Abstract：
:Embryonic development is a tightly regulated process, and many families of genes functions to provide a regulatory genetic network to achieve such a program. The homeobox genes are an extensive family that encodes transcription factors with a characteristic 60-amino acid homeodomain. Mutations in these genes or in the encoded proteins might result in structural malformations, physiological defects, and even embryonic death. Mutations in the short-stature homeobox gene (SHOX) is associated with idiopathic short stature in humans, as observed in patients with Turner syndrome and/or Leri-Weill dyschondrosteosis. A closely related human homolog, SHOX2, has not been linked to any syndrome or defect so far. In mice, a SHOX ortholog gene is not present in the genome; however, a true SHOX2 ortholog has been identified. Analyses of Shox2 knockout mouse models have showed crucial functions during embryonic development, including limb skeletogenesis, palatogenesis, temporomandibular joint formation, and cardiovascular development. During embryonic cardiac development, Shox2 is restrictedly expressed in the sinus venosus region, including the sinoatrial node (SAN) and the sinus valves. Shox2 null mutant is embryonically lethal due to cardiovascular defects, including a severely hypoplastic SAN and sinus valves attributed to a significantly decreased level of cell proliferation in addition to an abnormal low heartbeat rate (bradycardia). In addition, it has been demonstrated that Shox2 regulates a genetic network through the repression of Nkx2.5 to maintain the SAN fate and thus plays essential roles in its proper formation and differentiation.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Liu H,Espinoza-Lewis RA,Chen C,Hu X,Zhang Y,Chen Ydoi
10.1007/s00246-012-0179-xsubject
Has Abstractpub_date
2012-08-01 00:00:00pages
882-9issue
6eissn
0172-0643issn
1432-1971journal_volume
33pub_type
杂志文章abstract：:Twenty-three children with diabetes mellitus, their ages ranging from 0.2-9.8 years, but with no sign of diabetic microvascular disease were investigated by M-mode and Doppler echocardiography, along with a comparable group of control subjects. In the diabetics, the fractional shortening and the mean velocity of fract...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF02238405
更新日期：1991-04-01 00:00:00
abstract：:A 6-year-old girl with type 3 long QT syndrome was safely and successfully implanted with an implantable cardioverter-defibrillator (ICD) system. Prior to implantation, she had experienced uncontrollable life-threatening arrhythmia in spite of high-dose administration of mexiletine. An ICD coil lead for transvenous us...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-011-0065-y
更新日期：2011-10-01 00:00:00
abstract：:Assessment of ventricular dysfunction and asynchrony is very important in predicting the outcome for children with a single right ventricle. However, the assessment is inaccurate and subjective because of the unusual ventricular shape. This study aimed to evaluate the feasibility and clinical value of velocity vector ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-014-0908-4
更新日期：2014-10-01 00:00:00
abstract：:Killian-Pallister syndrome is a rare dysmorphic condition characterized by specific clinical manifestations and tetrasomy 12p. Although the association of this condition with congenital heart disease has been previously documented, no cases have been reported in association with Fallot's tetralogy. We report one such ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002469900420
更新日期：1999-03-01 00:00:00
abstract：:Myocardial contractility and relaxation are highly dependent on calcium homeostasis. Immature myocardium, as in pediatric patients, is thought to be more dependent on extracellular calcium for optimal function. For this reason, intravenous calcium chloride infusions may improve myocardial function in the pediatric pat...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-015-1322-2
更新日期：2016-03-01 00:00:00
abstract：:We sought to document morbidities and growth for patients with hypoplastic left heart syndrome (HLHS) to inform the initial surgical decision and understand healthcare needs. Data were obtained on 137 patients with HLHS, born between 1989 and 1994, who survived staged surgery ( n = 62) or transplantation ( n = 75) and...
