Summary
Esophageal atresia (EA) is a congenital defect of the esophagus involving the interruption of the esophagus with or without connection to the trachea (tracheoesophageal fistula TEF). EA/TEF ...may occur as an isolated anomaly, may be part of a complex of congenital defects (syndromic), or may develop within the context of a known syndrome or association. The molecular mechanisms underlying the development of EA are poorly understood. It is supposed that a combination of multigenic factors and epigenetic modification of genes play a role in its etiology.
The aim of our work was to assess the human gene expression microarray study in esophageal tissue samples. Total RNA was extracted from 26 lower pouches of esophageal tissue collected during thoracoscopic EA repair in neonates with the isolated (IEA) and the syndromic form (SEA).
We identified 787 downregulated and 841 upregulated transcripts between SEA and controls, and about 817 downregulated and 765 upregulated probes between IEA and controls. Fifty percent of these genes showed differential expression specific for either IEA or SEA. Functional pathway analysis revealed substantial enrichment for Wnt and Sonic hedgehog, as well as cytokine and chemokine signaling pathways. Moreover, we performed reverse transcription polymerase chain reaction study in a group of SHH and Wnt pathways genes with differential expression in microarray profiling to confirm the microarray expression results. We verified the altered expression in SFRP2 gene from the Wnt pathway as well as SHH, GLI1, GLI2, and GLI3 from the Sonic hedgehog pathway. The results suggest an important role of these pathways and genes for EA/TEF etiology.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, UILJ, UKNU, UL, UM, UPUK
Tracking the motion of lipid domains on a vesicle is a rheological technique allowing the measurement of surface shear viscosities of vesicular lipid phases. The ratio of surface to bulk viscosity ...defines a viscous length scale. Hydrodynamic interactions split the motion of the domains into different modes of diffusion. The measurability of surface shear viscosities from any mode of diffusion is limited to viscous length scales between the radius of the domains and the radius of the vesicle. The measurability of the surface shear viscosity results from the sensitivity of the diffusion to surface shear viscosities and from sufficient spatial resolution to resolve the diffusive motion. Switching between the various modes of diffusion is a trade between sensitivity gained and resolution lost by the hydrodynamic interactions leaving the measurability unchanged. Measurability drops with the number of domains making single-domain rheology the best technique to measure surface shear viscosities. Ultimately confinement of the domains to small vesicles renders measurements of surface rheological properties with domain-tracking rheology impossible. Experiments on domains in vesicles of a mixture of dioleoylphosphatidylcholine (DOPC), dipalmytoylphosphatidylcholin (DPPC) and cholesterol (Chol) exhibit diffusion that is entirely controlled by dissipation into the water. The diffusion is suppressed compared to the diffusion of isolated domains in a flat membrane due to confinement to the curved vesicle and by hydrodynamic interactions between the domains. Effects of surface shear viscosity can be neglected.
Children with multisystem involvement including congenital heart defect (CHD) are a very salient problem. The purpose of this study was to evaluate the incidence of CHD associated with malformations ...of other systems and to assess the modalities of treatment and perioperative mortality among patients referred to the department of pediatric cardiac surgery.
The medical records of 1856 children were reviewed retrospectively from 1997 to 2002 to establish CHD and types of associated malformations. The connections between CHD and other lesions were investigated. Furthermore, the influence of patient and perioperative variables on mortality risk was scrutinized. Univariate and multivariate analyses were used.
Eighty-four children (4.53%) had CHD and associated malformations. The malformations of digestive (35.7%), urinary (22.4%), and nervous (14.3%) systems were the most frequently observed associated defects. No relation was found between CHD and concomitant lesions. The results of multivariate logistic regression showed significant influence of patient age, primary cardiac procedure, and CHD type on mortality (ca 19%) in children with multiorgan lesions.
The treatment of children with CHD and associated multiple lesions is connected with higher mortality risk. The following factors: younger age, urgency of surgical procedure, and primary surgical procedure had negative impact on patient's outcome. However, these risks in certain cases are inevitable. The cardiac procedure preceding the surgical operation may improve the overall effect of treatment because of circulatory stabilization, provided that the condition of the patient does not preclude any intervention at all.
