bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ehlers‐Danlos syndrome – a historical review
Authors: Parapia Liakat A. , Jackson Carolyn 
Published: March 2008

SummaryEhlers‐Danlos syndrome is an inherited heterogeneous group of connective tissue disorders, characterized by abnormal collagen synthesis, affecting skin, ligaments, joints, blood vessels and other organs. It is one of the oldest known causes of bruising and bleeding and was first described by Hipprocrates in 400 BC. Edvard Ehlers, in 1901, recognized the condition as a distinct entity. In 1908, Henri‐Alexandre Danlos suggested that skin extensibility and fragility were the cardinal features of the syndrome. In 1998, Beighton published the classification of Ehlers‐Danlos syndrome according to the Villefranche nosology. From the 1960s the genetic make up was identified. Management of bleeding problems associated with Ehlers‐Danlos has been slow to progress.
β2‐Adrenergic receptor and adenylate cyclase gene polymorphisms affect sickle red cell adhesion
Authors: Eyler Christine E. , Jackson Terry , Elliott Laine E. , De Castro Laura M. , Jonassaint Jude , Ashley‐Koch Allison , Telen Marilyn J. 
Published: March 2008

SummarySickle red cell (SS RBC) adhesion is thought to contribute to sickle cell disease (SCD) pathophysiology. SS RBC adhesion to laminin increases in response to adrenaline stimulation of β2‐adrenergic receptors (β2ARs) and adenylate cyclase (ADCY6), and previous evidence suggests such activation occurs in vivo. We explored whether polymorphisms of the β2AR and ADCY6 genes (ADRB2 and ADCY6, respectively) affect RBC adhesion to laminin. We found that the β2AR arg16→gly substitution and two non‐coding ADCY6 polymorphisms were associated with elevated adhesion. We postulate that ADRB2 and ADCY6 polymorphisms may influence SCD severity through the mechanism of RBC adhesion.
Effects of extensive splenomegaly in patients with myelofibrosis undergoing a reduced intensity allogeneic stem cell transplantation
Authors: Ciurea Stefan O. , Sadegi Barry , Wilbur Andrew , Alagiozian‐Angelova Victoria , Gaitonde Sujata , Dobogai Lisa C. , Akard Luke P. , Hoffman Ronald , Rondelli Damiano 
Published: March 2008

SummaryChanges in spleen size postallogeneic haematopoietic stem cell transplantation (HSCT) in patients with primary myelofibrosis have been poorly characterized. We analysed 10 patients with myelofibrosis and splenomegaly following a reduced‐intensity allogeneic HSCT. All patients fully engrafted donor cells including five patients with extensive splenomegaly. Extensive splenomegaly was associated with a prolonged time to neutrophil and platelet recovery. In all 10 patients, a progressive reduction of splenomegaly was documented within 12 months post‐transplant and paralleled the reduction of marrow fibrosis. These findings suggest that myelofibrosis patients with extensive splenomegaly may proceed with allogeneic HSCT without prior splenectomy.
The non‐haematopoietic biological effects of erythropoietin
Authors: Arcasoy Murat O. 
Published: March 2008

SummaryIn the haematopoietic system, the principal function of erythropoietin (Epo) is the regulation of red blood cell production, mediated by its specific cell surface receptor (EpoR). Following the cloning of the Epo gene (EPO) and characterization of the selective haematopoietic action of Epo in erythroid lineage cells, recombinant Epo forms (epoetin‐alfa, epoetin‐beta and the long‐acting analogue darbepoetin‐alfa) have been widely used for treatment of anaemia in chronic kidney disease and chemotherapy‐induced anaemia in cancer patients. Ubiquitous EpoR expression in non‐erythroid cells has been associated with the discovery of diverse biological functions for Epo in non‐haematopoietic tissues. During development, Epo–EpoR signalling is required not only for fetal liver erythropoiesis, but also for embryonic angiogenesis and brain development. A series of recent studies suggest that endogenous Epo–EpoR signalling contributes to wound healing responses, physiological and pathological angiogenesis, and the body’s innate response to injury in the brain and heart. Epo and its novel derivatives have emerged as major tissue‐protective cytokines that are being investigated in the first human studies involving neurological and cardiovascular diseases. This review focuses on the scientific evidence documenting the biological effects of Epo in non‐haematopoietic tissues and discusses potential future applications of Epo and its derivatives in the clinic.
Therapy‐associated genetic aberrations in patients treated for non‐Hodgkin lymphoma
Authors: Basecke Jorg , Podleschny Martina , Becker Annegret , Seiffert Edda , Schwiers Ivonne , Schwiers Roman , Haase Detlef , Glass Bertram , Schmitz Norbert , Trumper Lorenz , Griesinger Frank 
Published: March 2008

