標題: Titlebook: Inborn Metabolic Diseases; Diagnosis and Treatm Jean-Marie Saudubray,Georges Berghe,John H. Walter Book 20125th edition Springer-Verlag Ber [打印本頁] 作者: Polk 時間: 2025-3-21 19:04
書目名稱Inborn Metabolic Diseases影響因子(影響力)
書目名稱Inborn Metabolic Diseases影響因子(影響力)學科排名
書目名稱Inborn Metabolic Diseases網(wǎng)絡公開度
書目名稱Inborn Metabolic Diseases網(wǎng)絡公開度學科排名
書目名稱Inborn Metabolic Diseases被引頻次
書目名稱Inborn Metabolic Diseases被引頻次學科排名
書目名稱Inborn Metabolic Diseases年度引用
書目名稱Inborn Metabolic Diseases年度引用學科排名
書目名稱Inborn Metabolic Diseases讀者反饋
書目名稱Inborn Metabolic Diseases讀者反饋學科排名
作者: BRACE 時間: 2025-3-21 21:52 作者: 猛烈責罵 時間: 2025-3-22 03:19 作者: FIS 時間: 2025-3-22 06:10
Diagnostic Procedures: Functional Tests and Post-mortem Protocolt give rise to toxicity or energy deficiency. The best functional test is elicited by nature itself during episodes that cause metabolic stress, including acute infection, inadvertent fasting, or consumption of a nutrient that induces a metabolic intolerance. If an inherited metabolic disease is sus作者: Asparagus 時間: 2025-3-22 11:08
Emergency Treatmentspresentation and the management depend mainly on the pathophysiology involved. This chapter focuses on the main clinical presentations in neonates and children with those inborn errors of metabolism for which emergency treatment may be life saving and outlines the first steps of such treatment up to作者: BOON 時間: 2025-3-22 14:31
The Glycogen Storage Diseases and Related Disordersresent with hypoglycaemia, marked hepatomegaly, and retarded growth [6]. GSD I is the most severe of these four conditions, because both glycogenolysis and gluconeogenesis are impaired. Patients with GSD III have a syndrome that includes hepatopathy, myopathy and often cardiomyopathy. Unlike the oth作者: 嚴厲批評 時間: 2025-3-22 18:26 作者: glisten 時間: 2025-3-22 22:04
Disorders of Fructose Metabolism fructose in the urine after the intake of fructose-containing food. In . (HFI), fructose may provoke prompt gastrointestinal discomfort and hypoglycaemia upon ingestion, symptoms that may vary from patient to patient and depend on the ingested dose. Fructose may cause liver and kidney failure when 作者: Infect 時間: 2025-3-23 02:05 作者: 抑制 時間: 2025-3-23 06:44
Disorders of Pyruvate Metabolism and the Tricarboxylic Acid Cycleynthesis, defects in pyruvate metabolism and in the TCA cycle almost invariably affect the central nervous system. The severity and the different clinical phenotypes vary widely among patients and are not always specific, the range of manifestations extending from overwhelming neonatal lactic acidos作者: 使人入神 時間: 2025-3-23 12:15 作者: expeditious 時間: 2025-3-23 14:16 作者: Paradox 時間: 2025-3-23 20:11
Defects of the Respiratory Chain restricted to the neuromuscular system, but proceeds in all cells that contain mitochondria. Most nonneuromuscular organs and tissues are, therefore, also dependent upon mitochondrial energy supply. Owing to the twofold genetic origin of respiratory enzymes (nuclear DNA and mitochondrial [mtDNA]), 作者: 直覺沒有 時間: 2025-3-23 23:21
Creatine Deficiency Syndromesamidinotransferase (AGAT) and guanidinoacetate methyltransferase (GAMT) deficiencies, and deficiency of the X-linked creatine transporter (SLC6A8). In all these disorders the common clinical hallmark is mental retardation, speech delay and epilepsy. Additional frequent manifestations include failure作者: Exhilarate 時間: 2025-3-24 03:42
ent analysis (DEA) technique and the Boone indicator to assess efficiency and competition, respectively. Besides presenting the efficiency estimations before and after the 2008 financial crisis that allows us to examine the evolution and relevance of efficiency compared with the previous results, we作者: overhaul 時間: 2025-3-24 09:03 作者: Incompetent 時間: 2025-3-24 13:09
