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Differential diagnosis in patients with suspected bile acid synthesis defects

World Journal of Gastroenterology 2012年第10期18卷 1067-1076页

作者： Dorothea Haas Hongying Gan-schreier Claus-Dieter Langhans Tilman Rohrer Guido Engelmann Maura Heverin David W Russell Peter T Clayton Georg F Hoffmann Jürgen G okun Department of General Pediatrics Division of Inborn Metabolic Diseases University Children's Hospital Heidelberg D-69120 Germany Department of Pediatric Endocrinology and Diabetology University Hospital Saarland Homburg D-66421 Germany Department of General Pediatrics Gastroenterology University Children's Hospital Heidelberg D-69120 Germany Karolinska Institut Karolinska University Hospital Huddinge Huddinge 14186 Sweden Department of Molecular Genetics UT Southwestern Medical Center Dallas TX 75390-9046 United States Clinical and Molecular Genetics Unit Institute of Child Health London WC1N 1EH United Kingdom

AIM: To investigate the clinical presentations associated with bile acid synthesis defects and to describe identification of individual disorders and diagnostic pitfalls. METHODs: We describe semiquantitative determination of 16 urinary bile acid metabolites by electrospray ionization-tandem mass spectrometry. sample preparation was performed by solid-phase extraction. The total analysis time was 2 min per sample. We determined bile acid metabolites in 363 patients with suspected defects in bile acid metabolism. REsULTs: Abnormal bile acid metabolites were found in 36 patients. Two patients had bile acid synthesis defects but presented with atypical presentations. In 2 other patients who were later shown to be affected by biliary atresia and cystic fibrosis the profile of bile acid metabolites was initially suggestive of a bile acid synthesis defect. Three adult patients suffered from cerebrotendinous xanthomatosis. Nineteen patients had peroxisomal disorders, and 10 patients had cholestatic hepatopathy of other cause. CONCLUsION: screening for urinary cholanoids should be done in every infant with cholestatic hepatopathy as well as in children with progressive neurological disease to provide specific therapy.

关键词： Cholestatic liver disease Bile acid synthesis defects Biliary atresia Electrospray-ionization tandemmass-spectrometry

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A test on adverse selection of farmers in crop insurance:Results from Inner Mongolia,China

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Journal of Integrative Agriculture 2017年第2期16卷 478-485页

作者： ZHAO Yuan-feng CHAI Zhi-hui michael s.Delgado Paul V.Preckel College of Economics and Management Inner Mongolia Agricultural University Department of Agricultural Economics Purdue University

Adverse selection is an operating risk of crop insurance. Based on survey data on crop insurance collected by questionnaires in Inner Mongolia, China, the paper uses non-parametric analysis and econometric models to estimate the relationship between conditions for crop production and farmers＇ insurance decision in order to test the existence of farmers＇ adverse selection. The results show farmers＇ adverse selection does exist, but settling a claim by negotiation and premium subsidy from governments at all levels can defuse farmers＇ adverse selection under the current system of crop insurance. Risk regionalization, heterogeneous insurance contract and product innovation may decrease adverse selection to some extent.

关键词： crop insurance farmers adverse selection

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selective androgen receptor modulators for the treatment of late onset male hypogonadism

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Asian Journal of Andrology 2014年第2期16卷 256-261,I0009页

作者： Christopher C Coss Amanda Jones michael L Hancock Mitchell s steiner James T Dalton GTx Inc. Memphis Tennessee USA.

several testosterone preparations are used in the treatment of hypogonadism in the ageing male. These therapies differ in their convenience, flexibility, regional availability and expense but share their pharmacokinetic basis of approval and dearth of long-term safety data. The brevity and relatively reduced cost of pharmacokinetic based registration trials provides little commercial incentive to develop improved novel therapies for the treatment of late onset male hypogonadism. selective androgen receptor modulators （sARMs） have been shown to provide anabolic benefit in the absence of androgenic effects on prostate, hair and skin. Current clinical development for sARMs is focused on acute muscle wasting conditions with defined clinical endpoints of physical function and lean body mass. similar regulatory clarity concerning clinical deficits in men with hypogonadism is required before the beneficial pharmacology and desirable pharmacokinetics of sARMs can be employed in the treatment of late onset male hypogonadism.