SummaryTherapy‐associated myelodysplastic syndromes and acute myeloid leukaemia (t‐AML/MDS) following high dose chemotherapy are significant problems, with a cumulative incidence of 20% or more in myeloablative treatment regimen. Retrospective findings indicated that t‐AML/MDS associated genetic aberrations can be observed directly after exposure to chemotherapy and can precede t‐AML by several months. To determine the incidence of post‐therapeutic aberrations and their predictive value, we prospectively investigated 316 samples of 95 patients with non‐Hodgkin lymphoma (NHL) who were treated with intermediate and high dose chemotherapy (Arm A and B of the megaCHOEP (cyclophosphamide, doxorubicin, etoposide, vincristine, prednisolone) trial of the German High Grade NHL study group). Molecular aberrations (RUNX1/RUNX1T1, PML‐RARA, CBFB‐MYH11, MLL‐MLLT1, BCR‐ABL1) were observed in 33·3% (Arm A) and 55·4% (Arm B) of patients and in 14·9% and 28·7% of respective samples. Cytogenetic analysis of 53 NHL patients after high dose therapy showed frequent chromosomal breakage. Clonal aberrations were found in three patients. None of these patients developed a t‐AML/MDS during a 3‐year clinical follow up period. We concluded that the high incidence of genetic aberrations reflected a dose‐dependent, transient therapy‐induced genetic damage which is not predictive of a t‐AML/MDS.
Long‐term reactions of Ni, Zn and Cd with iron oxyhydroxides depend on crystallinity and structure and on metal concentrations
Authors: Buekers J. , Amery F. , Maes A. , Smolders E. 
Published: March 2008

SummaryIron oxyhydroxides are long‐term sinks for toxic trace metals and are suggested as factors to explain long‐term metal immobilization in soil. It is unknown how crystallinity and crystal structure of iron (Fe) oxyhydroxides affect the long‐term kinetics of trace metal adsorption. Adsorption kinetics of nickel (Ni), zinc (Zn) and cadmium (Cd) on to four synthetic Fe oxyhydroxides (hydrous ferric oxide (HFO), ferrihydrite (FH), goethite (GT), haematite (HT)) were characterized in suspensions during 70 days at different initial metal concentrations and various pH values (3.8–7.0) maintained within 0.2 units. The slow reactions, defined as those occurring beyond day 1 were most pronounced in GT and least in HT. The extent of the slow reaction was surprisingly unrelated to crystallinity or microporosity as determined by N2 adsorption. Freundlich adsorption isotherms fitted to the data show that ageing between 1 and 70 days decreases metal concentrations in solution by factors of 2–30 (Ni), 1–20 (Zn) and 1–4 (Cd) depending on the type of oxyhydroxide. These factors become significantly larger at smaller concentrations for Ni and Zn in some oxyhydroxides and this concentration dependency suggests that slow reactions are not only related to diffusion. The short‐term adsorption strength of carrier‐free metal radio‐isotopes was similar at 1 or 70 days after reaction with these iron oxyhydroxides and excludes the possibility that long‐term reactions are related to solid‐phase transformations or changes in solution properties. The extent of slow reactions in GT decreased with decreasing pH and was less than a factor 2 within 70 days at pH 3.7. The results confirm that slow reactions in Fe oxyhydroxides can explain lessened metal availability by ageing in pH neutral soils. The uncertainty of their actual structure and crystallinity in soils does not allow quantitative predictions based on the behaviour of synthetic oxyhydroxides.
Sucking habits in childhood and the effects on the primary dentition: findings of the Avon Longitudinal Study of Pregnancy and Childhood
Authors: DUNCAN KAREN , MCNAMARA CLARE , IRELAND ANTHONY J. , SANDY JONATHAN R. 
Published: March 2008

