標題: Titlebook: Journal of Inherited Metabolic Disease; R. A. Harkness,R. J. Pollitt,G. M. Addison Book 1991 Springer Science+Business Media Dordrecht 199 [打印本頁] 作者: 搭話 時間: 2025-3-21 18:50
書目名稱Journal of Inherited Metabolic Disease影響因子(影響力)
書目名稱Journal of Inherited Metabolic Disease影響因子(影響力)學科排名
書目名稱Journal of Inherited Metabolic Disease網(wǎng)絡公開度
書目名稱Journal of Inherited Metabolic Disease網(wǎng)絡公開度學科排名
書目名稱Journal of Inherited Metabolic Disease被引頻次
書目名稱Journal of Inherited Metabolic Disease被引頻次學科排名
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書目名稱Journal of Inherited Metabolic Disease年度引用學科排名
書目名稱Journal of Inherited Metabolic Disease讀者反饋
書目名稱Journal of Inherited Metabolic Disease讀者反饋學科排名
作者: 范例 時間: 2025-3-21 20:40
Detoxification Pathways in the Liver,ore water-soluble metabolites which can be efficiently eliminated from the body via the urine. This protective ability of the liver stems from the expression of a wide variety of xenobiotic biotransforming enzymes whose common underlying feature is their ability to catalyse the oxidation, reduction 作者: Pruritus 時間: 2025-3-22 03:03
Hereditary Variation of Liver Enzymes involved with Detoxification and Neurodegenerative Disease,r’s disease, Parkinson’s disease and motor neurone disease. Defects were detected in sulphur pathways and also, in the case of Parkinson’s disease, in monoamine oxidase B. The possibility exists that the ability to cope safely with endogenous and exogenous substances which have neurotoxic properties作者: Nonflammable 時間: 2025-3-22 07:18 作者: 祖?zhèn)?nbsp; 時間: 2025-3-22 12:42 作者: floaters 時間: 2025-3-22 15:15 作者: 擔憂 時間: 2025-3-22 18:42
,α1-Antitrypsin Deficiency and Liver Disease: Clinical Presentation, Diagnosis and Treatment,mmonly identified in early infancy as a conjugated hyperbilirubinaemia with hepatitis (11%) or a bleeding state due to vitamin K malabsorption (2%). 50% of cases have cirrhosis and 25% die in the first decade of life. A further 2% present with cirrhosis in later childhood. Adult males are at risk of作者: 引起 時間: 2025-3-22 22:18
,,-Antitrypsin Deficiency and Liver Disease,n children it is associated with liver disease. Produced in the liver and released into the plasma, ..AT serves as the body’s major inhibitor of neutrophil elastase, a powerful proteolytic enzyme capable of degrading extracellular structural proteins. The pathogenesis of the liver disease associated作者: 換話題 時間: 2025-3-23 02:15
Investigation of Paediatric Liver Disease,er child (over 2 years) with hepatomegaly. The approach to investigation is directed by the clinical features and employs many different investigational methods including biochemistry, haematology, radiology, electrophysiology and histology. As the clinical presentation of many diseases is similar, 作者: cruise 時間: 2025-3-23 07:31 作者: 披肩 時間: 2025-3-23 11:10
Techniques for Studying Hepatic Metabolism ,,sensitive and frequently non-specific. Compounds labelled with stable isotopes can be used to study enzyme kinetics and substrate turnover, providing more detailed and specific information. Intracellular events may be studied using nuclear magnetic resonance spectroscopy..The results using these tec作者: 舉止粗野的人 時間: 2025-3-23 16:00 作者: Yag-Capsulotomy 時間: 2025-3-23 21:52
Investigation of the Molecular Basis of the Genetic Deficiency of UDP-Glucuronosyl-transferase in Cetectable in type I livers, and low levels (9–26% of controls) of monoglucuronide conjugates only were observed in type II livers. 1-Naphthol UDPGT activity was normal in most patients, where membrane integrity was maintained by correct sample procurement and preparation. Our data on type II livers 作者: 上釉彩 時間: 2025-3-23 23:03 作者: 征服 時間: 2025-3-24 04:12 作者: BOOM 時間: 2025-3-24 10:31
Treatment of Inherited Metabolic Disorders by Liver Transplantation,this indication runs second after extrahepatic biliary atresia..The aim of liver transplantation in inherited metabolic disorders is twofold: the first is to save a patient’s life, the second is to accomplish phenotypic and functional cure of his disease. These aims may be achieved in disorders pres作者: Incompetent 時間: 2025-3-24 11:10 作者: 高調(diào) 時間: 2025-3-24 17:42
Screening and Economics,in the cost-benefit calculation. The reasons are derived from a societal preference for consumption now over consumption tomorrow, combined with the productivity of capital, which enables goods today to be transformed into more goods tomorrow..It is also necessary to put costs and values on human li作者: 新陳代謝 時間: 2025-3-24 21:14 作者: 情節(jié)劇 時間: 2025-3-25 03:01 作者: arabesque 時間: 2025-3-25 04:09
D. M. Grantlarge scale proof engineering and maintenance..In particular, the presentation will draw a picture of what these verifications mean and how they fit together into a whole. Among these are a number of firsts: the first code-level functional correctness proof of a general-purpose OS kernel, the first 作者: 箴言 時間: 2025-3-25 09:02
