標題: Titlebook: Organic Acidurias; Proceedings of the 2 G. M. Addison,R. A. Chalmers,R. J. Pollitt Conference proceedings 1984 SSIEM and MTP Press Limited [打印本頁] 作者: intern 時間: 2025-3-21 19:22
書目名稱Organic Acidurias影響因子(影響力)
書目名稱Organic Acidurias影響因子(影響力)學科排名
書目名稱Organic Acidurias網(wǎng)絡公開度
書目名稱Organic Acidurias網(wǎng)絡公開度學科排名
書目名稱Organic Acidurias被引頻次
書目名稱Organic Acidurias被引頻次學科排名
書目名稱Organic Acidurias年度引用
書目名稱Organic Acidurias年度引用學科排名
書目名稱Organic Acidurias讀者反饋
書目名稱Organic Acidurias讀者反饋學科排名
作者: Infirm 時間: 2025-3-21 23:54
G. M. Addison,R. A. Chalmers,R. J. Pollitt,P. Divry,R. A. Harknessost enduring in popularity. Although it is over a hundred and twenty years since its first appearance, it remains a best-seller in both the United States and in Britain. The original novel is available in both hardcover and paperback editions on the permanent classics list of mainstream publishing h作者: chastise 時間: 2025-3-22 04:19
J. M. Saudubray,H. Ogier,C. Charpentier,E. Depondt,F. X. Coudé,A. Munnich,G. Mitchell,F. Rey,J. Rey,tributor to the genre of domestic narrative. . (1872), the first of her five family sagas which she wrote between 1872 and 1886, has much in common with . and the later chronicles of the March family. Essentially episodic in structure, it depicts the everyday activities of the six Carr children — th作者: interrogate 時間: 2025-3-22 05:13
R. Rousson,P. Guibaudtributor to the genre of domestic narrative. . (1872), the first of her five family sagas which she wrote between 1872 and 1886, has much in common with . and the later chronicles of the March family. Essentially episodic in structure, it depicts the everyday activities of the six Carr children — th作者: Substitution 時間: 2025-3-22 12:12 作者: Dorsal 時間: 2025-3-22 16:44
L. Sweetmanese visits are couched in the language of those late-Victorian explorers of the London slums for whom such visits were forays into an alien region (Keating, 1976, p. 14; Walkowitz, 1992, p. 19). According to Muriel ‘the houses seemed very queer to me’ and ‘they used to fascinate me’. Muriel’s commen作者: 缺乏 時間: 2025-3-22 20:39
N. J. Brandtese visits are couched in the language of those late-Victorian explorers of the London slums for whom such visits were forays into an alien region (Keating, 1976, p. 14; Walkowitz, 1992, p. 19). According to Muriel ‘the houses seemed very queer to me’ and ‘they used to fascinate me’. Muriel’s commen作者: Coronary 時間: 2025-3-22 21:28
N. Gregersenese visits are couched in the language of those late-Victorian explorers of the London slums for whom such visits were forays into an alien region (Keating, 1976, p. 14; Walkowitz, 1992, p. 19). According to Muriel ‘the houses seemed very queer to me’ and ‘they used to fascinate me’. Muriel’s commen作者: BLOT 時間: 2025-3-23 01:33
M. Duran,J. B. C. De Klerk,S. K. Wadman,L. Bruinvis,D. Ketting live births. Thus for some women at least, childbirth is now easier and safer than it has ever been in human history. However for others it remains extremely dangerous. More than half a million still die from pregnancy-related causes each year, almost all of them in the third world, where 86 per ce作者: 偽造 時間: 2025-3-23 08:42 作者: Acquired 時間: 2025-3-23 13:45
J. B. Clark,D. J. Hayes,J. A. Morgan-Hughes,E. Byrne live births. Thus for some women at least, childbirth is now easier and safer than it has ever been in human history. However for others it remains extremely dangerous. More than half a million still die from pregnancy-related causes each year, almost all of them in the third world, where 86 per ce作者: 冰雹 時間: 2025-3-23 16:16 作者: 珊瑚 時間: 2025-3-23 20:03
Prefaceed by the Society in 1972 (the 9th Annual SSIEM Symposium). Although relatively few of these disorders were recognized at that time, the symposium was prompted by the then recent identification between 1966 and 1970 of isovaleric acidaemia, methylmalonic aciduria, propionic acidaemia, pyroglutamic a作者: Allure 時間: 2025-3-24 02:12 作者: harrow 時間: 2025-3-24 03:46
Long Term Outcome of Organic Acidurias: Survey of 105 French Cases (1967–1983) acidaemia, 24 methylmalonic aciduria and some rare allied disorders) is reported. Main conclusions drawn from this survey are the poor overall prognosis and the slow improvement in the outcome of such disorders over the last 15 years. In MSUD, while early diagnosis and early management remain a bas作者: 蜈蚣 時間: 2025-3-24 09:20
