bluuebunny/crossref_metadata_2025 huggingface dataset:
Coming clean: the symbolic use of clinical hygiene in a hospital sterilising unit
Authors: Rawlings Barbara
Published: February 2004

The hospital Theatre Sterile Supplies Unit cleans and provides sterile instruments for use in operating theatres. The strict standards of cleanliness, based on scientific notions of clinical hygiene, are maintained at the everyday level by people such as ancillary workers, nurses and doctors organising their activities, movements and understandings according to known rules. Since clinical hygiene is related to advanced microbiological discoveries, an instrument cannot be judged sterile by the naked eye. The judgement is made on the basis of what is believed to be happening to the instrument (e.g. processes it goes through; places it has been). The paper, based on an empirical study, notes the conceptual relationship between primitive and western notions of hygiene, and examines the ways that rules are used in the everyday accomplishment of instrument sterilisation.
Ludwik Fleck on the social construction of medical knowledge
Authors: Löwy Iiana
Published: February 2004

Abstract The subject of the development and transmission of medical knowledge has remained, until recently, relatively little studied by medical sociologists. But as early as the 1930s the pioneering studies of Ludwik Fleck, a physician and historian of science, dealt with the evolution of medical knowledge and the genesis of medical facts. Starting with a reflection on his own experience as clinical bacteriologist and immunologist, Fleck developed highly original views on subjects such as the influence of patterns of specialization of physicians on the medical knowledge they produced, the impact of popular models of disease on expert ones, and the importance of the circulation of ideas between distinct, and ‐ according to Fleck ‐ incommensurable ‘thought collectives’(medical scientists, general practitioners and patients) for the development of innovations in medicine. The aim of this article is to analyze Fleck's vision of medicine and to select among his ideas those which may be of interest for sociologists of medicine today.
Addressing individual difficulties in reading: issues relating to Reading Recovery and Pause, Prompt, Praise
Authors: Wearmouth Janice
Published: February 2004

Recently the DfES has issued guidance on ways to address the needs of students who experience difficulties in literacy through Wave Three provision in the National Literacy Strategy (DfES, 2002). This guidance raises the issue of what kind of programmes might be initiated in mainstream schools that will improve what is available generally for pupils who experience difficulties. The original Literacy Taskforce report (1997) named Reading Recovery (RR) as one programme suitable for this purpose. It is not the only programme with 'proven' efficacy, however. This article compares RR and another New Zealand‐based programme, Pause, Prompt, Praise (PPP) with which it has a number of characteristics in common, in order to examine particular issues which are important to ensure that a particular programme can meet individual children's literacy needs and also have the potential for adoption by LEAs and schools in the current national curricular context. These issues are the underpinning rationales, assumptions about the reading process and questions of resources, ownership and control (Openshaw et al., 2002).
Moving from reader response to critical reading: developing 10–11‐year‐olds' ability as analytical readers of literary texts
Authors: McDonald Lorraine
Published: February 2004

This article presents aspects of a research study into how a group of ten‐ and eleven‐year‐old students (in 5th Grade in Sydney, Australia) were apprenticed to view a literary text from critical reading positions. These ways of reading were an alternative to their more typical reader response interpretations of texts. The article contrasts ‘non‐critical’ and ‘critical’ classroom talk through a close examination of transcript excerpts, and compares these kinds of talk as discrete ways of thinking and speaking. The article argues for a response‐analytical continuum which may support young readers in moving towards critical reading practices.
Transforming what they read: helping eleven‐year‐olds engage with Internet information
Authors: Pritchard Alan , Cartwright Victoria
Published: February 2004

This article explores an issue of great current concern: how do we help primary aged children to engage with, and to benefit from, information from the Internet? The notion of ‘engagement’ is considered in the context of constructivist learning theory. A short case study based on a small‐scale research project in a primary school classroom in England is presented in order to illustrate the difficulties that arise when children undertake information handling work involving access to vast amounts of information. The end products from two groups of children are found to be far from what might have been expected from children of their ability, background and experience. Engagement with the ideas contained in non‐fiction texts is isolated as a crucial factor. Approaches, including the EXIT model, are presented and considered as ways in which engagement might be encouraged.
Children writing funny stories: some reflections on the impact of collaborative talk
Authors: Pearson Caroline
Published: February 2004

This article describes work undertaken with a class of Scottish Primary Six children (aged 10) that encouraged them to write humorous stories. It reflects on the impact of different teaching approaches, in particular exploring how teacher‐led input combined with opportunities for peer talk might serve to influence children's writing. The aims were first, to investigate whether, through ‘mini lessons’, children's story schemas could be expanded to include ideas about incongruity, and second, to discover how independent talking in pairs might encourage children to take up and reformulate ideas given in the initial teacher‐led input. During the sessions following the ‘mini lessons’, many opportunities for paired talk were offered. These were found to lead to a reinterpretation of ideas of incongruity, and to affect the selection and devising of voices for characters. The children's talk often gave evidence of a marked ability to describe humour and act it out orally, but analysis of the written stories reveals difficulties in writing down comic events. This has implications for the role of the teacher in finding ways to intervene sensitively to introduce new strategies.
Insights from a social model of literacy and disability
Authors: Brewster Stephanie
Published: February 2004