关键词： hypogonadism late onset hypogonadism selective androgen receptor modulators testosterone

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Endocrine correlates of mate choice and promiscuity in females of a socially monogamous avian mating system with alternative male reproductive phenotypes

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Current Zoology 2014年第6期60卷 804-815页

作者： Hubert sCHWABL Willow R. LINDsAY Douglas G. BARRON michael s. WEBsTER school of Biological sciences and Center for Reproductive Biology Washington State University Pullman WA 99164-4236 USA Department of Department of Biological and Environmental sciences Goteborg University Box 463405 30 Goteborg Sweden Department of Psychology University of South Florida Tampa FL 33620-7200 USA Department of Neurobiology and Behavior and Comell Laboratory for Ornithology Comell University Ithaca NY 14850 USA

While our understanding of male reproductive strategies is informed by extensive investigations into endocrine mechanisms, the proximate mechanisms by which females compete for mates and adjust reproduction to social environment remains enigmatic. We set out to uncover endocrine correlates of mate choice, social environment, and reproductive investment in female red-backed fairy-wrens Malurus melanocephalus. In this socially monogamous, yet highly sexually promiscuous species, females experience discrete variation in the phenotype of their mates, which vary in both plumage signals and level of paternal care, and in the composition of their breeding groups, which consist of either the pair alone or with an additional cooperative auxiliary; fe- male investment varies according to these social parameters. We found that androgen, estrogen, and glucorticoid levels varied with reproductive stage, with highest androgen and estrogen concentrations during nest construction and highest corticosterone concentrations during the pre-breeding stage. These stage-dependent patterns did not vary with male phenotype or auxiliary presence, though androgen levels during pre-breeding mate selection were lower in females obtaining red/black mates than those obtaining brown mates. We found no evidence that androgen, estrogen, or corticosterone levels during the fertile period were re- lated to extra-pair young （EPY） frequency. This study demonstrates clear changes in steroid levels with reproductive stage, though it found little support for variation with social environment. We suggest hormonal responsiveness to social factors may be physiologically constrained in ways that are bypassed through exogenous hormone manipulations.

关键词： Androgen Estrogen Glucocorticoid Mate choice Promiscuity Alternative phenotypes

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Analysis of the microstructure of superconducting YBCO foams by means of AFM and EBsD

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Journal of Advanced Ceramics 2014年第4期3卷 317-325页

作者： michael Rudolf KOBLIsCHKA Anjela KOBLIsCHKA-VENEVA E.s.REDDY Gregor J.sCHMITZ Institute of Experimental Physics Saarland UniversityP.O.Box 151150D-66041 SaarbrückenGermany ACCEss Intzestrasse 552072 AachenGermany Nanonouvelle Pty Ltd. Marcoola QLD 4564Australia

YBa_(2)Cu_(3)O_(y)(YBCO)foam samples show an open,porous foam structure,which may have benefits for many applications of high-Tc *** the basic material of these foams is a pseudo-single crystalline material with the directional growth initiated by a seed crystal similar to standard melt-textured samples,the texture of YBCO is a very important ***,we analysed the local texture and grain orientation of the individual struts forming the foam by means of atomic force microscopy(AFM)and electron backscatter diffraction(EBsD).Due to the processing route starting with Y_(2)BaCuO_(5)(211),a two-phase analysis must be performed,so a high surface quality is necessary to enable an automated EBsD *** quality Kikuchi patterns were obtained from both the YBCO and 211 *** found an inhomogeneous distribution of the residual 211 particles,which are mainly randomly oriented and have sizes ranging between 200 nm and 15μ*** contrast to this,the YBCO matrix shows a dominating orientation with cracks with a typical distance of 1-10μ***,the analysis of strut cross-sections reveals that the entire strut is converted to the YBCO phase.