A. C. Williams,G. B. Steventon,S. Sturman,R. H. Waringto devote proper attention to specifications, and help maintain consistency between specification and implementation as the program evolves. The present study investigates how contracts are used in the practice of software development. Based on an extensive empirical analysis of 21 contract-equipped作者: Mortal 時間: 2025-3-25 12:33 作者: dissent 時間: 2025-3-25 17:39
W. F. Balistrerirecent years, compositional techniques have gained significant successes following a breakthrough in the ability to automate assume-guarantee reasoning. However, automation is still restricted to simple acyclic assume-guarantee rules..In this work, we focus on automating . assume-guarantee reasoning作者: 預示 時間: 2025-3-25 21:14
P. T. Claytonhe monitored systems, but recent developments in metric LTL and its extensions with aggregate operators allow some quantitative policies to be specified. Our interest in LTL-based policy languages is driven by applications in runtime Android malware detection, which requires the monitoring algorithm作者: 任命 時間: 2025-3-26 01:37
M. Hussain,G. Mieli-Vergani,A. P. Mowatsimultaneously, in a single run without generating any of the variants explicitly. They are also known as . or . analyses. The variability-aware analyses may be too costly or even infeasible for families with a large number of variants. In order to make them computationally cheaper, we can apply var作者: FAST 時間: 2025-3-26 05:15
P. Birrer,N. G. McElvaney,L. M. Chang-Stroman,R. G. Crystalnetwork consists of a set of physical components interconnected according to some reconfigurable network topology. The underlying model defines a local dynamics for each component in terms of a Differential-Algebraic Equation (DAE), and a set of network topologies by means of discrete switches. Each作者: Consequence 時間: 2025-3-26 11:35 作者: Metamorphosis 時間: 2025-3-26 15:40 作者: DEMUR 時間: 2025-3-26 19:20 作者: 粗語 時間: 2025-3-26 23:46 作者: 向下五度才偏 時間: 2025-3-27 05:10
The Place of Fetal Liver Transplantation in the Treatment of Inborn Errors of Metabolism,Enzyme levels were not significantly and durably increased in peripheral blood but the quantities of substrates detected in sera and urines were significantly reduced and tissue deposits were stabilized.作者: inclusive 時間: 2025-3-27 06:05 作者: moribund 時間: 2025-3-27 11:14 作者: 包裹 時間: 2025-3-27 14:40
Investigation of Paediatric Liver Disease,al methods including biochemistry, haematology, radiology, electrophysiology and histology. As the clinical presentation of many diseases is similar, it is appropriate to perform a variety of first-line tests, proceeding to more complex investigations only as indicated.作者: Venules 時間: 2025-3-27 19:03 作者: triptans 時間: 2025-3-28 00:43
Hereditary Variation of Liver Enzymes involved with Detoxification and Neurodegenerative Disease, is important in the pathogenesis of these diseases. Potentially such individuals could be identified preclinically and these diseases postponed by reduction in the load of toxin or modification of the relevant enzymic activity.作者: 希望 時間: 2025-3-28 04:47 作者: 就職 時間: 2025-3-28 07:48
,α1-Antitrypsin Deficiency and Liver Disease: Clinical Presentation, Diagnosis and Treatment,rability of the newborn liver are discussed in this review. A plea is made for a trial of infusions of α.-antitrypsin in early infancy, as is used safely but without proven efficacy in the emphysematous PIZZ subject. Prospects of therapy by gene modification are also reviewed.作者: 開始沒有 時間: 2025-3-28 11:38 作者: Wernickes-area 時間: 2025-3-28 16:06
Paediatric Liver Transplantation: Review of Current Experience,n to fit a child. This has allowed a greater number of children to be grafted, including emergency cases such as fulminant hepatic failure in whom there is insufficient time to wait for a size-matched donor.作者: Concerto 時間: 2025-3-28 19:07
Book 1991the Society for the Study of Inborn Errors ofMetabolism, Birmingham, UK, 1990, which was dedicated to `The Liverand Inherited Metabolic Disease‘ with a half-day workshop on`Screening and Economics‘. .The subjects covered include metabolic functions of the liver, bileacids, alpha-1-antitrypsin defici作者: 主動脈 時間: 2025-3-28 23:25 作者: Customary 時間: 2025-3-29 04:51 作者: Sinus-Node 時間: 2025-3-29 10:34 作者: troponins 時間: 2025-3-29 11:24
The Role of the Liver in Metabolic Homeostasis: Implications for Inborn Errors of Metabolism,he liver utilizes the energy generated by glucose oxidation to synthesize triglycerides. In the fasted state it utilizes that produced by .-oxidation of fatty acids to synthesize glucose. The mechanisms whereby a number of inborn errors of glycogen metabolism, of gluconeogenesis and of ketogenesis c作者: figure 時間: 2025-3-29 17:18
