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標(biāo)題: Titlebook: Inherited Metabolic Diseases; A Clinical Approach Georg F. Hoffmann,Johannes Zschocke,William L. Nyh Book 2017Latest edition Springer-Verla [打印本頁]

作者: oxidation    時(shí)間: 2025-3-21 16:13
書目名稱Inherited Metabolic Diseases影響因子(影響力)




書目名稱Inherited Metabolic Diseases影響因子(影響力)學(xué)科排名




書目名稱Inherited Metabolic Diseases網(wǎng)絡(luò)公開度




書目名稱Inherited Metabolic Diseases網(wǎng)絡(luò)公開度學(xué)科排名




書目名稱Inherited Metabolic Diseases被引頻次




書目名稱Inherited Metabolic Diseases被引頻次學(xué)科排名




書目名稱Inherited Metabolic Diseases年度引用




書目名稱Inherited Metabolic Diseases年度引用學(xué)科排名




書目名稱Inherited Metabolic Diseases讀者反饋




書目名稱Inherited Metabolic Diseases讀者反饋學(xué)科排名





作者: flourish    時(shí)間: 2025-3-21 21:29
Johannes Zschocke Dr.med.habil., PhDder Bitte um Weiterleitung an den . vortragen. Trotz dieses ?Filters“ haben die Bürger zunehmend von dieser neuen Institution Gebrauch gemacht. Im Jahre 2004 erhielt der . 57.000 (zul?ssige) Petitionen, von denen er fast 80% g?nzlich oder weitgehend im Sinne der Petenten erledigen konnte..
作者: 陪審團(tuán)    時(shí)間: 2025-3-22 02:28
Georg F. Hoffmannn, z?hes Festhalten am kleinen und mittleren Unternehmertum, Dominanz des Familienbetriebes, Fehlen technischen Fortschritts und wirtschaftlicher Dynamik, überproportionalit?t der Landwirtschaft; nach 1944: grenzenlose Verschuldung des Staates, erhebliche Zerst?rungen vor allem in den Industriezentr
作者: Muffle    時(shí)間: 2025-3-22 04:58
dien anzuregen. Trotz des begrenz- ten Raums, der für ein so komplexes System wie das der V. Republik zur Verfügung stand, wodurch zahlreiche, je nach Interessenlage wichtige Einzelheiten unberücksichtigt blieben bzw. nur marginal gestreift werden konnten, habe ich mich nicht auf den engeren Be- reich des Reg978-3-663-16298-8
作者: nullify    時(shí)間: 2025-3-22 09:00

作者: CRUE    時(shí)間: 2025-3-22 15:38

作者: Condescending    時(shí)間: 2025-3-22 17:53

作者: 按時(shí)間順序    時(shí)間: 2025-3-22 22:29
Elaine Murphy worden — in bewusster Entgegenstellung zur Kennzeichnung als Demokratie. Aus diesen Gründen folgt hier die Darstellung des Parteiensystems an prominenter Stelle. Die abrupten und radikalen Ver?nderungen im italienischen Parteiensystem machen jedoch zun?chst eine Differenzierung erforderlich.
作者: Lamina    時(shí)間: 2025-3-23 02:40
William L. Nyhan,Stefan K?lker,Georg F. Hoffmann worden — in bewusster Entgegenstellung zur Kennzeichnung als Demokratie. Aus diesen Gründen folgt hier die Darstellung des Parteiensystems an prominenter Stelle. Die abrupten und radikalen Ver?nderungen im italienischen Parteiensystem machen jedoch zun?chst eine Differenzierung erforderlich.
作者: moribund    時(shí)間: 2025-3-23 06:15
William L. Nyhan,Stefan K?lker,Georg F. Hoffmannalauslese und Kontrolle politischer und sozialer Konflikte. (3) Diese Anforderungen entsprechen den “funktionellen Imperativen”, denen alle sozialen Systeme und Subsysteme in der strukturell-funktionalen Theorie Talcott Parsons gerecht werden müssen: 1. Anpassung, 2. Zielbefriedigung, 3. Integration
作者: nostrum    時(shí)間: 2025-3-23 10:03
William L. Nyhan,Stefan K?lker,Georg F. Hoffmannngssystems Japans er?rtert und hierbei von dem Zusammenhang ausgeht, der zwischen dem Verfassungsrahmen, der Funktionsweise der Verfassungsinstitutionen, den politischen Verhaltensweisen, der sozialen Struktur und der wirtschaftlichen Entwicklung des Landes besteht.
作者: tic-douloureux    時(shí)間: 2025-3-23 15:41

