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 leading to the diagnosis of KSS. The cardiovascular complications of this syndrome are reviewed and discussed.
journal_name
Pediatr Cardioljournal_title
Pediatric cardiologyauthors
Chawla S,Coku J,Forbes T,Kannan Sdoi
10.1007/s00246-007-9040-zsubject
Has Abstractpub_date
2008-05-01 00:00:00pages
659-62issue
3eissn
0172-0643issn
1432-1971journal_volume
29pub_type
杂志文章abstract::Chylous effusions frequently occur after cardiac surgery due to severe damage to the lymphatic system, thus indicating that the insertion of a chest tube may be necessary. Factor XIII (FXIII) is discussed as being essential for wound healing. The aim of this retrospective study was to evaluate whether the application ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-005-0993-5
更新日期:2006-01-01 00:00:00
abstract::A 10-month-old boy was referred for tachypnea and heart murmur. An echocardiogram showed unexplained left heart dilation without evidence of an intracardiac shunt. A 64-slice computed tomographic contrast-enhanced angiography showed a large tortuous anomalous artery arising from the descending thoracic aorta and suppl...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-008-9287-z
更新日期:2009-01-01 00:00:00
abstract::In our institute, 1100 patients with a history of Kawasaki disease have been catheterized for selective coronary arteriography. Their age at examination ranged from four months to 13 years. Coronary artery lesions (CAL) were found in 262 patients. As far as the type of the CAL was concerned, occlusion was noted in 20 ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF02315475
更新日期:1986-01-01 00:00:00
abstract::A very rare combination including tetralogy of Fallot (TOF), absent pulmonary valve, and isolated left pulmonary artery is described. A 4-month-old girl presented with mild cyanosis and severe dyspnea. Echocardiography established the diagnosis of TOF and absent pulmonary valve with severe pulmonary regurgitation and ...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-008-9282-4
更新日期:2008-11-01 00:00:00
abstract::This review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for congenital heart disease. Five areas will be reviewed: (1) common language = nomenclature, (2) mechanism of data collection (database or registry) with an established uni...
journal_title:Pediatric cardiology
pub_type: 杂志文章,评审
doi:10.1007/s00246-006-1447-4
更新日期:2007-03-01 00:00:00
abstract::Sinus node disease is a problem encountered in patients after the Mustard surgery, requiring a pacemaker implantation. The knowledge of the complexity anatomy is crucial because of the possible challenges associated with this procedure. We report a case of a 24-year-old woman presented with symptomatic bradycardia, in...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-015-1155-z
更新日期:2015-08-01 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::Vitamin D has anti-inflammatory properties, and deficiency is prevalent in children. There is a paucity of data regarding vitamin D status and its correlation with low-grade inflammation and vasculature. We prospectively enrolled 25 children, 9-11 years old (13 male); 21 obese. Eight atherosclerosis-promoting risk fac...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-015-1162-0
更新日期:2015-10-01 00:00:00
abstract::Antenatal diagnosis of right heart enlargement has a wide spectrum of differential diagnosis from maternal, placental and fetal causes, and outcomes of all are not known. Coarctation of the aorta is in the differential diagnosis of right heart enlargement. In our study, we focused to measure multiple cardiac dimension...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-015-1168-7
更新日期:2015-10-01 00:00:00
abstract::With the advent of cardiac magnetic resonance imaging and high-resolution echocardiography, cardiac catheterization is unnecessary in clinical protocols in the "routine" single ventricle patient. Catheterization adds little to clinical care in these cases, and there are significant risks and costs associated with it. ...
journal_title:Pediatric cardiology
pub_type: 杂志文章,评审
doi:10.1007/s00246-004-0960-6
更新日期:2005-03-01 00:00:00
abstract::We performed a Waterston's anastomosis on a patient with complicated tricuspid atresia when she was two months of age. At age 14 years she required left pneumonectomy for massive, idiopathic hemoptysis. Four months after the pneumonectomy we substituted a modified Fontan anastomosis for the Waterston anastomosis. Rest...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF02238530
更新日期:1991-07-01 00:00:00
abstract::Left atrial isomerism includes a complex spectrum of cardiac and extracardiac anomalies. The records of all patients with left isomerism born during the period of 1973-2010 and treated at the Children's Hospital, Helsinki were reviewed. The short- and long-term outcomes were studied. The review included 38 patients (5...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0184-0
更新日期:2012-04-01 00:00:00
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 elu...
