bluuebunny/crossref_metadata_2025_split huggingface dataset:
Marrow Failure
Authors: Bagby Grover C. , Lipton Jeffrey M. , Sloand Elaine M. , Schiffer Charles A. 
Published: November 2004

Abstract
New discoveries in cell biology, molecular biology and genetics have unveiled some of the pathophysiological mysteries of some of the bone marrow failure syndromes. Many of these discoveries have revealed why these syndromes show so much clinical overlap and some hold the potential for influencing the development of new therapies. In children and adults with pancytopenia and hypoplastic bone marrows proper differential diagnosis requires that some attention be directed toward defining molecular and cellular pathogenetic mechanisms because, once identified, some of these mechanisms will clearly suggest rational therapeutic approaches, treatment options that should be avoided, or both.
In Section I, Drs. Jeffrey Lipton and Grover Bagby review the approach to diagnosis and management of patients with the inherited bone marrow failure syndromes, Fanconi anemia, dyskeratosis congenita, Diamond-Blackfan anemia, and the Shwachman-Diamond syndrome. Extraordinary progress has been made in identifying the genes bearing pathogenetically relevant mutations in these disorders, but slower progress has been made in defining the precise functions of the proteins these genes encode in normal cells, in part because it is increasingly obvious that the proteins are multifunctional. In practice, it is clear that in patients with dyskeratosis congenita and Fanconi anemia, the diagnosis must be considered not only in children but in adults as well.
In Section II, Dr. Elaine Sloand outlines a very practical and evidence-based approach to diagnosis and management of acquired hypoplastic states emphasizing overlap between non-clonal and clonal hematopoiesis is such conditions. The pathogenesis of T lymphocyte–mediated marrow failure is presented as a clear-cut rationale for use of immunosuppressive therapy and stem cell transplantation. Practical management of patients with refractory disease with and without evidence of clonal evolution (either paroxysmal nocturnal hemoglobinuria [PNH] or myelodysplasia [MDS]) is presented.
In Section III, the challenge of hypoplastic MDS is reviewed by Dr. Charles Schiffer. After reviewing the most up-to-date classification scheme, therapeutic options are reviewed, focusing largely on agents that have most recently shown some promising activity, including DNA demethylating agents, thalidomide and CC5013, arsenic trioxide, and immunosuppressive therapy. Here are also outlined the rationale and the indications for choosing allogeneic bone marrow transplantation, the only therapy with known curative potential.
Sickle Cell Disease
Authors: Buchanan George R. , DeBaun Michael R. , Quinn Charles T. , Steinberg Martin H. 
Published: November 2004

AbstractMuch progress has been made during the past several decades in gaining understanding about the natural history of sickle cell disease and management approaches aimed at treating or even preventing certain disease complications. The characterization of the human genome now offers the opportunity to understand relationships regarding how gene polymorphisms as well as how environmental factors affect the sickle cell disease phenotype, i.e., the individual patient’s overall clinical severity as well as their specific organ function. This chapter explores some of these recent advances in knowledge.In Section I, Dr. Michael DeBaun characterizes the problem of silent stroke in sickle cell disease, comparing and contrasting its clinical and neuroimaging features with overt stroke. Combined, these events affect virtually 40% of children with sickle cell anemia. New understanding of risk factors, associated clinical findings, and imaging technologies are impacting substantially on treatment options. The appreciable cognitive dysfunction and other sequelae of silent infarct demand more effective treatments and ultimate prevention.In Section II, Dr. Charles Quinn addresses the conundrum of why some patients with sickle cell disease do well whereas others fare poorly. Some risk factors have been known for years, based upon careful study of hundreds of patients by the Cooperative Study for Sickle Cell Disease and investigators studying the Jamaican newborn cohort. Other prognostic measures have only recently been defined. Dr. Quinn devotes special attention to stroke and chest syndrome as organ-related complications but also describes attempts to measure overall disease severity and to predict survival. Recently, investigators have attempted to predict factors responsible for early mortality in children and following onset of pulmonary hypertension in adults.In Section III, Dr. Martin Steinberg reviews pharmacologic approaches to sickle cell disease and the rationale for their use. In addition to the inhibition of hemoglobin S polymerization, newer targets have been defined during the past one to two decades. These include the erythrocyte membrane, changes in the red cell intracellular content (especially loss of water), endothelial injury, and free radical production. Hydroxyurea treatment attracted the greatest interest, but many uncertainties remain about its long-term benefits and toxicities. Newer “anti-sickling” agents such as decitabine and short-chain fatty acids also receive attention. Prevention of red cell dehydration, “anti-endothelial” therapy, and marshaling the potentially beneficial effects of nitric oxide are other new and exciting approaches.
The Molecular Basis of Leukemia
Authors: Gilliland D. Gary , Jordan Craig T. , Felix Carolyn A. 
Published: November 2004

