bluuebunny/crossref_metadata_2025_split huggingface dataset:
Diagnosis and management of glutaric aciduria type I – revised recommendations
Authors: Kölker Stefan , Christensen Ernst , Leonard James V. , Greenberg Cheryl R. , Boneh Avihu , Burlina Alberto B. , Burlina Alessandro P. , Dixon Marjorie , Duran Marinus , García Cazorla Angels , Goodman Stephen I. , Koeller David M. , Kyllerman Mårten , Mühlhausen Chris , Müller Edith , Okun Jürgen G. , Wilcken Bridget , Hoffmann Georg F. , Burgard Peter 
Published: March 2011

AbstractGlutaric aciduria type I (synonym, glutaric acidemia type I) is a rare organic aciduria. Untreated patients characteristically develop dystonia during infancy resulting in a high morbidity and mortality. The neuropathological correlate is striatal injury which results from encephalopathic crises precipitated by infectious diseases, immunizations and surgery during a finite period of brain development, or develops insidiously without clinically apparent crises. Glutaric aciduria type I is caused by inherited deficiency of glutaryl‐CoA dehydrogenase which is involved in the catabolic pathways of L‐lysine, L‐hydroxylysine and L‐tryptophan. This defect gives rise to elevated glutaric acid, 3‐hydroxyglutaric acid, glutaconic acid, and glutarylcarnitine which can be detected by gas chromatography/mass spectrometry (organic acids) or tandem mass spectrometry (acylcarnitines). Glutaric aciduria type I is included in the panel of diseases that are identified by expanded newborn screening in some countries. It has been shown that in the majority of neonatally diagnosed patients striatal injury can be prevented by combined metabolic treatment. Metabolic treatment that includes a low lysine diet, carnitine supplementation and intensified emergency treatment during acute episodes of intercurrent illness should be introduced and monitored by an experienced interdisciplinary team. However, initiation of treatment after the onset of symptoms is generally not effective in preventing permanent damage. Secondary dystonia is often difficult to treat, and the efficacy of available drugs cannot be predicted precisely in individual patients. The major aim of this revision is to re‐evaluate the previous diagnostic and therapeutic recommendations for patients with this disease and incorporate new research findings into the guideline.
Metabolic cutis laxa syndromes
Authors: Mohamed Miski , Kouwenberg Dorus , Gardeitchik Thatjana , Kornak Uwe , Wevers Ron A. , Morava Eva 
Published: March 2011

AbstractCutis laxa is a rare skin disorder characterized by wrinkled, redundant, inelastic and sagging skin due to defective synthesis of elastic fibers and other proteins of the extracellular matrix. Wrinkled, inelastic skin occurs in many cases as an acquired condition. Syndromic forms of cutis laxa, however, are caused by diverse genetic defects, mostly coding for structural extracellular matrix proteins. Surprisingly a number of metabolic disorders have been also found to be associated with inherited cutis laxa. Menkes disease was the first metabolic disease reported with old‐looking, wrinkled skin. Cutis laxa has recently been found in patients with abnormal glycosylation. The discovery of the COG7 defect in patients with wrinkled, inelastic skin was the first genetic link with the Congenital Disorders of Glycosylation (CDG). Since then several inborn errors of metabolism with cutis laxa have been described with variable severity. These include P5CS, ATP6V0A2‐CDG and PYCR1 defects. In spite of the evolving number of cutis laxa‐related diseases a large part of the cases remain genetically unsolved. In metabolic cutis laxa syndromes the clinical and laboratory features might partially overlap, however there are some distinct, discriminative features. In this review on metabolic diseases causing cutis laxa we offer a practical approach for the differential diagnosis of metabolic cutis laxa syndromes.
Antioxidant Activity and Total Phenolics of Propolis from the Basque Country (Northeastern Spain)
Authors: Bonvehí Josep Serra , Gutiérrez Arrate Lacalle 
Published: March 2011