journal_title：Pediatric cardiology
pub_type： 杂志文章,多中心研究
doi：10.1007/s00246-003-0471-x
更新日期：2004-01-01 00:00:00
abstract：:Severe atrioventricular valve (AVV) or semilunar valve (SLV) regurgitation in the setting of a single ventricle physiology may proceed to valve replacement if repair strategies fail. Outcome data for these children are limited. We present transplant-free survival of a case series of children with single ventricle phys...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-019-02234-9
更新日期：2020-01-01 00:00:00
abstract：:A 12-year-old male presented with a 6-week history of apparent digital vasculitis with color changes of the distal fingers and erythematous macules of the palms and soles. Physical examination revealed skin findings of Carney complex and an abnormal cardiac examination. Echocardiography demonstrated a large left atria...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-005-1238-3
更新日期：2006-03-01 00:00:00
abstract：:Fetal interventions have been performed for some congenital heart diseases. However, these procedures have not gained wide acceptance due to concerns about their efficacy and safety. The aim of this study was to report on a preliminary experience with fetal cardiac interventions in Brazil. Twenty-two cardiac intervent...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0792-3
更新日期：2014-03-01 00:00:00
abstract：:We present an infant with unusual anatomy and physiology of a persistent left superior vena cava (LSVC). The adverse physiologic consequences of the LSVC were corrected with percutaneous placement of vascular plugs. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-009-9617-9
更新日期：2010-05-01 00:00:00
abstract：:Increasingly the importance of how and why we make decisions in the medical arena has been questioned. Traditionally the aeronautical and business worlds have shed a light on this complex area of human decision-making. In this review we reflect on what we already know about the complexity of decision-making in additio...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-020-02295-1
更新日期：2020-03-01 00:00:00
abstract：:Left atrial tumor, diagnosed as myxoma at first investigation, was later demonstrated to be a malignant fibrosarcoma with myxoid change. Myxoma should be diagnosed and managed carefully because of its wide range of histopathologic features. ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s002460010218
更新日期：2001-05-01 00:00:00
abstract：:The hybrid pathway for the management of patients with hypoplastic left heart syndrome was pioneered at our institution and is the preferred approach compared with the traditional Norwood pathway. Patients undergoing this surgery are generally <6 months of age, and pain management in this age group after surgery for c...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0233-8
更新日期：2012-10-01 00:00:00
abstract：:The current pulse-oximetry screening (POS) protocol for detection of critical congenital heart defects (CCHDs) is recommended only for newborns in well-infant and intermediate care nurseries, and there is no evidence-based protocol for infants discharged from the neonatal intensive care unit (NICU). The objectives of ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-013-0793-2
更新日期：2014-03-01 00:00:00
abstract：:In 810 schoolchildren (aged 5-14 years) the prevalence of a grade 1-3/6 vibratory innocent heart murmur (VIHM) is 41%. Restricted to a grade 2 or 3 VIHM, the prevalence is 14%, decreasing from 21% in the age-class 5-6 years to 8% for children 13-14 years of age. The prevalence of a grade 3 VIHM is 1%. Together with a ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/BF00798120
更新日期：1994-11-01 00:00:00
abstract：:Congenital diverticulum of the ventricle is a rare disease, but most cases of congenital left ventricular diverticula are asymptomatic. We present a child with congenital left ventricular diverticulum whose routine electrocardiographic examination showed T-wave inversion in inferior and V4 to V6 leads. He was successf...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-010-9719-4
更新日期：2010-08-01 00:00:00
abstract：:Aortic aneurysm may develop after surgery for coarctation of aorta especially patch aortoplasty. The size of patch and of adjacent native aortic wall was analyzed to determine whether aortic dilatation represents a true aneurysm. Electron beam tomography (EBT) was done on 19 patients, three months to 17.5 years after ...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-002-0186-4
更新日期：2003-01-01 00:00:00
abstract：:Kaposiform hemangioendothelioma (KH) is a rare tumor of vascular origin that commonly affects the cutaneous tissues of the extremities. It can, however, affect the abdomen, thorax, head, or neck. Compared with juvenile hemangiomas, which tend to regress, KH tumors are locally aggressive and usually persist. Associated...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0280-1
更新日期：2013-01-01 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：: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：:While exercise stress testing (EST) is an important tool, little is known about its use for determining ischemia in patients with hypoplastic left heart syndrome (HLHS) and Fontan palliation. We sought to determine the frequency of ST segment changes during EST in HLHS patients after Fontan and examine results of furt...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-015-1312-4
更新日期：2016-03-01 00:00:00
abstract：:Postpericardiotomy syndrome is a frequent complication following cardiac surgery. It is characterized by fever, chest pain, pericardial friction rub or pericardial effusion, and laboratory signs of inflammation. Treatment includes the use of nonsteroidal antiinflammatory agents, corticosteroids, and pericardial draina...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-007-9025-y
更新日期：2008-03-01 00:00:00
abstract：:For neonates with right atrial isomerism (RAI), functional single ventricle (f-SV), and obstructive total anomalous pulmonary venous connection (TAPVC), primary TAPVC repair (TAPVCR) has a poor outcome. At our hospital, the survival rate at 1 year of such neonates undergoing primary TAPVCR between 1999 and 2010 (TAPVC...
