bluuebunny/crossref_metadata_2025_split huggingface dataset:
Enzyme replacement therapy improves peripheral nerve and sweat function in Fabry disease
Authors: Schiffmann Raphael , Floeter Mary Kay , Dambrosia James M. , Gupta Surya , Moore David F. , Sharabi Yehonatan , Khurana Ramesh K. , Brady Roscoe O. 
Published: November 2003

AbstractFabry disease is an X‐linked disorder caused by a deficiency of lysosomal α‐galactosidase A resulting in accumulation of α‐D‐galatosyl conjugated glycosphingolipids. Clinical manifestations include a small‐fiber neuropathy associated with debilitating pain and hypohidrosis. We report the effect of a 3‐year open‐label extension of a previously reported 6‐month placebo‐controlled enzyme replacement therapy (ERT) trial in which 26 hemizygous patients with Fabry disease received 0.2 mg/kg of α‐galactosidase A every 2 weeks. The effect of ERT on neuropathic pain scores while off pain medications, quantitative sensory testing, quantitative sudomotor axon reflex test (QSART), and thermoregulatory sweat test (TST) is reported. In the patients who crossed‐over from placebo to ERT (n = 10), mean pain‐at‐its‐worst scores on a 0–10 scale decreased (from 6.9 to 4.5). There was a significant reduction in the threshold for cold and warm sensation in the foot. At the 3‐year time‐point, pre‐ERT sweat excretion in 17 Fabry patients was 0.24 ± 0.33 μl/mm2 vs. 1.05 ± 0.81 in concurrent controls (n = 38). Sweat function improved 24–72 h post‐enzyme infusion (0.57 ± 0.71 μl/mm2) and normalized in four anhidrotic patients. TST confirmed the QSART results. We conclude that prolonged ERT in Fabry disease leads to a modest but significant improvement in the clinical manifestations of the small‐fiber neuropathy associated with this disorder. QSART may be useful to further optimize the dose and frequency of ERT. Muscle Nerve 28: 703–710, 2003
Characterization of two new dominant ClC‐1 channel mutations associated with myotonia
Authors: Grunnet Morten , Jespersen Thomas , Colding‐Jørgensen Eskild , Schwartz Marianne , Klaerke Dan A. , Vissing John , Olesen Søren‐Peter , Dunø Morten 
Published: November 2003

AbstractVoltage‐gated ClC‐1 chloride channels encoded by the CLCN1 gene have a major role in setting the membrane potential in skeletal muscle. More than 60 CLCN1 mutations have been associated with myotonia congenita. These mutations are traditionally classified as recessive (Becker's disease) or dominant (Thomsen's disease). In this study, we have electrophysiologically characterized two new dominant ClC‐1 mutations, thereby elucidating the observed phenotype in patients. The two ClC‐1 mutants M128V and E193K were identified, and the DNA was isolated from patients and subsequently expressed in Xenopus laevis oocytes for electrophysiological characterization. Both ClC‐1 mutants, M128V and E193K, showed a large rightward shift in the current–voltage relationship. In addition, the activation kinetics were slowed in the ClC‐1 M128V mutant, as compared to the wild‐type ClC‐1. Interestingly, ClC‐1 E193K revealed a change in reversal potential compared to wild‐type channels. This finding supports the notion that the E193 amino acid is an important determinant in the selectivity filter of the human ClC‐1 channel. The electrophysiological behavior of both mutants demonstrates a severe reduction in ClC‐1 channel conductance under physiologically relevant membrane potentials. These studies thereby explain the molecular background for the observed myotonia in patients. Muscle Nerve 28: 722–732, 2003
Atypical presentations of primary amyloid neuropathy
Authors: Vucic Steve , Chong Peter Siao Tick , Cros Didier 
Published: November 2003