Jean-Marie Saudubrayof a ... We can talk of the convertibility of a monetary system in two different senses. In a weaker and less familiar sense, convertibility involves a commitment by one or more banks of issue to maintain continuously or every so often a pre-announced exchange rate between the MOE they issue and som作者: 壯觀的游行 時間: 2025-3-24 17:44
Frédéric Sedelintermediaries exist because they improve on unintermediated markets in which the ‘ultimate’ parties (such as borrowers and savers, or firms and investors) deal directly with each other without the use of any intermediary. An intermediary might be useful in a situation where investors want informati作者: 流逝 時間: 2025-3-24 22:57 作者: 防銹 時間: 2025-3-25 00:00
Guy Touati,Fanny Mochel,Daniel Rabierentry and exit decisions. Three major factors are most important in the selection process. One is the evolutionary perspective which emphasizes the firm’s ability and competence to alter their market position and hence through strong increasing returns to scale, to alter the market structure signifi作者: photopsia 時間: 2025-3-25 06:41 作者: BIPED 時間: 2025-3-25 08:17 作者: 逃避責任 時間: 2025-3-25 14:09 作者: ATP861 時間: 2025-3-25 17:40 作者: 微塵 時間: 2025-3-25 20:11
Mirjam M. C. Wamelink,Vassili Valayannopoulos,Cornelis Jakobsing strict and specific provisions on competition rules within the EU–Ukraine AA, we can observe a tendency towards the “Europeanization” of competition law due to the transposition of commitments on the implementation of competition . into Ukrainian legal order. The competition chapter of the EU–Uk作者: 嗎啡 時間: 2025-3-26 00:29 作者: addition 時間: 2025-3-26 06:33 作者: conduct 時間: 2025-3-26 10:24
René Santer,J?rg Klepperral air services regime between States, which essentially precludes a totally liberalized air transport system, has led to protectionism for the past 70 years of regulated air transport. Although there are some open skies agreements between States, they have tended to ensure bilateral reciprocity fo作者: STRIA 時間: 2025-3-26 14:53
6 645 000 — a fall of almost one-third. In the same period employment in the service sector increased by nearly three million from 11 754 000 to 14 441 000. Many have suggested that the shift these figures show of around 3 million workers from the production of goods to the provision of services is 作者: elastic 時間: 2025-3-26 20:26 作者: 流動性 時間: 2025-3-26 22:23 作者: 蒼白 時間: 2025-3-27 05:05
Andrew A.M. Morris6 645 000 — a fall of almost one-third. In the same period employment in the service sector increased by nearly three million from 11 754 000 to 14 441 000. Many have suggested that the shift these figures show of around 3 million workers from the production of goods to the provision of services is 作者: Paraplegia 時間: 2025-3-27 07:58
Arnold Munnich,Agnès R?tig,Marlène Rio6 645 000 — a fall of almost one-third. In the same period employment in the service sector increased by nearly three million from 11 754 000 to 14 441 000. Many have suggested that the shift these figures show of around 3 million workers from the production of goods to the provision of services is 作者: spondylosis 時間: 2025-3-27 13:20 作者: 卵石 時間: 2025-3-27 14:31 作者: meditation 時間: 2025-3-27 19:23
ncy and that the currency would remain convertible thereafter. Convertibility arises and then persists because the public demand it, and they demand it because of the reassurance it provides them regarding the value of their currency. An implication of this position is that real-world fiat currencie作者: GEON 時間: 2025-3-27 22:25
