Effect of high-dose oral cholecalciferol on cardiac mechanics in children with chronic kidney disease.

Cardiovascular factors are an important cause of mortality in chronic kidney disease, and vitamin-D deficiency is common in this patient population. Therefore, we aimed to investigate the effect of oral cholecalciferol on cardiac mechanics in children with chronic kidney disease. A total of 41 children with chronic kidney disease - the patient group - and 24 healthy subjects - the control group - free of any underlying cardiac or renal disease with low 25-hydroxyvitamin-D3 levels were evaluated by conventional tissue Doppler imaging and two-dimensional speckle-tracking echocardiography, both at baseline and following Stoss vitamin-D supplementation.

Left ventricular mechanics are affected in children with celiac disease: A study based on two-dimensional speckle tracking echocardiography.

Background: An association between cardiomyopathy and celiac disease has been reported, but not frequently. We examined the effects on cardiac mechanics in children with celiac disease (CD) by two-dimensional speckle tracking echocardiography (2DSTE).

Methods: Eighty-one children with CD were compared with a control group comprising 51 healthy children by echocardiographic examination.

Acquired von Willebrand syndrome in children with aortic and pulmonary stenosis.

Introduction: This prospective study was planned to investigate the frequency and relationship of acquired von Willebrand syndrome (AVWS) with aortic and pulmonary stenosis in patients.

Methods: A total of 84 children, ranging from two to 18 years of age, were enrolled in this study. Of these, 28 had isolated aortic stenosis, 32 had isolated pulmonary stenosis and 24 were healthy.

Subclinical Left Ventricular Longitudinal and Radial Systolic Dysfunction in Children and Adolescents with Type 1 Diabetes Mellitus.

Introduction: There are few studies of subclinical systolic dysfunctions in children and adolescents with type 1 diabetes mellitus (DM), and so the available data are limited. The aim of this study was to determine early echocardiographic signs of LV systolic dysfunction in children and adolescents with type 1 DM using two-dimensional speckle tracking echocardiography (2DSTE).

Material And Methods: The study included 84 children and adolescents with type 1 DM and 32 sex-, age-, and body mass index-matched healthy subjects.

Diagnosis, treatment and follow up of neonatal arrhythmias.

Objective: This study aimed to evaluate the aetiology, spectrum, course and outcomes of neonates with arrhythmias observed in a tertiary neonatal intensive care unit from 2007 to 2012.

Methods: Neonates with rhythm problems were included. The results of electrocardiography (ECG), Holter ECG, echocardiography and biochemical analysis were evaluated.

Prevalence of overweight and obesity among patients with congenital and acquired heart disease in Kocaeli, Turkey.

Background: Childhood obesity has increased in the last half of the century. The aim of this study was to evaluate the frequency of obesity in the children with congenital or acquired heart disease.

Methods: A total of 1410 children were assessed in this study.

Predictors of vasovagal syncope recurrence in children and adolescents and value of head-up tilt table test.

Objective: The aim of the study was to define predictors of syncope recurrence in children and adolescents with vasovagal syncope and to determine the value of tilt test.

Methods: A retrospective observational study performed of prospective cohort of 150 patients aged between 8-18 years who were referred to our clinic because of fainting or who underwent tilt test with the pre-diagnosis of vasovagal syncope. The progress updated by telephone or face-to-face interview.

Effects that different types of sports have on the hearts of children and adolescents and the value of two-dimensional strain-strain-rate echocardiography.

Whether the hypertrophy found in the hearts of athletes is physiologic or a risk factor for the progression of pathologic hypertrophy remains controversial. The diastolic and systolic functions of athletes with left ventricular (LV) hypertrophy usually are normal when measured by conventional methods. More precise assessment of global and regional myocardial function may be possible using a newly developed two-dimensional (2D) strain echocardiographic method.

Crossed pulmonary arteries: a report on 20 cases with an emphasis on the clinical features and the genetic and cardiac abnormalities.

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, and certain genetic problems. To date, only a few cases have been reported, and most of these cases have been associated with complex cardiac abnormalities.

Functional pulmonary atresia in newborn with normal intracardiac anatomy: Successful treatment with inhaled nitric oxide and pulmonary vasodilators.

Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein's anomaly, Uhl's anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart.

