標(biāo)題: Titlebook: Inborn Metabolic Diseases; Diagnosis and Treatm John Fernandes,Jean-Marie Saudubray,N. R. M. Buist Book 19952nd edition Springer-Verlag Ber [打印本頁] 作者: 代表 時間: 2025-3-21 18:19
書目名稱Inborn Metabolic Diseases影響因子(影響力)
作者: 兩棲動物 時間: 2025-3-21 20:30
Disorders of Galactose Metabolismy. Thus, galactose consumption is highest in infancy, and in those who are galactose intolerant toxic effects occur early in life. Therefore, detection after birth, rapid diagnosis, and immediate therapy are mandatory.作者: 證明無罪 時間: 2025-3-22 03:29 作者: 有斑點 時間: 2025-3-22 06:37
TyrosineThree well-defined inborn errors of tyrosine catabolism are known:作者: cliche 時間: 2025-3-22 10:03
Urea Cycle DisordersFive inherited disorders of the . are now well described. These are characterised by . and disordered amino acid metabolism. The presentation is highly variable: those presenting in the newborn period usually have an overwhelming illness, but the presentation may be subtle in those who present later in childhood or adult life.作者: BIDE 時間: 2025-3-22 15:38 作者: 不可比擬 時間: 2025-3-22 18:39
http://image.papertrans.cn/i/image/463196.jpg作者: Bronchial-Tubes 時間: 2025-3-23 00:51
Disorders of Fructose Metabolismisphosphatase deficiency, sometimes also considered an inborn error of fructose metabolism, will be discussed in Chap. 8. It is manifested by the appearance of hypoglycemia and lactic acidosis during fasting and may also be life-threatening.作者: rectocele 時間: 2025-3-23 03:16 作者: fixed-joint 時間: 2025-3-23 07:26 作者: Mortar 時間: 2025-3-23 13:06
ization Study Group of the .Verein für..Sozialpolitik.. Most of his professional career has been spent atNürnberg, where he has helped to make the Economic Institute oneof the leading research centers in Industrial Organization. He hasalso been involved in various advisory activities. .The volume co作者: 針葉類的樹 時間: 2025-3-23 15:01 作者: follicle 時間: 2025-3-23 21:38
J.-M. Saudubray,H. Ogier de Baulny,C. Charpentier flexible in others. How gender intersects with management careers is highlighted most acutely among men and women who have dependent children. The collisions across work and home environments are difficult to navigate and can lead to decisions about how much time from the household is available to 作者: 神經(jīng) 時間: 2025-3-23 22:19 作者: jabber 時間: 2025-3-24 06:06
H. Ogier de Baulny,J.-M. Saudubraydly moving. Analysis ofmarket definition, contestibility and potential competition, the roleof innovation, network externalities, cost structures and marketingchannels present challenges for academics, policymakers and judgesalike. Evaluating consumer harm is problematic. Distinguishing betweenillegal conduct978-94-010-5894-0978-94-011-4407-0作者: corporate 時間: 2025-3-24 10:32 作者: INCUR 時間: 2025-3-24 11:44 作者: Juvenile 時間: 2025-3-24 16:22
G. Van den Berghen as an attempt to describe - dogenous market structures where the strategies, the expectations on the strategies of the others, and also the entry decisions are the fruit of rational behavior. In the last few decades, economic theory has put a lot of emphasis on the rational behavior in the choice 作者: pessimism 時間: 2025-3-24 19:00
C. A. Stanleyn as an attempt to describe - dogenous market structures where the strategies, the expectations on the strategies of the others, and also the entry decisions are the fruit of rational behavior. In the last few decades, economic theory has put a lot of emphasis on the rational behavior in the choice 作者: 變形 時間: 2025-3-25 03:00 作者: 百靈鳥 時間: 2025-3-25 04:22
I. Smith,D. P. Brentonromise any future prosperity, but because past practices simply can no longer hold water. In any case, neo-liberalist reforms alone will not resolve the deeper crisis of culture and value that has been the outcome of Korea’s state-guided capitalism. Korea must find within its older and more durable 作者: 開玩笑 時間: 2025-3-25 10:53
E. A. Kvittingen,P. T. Clayton,J. V. Leonardy, a central notion of communitarianism that is a modern version of virtue ethics. Next, I examine the positive and negative aspects of competition as an ideal type in economics and political philosophy with regard to trust. Lastly, I examine the relationship between trust and community from the vie作者: Lipoma 時間: 2025-3-25 14:32
J. V. Leonarde discipline of business ethics. By the end of 1970s, issues like bribery, deceptive advertising, price collusion, product safety, environmental damage were debated both in business and in the academia. Limited efforts were also made to describe how ethical decision making worked and what factors in作者: MERIT 時間: 2025-3-25 19:18
G. Andria,G. Sebastioromise any future prosperity, but because past practices simply can no longer hold water. In any case, neo-liberalist reforms alone will not resolve the deeper crisis of culture and value that has been the outcome of Korea’s state-guided capitalism. Korea must find within its older and more durable 作者: 寬容 時間: 2025-3-25 20:48
