作者: white-matter 時(shí)間: 2025-3-21 23:29 作者: 預(yù)測(cè) 時(shí)間: 2025-3-22 02:01
Springer-Verlag GmbH Germany 2016作者: ALTER 時(shí)間: 2025-3-22 05:30 作者: mortgage 時(shí)間: 2025-3-22 12:23
http://image.papertrans.cn/i/image/463193.jpg作者: 天賦 時(shí)間: 2025-3-22 16:52
Shamima Rahman,Johannes A. Mayrstaken regulatory policies, the clash between the early hype and the pioneering realities of the mobile Internet. But these are generalities that barely scratch the surface. The devil is in the details. And it is these details that .Competition for the Mobile Internet. addresses. .978-1-4613-4878-8978-1-4419-9290-1作者: aqueduct 時(shí)間: 2025-3-22 18:38 作者: 外來(lái) 時(shí)間: 2025-3-23 00:54
ulatory policies, the clash between the early hype and the pioneering realities of the mobile Internet. But these are generalities that barely scratch the surface. The devil is in the details. And it is these details that .Competition for the Mobile Internet. addresses. .作者: Boycott 時(shí)間: 2025-3-23 01:28
Diagnostic Proceduresr 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作者: 金絲雀 時(shí)間: 2025-3-23 07:18
Disorders of Galactose Metabolismers and can lead to the formation of nuclear cataracts and possibly also long-term developmental deficits, often without provoking acute symptoms of intolerance. Other secondary causes of impaired liver handling of galactose in the neonatal period are congenital portosystemic shunting and multiple h作者: 摘要 時(shí)間: 2025-3-23 13:05
Disorders of Glycolysis and the Pentose Phosphate Pathwayrogenase (LDH) deficiencies present with a purely myopathic syndrome characterized by exercise induced cramps and myoglobinuria. Glycerol kinase deficiency (GKD) is an X-linked disorder that is either an isolated condition presenting with hypoglycaemia and acidosis or part of a contiguous gene delet作者: 終點(diǎn) 時(shí)間: 2025-3-23 14:46 作者: Audiometry 時(shí)間: 2025-3-23 21:17
Disorders of Glucose Transportose and galactose carrier of hepatic, renal and pancreatic β-cells. Patients typically present with a combination of increased hepatic glycogen storage and generalised renal tubular dysfunction which includes severe glucosuria. Finally, . is an entity characterised by hyperelastic connective tissue 作者: fertilizer 時(shí)間: 2025-3-24 00:16 作者: 莊嚴(yán) 時(shí)間: 2025-3-24 03:33
Disorders of Ketogenesis and Ketolysisor the monocarboxylate transporter 1 (MCT1) present with episodes of ketoacidosis. This is often accompanied by dehydration and decreased consciousness. The organic acids usually show characteristic abnormalities in T2 deficiency but there are no specific findings in SCOT or MCT1 deficiencies and di作者: Infinitesimal 時(shí)間: 2025-3-24 09:04
Disorders of Oxidative Phosphorylationsubunits and assembly factors, and disorders of mitochondrial DNA maintenance, protein synthesis, cofactor biosynthesis and lipid metabolism. The complexity of underlying disease mechanisms, together with clinical, biochemical and genetic heterogeneity, creates enormous diagnostic challenges. Most m作者: immunity 時(shí)間: 2025-3-24 12:15 作者: airborne 時(shí)間: 2025-3-24 17:36
Gerard T. Berry,John Walter,Judith L. Fridovich-Keil作者: braggadocio 時(shí)間: 2025-3-24 20:14
Mirjam M.C. Wamelink,Vassili Valayannopoulos,Barbara Garavaglia作者: Absenteeism 時(shí)間: 2025-3-25 00:53
Linda de Meirleir,Angels Garcia-Cazorla,Michèle Brivet作者: transient-pain 時(shí)間: 2025-3-25 06:31
nd Small peptides, Lipid and Bile Acids; Nucleic Acid and Heme; Organelles –?Disorders affecting the synthesis and remodelling of complex lipids and fatty acid homeostasis are now included.?.978-3-662-49771-5作者: 笨拙處理 時(shí)間: 2025-3-25 09:57 作者: CHART 時(shí)間: 2025-3-25 14:51 作者: padding 時(shí)間: 2025-3-25 19:40 作者: patriot 時(shí)間: 2025-3-25 22:11
Clinical Approach to Inborn Errors of Metabolism in Pediatricsexpensive or unreliable and, as a consequence, a simple method of clinical screening is mandatory before initiating sophisticated biochemical investigations. This Chapter gives an overview of clinical clues to the diagnosis of IEM in pediatrics.作者: Comedienne 時(shí)間: 2025-3-26 02:16 作者: 哥哥噴涌而出 時(shí)間: 2025-3-26 07:09 作者: 命令變成大炮 時(shí)間: 2025-3-26 11:03 作者: 有組織 時(shí)間: 2025-3-26 12:48
Disorders of Fructose Metabolismdeficiency is also usually considered an inborn error of fructose metabolism although, strictly speaking, it is a defect of gluconeogenesis. The disorder is manifested by the appearance of hypoglycaemia and lactic acidosis (neonatally, or later during fasting or induced by fructose) and may be life-threatening作者: 聽寫 時(shí)間: 2025-3-26 19:30 作者: 蛛絲 時(shí)間: 2025-3-26 23:18 作者: 皺痕 時(shí)間: 2025-3-27 05:11 作者: 膽小鬼 時(shí)間: 2025-3-27 09:12