AbstractMajor strides have been made in our understanding of the molecular basis of adult and pediatric leukemias. More than one hundred disease alleles have been identified and characterized in cell culture and murine models of leukemia. In some instances, molecularly targeted therapies have been developed based on these insights that are currently in clinical trials, such as small molecule inhibitors of FLT3. In addition, it has recently been appreciated that, as with normal hematopoiesis, there is a hierarchical organization among leukemic cells that includes a rare population of leukemic stem cells that have properties of self-renewal. Understanding the characteristics of these leukemic stem cells may provide new insights into leukemia therapies that target self-renewal pathways.In Section I, Dr. Craig Jordan reviews the data that supports the existence of a “leukemia stem cell.” He provides an overview of the functional properties of leukemic stem cells, their relationship to hematopoietic stem cells, and the relevance of leukemic stem cells in other human malignancies including solid tumors. He briefly discusses what is known of the pathways that regulate properties of self-renewal.Dr. Gary Gilliland provides an overview of the genetics of adult leukemias in Section II and ongoing genome-wide strategies for discovery of new disease alleles. He describes the clinical and therapeutic implications of these findings and provides examples of bench-to-bedside translation of molecularly targeted therapies for AML, including the use of FLT3 inhibitors.In Section III, Dr. Carolyn Felix reviews recent advances in our understanding of the genetics and therapy of pediatric leukemias. She provides an overview of leukemias that are common in pediatric malignancies but rarely observed in adults, including the TEL-AML1 (ETV6-RUNX1) fusion associated with pediatric B-cell ALL, the OTT-MAL fusion associated with infant megakaryoblastic leukemia, PTPN11 mutations in juvenile myelomonocytic leukemia, and MLL fusion genes in leukemogenesis, among others.
TETRA: a web-service and a stand-alone program for the analysis and comparison of tetranucleotide usage patterns in DNA sequences
Authors: Teeling Hanno , Waldmann Jost , Lombardot Thierry , Bauer Margarete , Glöckner Frank Oliver 
Published: November 2004

Abstract
Background
In the emerging field of environmental genomics, direct cloning and sequencing of genomic fragments from complex microbial communities has proven to be a valuable source of new enzymes, expanding the knowledge of basic biological processes. The central problem of this so called metagenome-approach is that the cloned fragments often lack suitable phylogenetic marker genes, rendering the identification of clones that are likely to originate from the same genome difficult or impossible. In such cases, the analysis of intrinsic DNA-signatures like tetranucleotide frequencies can provide valuable hints on fragment affiliation. With this application in mind, the TETRA web-service and the TETRA stand-alone program have been developed, both of which automate the task of comparative tetranucleotide frequency analysis.
Availability:http://www.megx.net/tetra

Results
TETRA provides a statistical analysis of tetranucleotide usage patterns in genomic fragments, either via a web-service or a stand-alone program. With respect to discriminatory power, such an analysis outperforms the assignment of genomic fragments based on the (G+C)-content, which is a widely-used sequence-based measure for assessing fragment relatedness. While the web-service is restricted to the calculation of correlation coefficients between tetranucleotide usage patterns of submitted DNA sequences, the stand-alone program generates a much more detailed output, comprising all raw data and graphical plots. The stand-alone program is controlled via a graphical user interface and can batch-process a multitude of sequences. Furthermore, it comes with pre-computed tetranucleotide usage patterns for 166 prokaryote chromosomes, providing a useful reference dataset and source for data-mining.

