bluuebunny/crossref_metadata_2025_split huggingface dataset:
Progressive familial intrahepatic cholestasis
Authors: Davit-Spraul Anne , Gonzales Emmanuel , Baussan Christiane , Jacquemin Emmanuel 
Published: January 2009

Abstract
Progressive familial intrahepatic cholestasis (PFIC) refers to heterogeneous group of autosomal recessive disorders of childhood that disrupt bile formation and present with cholestasis of hepatocellular origin. The exact prevalence remains unknown, but the estimated incidence varies between 1/50,000 and 1/100,000 births.
Three types of PFIC have been identified and related to mutations in hepatocellular transport system genes involved in bile formation. PFIC1 and PFIC2 usually appear in the first months of life, whereas onset of PFIC3 may also occur later in infancy, in childhood or even during young adulthood. Main clinical manifestations include cholestasis, pruritus and jaundice. PFIC patients usually develop fibrosis and end-stage liver disease before adulthood. Serum gamma-glutamyltransferase (GGT) activity is normal in PFIC1 and PFIC2 patients, but is elevated in PFIC3 patients. Both PFIC1 and PFIC2 are caused by impaired bile salt secretion due respectively to defects in ATP8B1 encoding the FIC1 protein, and in ABCB11 encoding the bile salt export pump protein (BSEP). Defects in ABCB4, encoding the multi-drug resistant 3 protein (MDR3), impair biliary phospholipid secretion resulting in PFIC3.
Diagnosis is based on clinical manifestations, liver ultrasonography, cholangiography and liver histology, as well as on specific tests for excluding other causes of childhood cholestasis. MDR3 and BSEP liver immunostaining, and analysis of biliary lipid composition should help to select PFIC candidates in whom genotyping could be proposed to confirm the diagnosis. Antenatal diagnosis can be proposed for affected families in which a mutation has been identified. Ursodeoxycholic acid (UDCA) therapy should be initiated in all patients to prevent liver damage. In some PFIC1 or PFIC2 patients, biliary diversion can also relieve pruritus and slow disease progression. However, most PFIC patients are ultimately candidates for liver transplantation. Monitoring of hepatocellular carcinoma, especially in PFIC2 patients, should be offered from the first year of life. Hepatocyte transplantation, gene therapy or specific targeted pharmacotherapy may represent alternative treatments in the future.
Contralateral approach to a carotid bifurcation aneurysm in a case of multiple intracranial aneurysms: a case report
Authors: Tabatabai Seyed Ali F , Meybodi Ali Tayebi , Hashemi Mohammad , Habibi Zohreh 
Published: January 2009

Abstract

Background
Traditionally, surgery of the anterior circulation aneurysms of the cerebral vasculature is dictated by the site of the lesion, excluding such midline lesions as anterior communication artery aneurysms. Few reports address the issue of using a single craniotomy to obliterate multiple aneurysms located in both hemispheres.

Case presentation
A 51 year-old Caucasian right handed housewife lady (weight 61 kg, height 159 cm) presented with a headache of acute onset which proved to be caused by acute subarachnoid hemorrhage. Cerebral computed tomographic angiography revealed multiple aneurysms. The patient underwent a right pterional craniotomy to obliterate right middle cerebral, distal basilar and left carotid bifurcation aneurysms. The post-operative course was uneventful.

Conclusion
Despite technical difficulties of approaching cerebral vasculature through a contralateral craniotomy, this policy is advised in selected cases in which the benefits of unilateral craniotomy outweigh the risks of brain retraction.
Sedation practice in the intensive care unit: a UK national survey
Authors: Reschreiter Henrik , Maiden Matt , Kapila Atul 
Published: January 2009

Abstract
Introduction
The purpose of this study was to evaluate sedation practice in UK intensive care units (ICUs), particularly the implementation of daily sedation holding, written sedation guidelines, sedation scoring tools and choice of agents.

Methods
A national postal survey was conducted in all UK ICUs.

Results
A total of 192 responses out of 302 addressed units were received (63.5%). Of the responding ICUs, 88% used a sedation scoring tool, most frequently the Ramsey Sedation Scale score (66.4%). The majority of units have a written sedation guideline (80%), and 78% state that daily sedation holding is practiced. A wide variety of sedating agents is used, with the choice of agent largely determined by the duration of action rather than cost. The most frequently used agents were propofol and alfentanil for short-term sedation; propofol, midazolam and morphine for longer sedation; and propofol for weaning purposes.