作者: 糾纏,纏繞    時(shí)間: 2025-3-23 21:58

作者: 有常識(shí)    時(shí)間: 2025-3-24 01:29

作者: 提煉    時(shí)間: 2025-3-24 03:06

作者: 橢圓    時(shí)間: 2025-3-24 08:38
Metabolic Emergenciesds only the routine clinical laboratory to establish acidosis or alkalosis, hyperammonemia, ketosis, hypoglycemia, or lactic acidemia, and to detect acute organ dysfunction (e.g., acute renal failure, acute liver failure, cardiomyopathy, rhabdomyolysis, or pancreatitis). Appropriate tests must be or
作者: cruise    時(shí)間: 2025-3-24 11:36
Work-Up of the Patient with Hypoglycemiaisorders of carbohydrate metabolism and ketolysis as well as the transient disorder termed ketotic hypoglycemia. Hypoketotic hypoglycemia, in the absence of hyperinsulinemia, includes most of the disorders of fatty acid oxidation.
作者: Interlocking    時(shí)間: 2025-3-24 16:45
rent treatment approaches required for individual diseases. An extensive section structured according to organ systems outlines the correct approach in the context of specific symptoms and signs. The value of e978-3-662-57008-1978-3-662-49410-3
作者: 協(xié)迫    時(shí)間: 2025-3-24 19:06

作者: crutch    時(shí)間: 2025-3-25 01:25

作者: 粗語    時(shí)間: 2025-3-25 06:09
Patient Care and Treatmentconsanguinity, the possibility of carrier detection, and prenatal or preimplantation diagnosis can create a severe psychosocial burden for individuals and families as a whole. This implies the need for an equally diverse multidisciplinary approach to care and treatment.
作者: debris    時(shí)間: 2025-3-25 10:15
Adolescence, Emerging Adulthood, and Problems of Transitionion process is further investigated from the viewpoint of adolescence with special health-care needs giving emphasis to the transition from young to mature adulthood. This is followed by the discussion of existing guidelines and structures outlining options and future directives with emphasis on patients with inborn errors of metabolism.
作者: debris    時(shí)間: 2025-3-25 14:52

作者: anchor    時(shí)間: 2025-3-25 18:23
Approach to the Child Suspected of Having a Disorder of Fatty Acid Oxidation begin with assay of the acylcarnitines by MS/MS, and this may indicate the diagnosis and the appropriate enzymatic assay. Organic acid analysis should reveal the diagnosis in those with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency.
作者: 多節(jié)    時(shí)間: 2025-3-25 22:19

作者: 不感興趣    時(shí)間: 2025-3-26 03:12

作者: hieroglyphic    時(shí)間: 2025-3-26 05:37
Disorders of the Biosynthesis and Breakdown of Complex Moleculesand others. Other relevant disease groups include disorders of the metabolism of isoprenoids and sterols, disorders of posttranslation protein modification, disorders of bile acid and bilirubin metabolism, and inherited cholestasis and porphyrias disorders of lipoprotein metabolism.
作者: obstinate    時(shí)間: 2025-3-26 12:33
Structured Communication and Behavioural Medicinel experience allow the prediction of information needs and questions of patients, facilitating the preparation and training of communication skills and material supporting information transfer. Untrained professionals are at risk to overestimate their communicative competence and should be monitored by interprofessional supervision.
作者: Cacophonous    時(shí)間: 2025-3-26 16:28
Workup of the Patient with Metabolic Acidosis and Massive Ketosisan intestinal losses or a renal tubular acidosis from other causes, the former with acidic urine and the latter with alkaline urine. Quantitative organic acid analysis by gas chromatography-mass spectrometry is essential in differential diagnosis which may be faster achieved by acylcarnitine (MS/MS) analysis.
作者: 內(nèi)行    時(shí)間: 2025-3-26 19:25
Guidelines and Follow-Upt plan and follow-up is made by a healthcare professional or an interdisciplinary team of experts. This process includes the patient and his/her family; different options should be discussed with them and opportunities for individualization of treatment and follow-up considered.
作者: 逢迎春日    時(shí)間: 2025-3-27 00:39

作者: Mammal    時(shí)間: 2025-3-27 03:45
978-3-662-57008-1Springer-Verlag Berlin Heidelberg 2017
作者: AROMA    時(shí)間: 2025-3-27 08:38
https://doi.org/10.1007/978-3-662-49410-3DNA; Dietary Therapy; Enzyme Replacement Therapy; Gene Therapy; Inborn Errors of Metabolism; Metabolic Em
作者: 思想    時(shí)間: 2025-3-27 10:07

作者: freight    時(shí)間: 2025-3-27 16:23
http://image.papertrans.cn/i/image/466463.jpg
作者: STIT    時(shí)間: 2025-3-27 19:18
When to Suspect Metabolic DiseaseThe spectrum of clinical presentations of metabolic disease is quite broad. Clues leading to an investigation by the clinician come from the history and physical examination, as well as from initial laboratory results. Acidosis, alkalosis, and hyperammonemia are alerting signals. Changes in the color of a diaper may be key.
作者: cardiac-arrest    時(shí)間: 2025-3-28 00:54
Patient and Family AssociationsA voluntary organisation can provide many support services to patients and families. These associations are normally a registered charity or not for profit organisation, and the primary criterion is that it exists solely and exclusively for charitable purposes. Some of the services provided are outlined below.
作者: 失敗主義者    時(shí)間: 2025-3-28 02:49