Conclusions
Up to now, the analysis of skewed oligonucleotide distributions within DNA sequences is not a commonly used tool within metagenomics. With the TETRA web-service and stand-alone program, the method is now accessible in an easy to use manner for a broad audience. This will hopefully facilitate the interrelation of genomic fragments from metagenome libraries, ultimately leading to new insights into the genetic potentials of yet uncultured microorganisms.
Development of the Brunel Balance Assessment: a new measure of balance disability post stroke
Authors: Tyson Sarah F , DeSouza Lorraine H 
Published: November 2004

Objective: To report the psychometric properties of the Brunel Balance Assessment (BBA), a new test of balance disability post stroke. Design: Data from 92 subjects were collected and cohorts used to test hierarchical scaling, reliability and validity. Data from 80 people were used to test the hierarchical scaling using an inter-item correlation for redundancy, coefficient of reproducibility (CR) and scalability (CS) for the hierarchy and Cronbach's alpha coefficient for the internal consistency. Thirty-seven people participated in the reliability testing. Test / retest and inter-tester reliability were tested using the kappa coefficient. The testing was repeated on consecutive days to assess test / retest reliability and was scored simultaneously by two physiotherapists for inter-tester reliability. Fifty-five people participated in validity testing. The BBA was compared with the sitting Motor Assessment Scale (MAS), Berg Balance Test (Berg), Rivermead Mobility Index (RMI) using Spearman's rho. Setting: Physiotherapy stroke services of six UK NHS trusts. Participants: Hemiplegic stroke patients were recruited from physiotherapy services and the BBA used to assess their balance. Results: The order of the items was revised and the original 14-point scale reduced to 12 points in the scale development. The revised scale formed a hierarchical scale. Inter-item correlations were < 0.9, coefficients of reproducibility and scalability were 0.99 and 0.69 respectively and Cronbach's alpha was 0.92. Reliability was high (100% agreement) for both aspects of reliability. Correlations with other balance measures were significant (0.83 / 0.97, p<0.01) indicating validity as measure of balance disability. Conclusion: The BBA is a reliable, valid, hierarchical measure of balance disability post stroke that is suitable for use in the clinical setting.
An exploration looking at the impact of domiciliary and day hospital delivery of stroke rehabilitation on informal carers
Authors: Low J TS , Roderick Paul , Payne Sheila 
Published: November 2004

Objectives: To explore the impact of two methods of post-hospital stroke rehabilitation on both carers' perceptions of the health services offered and their quality of life. Setting: East Dorset Health Authority. Subjects: Forty-six informal carers were recruited from a sample of 106, initially identified from stroke patients participating in a larger randomized controlled trial. Design: Qualitative methods. Methods: Semi-structured interviews were used at baseline and six months to explore carers' perception of a good therapy, the advantages and disadvantages of the different services and their fulfilment with the services. In-depth thematic analysis was carried out to explore the impact of the two different methods of service delivery on carers' quality of life. Results: Day hospitals provided carers with respite opportunities, whilst domiciliary stroke teams provided carers with better educational opportunities to be involved in therapy. No qualitative difference was found in the impact that the different services had on carers' quality of life, which were influenced by factors such as the degree of disruption that caring had on their lives, the loss of a shared life and the availability of social support. Ultimately, carers saw the services as providing benefit for survivors and not themselves. Conclusions: Domiciliary stroke teams provided informal stroke carers with skills that could help improve postdischarge stroke rehabilitation amongst stroke survivors. Informal carers also benefited from the respite elements of day hospital. A mixed model using both domiciliary care and day hospital care, could provide carers with the benefits of education, convenience and respite.
Effects of visual feedback rhythmic weight-shift training on hemiplegic stroke patients
Authors: Cheng Pao-Tsai , Wang Chin-Man , Chung Chia-Ying , Chen Chia-Ling 
Published: November 2004