AbstractThe purpose of this study was to evaluate the antioxidant activity (AA) of 19 propolis extracts prepared in different solvent (ethanol and propylene glycol). It was observed that all the samples tested had AA, although results varied considerably between extracts, i.e. 420–1,430 μmol Trolox/g (ABTS), 108–291 mg ascorbic acid/g (DPPH), and 1,573–4,669 μmol iron++ sulfate/g (FRAP). The ethanol may enhance the potency of the AA, and the correlation coefficient between total phenolic content (TPC) (200–340 mg/g propolis extracts) and AA was statistically significant. Total flavonoids ranged from 72 to 161 mg/g propolis extracts. The results indicate that TPC and flavonoids contributed to AA.
Fatty Acids, Tocopherols and Sterols of Cephalocroton cordofanus in Comparison with Sesame, Cotton, and Groundnut Oils
Authors: Mariod Abdalbasit , Matthäus Bertrand , Hussein Ismail H. 
Published: March 2011

AbstractCephalocroton cordofanus, a perennial much‐branched shrub, is dominant in the eastern and western states of Sudan. The seeds of C. cordofanus sesame, groundnut, and cotton were compared for their oil and protein content as well as for fatty acids, tocopherols, and sterols. Fatty acids and sterols were analyzed by GC while tocopherols were analyzed by HPLC. The oil of C. cordofanus showed low levels of saturated fatty acids in comparison with the other three oils. The other reported fatty acids of C. cordofanus were 8.60 % oleic, 17.2% linoleic, 64.2% vernolic, and 2.0% coronaric acids. Neutral lipids, glycolipids, and phospholipids of C. cordofanus oil accounted for 77.5, 14.4, and 8.1% of the total lipid fraction, respectively. The oil of C. cordofanus showed higher levels of tocopherols (113.53 mg/100 g) in comparison to sesame, groundnut, and cottonseed oils, with 64.74, 27.96, and 77.83 mg/100 g, respectively. The primary tocopherol of C. cordofanus was γ‐tocopherol (106.21 mg/100 g), which amounted to 93.8% of the total tocopherols. β‐ and δ‐tocopherol were present at levels below 5.0 mg/100 g. In comparison to sesame, groundnut, and cottonseed oils, C. cordofanus oil contains more (304.4 mg/100 g) total sterols than ground nut (294.0 mg/100 g), but less than sesame (774.9 mg/100 g) and cotton seed (492.4) oils. Due to its high level of epoxy fatty acids, C. cordofanus oil is used for industrial rather than edible applications.
POINTWISE CONVERGENCE AND SEMIGROUPS ACTING ON VECTOR-VALUED FUNCTIONS
Authors: COWLING MICHAEL G. , LEINERT MICHAEL 
Published: March 2011

AbstractA submarkovian C0 semigroup (Tt)t∈ℝ+ acting on the scale of complex-valued functions Lp(X,ℂ) extends to a semigroup of operators on the scale of vector-valued function spaces Lp(X,E), when E is a Banach space. It is known that, if f∈Lp(X,ℂ), where 1<p<∞, then Ttf→f pointwise almost everywhere. We show that the same holds when f∈Lp(X,E) .
Striatal dopamine synthesis capacity in twins discordant for schizophrenia
Authors: Shotbolt P. , Stokes P. R. , Owens S. F. , Toulopoulou T. , Picchioni M. M. , Bose S. K. , Murray R. M. , Howes O. D. 
Published: March 2011

BackgroundElevated striatal dopamine synthesis capacity is thought to be fundamental to the pathophysiology of schizophrenia and has also been reported in people at risk of psychosis. It is therefore unclear if striatal hyperdopaminergia is a vulnerability marker for schizophrenia, or a state feature related to the psychosis itself. Relatives of patients with schizophrenia are themselves at increased risk of developing the condition. In this study we examined striatal dopamine synthesis capacity in both members of twin pairs discordant for schizophrenia.MethodIn vivostriatal dopamine synthesis capacity was examined using fluorine-18-l-dihydroxyphenylalanine (18F-DOPA) positron emission tomography (PET) scans in seven twin pairs discordant for schizophrenia and in a control sample of 10 healthy control twin pairs.ResultsStriatal 18F-DOPA uptake was not elevated in the unaffected co-twins of patients with schizophrenia (p=0.65) or indeed in the twins with schizophrenia (p=0.89) compared to the control group. Levels of psychotic symptoms were low in the patients with schizophrenia who were in general stable [mean (s.d.) Positive and Negative Syndrome Scale (PANSS) total=56.8 (25.5)] whereas the unaffected co-twins were largely asymptomatic.ConclusionsStriatal dopamine synthesis capacity is not elevated in symptom-free individuals at genetic risk of schizophrenia, or in well-treated stable patients with chronic schizophrenia. These findings suggest that striatal hyperdopaminergia is not a vulnerability marker for schizophrenia.
Agouti-related protein in patients with acute and weight-restored anorexia nervosa
Authors: Merle J. V. , Haas V. , Burghardt R. , Döhler N. , Schneider N. , Lehmkuhl U. , Ehrlich S. 
Published: March 2011