Jean-Marie Saudubrayral public by which they commit themselves to redeem and sell their liabilities for some medium (or media) of redemption (MOR) at an exchange rate determined by a preannounced formula.. There may be certain conditions attached to the banks’ obligation to buy and sell their liabilities (e.g., members作者: PRISE 時間: 2025-3-28 02:07 作者: cardiovascular 時間: 2025-3-28 10:20
Bridget Wilcken,Piero Rinaldo,Dietrich Maternted, and yet it has become increasingly apparent in recent years that the answers to some of the most basic issues in financial economics actually depend on it. If we do not understand why agents use the contract forms they do, then we can only have, at most, a limited understanding of firm capital 作者: 外露 時間: 2025-3-28 12:01 作者: FACET 時間: 2025-3-28 14:59
Carlo Dionisi-Vici,Hélène Ogier de Baulnyin their commitment and ability to innovate. Thus innovations in products and processes are largely endogenous to the firm through R&D investment and learning-by-doing. Thus vigorous innovation has been found to generate more competitive market structures, while innovations requiring large investmen作者: 一再煩擾 時間: 2025-3-28 19:52 作者: 有權 時間: 2025-3-29 00:10 作者: Mawkish 時間: 2025-3-29 04:51
Gerard T. Berry,John H. Waltercts. Furthermore, it discusses methodological issues of approximation of the IP legislation of third countries to EU law, including EU candidate countries, and illustrates the specifics of approximation of national legislation to EU law by focusing on certain IP subject matter.作者: 飲料 時間: 2025-3-29 10:21 作者: lethargy 時間: 2025-3-29 13:14 作者: 異端邪說2 時間: 2025-3-29 16:42 作者: adduction 時間: 2025-3-29 22:21 作者: FIG 時間: 2025-3-30 03:01
to the Oxford University Political Economy Club, ‘Do Goods and Services Differ?’ and she went on to publish her formulation of it (Hall, 1968). Margaret Hall’s question has long antecedents in the history of Political Economy. In this chapter we shall first examine critically the theoretical suppos作者: LINE 時間: 2025-3-30 06:00 作者: overbearing 時間: 2025-3-30 09:51 作者: Recess 時間: 2025-3-30 13:29
Andrew A.M. Morris to the Oxford University Political Economy Club, ‘Do Goods and Services Differ?’ and she went on to publish her formulation of it (Hall, 1968). Margaret Hall’s question has long antecedents in the history of Political Economy. In this chapter we shall first examine critically the theoretical suppos作者: 滔滔不絕地講 時間: 2025-3-30 19:58 作者: 阻礙 時間: 2025-3-30 23:00
Sylvia St?ckler-Ipsiroglu,Saadet Mercimek-Mahmutoglu,Gajja S. Salomons to the Oxford University Political Economy Club, ‘Do Goods and Services Differ?’ and she went on to publish her formulation of it (Hall, 1968). Margaret Hall’s question has long antecedents in the history of Political Economy. In this chapter we shall first examine critically the theoretical suppos作者: Spartan 時間: 2025-3-31 01:17
Book 20125th editiondiagnosis is being sought. This now includes a chapter on the clinical presentation of metabolic diseases in the older age range, using an analogous structure. If a particular diagnosis is already suspected, the reader may refer to the following sections of the book containing general chapters on di作者: intricacy 時間: 2025-3-31 08:36 作者: 萬神殿 時間: 2025-3-31 10:35
https://doi.org/10.1007/978-3-642-15720-2disorders of metabolism; inborn errors of metabolism; inherited metabolic diseases; metabolic diseases; 作者: 小故事 時間: 2025-3-31 15:50
Jean-Marie Saudubray,Georges Berghe,John H. WalterBeing up to date: Status Quo and Trends of Treatment.Standard textbook in this interdisciplinary field.Extensively revised and updated.Including errors affecting adults.Includes supplementary material作者: aerial 時間: 2025-3-31 20:38
