962 resultados para Cholestatic jaundice in infancy


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Workshop at Open Repositories 2014, Helsinki, Finland, June 9-13, 2014

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Hb Köln was identified by DNA analysis in a Brazilian patient. A four-year old Brazilian female, with jaundice since birth, presented an abnormal band, between A2 and S, in hemoglobin electrophoresis on a cellulose acetate membrane, and a band with electrophoretic migration similar to Hb C on agar gel. Thermic instability and isopropanol precipitation tests were positive. Heinz bodies were observed in the patient’s peripheral blood. Sequencing of the three exons of the b globin gene detected a transition from G to A in the first position of codon 98. This alteration does not create or abolish any known restriction site. In this case, confirmation of the mutation was accomplished by allele-specific oligonucleotide hybridization, which is a simple and fast identification method when the clinical data and hematological and electrophoretic patterns are suggestive of Hb Köln.

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Immunohistochemistry was used to evaluate the effects of neonatal handling and aversive stimulation during the first 10 days of life on the number of corticotrophs in the anterior lobe of the pituitary of 11-day-old male Wistar rats. Since adult rats handled during infancy respond with reduced corticosterone secretion in response to stressors and with less behavior inhibition in novel environments, we assumed that neonatal stimulation could affect pituitary morphology during this critical period of cell differentiation. Three groups of animals were studied: intact (no manipulation, N = 5), handled (N = 5) and stimulated (submitted to 3 different aversive stimuli, N = 5). The percentage of ACTH-immunoreactive cells in the anterior lobe of the pituitary (number of ACTH-stained cells divided by total number of cells) was determined by examining three slices per pituitary in which a minimum of 200 cells were counted by two independent researchers. Although animals during the neonatal period are less reactive to stress-like stimulation in terms of ACTH and corticosterone secretion, results showed that the relative number of ACTH-stained cells of neonatal handled (0.25 ± 0.01) and aversive stimulated (0.29 ± 0.03) rats was not significantly different from intact (0.30 ± 0.03) animals. Neonatal stimulation may have a differential effect on the various subpopulations of corticotroph cells in the anterior pituitary

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Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology. A number of questions regarding its etiology are unclear. CD4+CD25+ regulatory T cells (Tregs) play a critical role in self-tolerance and, for unknown reasons, their relative number is reduced in PBC patients. B-cell-activating factor (BAFF) is a key survival factor during B-cell maturation and its concentration is increased in peripheral blood of PBC patients. It has been reported that activated B cells inhibit Treg cell proliferation and there are no BAFF receptors on Tregs. Therefore, we speculated that excessive BAFF may result in Treg reduction via B cells. To prove our hypothesis, we isolated Tregs and B cells from PBC and healthy donors. BAFF and IgM concentrations were then analyzed by ELISA and CD40, CD80, CD86, IL-10, and TGF-β expression in B cells and Tregs were measured by flow cytometry. BAFF up-regulated CD40, CD80, CD86, and IgM expression in B cells. However, BAFF had no direct effect on Treg cell apoptosis and cytokine secretion. Nonetheless, we observed that BAFF-activated B cells could induce Treg cell apoptosis and reduce IL-10 and TGF-β expression. We also showed that BAFF-activated CD4+ T cells had no effect on Treg apoptosis. Furthermore, we verified that bezafibrate, a hypolipidemic drug, can inhibit BAFF-induced Treg cell apoptosis. In conclusion, BAFF promotes Treg cell apoptosis and inhibits cytokine production by activating B cells in PBC patients. The results of this study suggest that inhibition of BAFF activation is a strategy for PBC treatment.

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Biliary atresia (BA) is classically described at the neonatal age. However, rare cases of BA in older infants have also been reported. We report four cases of late-onset BA in infants older than 4 weeks (3 males, 1 female), and describe the diagnostic and management difficulties. One of the cases had a late-onset (29 weeks) presentation with a successful surgical procedure. We highlight the importance of this unusual differential diagnosis in infants with cholestatic syndrome, who may benefit from Kasai surgery, regardless of age.

