Total colonic aganglionosis (TCA) is a rare form of Hirschsprung disease (HD). It is difficult to be diagnosed on contrast enema because the radiographic findings are variable. The study aims to re-evaluate the contrast enema findings of TCA. From 2001 to 2009, 14 patients (11 males, 3 females; ages from 1 day to 6 months) with pathologically proven TCA were reviewed for demographic features, c...

BACKGROUND Although high throughput technologies for gene profiling are reliable tools, sample/tissue heterogeneity limits their outcomes when applied to identify molecular markers. Indeed, inter-sample differences in cell composition contribute to scatter the data, preventing detection of small but relevant changes in gene expression level. To date, attempts to circumvent this difficulty were ...

The bile salt export pump (BSEP) plays an important role in bile acid excretion. Impaired BSEP function may result in liver injury. Bile acids also undergo basolateral efflux, but the relative contributions of biliary (CLBile) versus basolateral efflux (CLBL) clearance to hepatocellular bile acid excretion have not been determined. In the present study, taurocholic acid (TCA; a model bile acid)...

HYPOTHESIS Hypothermic total circulatory arrest (TCA) in the resection and replacement of the thoracoabdominal and descending thoracic aorta is safe, will significantly decrease the incidence of postoperative renal failure, and should be preferentially performed over left heart bypass (LHB). DESIGN Retrospective review case series. SETTING Large, private, urban teaching hospital. PATIENTS...

The transferrin receptor is encoded by a diallelic locus which had been described as the T-cell antigenic system TCA. Using a human lymphoblastoid T-cell line (HSB) which reportedly carries a transferrin receptor and which we showed to be TCA-2 positive by serology, it appeared that only a TCA-2 but not a TCA-1 specific alloantiserum had the capacity to precipitate a protein with a molecular we...

BACKGROUND Evaluation of interindividual variability is a challenging step in risk assessment. For most environmental pollutants, including perchloroethylene (PERC), experimental data are lacking, resulting in default assumptions being used to account for variability in toxicokinetics and toxicodynamics. OBJECTIVE We quantitatively examined the relationship between PERC toxicokinetics and tox...

BACKGROUND Hirschsprung's disease (HD) is characterized by a lack of ganglion cells in the myenteric and submucosal plexus, associated with increased numbers of acetyl cholinesterase (AChE) positive nerve fibres. In approximately 10% of patients with HD the entire colon will be affected; a condition known as Total Colonic Aganglionosis (TCA). Aganglionosis of the appendix has long been consider...

A novel approach for the mathematical modeling of (13)C label incorporation into amino acids via the TCA cycle that eliminates the explicit calculation of the labeling of the TCA cycle intermediates is described, resulting in one differential equation per measurable time course of labeled amino acid. The equations demonstrate that both glutamate C4 and C3 labeling depend in a predictable manner...