Introduction.  Most previous research on non‐nutritive sucking habits has been cross‐sectional in nature. This study determined the prevalence of non‐nutritive sucking habits and the effects on the developing dentition within a longitudinal observational cohort.Methods.  The Children in Focus group of the Avon Longitudinal Study of Pregnancy and Childhood study was studied. Questionnaire data on non‐nutritive sucking habits were collected on the children at 15 months, 24 months, and 36 months of age. Dental examinations were performed on the same children at 31 months, 43 months, and 61 months of age.Results.  At 15 months, 63.2% of children had a sucking habit, 37.6% used just a dummy, and 22.8% used a digit. By 36 months, sucking had reduced to 40%, with similar prevalence of dummy and digit sucking. Both habits had effects on the developing dentition, most notably in upper labial segment alignment and the development of anterior open bites and posterior crossbites.Conclusions.  The majority of children had non‐nutritive sucking habits up until 24 months of age. Both digit and dummy sucking were associated with observed anomalies in the developing dentition, but dummy‐sucking habits had the most profound influence on the anterior and posterior occlusions of the children.
Bacterial invasion of dentinal tubules beneath apparently intact but hypomineralized enamel in molar teeth with molar incisor hypomineralization
Authors: FAGRELL TOBIAS G. , LINGSTRÖM PETER , OLSSON STINA , STEINIGER FRANK , NORÉN JÖRGEN G. 
Published: March 2008

Background.  The most common problems for a patient with molar incisor hypomineralization (MIH) are the collapse of enamel and cavitations, loss of fillings, and secondary caries, but most of all, severe hypersensitivity.Objective.  The aim of this paper was therefore to histologically study possible bacterial invasion of dentinal tubules beneath apparently intact, but hypomineralized enamel in permanent molars with MIH.Material and methods.  Five extracted permanent first molars diagnosed with MIH were fixated, demineralized, and sagittally serially sectioned in a bucco‐lingual direction in a microtome with a thickness of 4–5 µm. Sections were stained with a modified Brown and Benn staining for bacteria, unstained sections were analysed in field emission SEM.Results.  Stained sections from the cuspal areas, below the hypomineralized enamel, the staining indicated the presence of bacteria in the dentinal tubules. The HTX staining showed that the pulp in sections without any findings was normal and free from bacteria or infiltrates from inflammatory cells. In sections where bacteria were found in the cuspal areas or deeper in the dentin, a zone of reparative dentin was found, and in sections from one tooth, the coronal pulp showed an inflammatory reaction with inflammatory cells. In sections adjacent to those without any bacterial staining, the SEM analyses revealed empty dentinal tubules without any odontoblast processes or signs of bacteria. When odontoblast processes were found, the dentinal tubules were filled with bacteria located on the surface of the odontoblast processes. In some areas, a large number of tubules were found with bacteria. No bacteria were found close to the pulp. The odontoblast processes appeared larger in areas where bacteria were found.Conclusions.  The presence of bacteria in the dentinal tubules and inflammatory reactions in the pulp indicate that oral bacteria may penetrate through the hypomineralized enamel into the dentin, thus possibly contribute to hypersensitivity of teeth with MIH.
Nursing work in Jordan: an example of nursing work in the Middle East
Authors: Shuriquie Mona , While Alison , Fitzpatrick Joanne 
Published: March 2008

