndrome (KSS) is a rare mitochondrial disorder characterized by large-scale deletions of mitochondrial DNA. Neuromuscular and cardiac conduction systems are most commonly involved in these patients. Here, we discuss a 10-year-old patient with diabetes mellitus who presented in complete heart block leadin...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-007-9040-z
更新日期：2008-05-01 00:00:00
abstract：:Fifty infants with interrupted aortic arch (IAA), admitted between 1979 and 1988, were reviewed. They usually presented early in severe cardiac failure or shock. In the initial 5-year period, 17 of the 21 infants underwent diagnostic or confirmatory cardiac catheterization, in contrast with the latter 5 years when onl...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF00838779
更新日期：1992-10-01 00:00:00
abstract：:In 19 consecutive children with aortic valve stenosis, the left ventricle was entered retrogradely with a Gensini catheter guided by a tip-deflector guidewire which could produce any desirable degree of curvature at its tip. In all patients, the aortic valve was retrogradely traversed for measurement of the pressure g...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02328955
更新日期：1986-01-01 00:00:00
abstract：:In adult heart failure (HF) patients, a higher ventricular arterial (VA) coupling ratio measured non-invasively is associated with worse HF prognosis and response to treatment. There are no data regarding the relationship of VA coupling to outcome in pediatric dilated cardiomyopathy (DCM) patients. We investigated the...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-2021-6
更新日期：2019-02-01 00:00:00
abstract：:The purpose of the study was to evaluate school-age developmental and educational outcomes for children with and without a cardiac procedure in the first year of life to improve understanding of longer-term neurodevelopmental outcomes in children who have had a cardiac procedure for congenital heart disease, the most ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-018-2029-y
更新日期：2019-03-01 00:00:00
abstract：:Extracardiac valved conduits are routinely employed in the correction of complex congenital heart lesions. Compression of a conduit and/or of the heart may present serious operative and postoperative complications. We believe its occurrence can be minimized and hemodynamic results improved by adhering to simple surgic...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02242263
更新日期：1983-07-01 00:00:00
abstract：:Acute myopericarditis is a major cause of acquired heart disease worldwide in pediatric patients. Various viruses have been reported as the etiology, of which varicella zoster virus was first reported in 1953. However, since nationwide administration of the varicella vaccine, reports of varicella-associated myopericar...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9637-5
更新日期：2010-07-01 00:00:00
abstract：:The observation that endocardial fibroelastosis (EFE) can result from an immune response to maternal autoantibody deposition in the fetal myocardium raises the possibility that the fetal immune system may contribute to the pathogenesis of idiopathic EFE and dilated cardiomyopathy (DCM). This study sought to characteri...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-011-9980-1
更新日期：2011-12-01 00:00:00
abstract：:This prospective observational study conducted in a neonatal intensive care unit aimed to evaluate echocardiographic changes provoked by anemia and transfusion of packed red blood cells (pRBCs) in premature infants. In this study, 32 anemic premature infants had serial echocardiographic assessment of left ventricular ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0154-6
更新日期：2012-04-01 00:00:00
abstract：:The slope of the relation between the unadjusted QT interval and heart rate during the face immersion test has been reported to be useful as an index for predicting an abnormal lengthening of the QT interval for children with nonfamilial long QT syndrome. Our goals were to determine whether we can replace the slope of...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-001-0034-y
更新日期：2002-03-01 00:00:00
abstract：:The original version of the article unfortunately contained an alignment error in Table 1. The correct version of Table 1 is given below. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章,已发布勘误
doi：10.1007/s00246-018-1978-5
更新日期：2019-01-01 00:00:00
abstract：:As a transcription factor mainly expressed in cardiovascular system, T-box 20 (TBX20) plays an important role in embryonic cardiovascular system development and adult heart function. Previous studies have identified associations of two SNPs in the T-box DNA-binding domain of TBX20 with congenital heart disease (CHD) i...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-014-1073-5
更新日期：2015-04-01 00:00:00
abstract：:We report the case of a fetus diagnosed at 24 weeks' gestation with complete heart block associated with maternal collagen vascular disease and Sjögren's antibody. Serial fetal echocardiograms noted increased echogenicity along the tricuspid and mitral valves without insufficiency. Postnatally, severe tricuspid insuff...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF00800677
更新日期：1994-07-01 00:00:00
abstract：:A hemodynamically stable neonate with transposition of the great arteries suddenly went into cardiac arrest during preparation for transport to the operating room. Emergency echocardiography during cardiac massage detected coronary air embolism as the presumed cause of arrest. After about 15 minutes of resuscitation, ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-011-0088-4
更新日期：2011-12-01 00:00:00
abstract：OBJECTIVE:Heterotaxy syndrome is associated with complex cardiac malformations and cardiac conduction system abnormalities. Those with right atrial isomerism (RAI) have dual sinus nodes and dual atrioventricular nodes predisposing them to supraventricular tachycardia (SVT). Those with left atrial isomerism (LAI) lack a...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02087-2
更新日期：2019-06-01 00:00:00
abstract：:Information and communication technology has been widely applied to various fields, including clinical medicine. We report here a telediagnosis system using ultrasound image transmission. The effect of telediagnosis, using a medical link between local maternity hospitals and our children's medical center, was verified...
journal_title：Pediatric cardiology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00246-013-0834-x
更新日期：2014-04-01 00:00:00
abstract：:Atrial flutter/fibrillation (AFL/AF) is a late complication in adults with repaired tetralogy of Fallot (TOF). Its effects on long-term prognosis are not fully understood. We evaluate the impact of AFL/AF in adults with repaired TOF on global mortality and unplanned hospitalizations during follow-up, and the predictor...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02106-2
更新日期：2019-06-01 00:00:00