Book 19952nd editioniption of the metabolic derangements is restricted to the main pathophysiological features which provide the rationale for diagnosis and treatment. Methods to ascertain the diagnosis and diagnostic tests are listed comprehensively. As in the first edition, treatment is discussed extensively. Details作者: metropolitan 時間: 2025-3-26 03:26 作者: 憤憤不平 時間: 2025-3-26 07:52 作者: curriculum 時間: 2025-3-26 08:55 作者: 用手捏 時間: 2025-3-26 15:06 作者: 男學(xué)院 時間: 2025-3-26 18:01
uctures where the strategies, the expectations on the strategies of the others, and also the entry decisions are the fruit of rational behavior. In the last few decades, economic theory has put a lot of emphasis on the rational behavior in the choice 978-3-642-08052-4978-3-540-49601-4作者: 核心 時間: 2025-3-27 00:21 作者: ureter 時間: 2025-3-27 04:56 作者: itinerary 時間: 2025-3-27 05:21 作者: TERRA 時間: 2025-3-27 12:24 作者: 和平主義 時間: 2025-3-27 14:34 作者: 樹上結(jié)蜜糖 時間: 2025-3-27 18:58
Diagnostic Procedures: Function Tests and Postmortem Protocolnical Approach to Inherited Metabolic Diseases.” If no material is available or if the results are incomplete or ambiguous, a function test which challenges a metabolic route may provide a tentative diagnosis.作者: connoisseur 時間: 2025-3-27 23:32 作者: 籠子 時間: 2025-3-28 05:03 作者: hangdog 時間: 2025-3-28 10:00
Hyperphenylalaninaemiasuction and excretion of phenylketones and phenylamines (due to a combination of transamination, decarboxylation and deamination). The term “.” (or PKU) is often reserved, rather illogically, for more severe forms of deficiency in the enzyme phenylalanine hydroxylase in which urinary phenylketones are easy to detect by simple chemical methods.作者: Blazon 時間: 2025-3-28 11:01
Clinical Approach to Inherited Metabolic Diseasesiding to initiate sophisticated biochemical investigations. Clinical diagnosis of inborn errors of metabolism may at times be difficult. A number of generally accepted ideas contribute to this difficulty:作者: Inferior 時間: 2025-3-28 18:17
Psychosocial Care of the Child and Familysm. Although the special features of each condition dictate an individualized treatment approach, there are general issues of adaptation to the illness, styles of coping, and mechanisms of defense against anxiety that are pertinent to all families with affected children.作者: 舞蹈編排 時間: 2025-3-28 19:56
The Respiratory Chainal energy supply. For this reason, a respiratory chain deficiency can theoretically give rise to any symptom, in any organ or tissue, at any age with any mode of inheritance, due to the twofold genetic origin of respiratory enzymes (nuclear DNA and mitochondrial DNA, mtDNA).作者: 熱情贊揚 時間: 2025-3-29 00:01
Disorders of Gluconeogenesis of G-6Pase (Chap. 5) and FBPase usually spare the brain, unless it is damaged by recurrent hypoglycemia. The deficiencies of PEPCK and PC are associated with a progressive neurodegenerative disorder which is usually fatal.作者: OREX 時間: 2025-3-29 04:13 作者: Resistance 時間: 2025-3-29 09:42
nna (1997) suggests that, ‘Every woman entering the business world soon finds that, contrary to her academic experience, how well she performs is only one factor in creating a future for herself. Instead, an unwritten set of rules directs her fate — a Darwinian system that weeds out those with no st作者: 誓言 時間: 2025-3-29 13:07 作者: 悅耳 時間: 2025-3-29 16:33
J. Fernandes,J.-M. Saudubraynna (1997) suggests that, ‘Every woman entering the business world soon finds that, contrary to her academic experience, how well she performs is only one factor in creating a future for herself. Instead, an unwritten set of rules directs her fate — a Darwinian system that weeds out those with no st作者: acheon 時間: 2025-3-29 19:58
H. Ogier de Baulny,J.-M. Saudubraygainst Microsoft, and it is the question this volume isdesigned to answer. .America‘s antitrust laws were born out of the Industrial Revolution.Opponents of the antitrust laws argue that whatever merit theantitrust laws may have had in the past they have no place in adigital economy. Rapid innovatio作者: cognizant 時間: 2025-3-30 01:28 作者: violate 時間: 2025-3-30 06:48
Stackelberg, “Market and Equilibrium”, which contained pathbreaking studies on oligopolistic markets. In particular, it analyzed the behavior of a ?rm acting as a leader with a ?rst mover advantage in the choice of its production level over another ?rm acting as a follower. That analysis became the作者: 雜色 時間: 2025-3-30 11:09 作者: 排名真古怪 時間: 2025-3-30 12:30 作者: 躲債 時間: 2025-3-30 20:28
G. Van den Berghe. In particular, it analyzed the behavior of a ?rm acting as a leader with a ?rst mover advantage in the choice of its production level over another ?rm acting as a follower. That analysis became the foundation of the economic theory of market leaders and is the starting point of my book. In the fol
