Unifocalization and repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

Aim: To correlate anatomic and genetic features of paediatric patients with pulmonary atresia, ventricular septal defect (VSD) and multiple aortopulmonary collateral arteries with surgical outcome.

Methods: 44 consecutive patients aged 33 +/- 40 mo underwent either primary one-stage unifocalization (n = 32) or palliative right ventricular outflow tract reconstruction (n = 12) followed by secondary unifocalization and repair (n = 10) based on preoperative morphometric and functional evaluation of pulmonary blood sources. Chromosome 22q11.

Power dissipation associated with surgical operations' hemodynamics: critical issues and application to the total cavopulmonary connection.

The issue of the correct determination of the mechanical power dissipated by the blood flow in the circulatory system is very important. This parameter is particularly critical when the patient's circulation has to overcome structural impairments, such as, e.g.

Clinical outcome of 193 extracardiac Fontan patients: the first 15 years.

Objectives: We sought to evaluate the mid-term outcome of hospital survivors with extracardiac Fontan circulation.

Background: Few data exist about the mid-term and long-term results of the extracardiac Fontan operation.

Methods: From November 1988 to November 2003, 221 patients underwent an extracardiac Fontan procedure as primary (9 patients) or secondary (212 patients) palliation, at a mean age of 72.

[Evaluation of the Yersinia enterocolitica pathogenicity through some phenotypic and genotypic characters: CRMOX agar positivity and presence of the ail gene].

Sixty-nine strains of Y. enterocolitica isolated from environmental and human matrices (waste water, food and faeces) were studied in order to evidence the presence of ail gene, calcium-dependency and Congo Red absorption for pathogenic strains identification. Out of 24 clinical strains, the ail gene was present in 21 (87%), among which 79% were CRMOX-positive as well.

Genetic syndromes and outcome after surgical correction of tetralogy of Fallot.

Background: Genetic syndromes occur in 20% of patients with tetralogy of Fallot (TOF). The impact of genetic syndromes on surgical repair of TOF in infancy is still under investigation.

Methods: This retrospective study reviews the outcome of 306 consecutive patients (median age, 5.

S100B protects LAN-5 neuroblastoma cells against Abeta amyloid-induced neurotoxicity via RAGE engagement at low doses but increases Abeta amyloid neurotoxicity at high doses.

At the concentrations normally found in the brain extracellular space the glial-derived protein, S100B, protects neurons against neurotoxic agents by interacting with the receptor for advanced glycation end products (RAGE). It is known that at relatively high concentrations S100B is neurotoxic causing neuronal death via excessive stimulation of RAGE. S100B is detected within senile plaques in Alzheimer's disease, where its role is unknown.

Diagnosis-based risk adjustment and Australian health system policy.

Diagnosis-based risk adjustment is increasingly seen as an important tool for establishing capitation payments and evaluating appropriateness and efficiency of services provided and has become an important area of research for many countries contemplating health system reform. This paper examines the application of a risk-adjustment method, extensively validated in the United States, known as diagnostic cost groups (DCG), to a large Australian hospital inpatient data set. The data set encompassed hospital inpatient diagnoses and inpatient expenditure for the entire metropolitan population residing in the state of New South Wales.

S100B stimulates myoblast proliferation and inhibits myoblast differentiation by independently stimulating ERK1/2 and inhibiting p38 MAPK.

The Ca2+-modulated protein of the EF-hand type, S100B, was shown to inhibit rat L6 myoblast differentiation and myotube formation by interacting with a high affinity with an unidentified receptor (Sorci et al., 2003). We show here that S100B independently inhibits the MKK6-p38 MAPK pathway and stimulates the Ras-MEK-ERK1/2 pathway.

The amphoterin (HMGB1)/receptor for advanced glycation end products (RAGE) pair modulates myoblast proliferation, apoptosis, adhesiveness, migration, and invasiveness. Functional inactivation of RAGE in L6 myoblasts results in tumor formation in vivo.

We reported that RAGE (receptor for advanced glycation end products), a multiligand receptor of the immunoglobulin superfamily expressed in myoblasts, when activated by its ligand amphoterin (HMGB1), stimulates rat L6 myoblast differentiation via a Cdc42-Rac-MKK6-p38 mitogen-activated protein kinase pathway, and that RAGE expression in skeletal muscle tissue is developmentally regulated. We show here that inhibition of RAGE function via overexpression of a signaling deficient RAGE mutant (RAGE delta cyto) results in increased myoblast proliferation, migration, and invasiveness, and decreased apoptosis and adhesiveness, whereas myoblasts overexpressing RAGE behave the opposite, compared with mock-transfected myoblasts. These effects are accompanied by a decreased induction of the proliferation inhibitor, p21(Waf1), and increased induction of cyclin D1 and extent of Rb, ERK1/2, and JNK phosphorylation in L6/RAGE delta cyto myoblasts, the opposite occurring in L6/RAGE myoblasts.

Circumflex coronary artery from right pulmonary artery in hypoplastic left heart syndrome.

We report the case of a newborn with the very rare association of hypoplastic left heart syndrome and aberrant origin of the circumflex coronary artery from the right pulmonary artery. This condition can jeopardize the result of the Norwood palliation.

Inflammatory cytokines in pediatric cardiac surgery and variable effect of the hemofiltration process.