This article considers the role of literacy in the lives of four people with physical disabilities. Interviews with them reveal that despite experiencing potential barriers to participating in literacy activities, literacy is nevertheless an important dimension to their identities. Limitations of the traditional models of disability and literacy are explored and alternative, social models are found to offer a more helpful framework, in which engagement with text is seen in the context of people's lives. It is suggested that interventionists still have some way to go before their practice will fully reflect these perspectives.
“It came alive outside my head.” Developing literacies through comparison: the reading of classic text and moving image
Authors: Bousted Mary , Ozturk Alayne
Published: February 2004

This article reports on a term's work with students in Initial Teacher Education (ITE) in which the adult classic text Silas Marner was studied in both written and film form (Eliot, 1994; BBC/Fo, 1985). Through an extended consideration of the structures employed by different forms of narrative, students were invited to consider the knowledge and skills they brought to the development of their own response to different texts. Concurrently they were encouraged to consider the needs of their prospective pupils, as expert readers of moving image, and as novice readers of classic fiction. Crucial to the teaching and learning experience was the consideration of the different, expanding notions of literacy, including visual literacy, tele‐literacy and moving‐image literacy. A key consideration was the interrelationship between these, with narrative as the key link, and the exploration of how these literacies could be mutually supportive within the framework of ‘school’ literacy. The importance of paying explicit attention to the pedagogical practices that foster an engagement with and development of these different literacies was highlighted, as was the need to experience the reading of whole texts in order to foster the development of response.
From scripted instruction to teacher empowerment: supporting literacy teachers to make pedagogical transitions
Authors: Fang Zhihui , Fu Danling , Lamme Linda Leonard
Published: February 2004

The article describes a longitudinal professional development project in rural Florida (USA) schools that supports the efforts of inservice teachers to make pedagogical transitions from total reliance on prepackaged commercial programmes to making informed decisions about curriculum and pedagogy autonomously. It demonstrates that in order to initiate and sustain qualitative changes in teaching practices, conscious efforts must be made to address a multitude of barriers at the administrative, professional and personal levels. It suggests that professional development efforts in education must recognise the complex, multifaceted and lifelong nature of becoming and being an effective literacy teacher.
Balancing Social and Corporate Culture in the Global Economy: The Evolution of Japanese Corporate Structure and Norms
Authors: Sarra Janis , Nakaghigashi Masafumi
Published: February 2004

Japanese corporate law is undergoing a transition with new Commercial Code amendments in 2003 that allow corporations to opt for an Anglo‐American model of governance. Combined with shifts in securities regulation, shareholder activism, and the notion of Japanese lifetime employment, there is an important issue of whether these shifts represent a move towards an optimal corporate governance paradigm. The evolution is tempered by particular cultural norms that drive Japanese corporate law. While there is a perception in North America that Japanese corporations have shifted to an Anglo‐American model of corporate governance, the reality is more layered. It may be that ultimately, the governance model that evolves will adopt the best elements of increased transparency, corporate accountability, enhanced shareholder protection, employee long‐term employment protections, and production synergies, that would allow Japanese corporations to compete internationally, but retain those elements of corporate community that have long been considered a primary objective of the social and economic life of Japan.
Restructuring “Germany Inc.”: The Politics of Company and Takeover Law Reform in Germany and the European Union
Authors: Cioffi John W.
Published: February 2004

The reform of German company law by the Control and Transparency Law (KonTraG) of 1998 reveals the politics of corporate governance liberalization. The reforms strengthened the supervisory board, shareholder rights, and shareholder equality, but left intra‐corporate power relations largely intact. Major German financial institutions supported the reform's contribution to the modernization of German finance, but blocked mandatory divestment of equity stakes and cross‐shareholding. Conversely, organized labor prevented any erosion of supervisory board codetermination. Paradoxically, by eliminating traditional takeover defenses, the KonTraG's liberalization of company law mobilized German political opposition to the European Union's (EU) draft Takeover Directive and limited further legal liberalization.
The Bonding Effects of Directors’ Statutory Wage Liability: An Interactive Corporate Governance Explanation
Authors: Davis Ronald B.
Published: February 2004

Canadian corporate directors are personally liable to the corporation's employees for unpaid wages. The dominant rationale is the protection of vulnerable employees. A proposal under consideration to exonerate directors from this liability responds to claims that directors of financially troubled corporations resign prematurely, lessening the realized value potential of the firm. Scholars have also argued that a “liability chill” causes directors to make inefficient, risk‐averse investment decisions while the corporation is solvent. Paradoxically, exoneration may actually decrease the value of the firm because directors’ liability for employees’ wages increases efficiency in corporate governance by reducing agency costs. It serves as a bond by directors to corporate stakeholders that they will diligently restrain harmful managerial behavior.
Reactions of the London Stock Exchange to Company Trading Statement Announcements
Authors: Collett Nick
Published: February 2004