关键词： high-Tc superconductors foam microstructure atomic force microscopy(AFM) electron backscatter diffraction(EBsD) orientation

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Microcrack Formation During Gas Metal Arc Welding of High-strength Fine-Grained structural steel

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Acta Metallurgica sinica(English Letters) 2014年第1期27卷 140-148页

作者： Christoph Heinze Thomas michael Andreas Pittner michael Rethmeier Division 9.3 "Welding Technology" BAM Federal Institute for Materials Research and TestingUnter den Eichen 8712205 BerlinGermany Joining and Coating Technology Fraunhofer-Institute for Production Systems and Design Technology IPKPascalstraβe 8-910587 BerlinGermany

The recent development of high-performance-modified spray arc processes in gas metal arc welding due to modern digital control technology and inverter power sources enables a focused spray arc, which results in higher penetration depths and welding speed. However, rnicrocracks occurred in the weld metal while approaching the process limits of the modified spray arc, represented by a 20-mm double layer DV-groove butt-weld, These cracks were detected in structural steel exhibiting a yield strength level of up to 960 MPa and are neither dependent on the used weld power source nor a consequence of the modified spray arc process itself. The metallographic and fractographic investigations of the rather exceptional fracture surface lead to the classification of the microcracks as hot cracks, The effects of certain welding parameters on the crack probability are clarified using a statistical design of experiment. However, these microcracks do not impact the design specification for toughness in the Charpy V-notch test （absorbed energy at -40 ℃ for the present material is 30 J）.

关键词： High-strength low-alloy steel Welding Fracture Design of experiments Microcracking

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Correlation of in vivo tumor response and singlet oxygen luminescence detection in mTHPC-mediated photodynamic therapy

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Journal of Innovative Optical Health sciences 2015年第1期8卷 106-112页

作者： Brian C.Wilson michael s.Patterson Buhong Li Mark T.Jarvi Department of Medical Biophysics University of Toronto/University Health Network Toronto Ontario M5G 1L7Canada Juravinski Cancer Centre and McMaster University Hamilton Ontario L8V 5C2Canada MOE Key Laboratory of OptoElectronic science and Technology for Medicine Fujian Provincial Key Laboratory for Photonics Technology Fujian Normal University FuzhouFujian 350007P.R.China

Excited-state singlet axygen(^(1)O_(2)),generated during photodynamic therapy(PDT),is believed to be the primary cytotoxic agent with a number of clinically approved *** relative concentration in cells or tissues can be measured directly through its near-infrared(NIR)luminescence emission,which has correlated well with in vitro cell and in triro normal skin treatment ***,its correlation with the response of tumor tiss1e in vito is examined,using the photosensitizer meso-tetralydroxyphenylchlorin(mTHPC)in an animal model comprising luciferase-and green fluorescent protein(GFP)-transduced gliosarcoma grown in a dorsal window *** change in the bioluminescence signal,imaged pre-treatment and at 2,5 and 9 d post treatment,was used as a quantitative measure of the tumor response,which was classified in individual tumors as"non","moderate"and"strong"in order to reduce the variance in the *** the bioluminescence-based response vs the^(1)O_(2)counts demonstrated clear correlation,indicating tha^(1)O_(2)luminescence provides a valid do-simetric technique for PDT in tumor tissue.