AbstractPrimary amyloidosis (AL) may be complicated by peripheral neuropathy in 15–35% of cases. We report on four patients with atypical neurological presentations of AL neuropathy, whose diagnoses were delayed due to varied clinical presentations. The clinical presentation included painful sensory neuropathy (two patients), mononeuropathy multiplex (one patient), and primary demyelinating polyneuropathy (one patient). The latter two types of presentation have not been reported previously. The diagnosis was established by fat pad biopsy in two patients, lymph node biopsy in one, and sural nerve biopsy in one. Two patients were treated with high‐dose melphalan followed by stem cell rescue, and one was treated with oral melphalan and prednisone. All three cases experienced stabilization of neuropathic symptoms. We report these cases in order to raise awareness of the varied clinical presentation of AL neuropathy. Muscle Nerve 28: 696–702, 2003
Preserved cutaneous silent periods in severe entrapment neuropathies
Authors: Kofler Markus , Fröhlich Katrin , Saltuari Leopold 
Published: November 2003

AbstractNoxious digital nerve stimulation during isometric contraction of hand muscles leads to transient suppression of the electromyographic activity, the so‐called cutaneous silent period (CSP), which is mostly due to a spinal reflex mediated by A‐δ fibers. We investigated two patients with carpal tunnel syndrome (CTS) and two patients with ulnar entrapment at the elbow (UNE), in whom routine sensory conduction studies failed to document afferent fiber continuity across the lesion site. In three patients, motor nerve conduction studies and electromyography failed to demonstrate intact efferent fibers. Noxious stimulation of digit II elicited distinct CSPs in ulnar‐innervated hand muscles in both patients with CTS, and stimulation of digit V evoked CSPs in median‐innervated hand muscles in both UNE patients. The presence of a CSP can only be explained by preserved A‐δ fibers crossing the respective lesion site. Thus, preserved CSPs may serve to document residual nerve continuity in severe entrapment neuropathies when fast‐conducting fibers are so compromised that their continuity cannot be detected by standard electrodiagnostic techniques. Muscle Nerve 28: 711–714, 2003
Devolution and outsourcing of municipal services in Kampala city, Uganda: an early assessment
Authors: Golooba‐Mutebi Frederick 
Published: November 2003

AbstractThe late 1980s saw the beginning of wide‐ranging economic and political reforms in Africa, prompted by both external and internal pressures. Demands for political reform pushed for democratisation, including decentralisation of power and resources to lower levels of government. Alongside pressures for democratisation were those for economic liberalisation, including the rolling back of the state characterised by, among other things, reducing its role in service provision. This article looks at aspects of political and economic liberalisation in Uganda, involving devolution and outsourcing of service provision in Kampala city. It focuses on the city's experience with devolution and outsourcing of solid waste management. It shows that, pockets of resistance notwithstanding, the reforms enjoyed widespread popularity and led to many positive changes. In addition, it shows that they begot problems and encountered others that rendered the process of change more problematic than its advocates had anticipated. Its major conclusion is that while devolution and outsourcing are useful tools for improving service delivery, they cannot ensure long‐term success in the absence of financial, technical and managerial capacity on the part of contractors and contracting authorities. Copyright © 2003 John Wiley & Sons, Ltd.
Challenges for donor agency country‐level performance assessment: a review
Authors: Ireland Mark , McGregor J. Allister , Saltmarshe Douglas 
Published: November 2003

AbstractThis review of country‐level performance assessment in donor agencies is primarily based upon the experiences documented by bilateral donors to developing countries. The review suggests that four emerging themes can be identified in the literature on country‐level performance review: ownership, decentralisation and leadership, accountability and learning and complexity. The review considers the implementation of ‘results‐based’ approaches used by a number of international agencies and examines their relationship with ‘evidence‐based’ approaches. A key challenge, in the development of performance assessment, is bringing in a stronger evidence‐based approach into the planning and evaluation of donor country‐level programmes. Copyright © 2003 John Wiley & Sons, Ltd.
Quantiﬁcation of sideroxylonals in Eucalyptus foliage by high‐performance liquid chromatography
Authors: Wallis Ian R. , Herlt Anthony J. , Eschler Bart M. , Takasaki Midori , Foley William J. 
Published: November 2003