Agenesis of the inferior vena cava in H syndrome due to a novel SLC29A3 mutation.

We present a 10-year-old girl with typical clinical features of H syndrome. Complete agenesis of the inferior vena cava was found on echocardiography and radiologic studies. Mutation analysis of the SLC29A3 gene revealed a novel nonsense mutation.

Congenital left ventricular diverticulum associated with congenital ileo-jejunal atresia.

Congenital left-ventricular diverticulum (CVD) is a rare cardiac malformation. Echocardiography, magnetic resonance imaging, multislice computed tomography, and left-ventricular angiography are diagnostic tools. In this case report, we present a 5-month-old infant with CVD associated with congenital ileal atresia.

Management of an ascending aortic aneurysm associated with a bicuspid aortic valve and a coarctation in an adolescent.

Ascending aortic aneurysms are commonly associated with bicuspid aortic valves in adults but are rarely seen in childhood. We now report the management of an ascending aortic aneurysm in a 12-year-old male with an associated bicuspid aortic valve and a coarctation.

P-wave dispersion and heart rate variability in children with mitral valve prolapse.

Previous studies have reported that patients with mitral valve prolapse (MVP) may display autonomic dysfunction. Measurement of heart rate variability (HRV) and P-wave dispersion (PWD) may provide insights into the functional state of the autonomic nervous system. Heart rate variability (HRV) has been used as a noninvasive marker of autonomic activity.

Echocardiographic and three-dimensional computed tomographic diagnosis of crossed pulmonary arteries: report of three cases.

Crossed pulmonary arteries are rare anomalies of the pulmonary trunk and its branches. In this anomaly, the ostium of the left pulmonary artery originates superiorly and to the right of the right pulmonary artery. This anomaly is usually associated with other congenital cardiac and extracardiac diseases.

Hepatic adenomatosis in a 7-year-old child treated earlier with a Fontan procedure.

Patients who undergo a Fontan procedure experience some degree of liver disease. Hemodynamic changes such as central venous hypertension, depressed dynamic cardiac output, and late ventricular dysfunction combined with long-standing hypoxia preceding the Fontan procedure all are recognized risk factors for hepatic injury. The histopathologic changes associated with cardiac hepatopathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis.

Thiamine-responsive megaloblastic anemia: early diagnosis may be effective in preventing deafness.

Thiamine-responsive megaloblastic anemia syndrome is an autosomal recessive disorder characterized by diabetes mellitus, megaloblastic anemia and sensorineural hearing loss. Mutations in the SLC19A2 gene, encoding a high-affinity thiamine transporter protein, THTR-1, are responsible for the clinical features associated with thiamine-responsive megaloblastic anemia syndrome in which treatment with pharmacological doses of thiamine correct the megaloblastic anemia and diabetes mellitus. The anemia can recur when thiamine is withdrawn.

A novel matrix metalloproteinase 2 (MMP2) terminal hemopexin domain mutation in a family with multicentric osteolysis with nodulosis and arthritis with cardiac defects.

Multicentric osteolysis with nodulosis and arthropathy (MONA, NAO (OMIM no. 605156)) is an autosomal recessive member of the 'vanishing bone' syndromes and is notable for the extent of carpal and tarsal osteolysis and interphalangeal joint erosions, facial dysmorphia, and the presence of fibrocollagenous nodules. This rare disorder has been described previously in Saudi Arabian and Indian families.

Diffusion-weighted magnetic resonance imaging findings of a patient with neonatal citrullinemia during acute episode.

Reports on citrullinemia usually describe the imaging findings in the adult form of the disease. However, neuroimaging findings of neonatal cases are seldom reported. We report on the diffusion-weighted magnetic resonance imaging (MRI) findings of a one-year old infant with neonatal citrullinemia during an acute episode and discuss possible underlying mechanisms for abnormal diffusion findings.

Outcome for patients with isolated atrial septal defects in the oval fossa diagnosed in infancy.

Objective: Our aim was to investigate the change in diameter of holes within the oval fossa, and the role of aneurismal formation in reducing the size of the hole, in patients diagnosed during infancy with isolated defects in the floor of the fossa.

Patients: In a retrospective study, we included 100 patients diagnosed during the first year of life with an isolated defect in the floor of the oval fossa who had subsequently been observed for at least 5 years. There were 56 females and 44 males.