BackgroundAn imbalance in appetite-regulating neuropeptides of the central nervous system has been associated with anorexia nervosa (AN), but the mechanisms of action are poorly understood. Agouti-related protein (AGRP), an orexigenic mediator of the hypothalamus, increases food intake and decreases energy expenditure in times of negative energy balance. The aim of the present study was to investigate AGRP in acute and fully weight-restored patients with AN, as well as during weight gain.MethodPlasma AGRP and leptin levels were assessed using an enzyme-linked immunosorbent assay kit in a total of 175 female participants, including 75 patients with acute AN, 37 weight-restored AN patients and 63 healthy controls. Of the patients with acute AN, 33 were reassessed after partial weight gain.ResultsIn weight-restored AN patients plasma AGRP levels were similar to those in healthy controls, whereas in patients with acute AN, AGRP was elevated. AGRP was inversely correlated with indicators of undernutrition such as body mass index and plasma leptin. In addition, AGRP levels normalized during weight gain of longitudinally assessed AN patients.ConclusionsOur results underline the significance of undernutrition and hypoleptinemia for the interpretation of peripheral AGRP concentrations. This provides support for the hypothesis that abnormal AGRP plasma levels in AN patients reflect undernutrition, rather than disease-specific traits.
Movement disorders are associated with schizotypy in unaffected siblings of patients with non-affective psychosis
Authors: Koning J. P. , Tenback D. E. , Kahn R. S. , Vollema M. G. , Cahn W. , van Harten P. N. 
Published: March 2011

BackgroundMovement disorders and schizotypy are both prevalent in unaffected siblings of patients with schizophrenia and both are associated with the risk of developing psychosis or schizophrenia. However, to date there has been no research into the association between these two vulnerability factors in persons with an increased genetic risk profile. We hypothesized that unaffected siblings of patients with non-affective psychosis have more movement disorders and schizotypy than healthy controls and that these co-occur.MethodIn a cross-sectional design we assessed the prevalence and inter-relationship of movement disorders and schizotypy in 115 unaffected siblings (mean age 27 years, 44% males) and 100 healthy controls (mean age 26 years, 51% males). Movement disorders were measured with the Abnormal Involuntary Movement Scale (AIMS), the Unified Parkinson Disease Rating Scale (UPDRS), the Barnes Akathisia Rating Scale (BARS), and one separate item for dystonia. Schizotypy was assessed with the Structured Interview for Schizotypy – Revised (SIS-R).ResultsThere were significant differences in the prevalence of movement disorders in unaffected siblings versus healthy controls (10% v. 1%, p<0.01) but not in the prevalence of schizotypy. Unaffected siblings with a movement disorder displayed significantly more positive and total schizotypy (p=0.02 and 0.03 respectively) than those without. In addition, dyskinesia correlated with positive schizotypy (r=0.51, p=0.02).ConclusionsThe association between movement disorders (dyskinesia in particular) with positive and total schizotypy in unaffected siblings suggests that certain vulnerability factors for psychosis or schizophrenia cluster in a subgroup of subjects with an increased genetic risk of developing the disease.
Olanzapine versus placebo for out-patients with anorexia nervosa
Authors: Attia E. , Kaplan A. S. , Walsh B. T. , Gershkovich M. , Yilmaz Z. , Musante D. , Wang Y. 
Published: March 2011

BackgroundAnorexia nervosa (AN) is a serious psychiatric illness associated with significant morbidity and mortality. There is little empirical support for specific treatments and new approaches are sorely needed. This two-site study aimed to determine whether olanzapine is superior to placebo in increasing body mass index (BMI) and improving psychological symptoms in out-patients with AN.MethodA total of 23 individuals with AN were randomly assigned in double-blind fashion to receive olanzapine or placebo for 8 weeks together with medication management sessions that emphasized compliance. Weight, other physical assessments and measures of psychopathology were collected.ResultsEnd-of-treatment BMI, with initial BMI as a covariate, was significantly greater in the group receiving olanzapine [F(1, 20)=6.64, p=0.018]. Psychological symptoms improved in both groups, but there were no statistically significant group differences. Of the 23 participants, 17 (74%) completed the 8-week trial. Participants tolerated the medication well with sedation being the only frequent side effect and no adverse metabolic effects were noted.ConclusionsThis small study suggests that olanzapine is generally well tolerated by, and may provide more benefit than placebo for out-patients with AN. Further study is indicated to determine whether olanzapine may affect psychological symptoms in addition to BMI.
Structural divergence of the rotary ATPases
Authors: Muench Stephen P. , Trinick John , Harrison Michael A. 
Published: March 2011