Detoxification Pathways in the Liver, enzymes, implying that variations in the latter may be important predisposing factors for toxicity. Pharmacogenetic defects of xenobiotic biotransformation enzymes, a subclass of inborn errors of metabolism which are manifested only upon drug challenge, introduce marked variation into human populat作者: 臆斷 時間: 2025-3-29 20:03 作者: 半球 時間: 2025-3-30 00:47 作者: antipsychotic 時間: 2025-3-30 06:26
,Tyrosinaemia Type I — an Update,ial. Presence of a ‘pseudodeficiency’ gene for FAH prevents prenatal diagnosis by enzyme analysis in some families, and this gene also precludes identification of heterozygotes outside tyrosinaemia families. Immunoblot analyses show that acute patients and some chronic patients lack immunoreactive F作者: Seizure 時間: 2025-3-30 11:36
Niemann-Pick Disease Type C: An Update,ty in relation to abnormal LDL-processing has been defined in a large patient population. Clinical presentation of the disease is also reviewed and biochemical correlations are discussed. This discovery has had immediate medical applications, by providing the first strategy for reliable prenatal dia作者: 沙發(fā) 時間: 2025-3-30 12:37
Treatment of Inherited Metabolic Disorders by Liver Transplantation,uch as hepatoma or life-threatening progression. In comparison with other indications such as extrahepatic biliary atresia, postnecrotic liver cirrhosis or acute liver failure, the results of transplantation in patients with inherited metabolic disorders seem to be better, reaching up to 78–95% actu作者: FECK 時間: 2025-3-30 19:39 作者: 燒瓶 時間: 2025-3-30 23:10 作者: 搖曳 時間: 2025-3-31 03:30
D. M. Grantte with software and code. In the second part of the talk, I will report on our experience in proof engineering methods and tools, and pose a number of research questions that we think will be important to solve for the wider scale practical application of such formal methods in industry.作者: 鉤針織物 時間: 2025-3-31 07:56
A. C. Williams,G. B. Steventon,S. Sturman,R. H. Waring of program elements that include contracts is above 33% for most projects and tends to be stable over time; there is no strong preference for a certain type of contract element; contracts are quite stable compared to implementations; and inheritance does not significantly affect qualitative trends 作者: chisel 時間: 2025-3-31 11:39
J. K. Brown,H. Imamnstraints..We implemented our approach and compared it with an established learning-based method that uses an acyclic rule. In all cases, the assumptions generated for the circular rule were significantly smaller, leading to smaller verification problems. Further, on larger examples, we obtained a s作者: Vo2-Max 時間: 2025-3-31 15:08
W. F. Balistrerinstraints..We implemented our approach and compared it with an established learning-based method that uses an acyclic rule. In all cases, the assumptions generated for the circular rule were significantly smaller, leading to smaller verification problems. Further, on larger examples, we obtained a s作者: 協(xié)迫 時間: 2025-3-31 17:42
P. T. Claytonored in a trace-length independent way without sacrificing completeness, and provide a concrete algorithm to do so. We implement and test our algorithm in an existing Android monitoring framework and show that our approach can effectively specify and enforce quantitative policies drawn from real-wor作者: 不能和解 時間: 2025-3-31 22:52
M. Hussain,G. Mieli-Vergani,A. P. Mowatare static analysis. The idea is to use a . to estimate the impact of variability-specific parts of the program family on the analysis’s precision. Then we use the pre-analysis results to find out when and where the analysis should turn off or on its variability-awareness. We demonstrate the practic作者: Bricklayer 時間: 2025-4-1 04:12
P. Birrer,N. G. McElvaney,L. M. Chang-Stroman,R. G. Crystalfication tools that can cope with ODEs but not with DAEs..Since the number of network topologies is exponential in the number of switches, first, we propose a technique based on Satisfiability Modulo Theories (SMT) that can solve the validation problem symbolically, avoiding the explicit enumeration作者: 繁榮地區(qū) 時間: 2025-4-1 07:37 作者: 防水 時間: 2025-4-1 11:46
D. Kelly,A. Greens find deep bugs more efficiently. As an exemplar of an analysis that uses danger invariants, we design a bug finding technique based on a synthesis engine. We?implemented this technique and compute danger invariants for intricate programs taken from SV-COMP 2016.作者: Scleroderma 時間: 2025-4-1 14:54
ng potential of liver transplantation as treatment for severalinborn errors of metabolism. .This state-of-the-art review will be of interest to clinicians andresearch workers alike. .978-0-7923-8982-8978-94-011-9749-6作者: Hallmark 時間: 2025-4-1 21:14
http://image.papertrans.cn/j/image/501234.jpg作者: 潰爛 時間: 2025-4-2 02:39