Using event study methodology, we examine market reactions to nearly 2,000 trading statements during the period 1995‐2001. We find that profit warnings outnumber upgrades by 50%, and, in line with previous US studies, we find that market reaction to the actual announcements is considerably greater for profit warnings than for upgrades.Sub‐samples demonstrate significant market reaction to profit warnings for all sizecontrolled portfolios, but that reaction to the announcements is greatest for small companies.Examination of pre‐ and post‐announcement CARs shows no pre‐announcement market anticipation of the announcements.Post‐announcement there is a significant positive abnormal return on the day after the announcement of bad news for the small company subsample.Other post‐announcement results are small and insignificant.Trading volume results are consistent with this picture. Finally, when the trading statements are examined for news on turnover and margin changes, we find that the market reaction to margin changes is greater than market reaction to turnover changes.
Interim Reporting Frequency and Financial Analysts' Expenditures
Authors: Yee Kenton K.
Published: February 2004

This paper relates interim financial reporting frequency in a multiperiod Kyle framework to securities prices, trading volume, market liquidity, and analysts' information acquisition expenditures. The model supports conventional wisdom that more frequent interim reporting improves the information content of securities prices, reduces reporting day price volatility and trading volume, and enhances market liquidity. However, the model suggests that more frequent financial reporting induces analysts to increase their redundant information acquisition expenditures, which may be socially wasteful.
CAPM, Higher Co‐moment and Factor Models of UK Stock Returns
Authors: Hung Daniel Chi‐Hsiou , Shackleton Mark , Xu Xinzhong
Published: February 2004

In this paper we examine the variables that explain the cross‐section of UK stock returns. Previous studies have found that the CAPM beta has moderate or even insignificant explanatory power once the Fama French factors are included. However, we control for different realised risk premia in up and down markets by using the same methodology as Pettengill, Sundaram and Mathur (1995). Unlike previous work, we find that beta is highly significant in explaining the cross‐section of UK stock returns and more importantly remains significant even when the Fama French factors are included in the cross‐sectional regressions. We also investigate whether higher co‐moments (co‐skewness and co‐kurtosis) have any explanatory power but find that empirical support is weaker.
Human Immuno‐deficiency Virus and Infant Feeding in Complex Humanitarian Emergencies: Priorities and Policy Considerations
Authors: Leyenaar JoAnna
Published: February 2004

Issues surrounding mother‐to‐child transmission of HIV/AIDS pose considerable challenges in complex humanitarian emergencies. The risk of vertical transmission through breastfeeding is well recognised, but safe alternatives are limited by the social, economic and environmental conditions of emergency situations. In 2000, the World Health Organisation published a technical report on behalf of the UNFPA/UNICEF/WHO/UNAIDS Inter‐agency Task Team on Mother‐to‐child Transmission of HIV which outlined revised recommendations for infant feeding by HIV‐positive women. This paper outlines reasons why these recommendations may be insufficient during the initial stages of complex humanitarian emergencies and proposes recommendations for establishing infant‐feeding policy. Methods of mother‐to‐child transmission of HIV are reviewed and recent research findings are discussed. Rationale for modifying the 2000 UNFPA/UNICEF/WHO/UNAIDS infant‐feeding recommendations in complex emergency situations is explored from the perspective of the infant, the mother and humanitarian field staff. Ethical limitations and future priorities are considered. The paper concludes with recommendations and a policy decision‐making framework for consideration during the initial stages of humanitarian crises.
From Disaster to Sustainable Civil Society: The Kobe Experience
Authors: Shaw Rajib , Goda Katsuihciro
Published: February 2004

Nine years after the Kobe earthquake in Japan, social issues are still prominent, and the rehabilitation process is still ongoing. The earthquake caused two major changes in Japanese society: an increase in voluntary and non‐government activities, and the enhancement of cooperation between local government and the residents' association. People's participation in the decision‐making process was a significant achievement. To sustain the efforts generated after the earthquake, the Kobe Action Plan was formulated and tested in different disaster scenarios. The current study suggests that civil societies in urban areas are sustainable if, first, the activities related to daily services are provided by the resident's associations; and second, these are linked to economic incentives. Leadership plays a crucial role in collective decision‐making. Creation of the support system is essential for long‐term sustainability of civil‐society activities. These observations are exemplified in the case study in Nishi Suma, one of the worst‐affected areas in the Kobe city.
Housing Reconstruction After Two Major Earthquakes: The 1994 Northridge Earthquake in the United States and the 1999 Chi‐Chi Earthquake in Taiwan
Authors: Wu Jie Ying , Lindell Michael K.
Published: February 2004