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Very preterm birth is a risk for brain injury and abnormal neurodevelopment. While the incidence of cerebral palsy has decreased due to advances in perinatal and neonatal care, the rate of less severe neuromotor problems continues to be high in very prematurely born children. Neonatal brain imaging can aid in identifying children for closer follow-up and in providing parents information on developmental risks. This thesis aimed to study the predictive value of structural brain magnetic resonance imaging (MRI) at term age, serial neonatal cranial ultrasound (cUS), and structured neurological examinations during the longitudinal follow-up for the neurodevelopment of very preterm born children up to 11 years of age as a part of the PIPARI Study (The Development and Functioning of Very Low Birth Weight Infants from Infancy to School Age). A further aim was to describe the associations between regional brain volumes and long-term neuromotor profile. The prospective follow-up comprised of the assessment of neurosensory development at 2 years of corrected age, cognitive development at 5 years of chronological age, and neuromotor development at 11 years of age. Neonatal brain imaging and structured neurological examinations predicted neurodevelopment at all age-points. The combination of neurological examination and brain MRI or cUS improved the predictive value of neonatal brain imaging alone. Decreased brain volumes associated with neuromotor performance. At the age of 11 years, the majority of the very preterm born children had age-appropriate neuromotor development and after-school sporting activities. Long-term clinical follow-up is recommended at least for all very preterm infants with major brain pathologies.

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This article analyzes the emerging ethical and legal requirements for informed consent in pharmacogenomic research. It reviews how policies at the international, regional and national levels have responded to the ethical challenges raised by this new research area. It concludes that the pharmacogenomic policy framework is still in its infancy and needs to be further developed to answer the challenges raised by this important discipline.

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Fulminant hepatic failure (FHF) is a dramatic and challenging syndrome in clinical medicine. Although an uncommon disorder, it is usually fatal and occurs in previously healthy person. While the causes of FHF remain unclear, viral hepatitis and drug-induced liver injury account for the majority of cases. Hepatitis E causes large-scale epidemics of hepatitis in the Indian subcontinent, involving hundreds of thousands of cases with high mortality. FHF is associated with several clinical features like jaundice, shrunken liver, easy bruising, low levels of serum proteins, fatigue, multi-organ failure etc and metabolic derangements like hypoglycemia, hyperlipidemia, hyponatremia, defective protein synthesis, reduced energy production, decreased rate of urea production etc. These disturbances are predominantly attributed to oxidative stress, membrane destabilization and osmolytic imbalances. The options available for these patients are quite minimal with liver transplantation being one of them. But the procedure is ridden with issues causing it to find less favor among the patients and the caregivers. Use of hepatoprotective and cytoprotective drugs, is being considered to be a more acceptable alternative as a strategy to enhance liver regeneration. In this regard use of taurine a naturally occurring amino acid that plays a crucial role in many physiological processes would prove to be effective. In the present study, hepatoprotective effect of taurine on a rat model of induced FHF was studied. Taurine supplementation has effectively counteracted the metabolic and structural aberrations in the liver caused by D-galactosamine intoxication.

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Mangroves are considered to play a significant role in global carbon cycling. Themangrove forests would fix CO2 by photosynthesis into mangrove lumber and thus decrease the possibility of a catastrophic series of events - global warming by atmospheric CO2, melting of the polar ice caps, and inundation of the great coastal cities of the world. The leaf litter and roots are the main contributors to mangrove sediments, though algal production and allochthonous detritus can also be trapped (Kristensen et al, 2008) by mangroves due to their high organic matter content and reducing nature are excellent metal retainers. Environmental pollution due to metals is of major concern. This is due to the basic fact that metals are not biodegradable or perishable the way most organic pollutants are. While most organic toxicants can be destroyed by combustion and converted into compounds such as C0, C02, SOX, NOX, metals can't be destroyed. At the most the valance and physical form of metals may change. Concentration of metals present naturally in air, water and soil is very low. Metals released into the environment through anthropogenic activities such as burning of fossils fuels, discharge of industrial effluents, mining, dumping of sewage etc leads to the development of higher than tolerable or toxic levels of metals in the environment leading to metal pollution. Of course, a large number of heavy metals such as Fe, Mn, Cu, Ni, Zn, Co, Cr, Mo, and V are essential to plants and animals and deficiency of these metals may lead to diseases, but at higher levels, it would lead to metal toxicity. Almost all industrial processes and urban activities involve release of at least trace quantities of half a dozen metals in different forms. Heavy metal pollution in the environment can remain dormant for a long time and surface with a vengeance. Once an area gets toxified with metals, it is almost impossible to detoxify it. The symptoms of metal toxicity are often quite similar to the symptoms of other common diseases such as respiratory problems, digestive disorders, skin diseases, hypertension, diabetes, jaundice etc making it all the more difficult to diagnose metal poisoning. For example the Minamata disease caused by mercury pollution in addition to affecting the nervous system can disturb liver function and cause diabetes and hypertension. The damage caused by heavy metals does not end up with the affected person. The harmful effects can be transferred to the person's progenies. Ironically heavy metal pollution is a direct offshoot of our increasing ability to mass produce metals and use them in all spheres of existence. Along with conventional physico- chemical methods, biosystem approachment is also being constantly used for combating metal pollution