Conclusions
Most UK ICUs use a sedation guideline and sedation scoring tool. The concept of sedation holding has been implemented in the majority of units, and most ICUs have a written sedation guideline.
Studying alternative splicing regulatory networks through partial correlation analysis
Authors: Chen Liang , Zheng Sika 
Published: January 2009

Abstract
Background
Alternative pre-mRNA splicing is an important gene regulation mechanism for expanding proteomic diversity in higher eukaryotes. Each splicing regulator can potentially influence a large group of alternative exons. Meanwhile, each alternative exon is controlled by multiple splicing regulators. The rapid accumulation of high-throughput data provides us with a unique opportunity to study the complicated alternative splicing regulatory network.

Results
We propose the use of partial correlation analysis to identify association links between exons and their upstream regulators or their downstream target genes (exon-gene links) and links between co-spliced exons (exon-exon links). The partial correlation analysis avoids taking the ratio of two noisy random variables, exon expression level and gene expression level, so that it achieves a higher statistical power. We named this analysis procedure pCastNet (partial Correlation analysis of splicing transcriptome Network). Through studies of known alternative exons, conservation patterns, relative positions, functional annotations, and RT-PCR experiments, we concluded that pCastNet can effectively identify exon-gene or exon-exon links. We further found that gene pairs with exon-gene or exon-exon links tend to have similar functions or are present in the same pathways. More interestingly, gene pairs with exon-gene or exon-exon links tend to share cis-elements in promoter regions and microRNA binding elements in 3' untranslated regions, which suggests the coupling of co-alternative-splicing, co-transcription-factor-binding, and co-microRNA-binding.

Conclusions
Alternative splicing regulatory networks reconstructed by pCastNet can help us better understand the coordinate and combinatorial nature of alternative splicing regulation. The proposed tool can be readily applied to other high-throughput data such as transcriptome sequencing data.
Insight into the molecular requirements for pathogenicity of Fusarium oxysporum f. sp. lycopersici through large-scale insertional mutagenesis
Authors: Michielse Caroline B , van Wijk Ringo , Reijnen Linda , Cornelissen Ben JC , Rep Martijn 
Published: January 2009

Abstract
Background
Fusarium oxysporum f. sp. lycopersici is the causal agent of vascular wilt disease in tomato. In order to gain more insight into the molecular processes in F. oxysporum necessary for pathogenesis and to uncover the genes involved, we used Agrobacterium-mediated insertional mutagenesis to generate 10,290 transformants and screened the transformants for loss or reduction of pathogenicity.

Results
This led to the identification of 106 pathogenicity mutants. Southern analysis revealed that the average T-DNA insertion is 1.4 and that 66% of the mutants carry a single T-DNA. Using TAIL-PCR, chromosomal T-DNA flanking regions were isolated and 111 potential pathogenicity genes were identified.

Conclusions
Functional categorization of the potential pathogenicity genes indicates that certain cellular processes, such as amino acid and lipid metabolism, cell wall remodeling, protein translocation and protein degradation, seem to be important for full pathogenicity of F. oxysporum. Several known pathogenicity genes were identified, such as those encoding chitin synthase V, developmental regulator FlbA and phosphomannose isomerase. In addition, complementation and gene knock-out experiments confirmed that a glycosylphosphatidylinositol-anchored protein, thought to be involved in cell wall integrity, a transcriptional regulator, a protein with unknown function and peroxisome biogenesis are required for full pathogenicity of F. oxysporum.
Inhibition of Androgen Receptor Transcriptional Activity as a Novel Mechanism of Action of Arsenic
Authors: Rosenblatt Adena E. , Burnstein Kerry L. 
Published: January 2009