The Management and Long Term Outcome of Organic Acidaemias non-cofactor-responsive variants of methylmalonic acidaemia (eight patients), propionic acidaemia (eight patients) and isolated 3-methylcrotonyl CoA carboxylase deficiency (three patients). Their survival, growth, intellectual development and other clinical problems are analysed. With the exception作者: Ingrained 時間: 2025-3-24 14:05
Prenatal Diagnosis of the Organic Aciduriasy. The two major approaches to prenatal diagnosis are the assay for deficient activity of the enzymes in cultured amniocytes and the measurement of increased concentrations of the organic acids in the amniotic fluid. The latter, when done by stable isotope dilution analysis, is rapid, relatively ine作者: cogent 時間: 2025-3-24 15:05 作者: Default 時間: 2025-3-24 20:38
Glutaric Acidaemia Type II (Multiple Acyl-CoA Dehydrogenation Deficiency) transfer from primary FAD-containing dehydrogenases into the respiratory chain. Two proteins are involved in this process, i.e. electron transfer flavoprotein (ETF) and ETF dehydrogenase, an iron—sulphur flavoprotein with a distinctive EPR signal. Reliable catalytic assays for these proteins are no作者: fertilizer 時間: 2025-3-25 00:01
Carnitine Metabolism and Inborn Errorssible aetiology of the primary muscle and primary systemic carnitine deficiency syndromes are considered and the various genetic defects of intermediary metabolism which can give rise to secondary carnitine deficiency are indicated.作者: Chemotherapy 時間: 2025-3-25 06:13
Gas Chromatography—Mass Spectrometry (GC—MS) Diagnosis of Two Cases of Medium Chain Acyl-CoA Dehydro spectrometry (GC—MS) showed an excretion of dicarboxylic acids (adipic suberic and sebacic acids), unsaturated dicarboxylic acids (.-octenedioic and decenedioic acids), 5-hydroxyhexanoic acid, hexanoyl-glycine and suberylglycine. Deficiency of the medium chain acyl-CoA dehydrogenase (MCAD) in fibro作者: 原來 時間: 2025-3-25 11:01
The Differential Diagnosis of Dicarboxylic Aciduriaonditions several methods of investigation have been used: (1) analysis of urinary organic acids in both native and hydrolysed samples, (2) analysis of free and esterified carnitine, the latter by means of Chromatographic separation and identification of acyl moieties, (3) analysis of plasma organic作者: 熟練 時間: 2025-3-25 15:43 作者: fender 時間: 2025-3-25 18:24
Mitochondrial Oxidative Phosphorylation and Respiratory Chain: Reviewbed. Our knowledge concerning the functioning of respiratory chain, its structure, organization and topology inside the inner membrane of mitochondria has considerably improved in recent years. A central question — how does the respiratory chain cooperate with ATP-synthetase, also embedded in the in作者: 圓桶 時間: 2025-3-25 21:49 作者: 疼死我了 時間: 2025-3-26 02:55
Lactic Acidaemias group is deficiency of the pyruvate dehydrogenase complex. Of 23 cases we have diagnosed, 18 have a deficiency in the first component of the complex, the E. decarboxylase, while the other five have multiple ?-keto acid dehydrogenase deficiency due to a defect in lipoamide dehydrogenase. In additio作者: 衣服 時間: 2025-3-26 05:01
4-Hydroxybutyric Aciduria: A New Inborn Error of Metabolism. I. Clinical Reviewies e.g. IgA deficiency in Louis Bar syndrome (McKusick 20890), elevated serum levels of phytanic acid in Refsum’s disease (McKusick 26650) or a ?-lipoproteinaemia in Bassen-Kornzweig syndrome (McKusick 20010). In 1981 we observed a Turkish boy who suffered from a non-progressive ataxia with muscula作者: DENT 時間: 2025-3-26 11:32 作者: 帶來墨水 時間: 2025-3-26 13:02
Overview: 978-94-010-8975-3978-94-009-5612-4作者: padding 時間: 2025-3-26 18:53 作者: Confess 時間: 2025-3-26 23:29
https://doi.org/10.1007/978-94-009-5612-4Alanin; Glycogen; enzymes; metabolism; muscle作者: MONY 時間: 2025-3-27 01:51 作者: Chameleon 時間: 2025-3-27 07:23
Pyruvate Carboxylase DeficiencyThe causes of congenital lactic acidaemia are outlined. Isolated pyruvate carboxylase deficiency is reviewed in detail with a report of a recent case and a discussion of the biochemical consequences. Other causes of defective pyruvate carboxylation are described, particularly the combined carboxylase defects.作者: 嘲笑 時間: 2025-3-27 12:56 作者: Nonporous 時間: 2025-3-27 13:48
Prenatal Diagnosis of the Organic Aciduriasy. The two major approaches to prenatal diagnosis are the assay for deficient activity of the enzymes in cultured amniocytes and the measurement of increased concentrations of the organic acids in the amniotic fluid. The latter, when done by stable isotope dilution analysis, is rapid, relatively inexpensive and very reliable.作者: Heterodoxy 時間: 2025-3-27 20:47