The idea of pre‐impact recovery planning has recently been promoted by researchers and practitioners, but very little research has been done to evaluate its effects on disaster recovery. This study compared two jurisdictions — the city of Los Angeles, California and Taichung county in Taiwan — in their recovery from earthquakes. Although the two cases also differ with respect to variables other than the presence of pre‐impact recovery plans, the available data suggest that having a pre‐impact recovery plan facilitates housing reconstruction and allows local officials to make more effective use of the window of opportunity after disaster to integrate hazard mitigation into the recovery process.
Health Care in Appalachia: A Population‐Based Approach
Authors: Huttlinger Kathleen , Schaller‐Ayers Jennifer , Lawson Tony
Published: February 2004

Abstract  The health status of people who live in the rural, coal‐producing counties of southwest Virginia remains problematic despite an apparent high primary care provider‐to‐population threshold. This descriptive exploratory study surveyed 922 households representing 2,188 people, with regard to the availability, need, and access to health care services. Findings indicated a population who had a greater morbidity for chronic illnesses such as heart disease and hypertension than the rest of the state, a large number of people without health and prescription coverage, and an overall perception of fair‐to‐poor health status. Findings also indicated a substantial proportion of the population who were in need of dental and visual care and general preventive services and those who were dealing with depression at home without outside intervention. One disturbing finding was the large number of people who shared prescriptive medications with family and friends. The Community As Partner Model might be used by community health nurses in this region to help structure interventions. Overall findings suggest a need for interventions aimed at screening for depression, managing prescriptive medications, and identification of low‐cost and free preventive, dental, and visual care services.
Parenting Stress, Marital Quality, and Child Behavior Problems at Age 7 Years
Authors: Benzies Karen M. , Harrison Margaret J. , Magill‐Evans Joyce
Published: February 2004

Abstract The study examined the relationship of early family environment and infant characteristics with childhood behavior problems at age 7 years. Sixty‐two mothers and 56 fathers of preterm (30–36 weeks gestation and greater than 1500 g) and full‐term boys and girls completed the Parenting Stress Index, Dyadic Adjustment Scale, and a measure of socioeconomic status during the child's first year. When their child was age 7 years, parents completed the Eyberg Child Behavior Inventory Intensity (frequency of behavior) and Problem (impact of behavior) scales. Hierarchical regression analyses showed that parenting stress because of their child's distractibility during infancy predicted the frequency of childhood behavior problems at age 7 years for mothers and of the impact of behaviors on the mother and the father. The quality of the marital relationship during infancy predicted the frequency of behavior problems reported by fathers. Public health nurses are well positioned to assess parenting stress and marital quality and to provide support to families during the early stages of parenthood.
Diet and Behavior in At‐Risk Children: Evaluation of an Early Intervention Program
Authors: Worobey John , Pisuk Joan , Decker Kathleen
Published: February 2004

Abstract  This study describes outcomes for children enrolled in the Prevention‐Oriented System for Child Health Project, an early intervention program aimed at improving health and developmental status in at‐risk families. Through a series of home visits by public health nurses, 60 families received lessons on nutrition‐ and health‐related topics determined by the child and family's needs. On two occasions, some 8 months apart, the children were evaluated using the Developmental Assessment of Young Children, and their energy intake over the previous day was recorded. Analyses of the dietary and behavioral records indicated that the children's scores on the physical subtest improved significantly. A number of nutrition–development associations were found at follow‐up, suggesting that the intervention was successful. Implications of the results for at‐risk children are discussed.
Health Risk Behavior Assessment: Nutrition, Weight, and Tobacco Use in One Urban Seventh‐Grade Class
Authors: Dowdell Elizabeth Burgess , Santucci Mary Ellen
Published: February 2004

Abstract  Health attitudes and behaviors develop in childhood and progress through adolescence into adulthood. To better understand the health risk behaviors being undertaken by a seventh‐grade urban population, a study was conducted to identify what health risk behaviors a group of adolescents were participating in. The purpose of this study was to describe (1) the types of health risk behaviors being undertaken, (2) the frequency of their taking health risk behaviors, and (3) the age of initiation of the health risk behavior. The findings from this study indicate that this sample of 54, urban seventh graders suffers from multidimensional health needs. These students participated in a variety of health risk behaviors, as measured by the Youth Risk Behaviors Surveillance System Questionnaire. These students have begun to smoke regularly, which correlated with the use of alcohol. They rate their health as being good or excellent but rarely met the daily requirements for intake of fruits and vegetables. And, although they rate their weight as being acceptable, most are trying to lose weight. Additionally, differences between the boys and girls were noted in weight perception, with girls more likely than boys to use smoking as the primary method of weight control. Nursing is in an ideal position to provide effective primary care interventions and community health outreach to this adolescent population.
Alcohol Counseling as Part of General Wellness Counseling
Authors: Sieck Cynthia J. , Heirich Max , Major Christine
Published: February 2004

Abstract  Healthcare providers, and nurses in particular, are increasingly being called upon for health promotion counseling. Alcohol risk assessment is often included in general health screening questionnaires, but often little use is made of this information. Alcohol consumption is infrequently included as a focus of health promotion campaigns. Because alcohol intake influences many other health risks, it is logical and practical to include alcohol risk reduction in general wellness counseling. This article presents evidence that incorporating alcohol counseling lowers alcohol risk and lowers the risk of other chronic diseases. It also provides examples of ways nurses and other health professionals can discuss alcohol risk reduction in the context of general health, thus helping remove the stigma associated with alcohol risk and engage clients in efforts to lower their alcohol risk.
Autonomy and Adolescence: A Concept Analysis
Authors: Spear Hila J. , Kulbok Pamela
Published: February 2004