Cardiac surgery with cardiopulmonary bypass (CPB) elicits an inflammatory response and has a multitude of biological consequences, ranging from subclinical organ dysfunction to severe multiorgan failure. Pediatric patients are more prone to have a reaction that can jeopardize their outcome. Cytokines are supposed to be important mediators in this response: limiting their circulating levels is, therefore, appealing.

Abnormal vasomotor function of the epicardial coronary arteries in children five to eight years after arterial switch operation: an angiographic and intracoronary Doppler flow wire study.

Objectives: This study sought to test the vasoreactivity of the translocated coronary arteries after arterial switch operation (ASO) using quantitative angiographic analysis and intracoronary Doppler flow wire velocimetry.

Background: Late coronary artery events occur in 3% to 8% of patients after the ASO. Previous studies of coronary flow reserve have yielded disparate results.

S100b counteracts effects of the neurotoxicant trimethyltin on astrocytes and microglia.

Central nervous system degenerative diseases are often characterized by an early, strong reaction of astrocytes and microglia. Both these cell types can play a double role, protecting neurons against degeneration through the synthesis and secretion of trophic factors or inducing degeneration through the secretion of toxic molecules. Therefore, we studied the effects of S100B and trimethyltin (TMT) on human astrocytes and microglia with two glial models, primary cultures of human fetal astrocytes and a microglia cell line.

Distinct signaling pathways for induction of type II NOS by IFNgamma and LPS in BV-2 microglial cells.

Nitric oxide (NO) release upon microglial cell activation has been implicated in the tissue injury and cell death in many neurodegenerative diseases. Recent studies have indicated the ability of interferon-gamma (IFNgamma) and lipopolysaccharides (LPS) to independently induce type II nitric oxide synthase (iNOS) expression and NO production in BV-2 microglial cells. However, a detailed comparison between the signaling pathways activating iNOS by these two agents has not been accomplished.

Localisation of annexins in the retina of the rainbow trout-light and electron microscopical investigations.

We present a first description of annexin immunoreactivity within the teleost retina. Antibodies against annexins V and VI were used in light and electron microscopic sections of light- and dark-adapted retinae. Strong immunoreactivity could be found in retinal layers with high synaptic input, such as the outer and inner plexiform layers and dendritic regions within the inner plexiform layer, in cells that are involved in negative feedback control such as horizontal and amacrine cells, in the membrane metabolism of photoreceptor outer segments, and in close relation to cytoskeletal components.

Isolation of Trichosporon in a hematology ward.

During mycologic monitoring of the air in a hematology ward, we found massive air contamination caused by Trichosporon asahii, both in the room where neutropenic patients were staying and the corridor immediately outside the room. This fungal species had never been isolated in previous samplings. The urine culture taken from one of the patients in this room, whose urinary catheter had been removed immediately prior to air sampling, resulted positive for T.

Involvement of the S100B in cAMP-induced cytoskeleton remodeling in astrocytes: a study using TRTK-12 in digitonin-permeabilized cells.

1. Stellation of astrocytes in culture involves a complex rearrangement of microfilaments, intermediate filaments, and microtubules, which reflects in part the plasticity of these cells observed during development or after injury. 2.

S100B-stimulated NO production by BV-2 microglia is independent of RAGE transducing activity but dependent on RAGE extracellular domain.

The Ca(2+)-modulated protein, S100B, is expressed in high abundance in and released by astrocytes. At the low levels normally found in the brain, extracellular S100B acts as a trophic factor, protecting neurons against oxidative stress and stimulating neurite outgrowth through its binding to the receptor for advanced glycation end products (RAGE). However, upon accumulation in the brain extracellular space, S100B might be detrimental to neurons.

S100B increases proliferation in PC12 neuronal cells and reduces their responsiveness to nerve growth factor via Akt activation.

S100B is a Ca2+-modulated protein of the EF-hand type expressed in high abundance in a restricted set of cell types including certain neuronal populations. S100B has been suggested to participate in cell cycle progression, and S100B levels are high in tumor cells, compared with normal parental cells. We expressed S100B in the neuronal cell line PC12, which normally does not express the protein, by the Tet-Off technique, and found the following: (i) proliferation was higher in S100B+ PC12 cells than in S100B- PC12 cells; (ii) nerve growth factor (NGF), which decreased the proliferation of S100B- PC12 cells, was less effective in the case of S100B+ PC12 cells; (iii) expression of S100B made PC12 cells resistant to the differentiating effect of NGF; and (iv) interruption of S100B expression did not result in an immediate restoration of PC12 cell sensitivity to the differentiating effect of NGF.

Conotruncal heart defects: impact of genetic syndromes on immediate operative mortality.

Background: The surgical outcome of conotruncal heart defects in patients with genetic syndromes has been poorly studied. The aim of this prospective 5-year multicenter study was to elucidate the post-surgical death rate of children with conotruncal heart defects in relation to the presence of associated genetic syndromes.

Methods: Two institutions enrolled 350 consecutive inpatients with conotruncal heart defects, aged between 1 day and 60 months, who were submitted to surgery; all patients were evaluated by a clinical geneticist and had a standard metaphase chromosome analysis and a fluorescent in situ hybridization study searching for deletion of chromosome 22q11 (del22q11).