Aim.  The study aimed to investigate Jordanian nurses’ perceptions of their role in clinical practice.Background.  The lack of regulation of nursing practice by the profession across the Middle East until now has led to each institution setting its own policies regarding the role of the nurse and the practice of nursing. No study to date has examined the role of the nurse working in the acute hospital environment nor explored the practice of nursing in this region.Design and methods.  A cross‐sectional questionnaire survey was conducted using a quota sample. A total of 348 medical‐surgical staff nurses and practical nurses from the three healthcare sectors in Jordan participated in the study with a response rate of 77%. The results were analysed by constructing multiple response tables, chi‐square test, anova and log‐linear analysis.Results.  Staff nurses in Jordan were expected to carry out the majority of nursing care activities. The role of the practical nurse was limited to the physical and professional domains of nursing care. Activities requiring higher levels of emotional or intellectual labour and interdisciplinary communication were attributed only to the staff nurse. The majority of the respondents reported nursing had not been their first choice of career. Male nurses had a higher intention to leave the nursing profession. The predominant method of care delivery used by nurses was task‐oriented.Conclusion.  There was a general consensus regarding what constituted the nurse's work in the clinical area across the three healthcare sectors in Jordan. Role delineation between the two levels of nurses was also clear. There is a need to move from task‐oriented to patient‐centred care to promote quality patient care.Relevance to clinical practice.  This study explores the role of the nurse working in the acute hospital environment in Jordan.
Learning from the severe acute respiratory syndrome (SARS) epidemic
Authors: Chan Engle Angela , Chung Joanne WY , Wong Thomas KS 
Published: March 2008

Aims and objectives.  This study uses two models of nursing practice, conventional and modular design, to compare nursing activities, hand hygiene, time efficiency and nurse–patient satisfaction in medical and surgical wards.Background.  Learning from the SARS epidemic pointed to the importance of quality nursing practice considerations that minimize cross‐transmission of infection while maximizing patient‐focused care. Hence, a modular nursing model was adopted.Design and method.  This study comprised pre‐ and postintervention phases. Data collection tools to evaluate modular nursing practice included a work sampling observation checklist, focused group interviews with nurses, questionnaires addressing nurses’ perceived competence and caring attributes, a patient satisfaction questionnaire, and a hand hygiene audit. A series of education sessions were conducted between the two phases. Quantitative and qualitative analyses were used for data triangulation.Results.  Modular nursing practice, focusing on continuity of care, led to changes in the nature of direct care activities and improvement in patient/family education frequency. Also, a general increase in nurses’ hand washing frequency was noted. However, when nurses perceived time pressure, a lapse in hand hygiene compliance was found. Because of human resource and inefficiency issues, some nurses in the studied wards did not embrace geographical separation for infection control. Positive correlations were found for nurses’ perceived infection control practice competence and their perceived caring attributes.Relevance to clinical practice.  In examining nursing practice models within complex clinical situations, the significance lies not only in the model's effects but also in other operational outcomes.
Investigating the knowledge, attitudes and practice patterns of operating room staff towards standard and transmission‐based precautions: results of a cluster analysis
Authors: Chan Moon Fai , Ho Aly , Day Mary Christine 
Published: March 2008