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This study was on women's industries programme in Kerala, to assess the involvement of manpower in this field and to analyse the difficulties and problems faced by the women entrepreneurs which impede the growth and smooth functioning of units. It was supported by the views of 275 women entrepreneurs of Kerala. Census method was adopted and only 58 per cent of units responded by supplying necessary details. Details were collected from these: units through mailed questionnaires designed for the purpose. The study highlights the profile of workers in the women's industrial units, but the profile of the entrepreneurs is neglected. Problems faced by women entrepreneurs are analysed under the following major heads viz., capital, raw materials, marketing, competition from other units and availability of power. But the conclusions drawn from the survey are not on proper empirical support. It also includes suggestions of entrepreneurs. The major findings of the study are as follows : Nearly 82 per cent of the women's industrial units are functioning throughout the year. Proprietory concerns and co—operative societies are the popular ones. Majority of the units are running on profit. Women's units are still in their infancy and so the problems faced by them are many. The characteristics of having other business or sister concerns is lacking among women entrepreneurs. Nearly 94 per cent of the employees are permanent. About four-fifth (81%) of the workers are full time employees. Only a very small proportion of the employees (1%) get a reasonable income that is above Rs.50O per month. The workers are very young and 63 per cent workers have no experience at all.

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Since last few years open source integrated library systems gaining attention of library and information science professionals. This paper tries to identify the extent of adoption of Koha, an open source ILS in libraries around the world through a Web based study. The study found that Koha adoption in libraries is still at infancy

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This is the beginning of a prospective study on patients who have obstructive jaundice to see how the serum bilirubin falls after operative relief of the obstruction. Seven of such patients have been studied; four had carcinoma of the head of the pancreas while the other three had choledocholithiasis. The patients with carcinoma had relief of the jaundice through a biliary-enteric anastomosis and those with common bile duct stones had choledochotomy with stone extraction which was completed with insertion of a T-tube. Serial bilirubin estimations were then performed post-operatively to chart the pattern and rate of descent of this in each patient. Our observations suggest that the pattern of fall of serum bilirubin after successful decompression of the extra-hepatic biliary tree exhibit a distinct pattern regardless of the surgical procedure performed for the relief of the obstruction.

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The human colonic microflora has a central role in health and disease, being unique ill its complexity and range of functions. As such, dietary modulation is important for improved gut health, especially during the highly-sensitive stage of infancy. Diet call affect the composition of the gut microflora through the availability of different substrates for bacterial fermentation. Differences in gut microflora composition and incidence of infection exist between breast-fed and formula-fed infants, with the former thought to have improved protection. Historically, this improvement has been believed to be a result of the higher presence of reportedly-beneficial genera such as the bifidobacteria. As such, functional food ingredients such as prebiotics and probiotics could effect a beneficial modification in the composition and activities of gut microflora of infants by increasing positive flora components. The prebiotic approach aims to increase resident bacteria that are considered to be beneficial for human health, e.g. bifidobacteria and lactobacilli, while probiotics advocates the use of the live micro-organisms themselves in the diet. Both approaches have found their way into infant formula feeds and aim to more closely simulate the gut microbiota composition seen during breast-feeding.

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The aim of this study was to assess which behavioral characteristics of the newborn infant are associated with an increased risk of postnatal depression (PND) in the mother. A total of 497 mothers from a prospective cohort study were recruited during the last trimester of pregnancy. Infants were evaluated at 3 days with the Brazelton Neonatal Behavioral Scale. Maternal PND was assessed at 6 weeks postpartum with the Edinburgh Postnatal Depression Scale. Behavioral characteristics of the infant predicted the occurrence of PND, independent of other risk factors for PND: The lower the infants' orientation performance, the higher the risk that the mother would present with PND 6 weeks after delivery. As orientation capacities play a key role in the interactional skills developed between mothers and their infants, an infant who is difficult to engage in interaction may contribute to the risk of PND.

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The current study investigated a new, easily administered, visual inhibition task for infants termed the Freeze-Frame task. In the new task, 9-month-olds were encouraged to inhibit looks to peripheral distractors. This was done by briefly freezing a central animated stimulus when infants looked to the distractors. Half of the trials presented an engaging central stimulus, and the other half presented a repetitive central stimulus. Three measures of inhibitory function were derived from the task and compared with performance on a set of frontal cortex tasks administered at 9 and 24 months of age. As expected, infants' ability to learn to selectively inhibit looks to the distractors at 9 months predicted performance at 24 months. However, performance differences in the two Freeze-Frame trial types early in the experiment also turned out to be an important predictor. The results are discussed in terms of the validity of the Freeze-Frame task as an early measure of different components of inhibitory function. (C) 2007 Elsevier Inc. All rights reserved.