AbstractThis paper describes the extraction and quantiﬁcation of sideroxylonals, a group of formylated phloroglucinol compounds found in the foliage of some eucalypt species. Samples of dry, ground foliage were Soxhlet‐extracted with light petroleum spirit:acetone (4:1) and the resultant extract analysed (in the presence of internal standard) by reversed‐phase HPLC without further puriﬁcation. The yield of sideroxylonals was exponential with time and showed an inﬂection at ca. 4 h of extraction. It is recommended that samples be extracted for 6 h, giving a 92% recovery of the sideroxylonals. The title compounds deteriorate under various conditions, e.g. 10% are lost when foliage is oven‐dried at 40C compared to freeze‐drying. Storing samples in mobile phase led to a slow deterioration of sideroxylonals with a 7% loss after 4 days, while 22% of these compounds were lost from dry, ground eucalypt leaf stored at room temperature for 20 months. Copyright © 2003 John Wiley & Sons, Ltd.
Variation in the composition of the heartwood flavonoids of Prunus avium by on‐column capillary gas chromatography
Authors: Vinciguerra Vittorio , Luna Marcello , Bistoni Angela , Zollo Franco 
Published: November 2003

AbstractA fast and simple extraction procedure, coupled with on‐column gas chromatography, has been developed in order to investigate the relative amounts of ﬂavonoids in individual trees within several homogeneous populations of Prunus avium. Eight known ﬂavonoid aglycones were isolated from the heartwood of P. avium and multivariate analysis was employed in order to characterise population groups. Copyright © 2003 John Wiley & Sons, Ltd.
Prenatal manifestation of superior mesenteric artery syndrome
Authors: Caspi B. , Deutsch H. , Grunshpan M. , Flidel O. , Hagay Z. , Appelman Z. 
Published: November 2003

AbstractIntestinal obstruction is not a rarity in the newborn. Its etiology is diverse. Superior mesenteric artery syndrome (SMAS) is a phenomenon in which the duodenum is obstructed by the SMA. This causes bowel obstruction accompanied by duodenal dilatation. It has previously been described in adults and children but rarely in infants. We report for the first time on an intrauterine manifestation of SMAS. Copyright © 2003 John Wiley & Sons, Ltd.
Privileging Class: Toward a Critical Psychology of Social Class in the Context of Education
Authors: Ostrove Joan M. , Cole Elizabeth R. 
Published: November 2003

This issue of the Journal of Social Issues explores psychological meanings of social class in the context of education. In this article we propose an outline for a critical psychology of social class and discuss why education is a useful context for examining relations between class and individual psychology. We consider how research and theory in the study of race and gender can and cannot inform a psychology of social class. We introduce three themes that organize the issue and the articles that illustrate them. The articles in this issue address all levels of education, include data from within and outside of the United States, and investigate perspectives of individuals from a range of social class groups.
“What I remember most about school was that if you were poor you got no respect and no encouragement. I mean if you didn't have cute ringlets, an ironed new uniform, starched shirts, and a mother and father who gave money to the church, you weren't a teacher's pet and that meant you weren't encouraged.”   —a working‐class woman respondent interviewed in Luttrell, 1993

Class differences were boundaries no one wanted to face or talk about. It was easier to downplay them, to act as though we were all from privileged backgrounds, to work around them, to confront them privately in the solitude of one's room, or to pretend that just being chosen to study at such an institution meant that those of us who did not come from such privilege were already in transition toward privilege … . It was a kind of treason not to believe that it was better to be identified with the world of material privilege than with the world of the working class, the poor.            —hooks, 1989
Photogrammetric and laser altimetric reconstruction of water levels for extreme flood event analysis
Authors: Lane Stuart N. , James Timothy D. , Pritchard Hamish , Saunders Mark 
Published: November 2003

