Prognostic evidence of LEF1 isoforms in childhood acute lymphoblastic leukemia.

Introduction: The lymphoid enhancer factor 1 (LEF1) is a DNA-binding transcription factor that functions in the Wnt signaling pathway. Increased LEF1 activity is associated with progression of several types of cancer including leukemia. Here, we investigated LEF1 isoform expression and genomic variations in acute lymphoblastic leukemia (ALL).

Mitochondrial mutations in patients with congenital heart defects by next generation sequencing technology.

It has been shown that mitochondrial deoxyribo nucleic acid mutations may play an important role in the development of cardiomyopathy, and various types of cardiomyopathy can be attributed to disturbed mitochondrial oxidative energy metabolism. Several studies have described many mutations in mitochondrial genes encoding for subunits of respiratory chain complexes. Thus, recent studies confirm that pathologic mitochondrial deoxyribo nucleic acid mutations are a major reason of diseases and determining them by next-generation sequencing will improve our understanding of dysregulation of heart development.

A novel ATP8 gene mutation in an infant with tetralogy of Fallot.

We report the case of a novel mitochondrial DNA mutation in the MT-ATP8 gene in an infant with tetralogy of Fallot. Next-generation sequencing was applied to sequence whole mitochondrial DNA of the patient. A known Leber's hereditary optic neuropathy-associated mutation (G9804A), a heteroplasmic T7501C mutation (17%), and a novel C8481 T Pro > Leu missense mutation in the MT-ATP8 gene was identified.

Uniform, large surface-area polarization by modifying corona-electrodes geometry.

We report on the uniform, large scale polarization of ferroelectric materials by a newly designed corona charging technique developing nonconventional electrodes geometry. The results of pyroelectric measurements represented the spatial homogeneity of the polarization attained through a surface area of ~25 cm(2).

Primary myxoma of the pulmonary artery.

Cardiac myxomas are most commonly found within the left atrium. We now report a patient presenting with dyspnea, palpitation, and chest pain who was found to have myxoma arising from the pulmonary valve commissure. Urgent surgical treatment is curative and warranted for these lesions, in order to prevent outflow obstruction and thromboembolic phenomena.

Primary endobronchial schwannoma.

Primary intrabronchial neurilemmas, also called schwannomas, are extremely rare tumors that originate from Schwann cells. We report a case of intrabronchial schwannoma arising in the left main bronchus in an 8-year-old child. The diagnosis was made by bronchofiberscopic biopsy.

Pituitary apoplexy following coronary artery bypass grafting: report of a case.

Abstract. Pituitary apoplexy can rarely occur after surgical procedures, mainly due to severe haemorrhage or hypotension. It is a serious condition that may lead to devastating complications and even to death.

Quantitative measurements of adsorbate-adsorbate interactions at solid-liquid interfaces.

The interactions between adsorbates at a solid-liquid interface were studied by video-rate STM for the case of sulfur on Cu(100) electrode surfaces in HCl solution. Quantitative data were obtained by analyzing the S(ad) dimer dynamics within the surrounding c(2 x 2)-Cl adlattice as well as the adsorbate configurations. The interactions are repulsive for S(ad) separated by one or two lattice spacings and attractive at a separation of square root of 2 with energies comparable to adsorbates at the solid-vacuum interface.

In situ video-STM studies of adsorbate dynamics at electrochemical interfaces.

The dynamic behavior of individual adsorbates at electrochemical interfaces was studied directly by in situ high-speed scanning tunneling microscopy, using sulfur adsorbed on Cu(100) electrodes in 0.01 M HCl solution as an example. By dosing from diluted Na(2)S solutions S(ad) coverages of a few percent can be prepared, with the sulfur adsorbates occupying positions within the c(2x2) lattice of coadsorbed chloride.

Drugs with blocking effects on the renin-angiotensin-aldosterone system do not improve endothelial dysfunction long-term in hypertensive patients.

In essential hypertension, endothelial dysfunction has been documented many times and correlates with prognosis. The influence of the renin-angiotensin-aldosterone system (RAAS) on endothelial dysfunction has also been studied. The present study investigated the duration of the effects of RAAS-blocking drugs on endothelial function in 44 consecutive, never-treated, outpatients with mild to moderate hypertension.

Influence of methylprednisolone on levels of neuron-specific enolase in cardiac surgery: a corticosteroid derivative to decrease possible neuronal damage.

Background: Cerebral injury is a well-known complication after cardiac surgery with cardiopulmonary bypass (CPB), especially in adult patients. Specific biochemical markers like neuron-specific enolase (NSE) and S-100beta protein were developed previously for early detecting neuronal damage after CPB. Corticosteroids are shown to reduce multisystemic deleterious effects of cardiopulmonary bypass due to their anti-inflammatory characteristics.

Congenital heart disease in children with Down's syndrome: Turkish experience of 13 years.

Background: Down's syndrome (DS) is the most common chromosomal abnormality due to a trisomy of chromosome 21 commonly associated with congenital heart defects (CHDs). This study aimed to evaluate the frequency and types of CHD patterns in Turkish children with DS.

Method: The data relate to paediatric patients with DS who underwent cardiologic screening between 1994 and 2007 and were reviewed in our Paediatric Cardiology unit.

Thymoma: surgical results of 73 cases.

Purpose: Thymomas are uncommon tumours; they represent a broad spectrum of morphological and clinical behaviour. The purpose of this study is to identify the factors that influence survival.