AbstractThe rotary ATPase family of membrane protein complexes may have only three members, but each one plays a fundamental role in biological energy conversion. The F1Fo-ATPase (F-ATPase) couples ATP synthesis to the electrochemical membrane potential in bacteria, mitochondria and chloroplasts, while the vacuolar H+-ATPase (V-ATPase) operates as an ATP-driven proton pump in eukaryotic membranes. In different species of archaea and bacteria, the A1Ao-ATPase (A-ATPase) can function as either an ATP synthase or an ion pump. All three of these multi-subunit complexes are rotary molecular motors, sharing a fundamentally similar mechanism in which rotational movement drives the energy conversion process. By analogy to macroscopic systems, individual subunits can be assigned to rotor, axle or stator functions. Recently, three-dimensional reconstructions from electron microscopy and single particle image processing have led to a significant step forward in understanding of the overall architecture of all three forms of these complexes and have allowed the organisation of subunits within the rotor and stator parts of the motors to be more clearly mapped out. This review describes the emerging consensus regarding the organisation of the rotor and stator components of V-, A- and F-ATPases, examining core similarities that point to a common evolutionary origin, and highlighting key differences. In particular, it discusses how newly revealed variation in the complexity of the inter-domain connections may impact on the mechanics and regulation of these molecular machines.
Persistence of the Polarization in a Fusion Process
Authors: Didelez J.-P. , Deutsch C. 
Published: March 2011

AbstractWe propose an experiment to test the persistence of the polarization in a fusion process, using a terawatt laser hitting a polarized high density (HD) target. The polarized protons and deuterons heated in the plasma induced by the laser have a significant probability to fuse producing a3He and a γ-ray or a neutron in the final state. The angular distribution of the radiated γ-rays and the change in the corresponding total cross-section are related to the polarization persistence, but the resulting signal turns out to be weak. By comparison, the neutrons are produced hadronically with a larger cross-section and it is much easier to detect them. A significant reduction of the cross-section by parallel polarization of the deuterons as well as a structured angular distribution of the emitted neutrons is reliably predicted by the theory. Therefore, it is expected that the corresponding signal on the neutron counting rate could be seen experimentally. Magnetic fields, relaxation times and possibilities of local investigations are discussed.
Enhancement of line X-ray emission from iron plasma created by laser irradiation of porous targets
Authors: Fazeli R. , Mahdieh M.H. , Tallents G.J. 
Published: March 2011

AbstractEnhancement of the line X-ray emission from iron plasma is investigated by simulating laser irradiation of both porous and solid targets. Spectral line intensities are calculated for selected lines of the iron plasma within the extreme ultra-violet lithography wavelength range 13.3–13.7 nm. The calculations show that X-ray yield in porous targets can be enhanced significantly in comparison with solid density targets. The results also show that for specified conditions of the driving laser, there are optimums conditions of the porous target in which maximum yield can be obtained.
The maritime imagination and the paradoxical mind in Old English poetry
Authors: Harbus Antonina 
Published: March 2011

AbstractThe specifically maritime imagination of Anglo-Saxon poets resolves the potentially incongruous metaphorical models of the mind in this culture as both an enclosure and a wandering entity. The dual containing and travelling aspects of the ship provide a suitable model for the embodied yet metaphysical mind, and act in conjunction with the widespread metaphor of life as a sea voyage to produce a coherent means of imagining how the mind operates in relation to the body. The Wanderer and The Seafarer illustrate how acutely this conventionalized way of representing physical and mental experience relies on the sea voyage as both the setting for and metaphorical representation of a human consciousness that is both enclosed in the body and also able to transcend the physical.
Royal wisdom and the Alfredian Context of Cynewulf and Cyneheard
Authors: Leneghan Francis 
Published: March 2011