AbstractEnvironmental sodium arsenite is a toxin that is associated with male infertility due to decreased and abnormal sperm production. Arsenic trioxide (ATO), another inorganic trivalent semimetal, is an effective therapy for acute promyelocytic leukemia, and there is investigation of its possible efficacy in prostate cancer. However, the mechanism of arsenic action in male urogenital tract tissues is not clear. Because the androgen receptor (AR) plays an important role in spermatogenesis and prostate cancer, we explored the possibility that trivalent arsenic regulates AR function. We found that arsenic inhibited AR transcriptional activity in prostate cancer and Sertoli cells using reporter gene assays testing several androgen response element-containing regions and by assessing native target gene expression. Arsenic inhibition of AR activity was not due to down-regulation of AR protein levels, decreased hormone binding to AR, disruption of AR nuclear translocation, or interference with AR-DNA binding in vitro. However, chromatin immunoprecipitation studies revealed that arsenic inhibited AR recruitment to an AR target gene enhancer in vivo. Consistent with a deficiency in AR-chromatin binding, arsenic disrupted AR amino and carboxyl termini interaction. Furthermore, ATO caused a significant decrease in prostate cancer cell proliferation that was more pronounced in cells expressing AR compared with cells depleted of AR. In addition, inhibition of AR activity by ATO and by the AR antagonist, bicalutamide, was additive. Thus, arsenic-induced male infertility may be due to inhibition of AR activity. Further, because AR is an important target in prostate cancer therapy, arsenic may serve as an effective therapeutic option.
Constitutive Somatostatin Receptor Activity Determines Tonic Pituitary Cell Response
Authors: Ben-Shlomo Anat , Zhou Cuiqi , Pichurin Oxana , Chesnokova Vera , Liu Ning-Ai , Culler Michael D. , Melmed Shlomo 
Published: January 2009

AbstractSomatostatin (SRIF) binds G protein-coupled SRIF receptor subtypes (SST1, -2, -3, -4, and -5) to regulate cell secretion and proliferation. Hypothalamic SRIF inhibits pituitary growth hormone, thyroid stimulating hormone, and ACTH secretion. We tested SRIF-independent constitutive SST activity in AtT20 mouse pituitary corticotroph cells in which ACTH secretion is highly sensitive to SRIF action. Stable transfectants expressing SST2 or SST5 were sensitized to selective agonist action, and constitutive SST receptor activity was demonstrated by forskolin and pertussis toxin cAMP cell responses. Persistent constitutive SST activity decreased cell ACTH responses to CRH through decreased expression of CRH receptor subtype 1. Decreased dopamine receptor type 1 expression was associated with attenuated dopamine agonist action, whereas responses to isoproterenol were enhanced through increased β2-adrenoreceptor expression. Thus, integrated pituitary cell ACTH regulation is determined both by phasic SRIF action, as well as by tonic constitutive SST activity, independently of SRIF.
Factors associated with inappropriate inpatient prescribing of acid-suppressive therapy
Authors: Palkovic Lindsay B , Coley Kim C , Sokos Denise R 
Published: January 2009

Abstract

Objectives
Acid-suppressive therapy is used for 54–70% of inpatients, and is frequently prescribed for inappropriate indications. The objective of this study was to identify characteristics associated with inappropriate prescribing of acid-suppressive therapy.

Methods
A random sample of adult internal medicine inpatients admitted between 1 July 2005 and 30 June 2006 was screened for acid-suppressive therapy. Patients receiving such therapy without an accepted indication and those not prescribed acid-suppressive therapy were included in group 1 and group 2, respectively. Significant characteristics from separate univariate regression models were entered into a multivariate logistic regression to determine characteristics associated with inappropriate use. The setting was internal medicine units at a tertiary care academic medical centre.

Key findings
There were 108 patients in group 1 and 134 patients in group 2. Group 1 patients were older, had a longer median length of stay, a greater number of comorbidities, a greater median number of medications upon admission, and a higher rate of cirrhosis. Factors associated with use of acid-suppressive therapy without an accepted indication were use of a proton-pump inhibitor (odds ratio, 15.3; 95% confidence interval, 4.1–56.3) or histamine2 receptor antagonist (14.5; 2.8–74.8) prior to admission, cirrhosis (6.4; 1.02–39.5), use of inpatient anticoagulants (2.7; 1.4–5.2) and length of stay (1.1; 1.1-1.3).

Conclusions
The strongest factors associated with use of acid-suppressive therapy without an accepted indication were use of a proton-pump inhibitor or histamine2 receptor antagonist prior to admission, a diagnosis of cirrhosis and use of inpatient anticoagulants.
Associations of Genetic Variants in ATP-Binding Cassette A1 and Cholesteryl Ester Transfer Protein and Differences in Lipoprotein Subclasses in the Multi-Ethnic Study of Atherosclerosis
Authors: Tsai Michael Y , Li Na , Sharrett A Richey , Shea Steven , Jacobs David R , Tracy Russell , Arnett Donna , Arends Valerie , Post Wendy 
Published: January 2009