Objective: To assess the balance function of hemiplegic stroke patients and to investigate whether visual feedback rhythmic weight-shift training following acute stroke can decrease falls among patients with hemiplegic stroke. Design: A prospective study, using a Balance Master. Setting: Hospital-based rehabilitation units. Subjects: Fifty-two hemiplegic stroke patients (28 in the training group and 24 in the control group). Interventions: Conventional stroke rehabilitation programme plus visual feedback rhythmic weight-shift training. Training effect was evaluated by assessing the static and dynamic balance performance as well as comparing the occurrence of falls in the training and control groups at six-month follow-up. Main measures: Occurrence of falls; static balance in different sensory conditions; and dynamic balance performance, including on-axis velocity and directional control during rhythmic weight-shift. Results: Significant improvement in dynamic balance performance was found in hemiplegic patients in the training group. The improvement was sustained for six months. With regarding to static balance function, no significant improvement was found. At six-month follow-up, 5 of 28 patients (17.8%) in the training group had fallen, compared with 10 of 24 patients (41.7%) in the control group. The occurrence of falls decreased, although not statistically significantly (p = 0.059). Conclusions: Visual feedback rhythmic weight-shift training may improve dynamic balance function for hemiplegic stroke patients. The effects of training may be sustained for six months. The occurrence of falls decreased in the training group, but not statistically significantly.
Effects of aerobic and strength exercise on motor fatigue in men and women with multiple sclerosis: a randomized controlled trial
Authors: Surakka Jukka , Romberg Anders , Ruutiainen Juhani , Aunola Sirkka , Virtanen Arja , Karppi Sirkka-Liisa , Mäentaka Kari 
Published: November 2004

Objective: To investigate the effects of aerobic and strength exercise on motor fatigue of knee flexor and extensor muscles in subjects with multiple sclerosis (MS). Design: A randomized controlled trial. Setting: At Masku Neurological Rehabilitation Centre, Masku, and the Social Insurance Institution, Research Department, Turku, Finland. Subjects: Ninety-five MS patients with mild to moderate disability were randomized into exercise group (n = 47) and a control group (n = 48). Intervention: Participants in the exercise group attended in a supervised exercise period of three weeks, which was followed by a home exercise programme lasting for 23 weeks. Patients in the control group continued with their normal living. Outcome measures: Motor fatigue of knee flexor and extensor muscles was measured during a static 30-s maximal sustained muscle contraction. The decline in force (Nm) during the 30 s was recorded, and a fatigue index (FI) was calculated. Subjective fatigue was measured by using the Fatigue Severity Scale (FSS). The Ambulatory Fatigue Index (AFI) was calculated on the basis of a 500-m walking test. Assessment took place at baseline, at the third week (not for the control group) and at the 26th week. All outcome variables were analysed, men and women together, and some interesting contrasts were analysed by gender. Results: Associations were observed with changes in extension FI and Expanded Disability Status Scale (EDSS) score and mean extension torque (Nm), but not with changes in FI and aerobic or strength exercise activity, mean AFI, mean FSS or in mean knee flexion torque. AFI was decreased in all subject groups (p = 0.007). Motor fatigue was reduced in knee flexion (p = 0.0014) and extension (ns) among female but not in male exercisers after six months of exercise. The exercise activity of women was 25% higher than that of the men. Conclusions: Six months of exercise reduced motor fatigue in women, but not in men.
A multiple case design experiment to investigate the performance and neural effects of a programme for training hand function after stroke
Authors: Turton Ailie J , Butler Stuart R 
Published: November 2004

