bluuebunny/crossref_metadata_2025_split huggingface dataset:
Financial aspects of haemophilia care in Puerto Rico and other Latin American countries
Authors: Santiago‐Borrero  , Ortiz  , Rivera‐Caragol  , Maldonado  
Published: March 2003

The present cost of optimal care in haemophilia is very high. The paradigm of comprehensive care approach, including the essential elements of continuous integrated multidisciplinary health services and the provision of home care with early use of antihaemophilic products requires abundant economic resources that usually are not readily available in nonaffluent countries. A cost‐effective comprehensive paediatric haemophilia programme has been operating in Puerto Rico during the last 15 years that provides quality care to over 90% of paediatric haemophiliacs, and is financed mainly by the local government health system. Efforts are also being made to provide and/or coordinate health care to all adult haemophilic patients through the Puerto Rico Pediatric Hemophilia Treatment Center.Information on the haemophilia care available in Latin American countries is scanty. The data available indicate that with a few exceptions, haemophilia care in this vast region is suboptimal. Apparently, early diagnosis is not common, there is lack of accessibility of services in most countries, comprehensive care is not the rule, safe high‐purity AHF concentrates are used infrequently, and home care is used rarely. The main antihaemophilic products used are fresh frozen plasma and cryoprecipitate due to the high cost of modern antihaemophilic factor concentrates. The average per capita income of the main Latin American countries is about one‐quarter of that of the USA and Canada. The existing economic situation of most Latin American countries would make it very difficult for them to purchase modern antihaemophilic products regularly and provide quality comprehensive care to their haemophilia patients, unless they make special efforts and get some type of external help. As a result of this study recommendations are made to improve the quality and accessibility of services to haemophilia patients in Latin America and other nonaffluent countries.
Factor VIII gene polymorphisms in the Asian Indian population
Authors: Chowdhury M. R. , Herrmann F. H. , Schroder W. , Lambert C. T. , Lalloz M. R. A. , Layton M. , Kumbnani H. K. , Kabra M. , Menon P. S. N. , Verma I. C. 
Published: March 2003

Little is known about the heterozygous frequency of factor VIII gene markers in the Asian Indian population. The objective of this study was to establish the heterozygous frequency of polymorphic markers within and flanking the factor VIII gene in Indians and identify those most informative for carrier screening and prenatal diagnosis.Factor VIII gene polymorphism analysis at intragenic and extragenic sites was carried out by the polymerase chain reaction (PCR) method and Southern blot procedure. Sixty‐three Asian Indian haemophiliacs and their families were screened. A control group of 150 women from nonhaemophilic families were screened for two markers, HindIII and BclI.Among the intragenic markers studied, the HindIII restriction fragment length polymorphism (RFLP) showed the highest heterozygous frequency (0.52) followed by the intron 13 (0.47) and intron 22 (0.44) short tandem repeats (STRs). Among extragenic markers, TaqI had the highest heterozygous frequency (0.75) followed by BglII (0.54). The intron 22 inversion mutation was observed in eight (40%) of 20 severe cases.In the population studied the most diagnostic polymorphisms were the intragenic markers, intron 22 (70%) STR followed by the intron 13 (52%) STR and HindIII (52%) RFLP, and the TaqI (50%) extragenic marker. Application of HindIII, BclI and the intron 22 dinucleotide repeat combined were diagnostic in 87.2% of haemophilia A families studied.
Education and career development
Authors: MÁRQUEZ C. S. 
Published: March 2003

The main objective of this paper is to briefly analyse the different factors and circumstances that a person with haemophilia should take into account before undertaking the challenge of going to university and, in turn, choosing a profession or career.Although education systems in different countries or regions present fundamental differences, some characteristics remain the same, and these will be the focus of our study.The child with haemophilia will most likely encounter three challenges:(1) the challenge of choosing a career; (2) the challenge of choosing a college or university; and (3) the challenge of adjusting to college or university life.The young haemophiliac should be taught to exploit their strengths, and to feel that their only limit is their intellectual development. They should feel that they are only competing against themselves and that any adversities they might face due to haemophilia should not disfavour them; instead they should learn how to live with them and allow these to make them into stronger people.
Perioperative substitution therapy with plasma‐derived factor VIII concentrate in patients with von Willebrand disease
Authors: Wolf H‐H , Dorligschaw O , Eberspächer B 
Published: March 2003