AbstractThe often-anthologized story of Cynewulf and Cyneheard is typically regarded as the earliest example of heroic English prose, perhaps a summary of an earlier oral tale. Until recently, relatively little attention has been paid to its context within the A MS of the Anglo-Saxon Chronicle. Given this text's association with King Alfred, this article locates the tale within the broader context of Alfredian writing on the morality of rule and, in particular, royal wisdom. Rather than simply endorsing the loyalty of fighting men to their lord, the tale also warns of the dangers of royal folly and the consequences of unrighteous rule.
The Edgar poems and the poetics of failure in the Anglo-Saxon Chronicle
Authors: Smith Scott Thompson 
Published: March 2011

AbstractThe two poems dedicated to King Edgar in Anglo-Saxon Chronicle manuscripts ABC can be dated with some precision. This essay consequently reads the Edgar poems as contemporary products of two very different historical moments during the Benedictine reform. Written from a distinctly monastic perspective, the poem for 973 was one of many ideological texts and events from late in Edgar's reign committed to affirming the king's divinely-sanctioned sovereignty. The 973 poem realigns the function of verse in the Chronicle, adopting dynastic praise poetry to more ecclesiastical concerns. The poem for 975, however, breaks with the Chronicle precedent of panegyric verse and instead offers a critical complaint against the attacks on monastic landholdings in the wake of Edgar's death. The two Edgar poems can be best appreciated as historically-situated verse productions.
The Homiliary of Angers in tenth-century England
Authors: Rudolf Winfried 
Published: March 2011

AbstractLatin manuscripts used for preaching the Anglo-Saxon laity in the tenth century survive in relatively rare numbers. This paper contributes a new text to the known preaching resources from that century in identifying the Homiliary of Angers as the text preserved on the flyleaves of London, British Library, MS Sloane 280. While these fragments, made in Kent and edited here for the first time, cast new light on the importance of this plain and unadorned Latin collection for the composition of Old English temporale homilies before Ælfric, they also represent the oldest surviving manuscript evidence of the text.
Clustered lot quality assurance sampling: a tool to monitor immunization coverage rapidly during a national yellow fever and polio vaccination campaign in Cameroon, May 2009
Authors: PEZZOLI L. , TCHIO R. , DZOSSA A. D. , NDJOMO S. , TAKEU A. , ANYA B. , TICHA J. , RONVEAUX O. , LEWIS R. F. 
Published: March 2011

SUMMARYWe used the clustered lot quality assurance sampling (clustered-LQAS) technique to identify districts with low immunization coverage and guide mop-up actions during the last 4 days of a combined oral polio vaccine (OPV) and yellow fever (YF) vaccination campaign conducted in Cameroon in May 2009. We monitored 17 pre-selected districts at risk for low coverage. We designed LQAS plans to reject districts with YF vaccination coverage <90% and with OPV coverage <95%. In each lot the sample size was 50 (five clusters of 10) with decision values of 3 for assessing OPV and 7 for YF coverage. We ‘rejected’ 10 districts for low YF coverage and 14 for low OPV coverage. Hence we recommended a 2-day extension of the campaign. Clustered-LQAS proved to be useful in guiding the campaign vaccination strategy before the completion of the operations.
Host and environmental factors reducing mortality during the 1918–1919 influenza pandemic
Authors: PAYNTER S. , WARE R. S. , SHANKS G. D. 
Published: March 2011

SUMMARYMortality from influenza and pneumonia during the 1918–1919 pandemic was compared between subgroups of civilian and military populations from states in Australia and the USA. Exposures to crowded environments before and during the pandemic were used as proxies for exposure to respiratory infections. In three separate datasets, civilian mortality from influenza and pneumonia was higher in urban than rural populations. In contrast soldiers from these same urban backgrounds had significantly lower mortality than their rural counterparts. This suggests the lower mortality in rural civilians was due to the rural environment, probably due to the relative social isolation in rural areas. This is encouraging for pandemic planning, as it suggests social distancing interventions have the potential to reduce mortality in future pandemics. Soldiers recruited before 1918 had significantly lower mortality than those recruited in 1918, and this effect was separate from the protection given by urban origin to soldiers. Both these effects substantially reduced mortality in soldiers. Further research to identify the mechanisms of these separate protective effects may yield important evidence to inform pandemic planning strategies.