Abstract  During the developmental stage of adolescence, young people strive for independence and begin to make decisions that impact them for the rest of their lives. The purpose of this descriptive study was to examine the literature over the past 15 years to analyze the concept of autonomy in adolescence and to identify possible relationships between autonomy and health behavior. The findings indicate that few studies have been carried out that address the issue of autonomy as it relates to adolescent lifestyles or “ways of living,” particularly related to health behaviors. Additional study is warranted to develop more defined theoretical and operational definitions of the concept of autonomy, which will be of value for the development of instruments for measurement and continued research. Furthermore, understanding the antecedents and consequences of adolescent autonomy will facilitate the development of more appropriate health‐promoting interventions based on developmental needs.
Health Care Needs of Elderly in a Rural Community in Korea
Authors: Ahn Yang‐Heui , Kim Mi Ja
Published: February 2004

Abstract  The purpose of this study was to examine the need for health and social support services of the elderly who lived alone in a rural community in Korea. All 97 elderly who lived alone in 32 communities in H Eub(county), Kangwon Province participated in the study. Comprehensive assessment was conducted on their need for these services and on the status of their physical, functional, cognitive, emotional, nutritional, social, and environmental factors. Data were collected by eight public health nurses and were analyzed using descriptive statistics and stepwise multiple regression. Elderly subjects living alone had more than one disease, limited gait ability, limited functional capabilities as measured by the combination of activities of daily living and instrumental activities of daily living, and poor nutritional state. They had low level of cognitive ability, high level of depression, and high level of need for financial support. They engaged in no or little exercise. Service need for physical care was highest, and the factors contributing to the service need were gait ability, functional capabilities, nutrition state, and perceived health status. The results of this study indicate that their needs are multiple and complex. Well‐coordinated health services that integrate social welfare services for elderly population who live alone are recommended.
Homeless Persons' Decisions to Accept or Reject Public Health Disease‐Detection Services
Authors: Swigart Valerie , Kolb Randall
Published: February 2004

Abstract  The purpose of this study was to describe the factors that homeless persons report as influencing their decisions to utilize or reject a public health disease‐detection program. Although there is copious literature on homelessness, few studies report the real‐life perspectives of homeless persons toward health or health promotion. A convenience sample of 55 sheltered and street‐dwelling homeless persons, who either resided in or were visiting seven shelters in a large northeastern U.S. city, were interviewed. The interview questions focused on the bases for decisions to accept or reject tuberculosis screening. The in‐depth semistructured audio‐taped interviews were transcribed, coded, and categorized using Ethnograph software. Interviews were analyzed using the constant comparative content analysis methods. The findings describe homeless persons' reasons for accepting or rejecting a tuberculosis‐detection service, the prominent role of shelter personnel in recruitment for health‐related interventions, and the confidentiality needs of women with children. This information can assist community health practitioners in designing and advertising health‐promotion and disease‐detection programming.
Evaluation of Needle Exchange Programs
Authors: Delgado Cheryl
Published: February 2004

Abstract  Needle exchange programs exist in every major population area in the United States and in many other countries. Some operate legally under emergency health decrees issued by local departments of health, with the stated intention of risk reduction through the removal of used injection equipment from use by injection drug users. It is theorized that this results in a reduced transmission of human immunodeficiency virus, hepatitis, and, possibly, other blood‐borne diseases. Needle exchange programs also offer access to drug treatment programs for the participants. It is a difficult but necessary task to evaluate these programs. This article examines examples of evaluations attempted in the past and discusses the challenges of such evaluations. Experimental evaluations, economic program analysis, legal aspects, and risk–benefit assessment along with ethical aspects are considered. An outline of program evaluation is proposed. Needle exchange programs offer an opportunity to encourage risk reduction and to offer counseling and access to health care for individuals at high risk. It is essential that such programs demonstrate their effectiveness. Assumptions of efficacy are insufficient for health care in the twenty‐first century.
The Public Health Nursing Practice Manual: A Tool for Public Health Nurses
Authors: Sakamoto Sharon D. , Avila Margaret
Published: February 2004