Aims.  To examine the relationship on knowledge, attitudes and practice levels of operating room staff towards the standard precautions and transmission‐based precautions, and to identify profiles of them based on their demographic variables and their knowledge, attitudes and practices towards the standard precautions and the transmission‐based precautions.Research method.  During January 2006, 113 staff working in the operating room of a public hospital completed a self‐reported questionnaire.Outcome measures.  Demographic information, knowledge, attitudes and practices scores were collected.Results.  Two‐step cluster analysis yielded two clusters. Clusters 1 and 2 consisted of 50·4% (n = 57) and 49·6% (n = 56), respectively. Cluster 1 subjects were younger, had a higher educational attainment level and worked at a more senior level than Cluster 2 subjects. They reported good knowledge, positive attitudes and practices. Cluster 2 subjects were characterized by relatively poor knowledge, negative attitudes and practices. Significant differences towards standard and transmission‐based precautions were found between clusters, except attitudes towards choosing protective personal equipment (p = 0·095) and practices on wearing gowns and eye shields/goggles (p = 0·759). Attitudes of Cluster 2 staffs were highly significant, but weakly correlated with practices (rs = 0·39, p < 0·05).Conclusion.  This study clearly profiles knowledge, attitudes and practice patterns of operating room staff, which may benefit healthcare educators in planning and developing appropriate educational programmes, may help organizations to provide a safe workplace climate and may aid healthcare workers to learn the importance of personal responsibility in preventing infectious disease transmission to patients, co‐workers and even themselves.Relevance to clinical practice.  To date, the only protection against infection is to minimize risk by modifying behaviour and practice patterns. Education and communication play a major role of the precautions. Tailoring interventions to fit different specific groups of operating room staff is needed to improve compliance with the standard and transmission‐based precautions.
A review of the psychosocial issues for nurses in male genitalia‐related care
Authors: Zang Yu‐Li , Chung Loretta YF , Wong Thomas KS 
Published: March 2008

Aim.  This literature review aimed to highlight psychosocial issues for nurses in the practice of male genitalia‐related care so as to guide the improvement of the teaching and practice of male genitalia‐related care.Background.  Male genitalia‐related care is common in hospitals and in the community. In several conditions, e.g. incontinence, postradiotherapy or following operation for cancer of genitalia, bladder, colon or rectum, patients will require male genitalia‐related care. Patients who require male genitalia‐related care may encounter psychosocial and/or sexual dysfunction. In the holistic approach to men's health, nurses are expected to meet patients’ psychosocial and sexual needs, while the literature suggests that nurses’ perceptions and attitudes in providing certain male genitalia‐related care, e.g. genital hygiene, sexual counselling, are negative.Method.  Systematic literature review.Conclusion.  Issues surrounding male genitalia‐related care for nurses are complicated and may be related to privacy, intimacy, sexuality, dirty work and emotional discomfort. Age, gender, race and social class could compound these issues.Relevance to clinical practice.  Nurses’ negative perceptions, responses and attitudes towards male genitalia‐related care may exacerbate patients’ conditions under which male genitalia‐related care is required. Appropriate strategies should be developed to overcome these problems.
From critical care to comfort care: the sustaining value of humour
Authors: Dean Ruth Anne Kinsman , Major Joanne E 
Published: March 2008

Aims and objectives.  To identify commonalities in the findings of two research studies on humour in diverse settings to illustrate the value of humour in team work and patient care, despite differing contexts.Background.  Humour research in health care commonly identifies the value of humour for enabling communication, fostering relationships, easing tension and managing emotions. Other studies identify situations involving serious discussion, life‐threatening circumstances and high anxiety as places where humour may not be appropriate. Our research demonstrates that humour is significant even where such circumstances are common place.Method.  Clinical ethnography was the method for both studies. Each researcher conducted observational fieldwork in the cultural context of a healthcare setting, writing extensive fieldnotes after each period of observation. Additional data sources were informal conversations with patients and families and semi‐structured interviews with members of the healthcare team. Data analysis involved line‐by‐line analysis of transcripts and fieldnotes with identification of codes and eventual collapse into categories and overarching themes.Results.  Common themes from both studies included the value of humour for team work, emotion management and maintaining human connections. Humour served to enable co‐operation, relieve tensions, develop emotional flexibility and to ‘humanise’ the healthcare experience for both caregivers and recipients of care.Conclusions.  Humour is often considered trivial or unprofessional; this research verifies that it is neither. The value of humour resides, not in its capacity to alter physical reality, but in its capacity for affective or psychological change which enhances the humanity of an experience, for both care providers and recipients of care.Relevance to clinical practice.  In the present era which emphasises technology, efficiency and outcomes, humour is crucial for promoting team relationships and for maintaining the human dimension of health care. Nurses should not be reluctant to use humour as a part of compassionate and personalised care, even in critical situations.