Inborn Errors of Metabolism in Adults: A Diagnostic Approach to Neurological and Psychiatric Presenthiatric or neurological manifestations, including atypical psychosis or depression, unexplained coma, peripheral neuropathy, cerebellar ataxia, spastic paraparesis, dementia, movement disorders and epilepsy. Physicians caring for adult patients with IEM are also involved in the management of those w作者: 填料 時(shí)間: 2025-3-27 10:07
Diagnostic Proceduresemain the gold standard for many clinical presentations (hypoglycaemia, liver disease, epilepsy, neurodevelopmental delay, movement disorders, neuro-sensorial deficit, peripheral neuropathy, etc.). If an IEM is suspected, then blood, urine and cerebrospinal fluid should be collected for the appropri作者: debacle 時(shí)間: 2025-3-27 14:32 作者: acclimate 時(shí)間: 2025-3-27 18:20 作者: 壓倒性勝利 時(shí)間: 2025-3-27 22:05
Disorders of Galactose Metabolismncy of galactose-1-phosphate uridylyltransferase (GALT). Classic galactosemia can be life threatening in infancy with multiorgan involvement; long-term developmental and other complications are also common. Partial GALT deficiency ranges from having serious consequences in the newborn period to bein作者: 沉著 時(shí)間: 2025-3-28 03:21 作者: opinionated 時(shí)間: 2025-3-28 09:44 作者: 向宇宙 時(shí)間: 2025-3-28 10:53 作者: 滴注 時(shí)間: 2025-3-28 16:54 作者: 拱形大橋 時(shí)間: 2025-3-28 21:57
Disorders of Pyruvate Metabolism and the Tricarboxylic Acid Cyclepyruvate 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 acidosis and early death to作者: PATHY 時(shí)間: 2025-3-29 00:47 作者: Ondines-curse 時(shí)間: 2025-3-29 05:57
Disorders of Ketogenesis and Ketolysisress. There are two defects of ketogenesis, 3-hydroxy-3-methylglutaryl (HMG)-CoA lyase deficiency and HMG-CoA synthase deficiency. In these, decompensation leads to encephalopathy, with vomiting and a reduced level of consciousness, often accompanied by hepatomegaly. The biochemical features – hypok作者: 朋黨派系 時(shí)間: 2025-3-29 09:43
Disorders of Oxidative Phosphorylationhigh energy requiring organs. The central and peripheral nervous systems, skeletal and cardiac muscle, eyes, ears, kidneys and liver are frequently involved. Some well-characterised mitochondrial syndromes are recognised, but many patients have overlapping features not corresponding to a specific sy作者: 偽善 時(shí)間: 2025-3-29 12:35
Creatine Deficiency Syndromesdinoacetate methyltransferase (GAMT) (MIM 601240) deficiencies), and transport [the X-linked creatine transporter (CRTR)] (MIM 300036) deficiency. CDS typically present with cerebral creatine deficiency and global developmental delay/ intellectual disability along with various neurological manifesta作者: faultfinder 時(shí)間: 2025-3-29 17:02
Andrew A. M. Morris,Ute Spiekerkoetter consequences for community structure. While my own contribu- tion ----and the bulk of this volume --- lies in mathematical analysis of the phenomenon, I have also tried to summarize the most important natural historical aspects of these communities, and have devoted much effort to relating the math作者: 影響深遠(yuǎn) 時(shí)間: 2025-3-29 22:05
Andrew A. M. Morris consequences for community structure. While my own contribu- tion ----and the bulk of this volume --- lies in mathematical analysis of the phenomenon, I have also tried to summarize the most important natural historical aspects of these communities, and have devoted much effort to relating the math作者: 和平 時(shí)間: 2025-3-30 01:29 作者: 處理 時(shí)間: 2025-3-30 04:05
Sylvia St?ckler-Ipsiroglou,Saadet Mercimek-Mahmutoglu,Gajja S. Salomonsdio frequency spectrum needed to offer so-called "Third Generation" (3G) mobile services. These services include high-speed data, mobile Internet access and entertainment such as games, music and video programs. Indeed, as voice communications are substituted by data communications, software -rather作者: Sedative 時(shí)間: 2025-3-30 08:25
ncy spectrum needed to offer so-called "Third Generation" (3G) mobile services. These services include high-speed data, mobile Internet access and entertainment such as games, music and video programs. Indeed, as voice communications are substituted by data communications, software -rather than term作者: Aboveboard 時(shí)間: 2025-3-30 15:07 作者: 無(wú)思維能力 時(shí)間: 2025-3-30 20:31
https://doi.org/10.1007/3-540-13389-0Kritisches Ph?nomen; Phenomena; Systems; Ungeordnetes System作者: nostrum 時(shí)間: 2025-3-30 23:02
https://doi.org/10.1007/978-3-319-47967-5American Comedy Films; Ludic elements in movies; Comic Hero; Roleplaying; Huzinga; Caillois; óneiros; Pragm