关键词： Photodynamice therapy singlet axygen lumninescence dosinetry bioluminescence

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Analysis of roof and pillar failure associated with weak floor at a limestone mine

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International Journal of Mining science and Technology 2016年第3期26卷 471-476页

作者： Murphy michael M. Ellenberger John L. Esterhuizen Gabriel s. Miller Tim Office of Mine safety and Health Research National Institute of Occupational Safety and Health East Fairfield Company

A limestone mine in Ohio has had instability problems that have led to massive roof falls extending to the surface. This study focuses on the role that weak, moisture-sensitive floor has in the instability *** NIOsH research related to this subject did not include analysis for weak floor or weak bands and recommended that when such issues arise they should be investigated further using a more advanced analysis. Therefore, to further investigate the observed instability occurring on a large scale at the Ohio mine, FLAC3 D numerical models were employed to demonstrate the effect that a weak floor has on roof and pillar stability. This case study will provide important information to limestone mine operators regarding the impact of weak floor causing the potential for roof collapse, pillar failure, and subsequent subsidence of the ground surface.

关键词： Underground limestone Ground control Case study Weak floor Instability

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The Determinants of China's Minimum Wage Rates

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China & World Economy 2023年第3期31卷 59-91页

作者： Achim schmillen michael stops Dewen Wang Human Development World BankIndonesia Institute for Employment Research Germany senior social Protection Economist World BankChina.

We use a highly disaggregated panel of macro data and minimum wages at the county level to investigate the processes behind minimum wage adjustments in *** on random effects models,spatial econometrics techniques,and multilevel analyses,we document that a comparatively small number of economic variables-including the local price level and GDP per capita-are important determinants of minimum wage *** between adjacent counties and counties of the same administrative type,and centralized mechanisms,particularly at the provincial level,also play an important role in explaining the variance in minimum wage rates across ***,we show that China's provinces are the key players for setting minimum wage rates and that,when they do so,they are not uniform in the way they weigh different economic variables.

关键词： applied spatial econometrics China highly disaggregated macro data minimum wage setting multilevel analysis strategic interactions between subnationa governments

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Predictive factors associated with carcinoid syndrome in patients with gastrointestinal neuroendocrine tumors

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World Journal of Gastroenterology 2017年第40期23卷 7283-7291页

作者： Beilei Cai michael s Broder Eunice Chang Tingjian Yan David C Metz Novartis Pharmaceuticals Corporation One Health Plaza Partnership for Health Analytic Research LLC 280 S. Beverly Dr. Division of Gastroenterology University of Pennsylvania Health System 3400 Civic Center Boulevard Perelman Center for Advanced Medicine

AIM To discover unknown factors associated with carcinoid syndrome(Cs) with the goal of earlier diagnosis of *** In this retrospective case-control study using United states administrative claims, patients(≥ 18 years) newly-diagnosed with gastrointestinal neuroendocrine tumors(GI NETs) without Cs(controls) were exactly matched to patients with Cs(cases) based on NET diagnosis date at a 3-to-1 ratio. study index date was first Cs diagnosis(controls: same distance from NET diagnosis as cases). The most observed conditions, excluding Cs-associated symptoms/diagnoses, during the year before index date were assessed. Forwardstepwise logistic regression models were used to derive predictors, and were validation within another claims database. REsULTs In the development database, 1004 patients with GI NETs were identified; 251(25%) had Cs and 753(75%) were controls. In the validation database, 724 patients with GI NETs were identified; 181(25%) had Cs and 543(75%) were controls. A total of 33 common diagnoses(excluding conditions already known to be associated with Cs) in the development database were entered in forward step-wise logistic regression models. In the final, validated logistic regression model, three factors prior to Cs diagnosis were found consistently associated with higher risks for Cs, including liver disorder [odds ratio(95%CI): 3.38(2.07-5.51)], enlargement of lymph nodes [2.13(1.10-4.11)], and abdominal mass [3.79(1.87-7.69)].CONCLUsION GI NET patients with Cs were 2-4 times as likely to have preexisting diagnoses(i.e., liver disorder, enlarged lymph nodes, abdominal mass) than non-Cs patients.

关键词： Carcinoid syndrome Gastrointestinal neuroendocrine tumors Predictive factors Data mining

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