Abstract  Public health nursing is recognized as an important and critical component of the public health workforce, and today, it makes up the largest single category of public health professionals, according to the U.S. Department of Health and Human Services. Preparation of generalist public health nurses with the knowledge base, skills, and training to effectively respond to public health challenges is essential. The County of Los Angeles Department of Health Services, Public Health Nursing Section, in response to this need, implemented the Public Health Nursing Practice Manual to provide public health nurses with interventions and guidelines to articulate their role and practice as outlined by the framework of the Public Health Nursing Practice Model developed by Los Angeles County. Identification and development of new methods to support the practice of nursing is imperative in facilitating a more sophisticated and expanded level of practice, as well as providing a means of improving, protecting, and enhancing the quality of health for all people. The Public Health Nursing Practice Manual is an effective tool to monitor performance improvement and provide standardization of the generalist public health nurse practicing in Los Angeles County.
Novel keratin 14 gene mutations in patients from Hungary with epidermolysis bullosa simplex
Authors: Csikós Márta , Szalai Zsuzsanna , Becker Krisztina , Sebõk Béla , Schneider Imre , Horváth Attila , Kárpáti Sarolta
Published: February 2004

Abstract:  Mutations in genes keratin 5 (KRT5) and 14 (KRT14) encoding the basal type keratin intermediate filaments have been identified in epidermolysis bullosa simplex (EBS) families and are likely to cause skin fragility. Three novel keratin 14 mutations in cases from the Hungarian Epidermolysis Bullosa Centre are reported. In a 7‐year‐old boy with Dowling‐Meara type EBS (DM‐EBS), who had severe skin symptoms with extended herpetiform blisters, a novel amino acid substitution N123K in keratin 14 had been detected. A 26‐year‐old woman with mild DM‐EBS with prominent palmoplantar hyperkeratosis and without active blister formation had a novel R125G mutation in keratin 14. In a 6‐year‐old girl, with Weber‐Cockayne type EBS (WC‐EBS) with palmoplantar blisters and moderate mental retardation, a novel V133L substitution was detected. Her pedigree showed autosomal dominant mode of inheritance; in the two other families, only the index patients were affected. The N123K and R125G mutations causing DM‐EBS phenotypes are located within the helix initiation motif of the rod domain, whereas the very close V133L mutation underlying the WC‐EBS phenotype is outside of this region. These novel amino acid substitutions provide further information for genotype–phenotype correlation in KRT14 mutations, and demonstrate the first molecular genetic data in EBS patients from Hungary.
Genetic variation in COL17A1 and the development of bullous pemphigoid
Authors: Winsey Samantha , Lonie Lorne , Allen Jill , Bunce Mike , Marshall Sara E. , Wojnarowska Fenella
Published: February 2004

Background:  Bullous pemphigoid (BP) is an autoimmune blistering disease of the skin characterized by autoantibody attack on collagen XVII.

Objectives:  To characterize the genetic complexity of COL17A1, the gene which encodes for the autoantigen collagen XVII. The data will be used to determine whether there is an association between polymorphisms and haplotypes of COL17A1 and genetic susceptibility to development of BP.

Methods:  The genetic complexity in COL17A1 was deduced by screening and then sequencing the gene. Haplotypes were constructed from the resulting polymorphisms using the statistical programme PHASE. The linkage disequilibrium (D′) between the polymorphisms was deduced from haplotypic data using the statistical programme GOLD. Association of the polymorphisms and haplotypes was tested for, in a cohort of BP patients and controls.

Results:  Screening of COL17A1 for genetic variation was carried out in 29 individuals of North European caucasoid origin, and it revealed 19 single‐nucleotide polymorphisms in approximately 14.7 kb of sequence. These variants resulted in 60 different haplotypes in 191 individuals, of which 13 occurred above 1% in the population. D′ between the variants was found to be extensive, have a low correlation with physical distance and to extend over 33.8 kb. No association was found with any of the polymorphisms or haplotypes and development of BP, when tested for, in a cohort of patients and controls.

Conclusion:  This study provides an extensive description of the genetic variation in COL17A1 and shows no association of the genetic variants with susceptibility to BP.
Human skin: source of and target organ for angiotensin II
Authors: Steckelings U. Muscha , Wollschläger Tanja , Peters Jörg , Henz Beate M. , Hermes Barbara , Artuc Metin
Published: February 2004

Abstract:  The present study examined the expression of angiotensin receptors in human skin, the potential synthesis of angiotensin II (Ang II) in this location and looked for a first insight into physiological functions. AT1 and AT2 receptors were found within the epidermis and in dermal vessel walls. The same expression pattern was found for angiotensinogen, renin and angiotensin‐converting enzyme (ACE). All components could additionally be demonstrated at mRNA level in cultured primary keratinocytes, melanocytes, dermal fibroblasts and dermal microvascular endothelial cells, except for AT2 receptors in melanocytes. The ability of cutaneous cells to synthesize Ang II was proved by identifying the molecule in cultured keratinocytes. Furthermore, in artificially wounded keratinocyte monolayers, ACE‐mRNA expression was rapidly increased, and enhanced ACE expression was still found in cutaneous human scars 3 months after wounding. These findings suggest that the complete renin–angiotensin system is present in human skin and plays a role in normal cutaneous homeostasis as well as in human cutaneous wound healing.
Recapitulation of the hairless mouse phenotype using catalytic oligonucleotides: implications for permanent hair removal
Authors: Cserhalmi‐Friedman Peter B. , Panteleyev Andrey A. , Christiano Angela M.
Published: February 2004