AbstractBackground: ATP-binding cassette A1 (ABCA1) and cholesteryl ester transfer protein (CETP) play important roles in the reverse cholesterol transport pathway. The associations of ABCA1 and CETP polymorphisms with lipoprotein subclasses have not been extensively studied.Methods: We genotyped 2 ABCA1 and 5 CETP polymorphisms in 999 participants of the Multi-Ethnic Study of Atherosclerosis (MESA) and studied their associations with HDL and LDL subclass particle concentrations, measured by nuclear magnetic resonance spectroscopy.Results: ABCA1 and CETP polymorphisms were associated with different and distinct changes in lipoprotein subclass concentrations. The ABCA1 1051G/A AA genotype, previously found to be associated with cardioprotective effects in this cohort, was associated with a 5.5% higher concentration of small HDL particles (P = 0.024). The CETP TaqIB B2B2, −2505C/A AA, and −629C/A AA genotypes, previously demonstrated to lack cardioprotective effects, were associated with 15.2%, 15.4%, and 11.7% higher HDL cholesterol concentrations, respectively, and 36.5%, 40.7%, and 25.4% higher large HDL particle concentrations (P &lt; 0.0001). The minor alleles of the A373P and R451Q polymorphisms were associated with lower large HDL particle concentrations.Conclusions: Our study of the influence of ABCA1 and CETP genetic variants on lipoprotein subclasses demonstrates the importance of interpreting lipoprotein subclasses within the context of the biochemical processes involved in the alterations. In the case of HDL, the study of subclass particle numbers and sizes may not be sufficiently informative. Assays for HDL function may be needed to supplement quantification of HDL cholesterol and HDL particle numbers and sizes.
Determination of Selected Geotechnical Properties of Soil Using Electrical Conductivity Testing
Authors: Sebastian Bryson L. , Bathe Abhijit 
Published: January 2009

Abstract
The use of electrical conductivity measurements, at relatively low frequencies, has been shown to be an effective tool for characterizing soils for hydrogeological studies. Many of the properties that affect the hydraulic and mechanical behavior of a soil also affect the electrical response. Thus, there is a likelihood that electrical measurements of soils will provide useful information for predicting geotechnical parameters. This paper presents the results of efforts to develop an electrical conductivity testing system that can be used specifically to evaluate geotechnical properties of soils. The testing system consisted of a robust data acquisition and control system that allowed for autonomous testing of various sand-clay mixtures and a testing apparatus that was rugged enough to allow soil samples to be compacted directly into the cell. The testing apparatus utilized a multi-electrode configuration which facilitated the investigation of anisotropic electrical measurements of the compacted soil samples. The data obtained during the evaluation phase of this research showed that low-frequency electrical conductivity measurements are viable for evaluating and predicting geotechnical properties of soils such as void ratio and volumetric water content, and the use of the multi-electrode configuration is very promising for evaluating anisotropy in soils.
Ein Verfahren zur modellbasierten On-Board Diagnose von Drei-Wege-KatalysatorenA Method for Model-based On-board Diagnosis for Three-Way Catalytic Converters
Authors: Feßler Dirk K. , Krebs Volker 
Published: January 2009

Zusammenfassung
Dieser Beitrag beschreibt ein neues modellbasiertes On-Board Diagnoseverfahren für Drei-Wege-Katalysatoren, das Information über die Wärmeenergie nutzt, die durch die im Katalysator stattfindenden, stark exothermen chemischen Reaktionen entsteht. Bei diesem Verfahren kommt ein Sigma-Punkt-Kalman-Filter zum Einsatz, das fortlaufend den aktuellen Zustand des Katalysators schätzt. Erreicht dieser einen bestimmten Grenzwert, erfolgt eine Fehlermeldung. Das Filter basiert auf einem physikalischen Modell des Drei-Wege-Katalysators und verwendet als wesentliche Messgröße die Abgastemperatur am Ausgang des Katalysators.
Brennraumdruckbasierte Extremwertregelung für direkteinspritzende OttomotorenSelf-Tuning Extremum Control Based on Cylinder Pressure Sensors for Direct Injection Spark Ignition Engines
Authors: Kohlhase Martin , Isermann Rolf 
Published: January 2009

Zusammenfassung
Es wird eine brennraumdruckbasierte Extremwert-Regelung zur Online-Optimierung der Verbrennungsschwerpunktlage bzw. des Zündwinkels hinsichtlich maximaler Drehmomentabgabe bzw. maximalem indiziertem Mitteldruck vorgestellt. Die Regelung hält zylinder-individuell — unabhängig von Fertigungstoleranzen, Alterung und Sensorfehlern — den für den jeweiligen Motorbetriebspunkt optimalen Wert von Zündzeitpunkt bzw. Verbrennungsschwerpunktlage und regelt diese unabhängig von Störungen.
Burma and Asean: Estranged Bedfellows
Authors: McCarthy Stephen 
Published: January 2009