Objective: To investigate the effects of task-specific practice on hand function in stroke subjects who were given computer-assisted training and to look for associated changes in corticospinal connectivity. Design: Single case design experiments. Setting: Subjects’ homes. Subjects: Seven chronic stroke subjects with impaired hand function. Interventions: Daily intensive practice of fast and accurate force changes in pinch grip and knob turning grip for four weeks. Main measures: Pinch and power grip strength and dexterity measured using the Ten-hole Peg Test and a timed nuts and bolts test were recorded twice a week throughout the study. Corticospinal connectivity was assessed by latency of electromyography (EMG) responses to transcranial magnetic stimulation (TMS) recorded during the baseline weeks before the training and again immediately after the training period. Results: Subjects completed between 5760 and 18 560 force changes. Clinically relevant improvements in grip strength were evident in only two subjects. None of the subjects showed clinically significant improvement in dexterity measures. Statistical analysis showed that improved performance was related to the training phase in only two subjects. A reduction in latency of EMG responses to TMS was seen in two subjects but could not be attributed to the training phase. Conclusions: The intensive training did not improve hand function in most subjects. Latencies of EMG responses to TMS were also unable to detect any change.
Emission Control, Industrial
Authors: Berglund Ronald L. 
Published: November 2004

AbstractExhaust emissions are increasingly legislated with additional emphasis on fugitive emissions. Much of the research and development in the chemical process, petroleum, power generation, and manufacturing industries is driven by the need to control exhausts utilizing postprocess emissions treatment. Oxidative combustion technology is commonly employed for elimination of volatile organic components. This technology may be strictly thermal (incineration) or employ oxidation catalysis. The catalysts, mechanistic models, and design, operation, and selection of industrial catalytic exhaust control systems are discussed, as is catalytic inhibition. Exhaust control technology for criteria air pollutants, such as the nitrogen oxides, is presented and the incorporation of biofiltration technology in selected applications. The use of catalytic oxidation techniques for control of specific emissions within particular industries is also discussed.
Flow Measurement
Authors: Burgess Thomas H. 
Published: November 2004

Abstract
The measurement of flow is essential to the production, distribution, consumption and disposal of all liquids and gases including fuels, chemicals, foods and wastes. Emphasis in this chapter is placed on the broad types of flow measuring equipment that are commercially available and the general selection criteria for their proper use. The various measurement methods and corresponding hardware are discussed in terms of their principles of operation, variations in available forms, advantages and limitations, and commonly used areas of application.
Heat Pipes
Authors: Kosoy Boris 
Published: November 2004

AbstractHeat pipes are used to perform several important heat‐transfer roles in the chemical and closely allied industries. Examples include heat recovery, the isothermalizing of processes, and spot cooling in the molding of plastics. In its simplest form, the heat pipe possesses the property of extremely high thermal conductance, often several hundred times that of metals. As a result, the heat pipe can produce nearly isothermal conditions making an almost ideal heat‐transfer element. In another form, the heat pipe can provide positive, rapid, and precise control of temperature under conditions that vary with respect to time.The heat pipe is self‐contained, has no mechanical moving parts, and requires no external power other than the heat that flows through it.
Iron Compounds
Authors: Stolzenberg Alan M. 
Published: November 2004

Abstract
This article provides a brief overview of the preparation, composition, properties, and uses of the principal compounds of iron. The compounds are divided into salts and simple coordination compounds, chelate compounds, organometallic compounds, and compounds of biological relevance. Whereas compounds of iron in the + 2 and + 3 oxidation state are the most common, eg, salts of iron(II) are known for almost all the common anions, higher oxidation states are known to exist, even in biological compounds. Moreover, organometallic iron is formally lower in the oxidation state. Iron compounds find commercial applications throughout the chemical industry as catalysts, pharmaceuticals, dyes, pigments, and indicators. Many iron compounds are magnetic and may be found in magnets and in recording media as well as in microwave technology. Sections on economic aspects, analytical methods, and health and safety considerations are included.