Fatty Acyl-CoA Dehydrogenase Deficiency: Enzyme Measurement and Studies on Alternative Metabolisml-CoA., general (medium chain) acyl-CoA and long chain acyl-CoA dehydrogenases. Patients with proven or indicated defects in either general (medium chain) or long chain acyl-CoA dehydrogenase have been reported.作者: 驚惶 時間: 2025-3-27 23:23 作者: 漫不經(jīng)心 時間: 2025-3-28 04:16
s exploration of juvenile behaviour isolated from direct contact with adults. Unlike Susan Warner, or even Charlotte Yonge, in whose The . (1856) the young Mays interact largely within an adult context, both Alcott and Coolidge write to a considerable extent from a child or adolescent-centred perspe作者: chemoprevention 時間: 2025-3-28 07:14
G. M. Addison,R. A. Chalmers,R. J. Pollitt,P. Divry,R. A. Harknessn an essay on the phenomenon of ., has noted how a number of twentieth-century women, as diverse in their origins and achievements as Gertrude Stein, Simone de Beauvoir and Adrienne Rich have all been prepared to admit to the impact of the novel as a formative influence on their lives.作者: endure 時間: 2025-3-28 10:39
J. M. Saudubray,H. Ogier,C. Charpentier,E. Depondt,F. X. Coudé,A. Munnich,G. Mitchell,F. Rey,J. Rey,s exploration of juvenile behaviour isolated from direct contact with adults. Unlike Susan Warner, or even Charlotte Yonge, in whose The . (1856) the young Mays interact largely within an adult context, both Alcott and Coolidge write to a considerable extent from a child or adolescent-centred perspe作者: 保守黨 時間: 2025-3-28 15:59 作者: 現(xiàn)任者 時間: 2025-3-28 18:46 作者: 事物的方面 時間: 2025-3-29 02:02 作者: 兵團 時間: 2025-3-29 04:51
N. J. Brandtisited by Muriel were a jumble of small workshops and back-to-back housing, crisscrossed by a network of canals and railways lines (Bournville Village Trust, 1941; Briggs, 1952, p. 284). In contrast, Rednal, where Muriel grew up, was a suburb, eight miles from Birmingham’s city centre at the end of 作者: 上下連貫 時間: 2025-3-29 08:06 作者: excrete 時間: 2025-3-29 14:47
M. Duran,J. B. C. De Klerk,S. K. Wadman,L. Bruinvis,D. Kettinghe past few years in many post-communist states. Maternal mortality in the newly independent states of the former Soviet Union is now twice as high as in Central and Eastern Europe and four times higher than in Western Europe (World Health Organisation, 1994).作者: 我不明白 時間: 2025-3-29 17:45
D. C. Gautheronhe past few years in many post-communist states. Maternal mortality in the newly independent states of the former Soviet Union is now twice as high as in Central and Eastern Europe and four times higher than in Western Europe (World Health Organisation, 1994).作者: 緩解 時間: 2025-3-29 23:10
J. B. Clark,D. J. Hayes,J. A. Morgan-Hughes,E. Byrnehe past few years in many post-communist states. Maternal mortality in the newly independent states of the former Soviet Union is now twice as high as in Central and Eastern Europe and four times higher than in Western Europe (World Health Organisation, 1994).作者: ironic 時間: 2025-3-30 02:14 作者: 不可思議 時間: 2025-3-30 07:40 作者: 大雨 時間: 2025-3-30 10:42
Mitochondrial Oxidative Phosphorylation and Respiratory Chain: Reviewricted to mitochondrial oxidative phosphorylation but appears to provide a general explanation to the synthesis of ATP in all transducing membranes: inner mitochondrial membrane, bacterial plasma membrane, thylakoid membranes in chloroplasts of green plants.作者: MITE 時間: 2025-3-30 14:56 作者: champaign 時間: 2025-3-30 19:54 作者: 討好女人 時間: 2025-3-30 23:07 作者: 熱情的我 時間: 2025-3-31 03:33
K. Bartlett,H. K. Ghneim,J.-H. Stirk,G. Dale,K. G. M. M. Alberti作者: DEFT 時間: 2025-3-31 08:42 作者: 言行自由 時間: 2025-3-31 11:53 作者: 討厭 時間: 2025-3-31 17:18
Long Term Outcome of Organic Acidurias: Survey of 105 French Cases (1967–1983)ic requirement, intellectual development did not improve as much as expected. In propionic and methylmalonic acidaemia modern treatment does not prevent a fatal outcome in the classical neonatal forms. It should be also emphasized that in the rare cases where a coenzyme deficiency has been demonstrated, vitamin therapy is very often ineffective .作者: 鳴叫 時間: 2025-3-31 17:55 作者: Decrepit 時間: 2025-4-1 01:14
The Differential Diagnosis of Dicarboxylic Aciduria acids, including the so-called free fatty acids, (4) a prolonged fasting test with serial measurements of the aforementioned parameters and close monitoring of the blood glucose and (5) an oral loading test with medium chain triglycerides accompanied by the same measurements as those named in item (4).