journal_title：Pediatric cardiology
pub_type： 杂志文章,收录出版
doi：10.1007/s00246-018-1902-z
更新日期：2018-10-01 00:00:00
abstract：:To reveal the association between DNMT1 polymorphisms and congenital heart disease (CHD) in child patients, a total of 224 CHD child patients as well as 199 healthy individuals were enrolled in the present study. The DNA was extracted from whole blood, and four SNPs including rs16999593, rs2228612, rs2288349 and rs104...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-015-1093-9
更新日期：2015-06-01 00:00:00
abstract：:Routine use of intraoperative transesophageal echocardiography (TEE) is a safe monitoring and diagnostic method during pediatric congenital cardiac surgery. However, the question of whether intraoperative TEE is accurate and cost effective for patients with tetralogy of Fallot (TOF) has not been raised. This study aim...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-012-0423-4
更新日期：2013-02-01 00:00:00
abstract：:Studies of larger patient groups for systematic assessment of the anatomical accuracy of magnetic resonance imaging (MRI) for partial anomalous pulmonary venous drainage (PAPVD) have been performed so far only in adults. This study was undertaken to evaluate whether MRI can precisely depict pulmonary venous anatomy in...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-008-9367-0
更新日期：2009-05-01 00:00:00
abstract：:Ductal stenting (DS) palliates duct-dependent lesions using coronary stents. Sirolimus-eluting stents have replaced bare-metal stents in coronary interventions. Concerns exist about sirolimus levels in neonates. Therapeutic immunosuppressive sirolimus level is 5-15 ng/ml. After neonatal DS, drug levels were assessed a...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-020-02381-4
更新日期：2020-10-01 00:00:00
abstract：:Hyperplastic left heart syndrome (HLHS) patients are palliated by creating a Fontan-type circulation passing from different surgical stages. The aim of this work is to describe the evolution of ventricular energetics parameters in HLHS patients during the different stages of palliation including the hybrid, the Norwoo...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-017-1704-8
更新日期：2017-12-01 00:00:00
abstract：:Arrhythmias in pregnancy are becoming more common given more available and effective medical, ablation and device treatment options. Several changes associated with pregnancy, increased blood volume, cardiac output, and heart rate secondary to an increased sympathetic state, facilitate more frequent occurrences of arr...
journal_title：Pediatric cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00246-015-1232-3
更新日期：2015-10-01 00:00:00
abstract：:Cardiac surgery for congenital heart disease often necessitates a period of myocardial ischemia during cardiopulmonary bypass and cardioplegic arrest, followed by reperfusion after aortic cross-clamp removal. In experimental models, myocardial ischemia-reperfusion is associated with significant oxidative stress and ve...
journal_title：Pediatric cardiology
pub_type： 杂志文章
doi：10.1007/s00246-016-1345-3
更新日期：2016-04-01 00:00:00
