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	题名	作者	年代	出处	被引量
1	Differential diagnosis in patients with suspected bile acid synthesis defects显示文摘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.	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	2012	World Journal of Gastroenterology2012，18，10：	2
2	基于一个大型随机试验预测稳定性心绞痛队列患者死亡、心肌梗死和卒中的危险评分显示文摘目的对需要抗心绞痛治疗并且左心室功能代偿症状稳定的心绞痛患者建立预测全因死亡、心肌梗死和导致致残性卒中的复合危险评分。设计大型多中心临床试验数据的多变量Cox回归分析。背景西欧、以色列、加拿大、澳大利亚和新西兰的心脏专科门诊患者。入选者7311例具有全部需要资料的患者被入选。主要结局的测量指标平均随访4.9年时间内所有原因的死亡、心肌梗死或者致残性卒中。结果1063例患者发生任何原因死亡或者持久性心肌梗死或者致残性卒中。这些复合终点的5年危险性在危险性最低的十分位患者为4%,而在危险性最高的十分位患者为35%。危险评分结合16项临床常规变量的降序排序是:年龄、左心室射血分数、吸烟、白细胞计数、糖尿病、随机血糖浓度、肌酐浓度、既往卒中病史、1周至少1次心绞痛发作、冠状动脉造影发现(如果能够获得)、降脂治疗、QT间期、收缩期血压≥150mmHg、抗心绞痛药物数量、陈旧性心肌梗死以及性别。把这个模型分别应用于任何原因所致的死亡、心肌梗死和卒中进行预测评估,所得的结果相似。危险评分似乎不能预测事件性质(39%的死亡、46%心肌梗死以及15%致残性卒中)或者冠状动脉造影或血管重建率(占29%的患者)。结论该危险评分对客观地决定稳定性心绞痛患者进一步治疗措施以减少重要预后事件发生是有帮助的。该危险评分同样可以用于将来的计划性试验。	Tim C Clayton Stuart J Pocock Jacobus Lubsen Zoltán Vokó Bridget-Anne Kirwan Keith A A Fox Philip A Poole-Wilson	2006	英国医学杂志中文版2006，9，1：	2
3	Oxygen isotope fractionation divalent metal carbonates显示文摘	O'Neil J R Clayton R N Mayada T K	1969	Chemistry Geophysics1969，51，：	1
4	The Impact of CEO Turnover on Equity Volatility 显示文摘	Clayton M C Hartzell J C Rosenberg J	2005	Journal of Business2005，，78：	1
5	Human tumor-de- rived exosomes selectively impair lymphocyte responses to in- terleukin-2 显示文摘	Clayton A Mitchell J P Court J	2007	Cancer Res2007，67，15：	1
6	Species differences in accumulation of nitrate pools in phytoplankton显示文摘	Dortch Q Clayton J R Thoresen S S	1984	Mar Biol1984，81，3：	1
7	Identification of transcrip- tional regulatory elements in human mitochondrial DNA by linker substitution analysis显示文摘	TOPPER J N CLAYTON D A	1989	Mol Cell Biol1989，9，3：	1
8	Effects of dewatering on the pyrolysis and gasification reactivity of victorian brown coal显示文摘	ZENG C CLAYTON S WU H HAYASHI J LI C	2007	Energy Fuels2007，21，2：	1
9	Mapping of deletion and transloeation breakpoints in 1q44 implicates the serine/threonine kinase AKT3 in postnatal microeephaly and agenesis of the corpus callosum显示文摘	Boland E Clayton - Smith J Woo VG	2007	Am J Hum Genet2007，81，2：	1
10	A randomized controlled trim of laser treatment among hirsute women with polycystic ovarysyndrome显示文摘	Clayton WJ Lipton M Elford J	2005	Br J Dermatol2005，152，5：	1
11	Hepatitis B virus transgenic SLID mouse model of chronic liver disease显示文摘	Larkin J Clayton MM Sun B	1999	Nat Med1999，5，8：	1
12	Dynamic knowledge map:Reusing expert's tacit knowledge in the AEC industry显示文摘	Woo Jeong-han Clayton Mark J Johnson Robert E	2004	Automation in Construction2004，13，10：	1
13	Petroleum generation and migration in Denver Basin显示文摘	CLAYTON J L SWELAND P J	1980	AAPG Bulletin1980，64，：	1
14	Analysis of antigen presenting derived exosomes, based on immuno-magnetic isolation and flow cytometry显示文摘	Clayton A Court J Navabi H	2001	J Immunol Methods2001，247，12：	1
15	Functional and molecular characterisation of mammary side population cells 显示文摘	Alvi A J Clayton H Joshi C	2003	Breast Cancer Res2003，5，：	1
16	Debt Investment, and Product Market Competition : A Note on the Limited Liability Effect 显示文摘	Clayton M J	2009	Journal of Banking & Finance2009，33，4：	1
17	Analysis of the role of 13 major fimbrial subunits in colonisation of the chicken intestines by Salmonella enterica serovar Enteritidis reveals a role for a novel locus 显示文摘	Clayton D J Bowen A J Hulme S D	2008	BMC Microbiol2008，8，：	1
18	Helicobacter pylori porCDAB andoorDABC genes encode distinct pyruvate:flavodoxin and 2-oxoglutarate:acceptor oxidoreductases whichmediate electron transport to NADP显示文摘	Nicky J Hughes Chris L Clayton Peter A Chalk	1998	Journal of bacteriology1998，180，5：	1
19	Time series modeling and maximum entropy显示文摘	T J Ulrych R W Clayton	1976	Phys Earth Planet Interiors1976，12，23：	1
20	Homology and structural relationship between the dimeric and monomeric circular forms of mitochondrial DNA from human leukemic leukocytes显示文摘	Clayton DA Davis RW Vinograd J	1970	J Mol Biol1970，47，2：	1

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