Method: Seventy-three patients with thymoma who underwent thymectomy between 1980 and 2002 were reviewed.

Left-sided aortic arch, right-sided descending aorta, and right-sided arterial duct associated with interruption of the aortic arch and presence of Abbott's artery.

We describe a male infant, seen at the age of 10 days, with a very rare form of vascular ring. The aortic arch was left-sided, but the aorta descended on the right, with a right-sided persistently patent arterial duct associated with interruption of the aortic arch and presence of Abbott's artery. We performed end-to-side anastomosis of the descending aorta to the ascending aorta, divided the Abbott's artery and the right-sided arterial duct, and banded the pulmonary trunk.

Echocardiographic diagnosis of a giant thrombus passing through a patent foramen ovale from right atrium to the left atrium.

Pulmonary embolus sourced by right atrial thrombus trapped in a patent foramen ovale is an unusual, rare condition. Thus in suspicion of massive pulmonary thromboembolus, echocardiographic examination carries great importance evaluate right ventricular functions and diagnose right sided intracardiac thrombus. We report a 76-year-old female with massive pulmonary embolism caused by a gigantic thrombus trapped in a patent foramen ovale.

Successful bleomycin pleurodesis in a patient with prolonged pleural effusion after extracardiac fontan procedure.

Pleural effusions after modified Fontan operations are unpredictable and contribute significantly to the morbidity and prolonged hospitalization. Chemical pleurodesis is widely performed in adults for malignant pleural effusions, but has found less chance of usage for benign cases or the cases with unidentified etiology. Although it is a safe and easily performed procedure, the use of bleomycin for the treatment of pleural effusions in the pediatric age population has been rarely reported in the literature.

Over 14 years of experience with cardiac myxomas.

Atrial myxomas are the most commonly encountered tumours of the heart and can present at different ages with different clinical symptoms. They are one of the curable tumours of the heart. Appropriate surgical treatment and surgery must be performed with great precautions in order to prevent fatal systemic embolizations.

Childhood mediastinal masses in infants and children.

We reviewed all cases of primary pediatric mediastinal masses diagnosed and treated over a 24-year period. In this study, out of 187 primary mediastinal mass cases diagnosed between 1980 and 2004 in Istanbul University Istanbul Faculty of Medicine, Cardiovascular Surgery Department, 37 pediatric primary mediastinal mass cases were retrospectively evaluated according to age, sex, symptoms, diagnostic procedure, anatomical location, surgical treatment, histopathological evaluation and postoperative adjuvant therapy. The patients ranged in age from 2 months to 15 years at the time of diagnosis, with a mean age of 8 years.

Video STM studies of adsorbate diffusion at electrochemical interfaces.

Direct in situ studies of the surface diffusion of isolated adsorbates at an electrochemical interface by high-speed scanning tunneling microscopy (video STM) are presented for sulfide adsorbates on Cu(100) in HCl solution. As revealed by a quantitative statistical analysis, the adsorbate motion can be described by thermally activated hopping between neighboring adsorption sites with an activation energy that increases linearly with electrode potential by 0.50 eV per V.

Serum lactate level has prognostic significance after pediatric cardiac surgery.

Objective: The determination of postoperative course after cardiac surgery has always been a challenging issue. It is more sophisticated in the pediatric age group. The aim of this investigation was to identify whether increased concentrations of lactate in arterial blood has a predictive value for postoperative morbidity and mortality after heart surgery.

Anomalous dual drainage of the right pulmonary veins in a patient with cor triatriatum: report of a case without scimitar sign.

The majority of patients with partial anomalous drainage of pulmonary veins are asymptomatic during infancy and childhood. Patients with significant left-to-right shunt develop symptoms and benefit from early corrective surgery. Anomalous pulmonary veins draining into inferior vena cava is very rare and frequently encountered in association with scimitar syndrome.

Results of surgical treatment for infective endocarditis in children.

Objective: Infective endocarditis is uncommon condition, with a high degree of morbidity and mortality. It is less common in children, albeit tending to be associated with congenital cardiac malformations. We describe our experience of the need for surgical treatment in children with infective endocarditis.

Idiopathic aneurysm of the inferior caval vein.

Idiopathic aneurysms of the inferior caval vein are uncommon in children and adults. We describe a 14 year old boy with a saccular aneurysm of the inferior caval vein, in whom no surgical intervention was required to repair the aneurysm. The patient is being followed-up periodically for the evaluation of any increase in the diameter of the inferior caval vein.

Epitheloid hemangioendothelioma of the heart in infancy.

We report a case of primary cardiac epithelioid hemangioendothelioma arising from the right atrium of a 2-month-old infant. The tumor was found incidentally during exploratory sternotomy for recurrent pericardial effusion. This case represents a very rare situation, because this is the youngest patient found in relevant literature, and because it involves extensive infiltration by the tumor without any development of intracardiac mass appearance.

The benefits of continuous ultrafiltration in pediatric cardiac surgery.

Background: Systemic inflammatory response and capillary leak syndrome, caused by extracorporeal circulation, have negative effects on the function of vital organs during the postoperative period. Modified ultrafiltration (MUF) has been developed as an alternative method to reduce the detrimental effects of cardiopulmonary bypass. The aim of this prospective, randomized study is to analyze the effects of MUF in a pediatric population undergoing congenital cardiac surgery.