Von Willebrand (vW) disease is a common hemorrhagic disorder characterized by various clinical manifestations and a variety of bleeding symptoms. By classification 3 types of vW disease are described concerning synthesis and multimeric constitution of vW factor. Especially the type 3 disorder is often complicated by perioperative hemorrhages.vW factor may be released from endothelial depots due to hormonal regulation or stress. Therefore, patients (pts) with vW disease type 1 or 2 may suffer from haemorrhagic complications after major surgery even if preoperative coagulation tests had found to be normal. Especially in pts with combined plasmatic disorders as coagulation factor (F) V or F XI deficiency bleeding may start once more after initial haemostasis.Until now, recombinant vW factor is not available. There are no clear dosage regimens of plasma derived FVIII concentrate. Substitution therapy with plasmatic F VIII / vW factor concentrates usually has to be adjusted to plasma concentrations of vW ristocetin cofactor.We report 5 pts with vW disease type 1 and 1 patient type 2 and history of bleeding disorders who received F VIII / vW factor concentrate (Immunate) meanwhile surgery. In 2 pts there was evidence of additional F XI or F V deficiency. After initial bolus administration (80 – 120 IU / kg body weight) we started continuous infusion (3.4 – 9.8 IU / kg body weight) for 48 h. The initial dose of continuous infusion was reduced to 50% after 24 h and to 25% after 36 h, respectively.In type 1 pts we saw a 3.4‐fold increase in collagen binding activity and 1.9‐fold increase in plasma concentration of vW ristocetin cofactor, respectively. Clinical effectiveneness of substitution therapy was good.We conclude that continuous infusion of F VIII/vW Factor concentrate (Immunate) may provide sufficient haemostasis in pts with vW disease. Increase in collagen binding activity has been shown to correspond well to substitution. Further data are necessary to prove whether collagen binding activity rather than plasma concentration of vW ristocetin cofactor could be a more relevant parameter concerning dosage administration.
Complications of central venous access devices in paediatric haemophilia patients
Authors: Domm J. A. , Hudson M. G. , Janco R. L. 
Published: March 2003

Summary. We conducted a retrospective survey of our experience with central venous access devices (CVADs) implanted in children with haemophilia seen at the Vanderbilt Hemostasis–Thrombosis Clinic from 1986 to 2000. Following discussion with parents on the merits and risks associated with the use of CVADs for immune tolerance induction or factor prophylaxis, catheters were inserted under sterile technique in the operating room. One nurse provided demonstration and teaching about catheter care and access. Thirty central venous catheters were inserted in 22 children. Our survey revealed that the two most common complications associated with central venous catheters were bacteraemia and thrombosis. We found a sepsis rate of 0.30/1000 catheter‐days or one episode of bacteraemia for every 3346 days of catheter use. The thrombosis rate of our cohort was 0.13/1000 catheter‐days or one episode of thrombosis for every 7529 days of catheter use. Uncomplicated venous access is essential in children with severe haemophilia who require prophylaxis or immune tolerance induction. While infection was the most common complication observed in our series, we experienced a lower overall infection rate than several reported series. Catheter thrombosis and subsequent obstruction may occur as a result of intraluminal fibrin deposits. We conclude that the use of implantable central venous catheters is an effective method for accessing children with haemophilia. We accept that the benefits of CVADs in the treatment of paediatric haemophilia patients outweigh the previously documented risks. Future prospective studies should be designed to define all associated risks and to determine effective strategies to reduce them.
Periapical status and quality of root fillings and coronal restorations in a Danish population
Authors: Kirkevang L.‐L. , Ørstavik D. , Hörsted‐Bindslev P. , Wenzel A. 
Published: March 2003

AbstractAimThe aim of this study was to investigate the quality of endodontic and coronal restorations and the association with periapical status in a Danish population.MethodologyA total of 614 randomly selected individuals (20–60+ years of age) from Aarhus County had a full‐mouth radiographic examination. The quality of endodontic and coronal restorations and the periapical status of endodontically treated teeth were assessed by radiographic criteria. Root fillings were categorized as ‘adequate’ or ‘inadequate’ with regard to root filling length and lateral seal. Coronal restorations were categorized into ‘adequate’ and ‘inadequate’, defined by the absence or presence of radiographic signs of overhangs or open margins. Results were analysed statistically using the chi‐squared test.ResultsThe total number of endodontically treated teeth was 773, and 52.3% had apical periodontitis (AP). Root‐filled teeth with an adequate lateral seal had a lower incidence of AP than teeth with an inadequate seal (44.3% vs. 57.8%), and teeth with an adequate root filling length were associated with a better periapical status than teeth with inadequate length of the root filling (42.0% vs. 67.6%). Similarly, adequate coronal restorations were associated with better periapical status than inadequate restorations (48.0% vs. 63.9%). When both root filling and coronal restoration quality were assessed, the incidence of AP ranged from 31.2% (optimal quality) to 78.3% (all parameters scored as inadequate).ConclusionsInadequate root canal and coronal restorations were associated with an increased incidence of AP.
A study of endodontic treatment carried out in dental practice within the UK
Authors: Jenkins S. M. , Hayes S. J. , Dummer P. M. H. 
Published: March 2003