Abstract
Burma's relations with ASEAN have changed signifi cantly since 1997. This article examines how Burma-ASEAN relations have been influenced by intense international pressure and ASEAN's desire to maintain regional credibility. As ASEAN continues to redefine its position with Burma, the generals' relations elsewhere may continue to define it for them.
India's Ambitions in Burma: More Frustration Than Success?
Authors: Egreteau Renaud 
Published: January 2009

Abstract
India has been pursuing a new Burma policy since 1993 to meet its emerging strategic interests in the region. This article assesses 15 years of this “velvet policy” toward Burma's military regime and argues that New Delhi has faced more frustration than satisfaction in achieving its strategic objectives as a result of this shift in policy.
Burma's Insatiable State
Authors: Turnell Sean 
Published: January 2009

Abstract
The bounty presently accruing to Burma from rising exports of natural gas promises to transform the country's finances. Redeeming this promise, however, will require wholesale reform of Burma's fiscal and financial arrangements. Such reforms are unlikely. This article explores the financial potential of Burma's gas exports, the danger that they could yield a “resources curse,” and the extent to which the state's fiscal demands compromise Burma's economic development.
The Lost Boys of Baltimore: Beauty and Desire in the Hood
Authors: Williams James S. 
Published: January 2009

Abstract
This article argues that despite the omnipresence of homophobia relayed linguistically and thematically in The Wire, the show also includes some remarkable instances of male homoeroticism in the way it visualizes “cinematically” its black male characters. The result is a groundbreaking exploration of the relations between race, television realism, and spectatorship.
Quality of life is reduced in patients with Klinefelter syndrome on androgen replacement therapy
Authors: de Ronde Willem , de Haan Anne , Drent Madeleine L 
Published: January 2009

ContextKlinefelter syndrome (KS) is a genetic disorder, characterized by an XXY karyotype, hypergonadotrophic hypogonadism and infertility, which affects ∼1 in 700 men. KS has also been associated with neuropsychological alterations.ObjectiveTo investigate whether quality of life (QoL) is reduced in patients with KS on androgen treatment.DesignObservational study.MethodsQuestionnaires (RAND-36) were sent to 46 KS patients on androgen treatment who regularly visit the outpatient clinic of an academic medical centre. QoL scores were compared with a Dutch male reference group.ResultsForty (87%) questionnaires were returned which were suitable for evaluation. KS patients had significantly lower (worse) scores on eight of the nine domains of the RAND questionnaire compared with the male reference group. In KS patients, higher education was associated with significantly better QoL scores.ConclusionsQoL is reduced in patients with KS on androgen treatment.
Screening for membrane hormone receptor expression in primary aldosteronism
Authors: Zwermann O , Suttmann Y , Bidlingmaier M , Beuschlein F , Reincke M 
Published: January 2009

ObjectiveThe investigation of expression and functional relevance of G-protein coupled receptors in primary aldosteronism (PA) by molecular and clinical studies.Patients and methodsTissues of 14 aldosterone-producing adenomas (APA), of one unilateral adrenal hyperplasia and of six healthy adult adrenal glands; 12 patients with confirmed PA due to APA; (n=5), idiopathic hyperaldosteronism (n=7) and 8 control subjects (C). The tissues were subjected to a quantitative PCR for determination of mRNA expression levels of AT2R1, GIPR, MC2R, GnRHR, LHR, TRHR, TSHR, glucagon-R, V1aR, V2R, and 5-HT4R. The patients and controls were enrolled in a test protocol consisting of stimulation by posture, mixed meal, ACTH, GnRH, TRH, glucagon, vasopressin, and metoclopramide (MCP). Three patients could be analyzed by both studies. A positive response was defined as an aldosterone increase of more than 50% following stimulation.ResultsAll the tissues revealed AT2R1, MC2R, AVPR, and 5-HT4R mRNA expression. LHR mRNA was found in normal adrenals and 13 adrenal tumors. By contrast with normal adrenals tumorous adrenal tissue expressed GnRHR mRNA (4/15) and TSHR mRNA (1/15). Both the patients and controls responded to posture, ACTH, glucagon, AVP, and MCP. Specific responses were seen in one patient following TRH and three patients following GnRH stimulation.ConclusionsWe provide evidence for peptide hormone responsiveness to various peptide hormones in patients with PA, including GnRH and TRH. A good correlation between clinical and molecular testing could be observed, making an involvement of the receptor expressed in PA possible.