Abstract:  Ribozyme technology is widely used to target mRNA in a sequence‐specific fashion and thus change the expression pattern of cells or tissues. While the goal of mRNA targeting is usually the cleavage of mutant mRNAs with the prospect of gene therapy for inherited diseases, in certain instances, targeting of wild‐type genes can be used therapeutically. Lack of expression of the mouse hairless gene due to inherited mutations leads to the complete and irreversible loss of hair known as atrichia. We designed this study to recapitulate the hairless phenotype in a restricted manner by topical application of deoxyribozyme‐targeting molecules to specifically cleave the mouse hairless mRNA. Histological samples taken from treated skin at different times demonstrated a decreased number of hair follicles, an involution of the remaining follicles, a separation of the dermal papillae, and the presence of dermal cysts, all characteristics of the hairless phenotype, but not normally present in the skin of C57Bl/6 J mice. In this study, we successfully recapitulated the hairless phenotype using topically applied target‐specific catalytic oligonucleotides designed to cleave the mouse hairless mRNA. Our results demonstrate the feasibility of using ribozyme technology to alter the gene expression in the skin via topical application and provide proof of principle for the development of this strategy for permanent hair removal.
Identification of calcium‐inducible genes in primary keratinocytes using suppression‐subtractive hybridization
Authors: Seo Eun‐Young , Lee Woong‐Hee , Piao Yong‐Jun , Kim Kyoung‐Hoon , Lee Kyu‐Mi , Ahn Kwang‐Sung , Yang Jun‐Mo , Seo Young‐Joon , Kim Chang Deok , Park Jang‐Kyu , Lee Jeung‐Hoon
Published: February 2004

Abstract:  Terminal differentiation in epidermal keratinocytes involves major biochemical changes including the expression of many new differentiation‐specific genes. To further understand this process, we performed suppression‐subtractive hybridization of keratinocytes cultured under high‐calcium condition, known to induce differentiation in vitro. We randomly isolated 300 clones representing 90 different genes. By reverse Northern blot analyses, 20 different genes were found to be overexpressed, of which 13 were confirmed as differentially expressed genes during keratinocyte differentiation by Northern blot analysis. Of those, five genes, transglutaminase 1, keratin 6, interleukin‐1 receptor antagonist, kallikrein 7, and heat shock protein 27, are known to be up‐regulated during epidermal differentiation. Six genes, ferritin‐L chain, ribosomal protein S6, tumor‐associated calcium signal transducer 2, neuroendocrine secretory protein 55, phosphoserine aminotransferase, and neutrophil gelatinase‐associated lipocalin, heretofore were not known to be up‐regulated during keratinocyte differentiation. We also identified two novel genes. One of these maps to chromosome 1q21 of the epidermal differentiation complex, and its expression level was strongly increased in differentiating keratinocytes. These differentially expressed genes may provide significant opportunities for further understanding of the epidermal keratinocyte differentiation.
Cytoprotective effect of gallotannin in oxidatively stressed HaCaT keratinocytes: the role of poly(ADP‐ribose) metabolism
Authors: Bakondi Edina , Bai Péter , Erdélyi Katalin , Szabó Csaba , Gergely Pál , Virág László
Published: February 2004

Abstract:  Oxidative stress‐induced cytotoxicity is mediated in part by accelerated poly‐ADP ribosylation. Peroxynitrite and hydrogen peroxide cause DNA breakage triggering the activation of the DNA nick sensor enzyme poly(ADP‐ribose) polymerase‐1 (PARP‐1). Overactivation of PARP‐1 leads to cell dysfunction and cell death mainly due to depletion of NAD+ (the substrate of PARP‐1) and ATP. PARP‐1 attaches most ADP‐ribose residues onto itself, leading to downregulation of enzyme activity. Here, we have investigated the role of poly(ADP‐ribose) glycohydrolase (PARG), the poly(ADP‐ribose)‐catabolyzing enzyme in oxidative stress‐induced cytotoxicity in HaCaT cells. We have found that inhibition of PARG by gallotannin (GT) (50 µM) provided significant cytoprotection to peroxynitrite‐ or hydrogen peroxide‐treated HaCaT cells, as assessed by lactate dehydrogenase release and propidium iodide uptake (parameters of necrotic cell death) as well as caspase activation (apoptotic parameter). GT pretreatment has also inhibited the depletion of cellular NAD+ pools in hydrogen peroxide‐ or peroxynitrite‐treated HaCaT cells. GT caused the accumulation of poly(ADP‐ribose) and concomitant inhibition in cellular PARP activity in oxidatively stressed cells. Therefore, PARG is likely to contribute to maintaining the active state of PARP‐1 by continuously removing inhibitory ADP‐ribose residues from PARP‐1.
Expression of 67‐kDa elastin receptor in annular elastolytic giant cell granuloma: elastin peptides induce monocyte‐derived dendritic cells or macrophages to form granuloma in vitro
Authors: Fujimoto Norihiro , Akagi Atsushi , Tajima Shingo
Published: February 2004