Cultures of Candida glabrata treated with CdCl2 form intracellular Cd(II) complexes that evolve with the time of culturing. Initially, glutathione (gamma ECG) appears to be the major buffering ...component. One type of Cd(II)-glutathione complex exists as a cadmium:sulfide (CdS) crystallite coated with glutathione. A time dependent change in the coating of the CdS particles occurs with a decrease in the (gamma ECG) content and a corresponding increase in the abundance of (gamma EC)nG peptides with (gamma EC)2G becoming the predominant peptide. The desGly variant (gamma EC)2 appears in significant concentration only in late cultures. The evolution in isopeptide coating appears to be dependent on the sulfide content of the CdS particles. Cellular conditions that enhance the generation of sulfide ions facilitate the conversion from gamma ECG to (gamma EC)2G
Mutants of Saccharomyces cerevisiae, deficient in cytosolic superoxide dismutase and catalase activities were used to study the role of various oxygen species in the process of lipid peroxidation in ...yeast cells. Lipid peroxidation does not occur normally in yeast, because this organism is unable to form fatty acids with more than one double bond, whereas under physiological conditions, only fatty acids with at least two double bonds undergo this process. The fatty acid content of cellular lipids was modified by growing the cells in anoxia in the presence of oleic or linolenic acid. Toxic effects of oxygen were observed almost exclusively in those cells of yeast mutants deficient in superoxide dismutase, which contain linolenic acid in cellular lipids. Hypersensitivity of the mutant cells, however, results mainly from toxic effects of the products of autooxidation of extracellular fatty acids. These facts suggest that superoxide dismutases are in some way involved in preventing toxic effects of the products of lipid peroxidation and to some extent prevent the process of lipid peroxidation.
Two ways of iron oxidation by yeast Błaszczyński, M; Krawiec, Z; Biliński, T
Acta microbiologica Polonica,
1993, Letnik:
42, Številka:
1
Journal Article
It has been found that yeast cells suspended in saline containing ferrous salts could oxidize them to the ferric form by excreting H2O2 and ammonia. Excretion of ammonia accelerates spontaneous ...oxidation of iron by molecular oxygen. Ammonia generation probably results from the degradation of amino acids within starving cells.
The analysis presents 622 children in the age ranged from 4 months to 14 years, operated on vesicoureteral reflux. The treatment was carried on in Department of Pediatric Surgery in Poznan between ...1983 and 1992. All these children were operated on with antireflux Politano-Leadbetter technique, modified by Sarrazin, protecting reimplanted ureters with stenting catheters. Duplicated ureters were reimplanted "en bloc" according to Bettex technique, also with Sarrazin modification. Preoperative parameters included: history, radiological evaluations: voiding cystourethrogram, intravenous pyelogram or ultrasound imaging, endoscopic evaluations--cystourethroscopy (with meatal calibration in girls), laboratory blood and urine results. The reflux degree was estimated according to international classification IRSC. There were 12 (2%) children with II degree, 498 (80%) with III degree, 101 (16%) with IV degree and 11 (2%) patients with V degree reflux. In order to compare the results of operative treatment, all analyzed children were subdivided into groups: 303 (48.7%) patients with primary reflux, 77 (12%) with reflux to duplicated pyelo-ureteral systems, 17 (2.7%) with refluxing megaureters, 66 (10%) with reflux to hypoplastic kidney, 99 (15.9%) with cystic cystitis and 114 (18%) children with urethral meatal stricture. Good recovery with uncomplicated postoperative course was obtained in 606 (97.4%) patients. Early postoperative complications occurred in 16 (2.6%). In outpatient control the results of urinalysis and urine cultures, ultrasound imaging and voiding cystourethrograms were tested. Urine cultures were negative in 501 (80.5%) patients 3-4 weeks after discharge increasing to above 92% of patients 6 months after operation. In postoperative imaging control, 600 (96.5%) patients presented with normal status of upper urinary tract, in 14 (2.2%) transient dilatation of reimplanted ureters was observed and in 8 (1.3%) ureters had to be reimplanted because of secondary dilatation. After 6 months postoperative voiding cystourethrograms showed cessation of reflux in 589 (94.6%) patients, in 33 (5.2%) recurrent reflux was observed, which disappeared in 20 of them in control. In 13 patients urodynamic evaluation showed vesicourethral dysfunction, medically curable.
The use of vesicoamniotic shunt in the case of obstructive uropathy was described. On the base of ultrasound screening it has been diagnosed urethral obstruction. This abnormality of urinary tract ...was found in male fetus. It has been diagnosed the posterior urethral valve. Qualification to intrauterine therapy was based on normal parameters of renal function. Under ultrasound guidance the vesicoamniotic shunt was installed. The normal drenage was observed within 6 weeks, until it was dislocated. In 36th week of gestation the cesarean section was performed. The indications were signs of urethral obstruction. The boy was born in whom posterior urethral valves were recognized. He was born in good condition without any biochemical features of renal damage as well as signs of hypoplastic lung.