AbstractAimThe purpose of this study was to gather both qualitative and quantitative information on the nature of root canal treatment carried out by a group of dentists working within the United Kingdom.MethodologyA two‐part questionnaire was posted to 720 dentists who graduated from the Dental School, Cardiff, Wales, UK. The first part requested basic information regarding age, year of qualification, field of practice, etc. The second part consisted of 15 questions on endodontic practice and root canal treatment.ResultsThe response rate was 41.5%. Two hundred and ninety‐nine questionnaires contained useful information. The majority of practitioners did not use rubber dam during root canal treatment. The vast majority (89%) exposed a radiograph with an instrument of known length in situ to gauge the ‘working length’, a small number relied upon tactile sensation. Most practitioners used local anaesthetic solution as an irrigant during instrumentation of the root canal. A wide variety of instruments were used for root canal treatment; a stepback technique was preferred by almost half the practitioners. Antiseptic solution was preferred as an interappointment dressing. More than half of the respondents used laterally condensed gutta‐percha to obturate root canals in anterior teeth but only one‐third used the same technique in posterior teeth. Less than half the respondents exposed a radiograph to check the fit of the master point prior to obturation. Two‐thirds of practitioners used a zinc oxide based material as their root canal sealer. Three‐quarters of the practitioners exposed a post obturation radiograph.ConclusionsThe results of this study suggest that although some dentists are using the techniques taught during their undergraduate careers, a large percentage now use techniques with no evidence of clinical effectiveness.
The political role of illness narratives
Authors: Sakalys Jurate A. 
Published: March 2003

The political role of illness narratives Cultural criticism is used to describe the political role of autobiographical illness narratives or pathographies. In expressing the subjective experience of illness, authors of pathographies illuminate ideological differences between patient and health care cultures, reveal the dominance of health care ideologies, and explicate patients’ moral and political claims. The contributions of these literary works to nursing practice provide direction for relational restructuring. Gadow’s concept of the relational narrative is proposed as a way to restore patient subjectivity and agency and establish the dialogue necessary for cultural pluralism in nursing and health care.
Modelling the vegetation of China using the process‐based equilibrium terrestrial biosphere model BIOME3
Authors: Ni Jian , Sykes Martin T. , Prentice I. Colin , Cramer Wolfgang 
Published: March 2003

Abstract1 We model the potential vegetation and annual net primary production (NPP) of China on a 10′ grid under the present climate using the processed‐based equilibrium terrestrial biosphere model BIOME3. The simulated distribution of the vegetation was in general in good agreement with the potential natural vegetation based on a numerical comparison between the two maps using the ΔV statistic (ΔV = 0.23). Predicted and measured NPP were also similar, especially in terms of biome‐averages.2 A coupled ocean–atmosphere general circulation model including sulphate aerosols was used to drive a double greenhouse gas scenario for 2070–2099. Simulated vegetation maps from two different CO2 scenarios (340 and 500 p.p.m.v.) were compared to the baseline biome map using ΔV. Climate change alone produced a large reduction in desert, alpine tundra and ice/polar desert, and a general pole‐ward shift of the boreal, temperate deciduous, warm–temperate evergreen and tropical forest belts, a decline in boreal deciduous forest and the appearance of tropical deciduous forest. The inclusion of CO2 physiological effects led to a marked decrease in moist savannas and desert, a general decrease for grasslands and steppe, and disappearance of xeric woodland/scrub. Temperate deciduous broadleaved forest, however, shifted north to occupy nearly half the area of previously temperate mixed forest.3 The impact of climate change and increasing CO2 is not only on biogeography, but also on potential NPP. The NPP values for most of the biomes in the scenarios with CO2 set at 340 p.p.m.v. and 500 p.p.m.v. are greater than those under the current climate, except for the temperate deciduous forest, temperate evergreen broadleaved forest, tropical rain forest, tropical seasonal forest, and xeric woodland/scrub biomes. Total vegetation and total carbon is simulated to increase significantly in the future climate scenario, both with and without the CO2 direct physiological effect.4 Our results show that the global process‐based equilibrium terrestrial biosphere model BIOME3 can be used successfully at a regional scale.
Stimulatory and inhibitory epitopes in the T cell responses of mice to Der p 1
Authors: Jarnicki A. G. , Thomas W. R. 
Published: March 2003