journal_title:Pediatric cardiology
pub_type: 杂志文章,评审
doi:10.1007/s00246-019-02180-6
更新日期:2019-10-01 00:00:00
abstract::To evaluate the feasibility of implementing a pulse oximetry screening protocol at a city of mild elevation with a specific focus on the false-positive screening rate. Pulse oximetry screening was performed according to the proposed guidelines endorsed by the American Academy of Pediatrics at a center in Tucson, AZ, a...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-013-0716-2
更新日期:2013-01-01 00:00:00
abstract::A case of aneurysmal enlargement of the right atrium was found accidentally during a routine examination. The patient was asymptomatic and free of palpitations or proven rhythm disturbances. The results of echocardiography, magnetic resonance imaging, and cardiac catheterization excluded cardiac malformation, which co...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s002469900341
更新日期:1998-09-01 00:00:00
abstract::Anatomic evidence of intrauterine closure of ventricular septal defects (VSDs) has been reported rarely. Between 1985 and 1990, 112 autopsies were performed at the Mayo Clinic on third trimester stillborns and infants who died during the first week of life. There were 21 (19%) cases of congenital heart disease. VSD wa...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00797004
更新日期:1994-01-01 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::The nuclear cross-sectional area of the muscular fibers from a series of myocardial bridges was analyzed and compared to both the adjacent and underlying myocardial fibers. The series came from 12 hearts of children, ranging in age from 24 h to 2 years, with diverse forms of congenital heart disease, which caused left...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF02238364
更新日期:1990-10-01 00:00:00
abstract::Hospital volume has been associated with improved outcomes in congenital cardiac surgery. However, the relationship between hospital volume and hospitalization cost remains unclear. This study examines the relationship between hospital surgical volume and hospitalization costs, while accounting for measures of quality...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-014-0987-2
更新日期:2015-01-01 00:00:00
abstract::Eighteen of 25 survivors of aortic valvotomy in infancy were reinvestigated by cross-sectional echocardiography a mean of 7.5 (2.3-13.4) years after surgery. They had been operated at a median age of 38 (5-330) days. At the follow-up examination the gradient across the aortic valve was 41 +/- 19 (15-85) mmHg and the e...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF00788221
更新日期:1992-01-01 00:00:00
abstract::The longer survival of patients with Duchenne muscular dystrophy due to advances in clinical care has increased the incidence of Duchenne muscular dystrophy-associated cardiomyopathy, a nearly consistent feature in the third decade of life. A 26-year-old patient with Duchenne muscular dystrophy experienced severe acut...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-019-02072-9
更新日期:2019-03-01 00:00:00
abstract::Children with trisomy 21 have a high incidence of congenital heart disease that frequently requires surgical repair. These patients often require multiple and higher dosage sedatives during the postoperative period. Dexmedetomidine is an alpha(2)-adrenergic receptor agonist that has sedative, analgesic, and anxiolytic...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-006-0072-6
更新日期:2007-09-01 00:00:00
abstract::Intravenous immunoglobulin (IVIG) administered in the acute stage of Kawasaki disease (KD) is the standard therapy. Few reports describe nonresponders to initial treatment with IVIG in KD, which remains the most consistent risk factor for coronary artery lesions (CALs). This study aimed to investigate whether the seru...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-013-0724-2
更新日期:2013-01-01 00:00:00
abstract::Catheter ablation in children requires placement of multiple large femoral venous sheaths and catheters. Magnetic resonance angiography (MRA) was used to evaluate the effect of indwelling lines on femoral venous blood flow. Between October 1993 and February 1994 a total of 17 patients scheduled for catheter ablation u...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s002469900151
更新日期:1997-05-01 00:00:00
abstract::Patients post-bidirectional Glenn (BDG) operation are at risk of left and right pulmonary artery (LPA and RPA) hypoplasia. Transthoracic echocardiograms (TTE) in active children can miss essential elements of anatomy. Procedural sedation improves image quality but increases risk of adverse events. We hypothesized that...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-020-02342-x
更新日期:2020-06-01 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::This study is presented to identify and characterize the spectrum of the cardiovascular anomalies in children presenting with Williams-Beuren syndrome and cardiovascular anomalies at The Hospital for Sick Children, Toronto from 1966 to 1988. Forty-nine children were diagnosed and followed. The female to male ratio was...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/BF02310569
更新日期:1991-10-01 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::Common arterial trunk is relatively a straightforward diagnosis on echocardiography. We describe a neonate who was referred to our centre as a case of common arterial trunk but on evaluation was found to have pulmonary atresia with ventricular septal defect and aortopulmonary window, for which he underwent repair with...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s00246-012-0240-9
更新日期:2012-10-01 00:00:00
abstract::We report successful emergency pacing followed by permanent pacemaker implantation due to complete block in an otherwise healthy premature infant of 1,770 g. Via the umbilical vein a temporary bipolar pacing lead was placed in the right ventricle. The lack of spontaneous improvement warranted implantation of a permane...
journal_title:Pediatric cardiology
pub_type: 杂志文章
doi:10.1007/s002460010112
更新日期:2000-09-01 00:00:00