Abstract: Annular elastolytic giant cell granuloma (AEGCG) is characterized by non‐palisading granuloma and elastophagocytic giant cells. Granulomas consist of structured masses of macrophages, dendritic cells, and T lymphocytes which play an essential role in granuloma formation. Two lineage systems of dendritic cells and macrophages originated from peripheral blood monocytes have been established in vitro. To know how elastin fragments are involved in the granuloma formation in AEGCG, we tested in vitro whether elastin fragments potentially induce monocyte‐derived macrophages or dendritic cells to form granuloma and multinucleated giant cells. Immunohistochemical studies of the lesional skins of AEGCG (n = 5) revealed that the 67‐kDa elastin receptor was specifically expressed in the epithelioid or multinucleated giant cells. Proliferation of factor XIIIa+ cells and CD68+ cells was also seen in the lesional skins of AEGCG. Factor XIIIa+ dendritic cells or CD68+ macrophages were established by the treatment of granulocyte/macrophage‐colony stimulating factor (GM‐CSF)/interleukin‐4 or M‐CSF, respectively. Further treatments of these dendritic cells or macrophages with elastin peptide resulted in the formation of granuloma or multinucleated giant cells which were immunoreactive with anti‐67‐kDa elastin receptor antibody. These findings suggest that elastic tissue induces factor XIIIa+ cells and CD68+ macrophages to form granuloma or multinucleated giant cells and plays an essential role in the formation of granuloma in AEGCG.
Quality of life assessment in haemophilia
Authors: Bullinger Monika , Von Mackensen Sylvia
Published: February 2004

Summary.  Quality of life (QoL) is a recent focus of research in haemophilia. It can be defined − in analogy to the World Health Organization (WHO) definition of health − as patient‐perceived wellbeing and function in terms of physical, emotional, mental, social and behavioural life domains. The paper describes conceptual, methodological and practical foundations of QoL research in adults and children at an international level. It then proceeds to review the QoL literature in the field of haemophilia. With regard to assessment of QoL in haemophilia patients, both generic and very recently targeted instruments have been applied. Recent publications have focused on describing QoL in adults, showing specific impairments in terms of physical function (arthropathy) and mental wellbeing (HIV infection) as well as focusing on the cost–benefit (QoL) ratio of haemophilia care. In paediatric haemophilia, research has suggested the beneficial QoL outcomes with prophylaxis and stressed the role of the family for patients' wellbeing and function. QoL research is a relevant area for haemophilia research which should be pursued further.
Development of a health‐related quality of life measure for boys with haemophilia: the Canadian Haemophilia Outcomes – Kids Life Assessment Tool (CHO‐KLAT)
Authors: Young N. L. , Bradley C. S. , Blanchette V. , Wakefield C. D. , Barnard D. , Wu J. K. M. , McCusker P. J.
Published: February 2004

Summary.  Several measures of quality of life (QoL) are available for children with haemophilia. However, most are not disease‐specific and few focus on children's perspectives. The purpose of this study was to develop a psychometrically sound measure of QoL that included the perspectives of boys with haemophilia. A list of potential items was developed from the literature, other measures, and input from five discussion sessions with adults with haemophilia, children with haemophilia and their parents and haemophilia nurses. The list was augmented with items generated by three focus groups with children and three focus groups with parents. These groups also prioritized items and recommended a domain structure. Supplemental information was gathered by surveying haematologists. Data from all sources were analysed to reduce the number of items using a two‐step approach, based on rules that weighted the children's priorities most heavily. The remaining items were compiled into a questionnaire that was pilot tested with 10 children and their parents. The total item pool contained 228 potential items. Of these, 33 were removed based on three focus groups and survey responses, 72 were removed after the completion of all focus groups and 46 were removed due to redundancy. This resulted in a 77‐item version of the CHO‐KLAT. Pilot testing identified the need to subdivide two items, resulting in a 79‐item CHO‐KLAT. The CHO‐KLAT is a promising disease‐specific measure of QoL that reflects children's unique perspectives. This child‐centric focus distinguishes the CHO‐KLAT from alternative measures of QoL. Further research will assess the measurement properties of the CHO‐KLAT.
Economic evaluation: what are we looking for and how do we get there?
Authors: Steen Carlsson K. , Höjgård S. , Lethagen S. , Berntorp E. , Lindgren B.
Published: February 2004

Summary. The interest in economic evaluation of alternative strategies for haemophilia treatment has increased through the years. Few studies have actually been undertaken, however, and most of them have been simple cost‐minimization or cost‐effectiveness analyses. From the perspective of the binational project ‘Treatment strategies for severe haemophilia − prophylaxis vs. on‐demand’, the present paper discusses the pros and cons of different methods for economic evaluation and their data requirements. Severe haemophilia is a rare disease that requires lifelong treatment. In addition, treatment has both short‐ and long‐term effects which are likely to differ between strategies. Accordingly, regardless of the chosen evaluation method, data requirements are non‐trivial. Hence, the various problems connected to the generation of data, as well as how they may be addressed, are also discussed.