AbstractThis paper assesses the feasibility of estimating water levels using digital photogrammetry. A common problem during an extreme flood event is that automated water level recorders do not record the highest water levels, as a result of instrument malfunctioning. This paper explores two possible solutions to this problem based upon data acquired using synoptic remote sensing methods. The first method requires: (a) high‐resolution elevation data (for example, in the form of a digital elevation model for the floodplain); and (b) information on the planimetric position of the maximum flood extent, such as from debris lines (known as wrack lines) visible on aerial imagery flown after the event. The planimetric data can then be used to segment the topographic data in order to identify water level elevations. The second method uses a digitial photogrammetric approach and is suitable where no topographic data are available, but aerial imagery is available, flown after the event. Provided this imagery is of the right scale, digital photogrammetric analysis may be used to identify the elevations of wrack lines visible on the imagery. In this paper, the second of these options is compared with the first. The research shows that desktop photogrammetric methods, using 1:4500 scale imagery, can yield water level estimates that are precise to ±0·147 m, on the basis of check data obtained from lidar data. This is a worst possible estimate of the acquired precision given uncertainties in the lidar data. When compared with the first option, based upon segmenting lidar data using flood outlines, the photogrammetric approach was found to be preferable given both the quality of the lidar and uncertainties over how to segment it.
Macrofauna Communities in the Eastern Mediterranean Deep Sea
Authors: Kröncke Ingrid , Türkay Michael , Fiege Dieter 
Published: November 2003

Abstract.  During two expeditions with RV ‘Meteor’ in summer 1993 and winter 1997/98 the structural and functional diversity of the benthic system of the highly oligotrophic eastern Mediterranean deep sea was investigated. The macrofauna communities were dominated by polychaetes even at the deepest stations.The fauna at shallow stations was dominated by surface deposit feeders, whereas subsurface deposit feeders and predators generally increased with depth. A high percentage of suspension‐feeding Porifera was found in the Levantine Basin. Mean abundance and number of taxa of both expeditions were significantly correlated to depth and distance to the nearest coast as well as to the total organic carbon (TOC) content in sediments.Numbers of taxa and abundance decreased generally with depth, although lowest numbers were not found at the deepest stations but in the extremely oligotrophic Levantine and Ierapetra Basin. Biomass measured during the second cruise was extremely low in the Ierapetra Basin and comparable to other extreme oligotrophic seas.The significant correlations found for TOC contents and macrofauna with distance to coast during both expeditions apparently reflect the role of hydrographically governed transport of organic matter produced in coastal regions into greater and extreme depths of the Mediterranean Sea.Seasonal differences in macrofauna communities due to seasonal differences in food supply were not found. However, recent large‐scale hydrographic changes (Eastern Mediterranean Transient, EMT) might change the oligotrophy and, thus, the structure of the benthic communities in the Eastern Mediterranean deep sea.
Mitochondrial DNA diversity of Pakistani goats
Authors: Sultana S. , Mannen H. , Tsuji S. 
Published: November 2003

SummaryPakistan contains numerous domestic goat breeds, but until now there has been no comprehensive study on genetic diversity or a phylogenetic analysis of Pakistani goats. In this study, we analysed the complete mitochondrial DNA D‐loop and the cytochrome b gene of 13 Pakistani domestic goat breeds (Capra hircus) and one wild goat, the Sindh Ibex (Capra aegagrus blythi). The phylogenetic analyses and sequence divergence (SD) established four distinct mt‐lineages termed as A, B and C (previously reported) and a new lineage D. The Sindh Ibex appeared as an outgroup of domestic goats. The estimated divergence times between the most recently evolved mt‐lineages A and D were from 260 483 to 371 052 YA. This suggested that at least four different strains of wild Capra might have been the source of the modern domestic goats. The new mt‐lineage D revealed high SD from mt‐lineage A and may be the oldest branch under domestication, while mt‐lineages B and C showed lower SD and might have been domesticated during an advanced stage of the domestication process.
A comparison of deep vs. awake removal of the laryngeal mask airway in paediatric dental daycase surgery. A randomised controlled trial
Authors: Dolling S. , Anders N. R. K. , Rolfe S. E. 
Published: November 2003

SummaryDental anaesthesia provides a potential conflict between anaesthetist and surgeon because of the shared airway. The laryngeal mask airway (LMA) has helped to improve airway control for these procedures, but there is little evidence for best practice on the timing of their removal after airway surgery in the paediatric population. We compared ‘awake’ and ‘deep’ removal of the LMA in 196 patients aged from 2 to 15 years in a randomised, controlled study. We found that average peripheral oxygen saturation (SpO2) was lower in the deep group and this was statistically significant (96.2% vs. 94.9%, p = 0.04). It was also found that the deep group had a higher incidence of patients with SpO2 < 95% (p = 0.003) and of patients who coughed (p = 0.003). We conclude that the LMA should be taken out awake in these patients.
Life history consequences for Daphnia pulex feeding on nutrient‐limited phytoplankton
Authors: LÜRLING M. , VAN DONK E. 
Published: November 2003