SummaryBackground The responses of mice to the mite allergen Der p 1 have been used to study the mechanisms of allergic sensitization and the development of new types of immunotherapy. Many of the studies require a knowledge of the T cell epitopes, and because Der p 1 is polymorphic, the effect of natural amino acid substitution in the allergen. The intranasal administration of peptides containing T cell epitopes can induce a mucosal tolerance but it is not known if the major activity is limited to stimulatory peptides and if, as found for autoimmunity, some epitopes are not inhibitory.Objective To determine and compare the sequences of Der p 1 which contain stimulatory epitopes for the high responding H‐2b and H‐2q mice and the sequences which induce tolerance by intranasal administration of peptides.Methods T cell responses of mice immunized with Der p 1 were measured by in vitro T cell stimulation assays so an extensive study of epitope recognition and intranasal tolerance could be made. Synthetic peptides were used to examine the stimulatory and inhibitory ability of all Der p 1 sequences and to map the major H‐2b epitope in detail. This included the effect of the common polymorphic amino acid 124 substitution found within this epitope.Results Three and two regions, respectively, were found to contain stimulatory T cell epitopes for H‐2b and H‐2q mice. The peptides in these regions were also the most active at inducing intranasal tolerance for the responding haplotype. The correspondence between inhibitory and stimulatory peptides was maintained for the fine mapping of the major H‐2b epitope. This was found about a core region of 118–126 which was overlapping but separate to a consensus sequence for the binding of endogeneous peptides. Peptides with alanine at the naturally polymorphic residue 124 stimulated and inhibited responses to Der p 1 more effectively, while peptides with the valine 124 variant were immunogenic but poorly cross‐reactive.Conclusions The intranasal administration of peptides representing each of five epitopes recognized by two strains of mice were able to induce mucosal tolerance and the major tolerizing activity was limited to these epitopes. The position of the core major epitope for C57 mice, which differs from a previously predicted epitope, and its specificity for the natural alanine 124 variant is described.
The use of a feather quilt, childhood asthma and allergic rhinitis: a prospective cohort study
Authors: Nafstad P. , Nystad W. , Jaakkola J. J. K. 
Published: March 2003

SummaryBackground Feather bedding has long been considered as a potential source of allergen exposure and thus a potential risk factor for allergic diseases. However, recent cross‐sectional studies have reported a higher risk of allergic diseases among users of synthetic bedding compared with feather‐bedding users.Objective To explore associations between early life exposure to feather bedding and the risk of developing asthma allergic rhinitis in childhood.Methods We assessed the association between early life exposure to feather quilts and the risk of bronchial obstruction during the first 2 years of life and asthma and allergic rhinitis in a prospective 4‐year cohort study of 2531 Norwegian children.Results At the age of 6 months, 24% of the children had a quilt with feathers, decreasing to 20% at the age of 2 years. The adjusted odds ratio for bronchial obstruction 0 to 2 years by exposure to a feather quilt at the age of 6 months was 0.59, 95% confidence interval 0.41 to 0.86, for asthma at the age of 4 years 0.38, 0.23 to 0.64 and for allergic rhinitis at the age of 4 years 0.73, 0.43 to 1.18.Conclusion The use of a feather quilt in early life does not seem to increase the risk of asthma and allergic rhinitis later in childhood.
Ethanol as a cause of hypersensitivity reactions to alcoholic beverages
Authors: Ehlers I. , Hipler U.‐C. , Zuberbier T. , Worm M. 
Published: March 2003

SummaryBackground Adverse reactions after ingestion of alcoholic beverages are common. Metabolic differences in individuals and also the histamine content in alcoholic beverages have been implicated. By contrast pure ethanol has rarely been reported as a cause of hypersensitivity reactions and its mechanism has not been clarified yet.Objective To determine whether ethanol itself accounts for alcohol hypersensitivity in patients with anaphylactic reactions after alcohol intake. In search of possible pathomechanisms all patients were analysed by skin prick testing and sulfidoleukotriene production of peripheral leucocytes using ethanol and its metabolites.Methods Double‐blind, placebo‐controlled food challenges with a cumulated amount of 30 mL ethanol were performed in 12 adult patients with a positive history of adverse reactions after consumption of different alcoholic beverages. Skin prick tests and measurement of sulfidoleukotriene production were performed using different concentrations of ethanol and acetaldehyde from 50 to 1000 mm.Results Oral challenges with pure ethanol were positive in six out of eleven patients. All challenge‐positive patients, but also four out of five challenge‐negative patients, showed an increased sulfidoleukotriene production in‐vitro compared with healthy controls. Skin prick tests using alcoholic beverages, ethanol, acetaldehyde and acetic acid were negative in all patients (12/12).Conclusion Our study shows that ethanol itself is a common causative factor in hypersensitivity reactions to alcoholic beverages. These reactions occur dose‐dependent and a non‐IgE‐mediated pathomechanism is likely, because skin prick tests were negative in all cases. Increased sulfidoleukotriene production was determined in some patients, but is no reliable predictor. Therefore oral provocation tests remain indispensable in making the diagnosis of ethanol hypersensitivity.