作者: 爵士樂    時(shí)間: 2025-3-28 07:46

作者: 離開真充足    時(shí)間: 2025-3-28 12:27

作者: 打擊    時(shí)間: 2025-3-28 15:29
hen Politikwissenschaftlern bisher nur wenig Interesse ge- funden. Au?er Klaus H?nsch‘ kurzer, informativer Landeskunde gibt es keine Monographie, die einen zusammenfassenden überblick über Frankreichs Politik, Wirtschaft und Gesellschaft seit 1958 aufzu- zeichnen versucht. Die wenigen deutschsprach
作者: 大都市    時(shí)間: 2025-3-28 20:44

作者: 奴才    時(shí)間: 2025-3-28 23:37
Steve Hanniganür, dass Indien in den n?chsten Jahren zu einem Wachstumsmotor der Weltwirtschaft werden wird. Obwohl Indien bislang noch im Schatten Chinas steht, sprechen einige Aspekte sogar dafür, dass Indien langfristig die besseren Aussichten hat. Mit Blick auf die Bereiche Wirtschaft und Politik stehen beide
作者: enmesh    時(shí)間: 2025-3-29 03:52
Ursula Pl?ckingerür, dass Indien in den n?chsten Jahren zu einem Wachstumsmotor der Weltwirtschaft werden wird. Obwohl Indien bislang noch im Schatten Chinas steht, sprechen einige Aspekte sogar dafür, dass Indien langfristig die besseren Aussichten hat. Mit Blick auf die Bereiche Wirtschaft und Politik stehen beide
作者: 愛得痛了    時(shí)間: 2025-3-29 09:08
Elaine MurphyStelle behandelt, sondern erst nach den zentralen politischen Institutionen wie Parlament und Regierung. Im Falle Italiens wird sich jedoch zeigen, dass die Parteien und spezielle Eigenheiten des Parteiensystems nicht nur die zentralen Determinanten, sondern auch Ursachen für zahlreiche Eigenheiten
作者: ELUC    時(shí)間: 2025-3-29 13:40

作者: 殘忍    時(shí)間: 2025-3-29 16:54

作者: 小木槌    時(shí)間: 2025-3-29 23:20

作者: Recessive    時(shí)間: 2025-3-30 01:42

作者: Eructation    時(shí)間: 2025-3-30 04:56

作者: thrombus    時(shí)間: 2025-3-30 11:29
Disorders of the Biosynthesis and Breakdown of Complex Moleculesd are less likely to cause acute metabolic crises. They are not usually recognized by basic metabolic analyses but require specific investigations for their diagnosis. Disorders of purine and pyrimidine metabolism cause a range of nephrological, neurological, musculoskeletal, hematological, and othe
作者: 人工制品    時(shí)間: 2025-3-30 13:58

作者: 善辯    時(shí)間: 2025-3-30 16:40
Patient Care and Treatmentrehensive understanding of the molecular basis and the pathophysiological consequences of gene defects. Continuous sympathetic company and guidance of patients and their families are essential for optimal outcome. Inherited metabolic diseases are chronic conditions that involve various different org
作者: 整潔漂亮    時(shí)間: 2025-3-30 22:11
Inherited Metabolic Diseases in the Context of Rare/Orphan Diseasestening and increasingly treatable diseases. Unchanged is the orphan nature of these disorders. In 1999 the World Health Organization (WHO) declared rare or orphan diseases as a major future health challenge followed by similar initiatives of the European Union. The prevalence was defined as less tha
作者: geriatrician    時(shí)間: 2025-3-31 02:32
Structured Communication and Behavioural Medicineication with parents and patients requires teaching skills like explanation by analogy and visualisation. Comprehensive repeated information and counselling is necessary, because treatment has to be executed lifelong by patients and families themselves mostly at home or in their everyday life enviro
作者: 恃強(qiáng)凌弱的人    時(shí)間: 2025-3-31 05:43

作者: 畏縮    時(shí)間: 2025-3-31 11:15
Adolescence, Emerging Adulthood, and Problems of Transitione needs survive into adulthood passing through adolescence and young adulthood and may live successful personal and professional lives. Unfortunately, the social and health-care structures needed for successful transition of each of these steps still lack behind with sometimes severe medical consequ
作者: Prostatism    時(shí)間: 2025-3-31 15:39

作者: 不持續(xù)就爆    時(shí)間: 2025-3-31 19:19
Metabolic Emergenciesderlying cause. The classic presentation of inborn errors of metabolism is with a free period of apparent health that may last days or even years, but then followed by an overwhelming life-threatening disease. Metabolic decompensation is often precipitated by acute infection and its attendant catabo




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