1. The growth and feeding of Daphnia pulex De Geer on different algal species was examined. The green algae Chlamydomonas reinhardtii Dangeard and Scenedesmus acutus Meyen, the diatom Synedra tenuissima Kützing, the cryptophyte Cryptomonas pyrenoidifera Geitler and the cyanobacterium Microcystis aeruginosa Kützing were cultured in non‐limiting and in N‐ or P‐limiting medium and used as food for D. pulex.2. Growth limitations were reflected in the elemental and biochemical composition and the morphological characteristics of the algal resources.3. The clearance rates of D. pulex feeding on nutrient‐limited algae were reduced. This was not observed when nutrient‐limited mutant Chlamydomonas cells without cell walls were used as food, indicating that the cell wall may play an important part.4. In comparison with animals grown on nutrient‐sufficient cells, nutrient‐limited algae resulted in smaller body length, reduced brood sizes, reduced size at maturity, increased age at first reproduction and, consequently, in reduced Daphnia population growth rates.5. Daphnia population growth rates (r) were negatively correlated with the C : P ratio and the carbohydrate content of the food. Moreover, significant correlations between r and clearance rates were found.6. The observed differences in the grazing and the life history parameters of Daphnia feeding on non‐limited and nutrient‐limited algae may be the result of both reduced nutritional value and reduced digestibility of nutrient‐limited algae.
Relationships between heterotrophic nanoflagellates and the demographic response of Daphnia longispina in a eutrophic lake with poor food quality conditions
Authors: MONTEL MARIE‐LAURE , LAIR NICOLE 
Published: November 2003

1. Based on both field data and laboratory studies, the summer population of Daphnia longispina living in a stratified eutrophic lake was examined in relation to the abundance of algae, nanoflagellates and picocyanobacteria.2. In early July, the Daphnia population replaced Bosmina and remained the dominant zooplankter during summer 1994. Its development in July was concomitant with an increase of edible algae but, despite the apparent abundance in available food, the Daphnia population decreased throughout August suggesting poor food conditions.3. From mid‐August to the beginning of September, the biomass of inedible phytoplankton was greater than that of the smaller, edible fraction. Consequently the average rate of increase, birth rates and fecundity of Daphnia remained low. Although the biomass of heterotrophic nanoflagellates was consistently low, the demographic parameters of Daphnia were correlated throughout this period with these protozoans.4. Life table experiments run in the laboratory showed that epilimnetic food supported both the growth in length of individual Daphnia and an increase in fecundity, but metalimnetic food supported only individual growth. D. longispina probably failed to reproduce because of the abundance of detritus mixed with the heterotrophic nanoflagellates in the metalimnetic water at that period of the year. The vertical migration of Daphnia into these deeper layers could be caused by a predator avoidance mechanism.
Effect of thermal treatment of incubated potato juice on the formation of Maillard volatiles
Authors: Davids Stephen J. , Yaylayan Varoujan A. , Turcotte Ginette 
Published: November 2003

SummaryPotato juice samples, obtained from potatoes stored at different temperatures (0–24 °C) and times (8–20 weeks), were incubated (45–57 °C for 42 h) with or without commercial proteases. The samples were then used for thermal browning experiments by placing in either a heated oven at 125 °C overnight or in an autoclave at 132 or 121 °C for 1 h, these experiments promoted the occurrence of the Maillard reaction. After the heat treatment step, all samples were extracted with methylene chloride and analysed by gas chromatography/mass spectrometry. The data indicated that, by subjecting the potato juice to thermal treatments, a wide variety of volatiles generated by the Maillard were produced and these are known to be associated with specific flavour notes, examples are alkyl and acetyl pyrazines, piperazinediones, furans and pyrroles. These findings suggest that the study of potato juice extract as a new medium for flavour development, in particular in those applications which presently use malt, coffee, soybean, meat and yeast extracts, is a worthwhile endeavour.