bluuebunny/crossref_metadata_2025 huggingface dataset:
Review: Contraception in adolescents with systemic lupus erythematosus
Authors: Tincani A. , Nuzzo M. , Lojacono A. , Cattalini M. , Meini A.
Published: August 2007

In the management of adolescents with systemic lupus erythematosus (SLE), sexual activity and prevention of unwanted pregnancies are important topics. Many contraceptive methods are available nowadays. Oral contraceptives (OCs) are the preferred choice among adolescents in general. However, the use of these medications in adolescents with SLE raises serious concerns, particularly the risk of thrombotic events from estrogen exposure and the impact of these medications on lupus activity. In this article, different contraceptive methods available are reviewed and their application in adolescents with SLE is discussed. In conclusion, OCs are the methods of choice in adolescents with stable disease and no antiphospholipid antibodies (aPL) detected. In patients with aPL, fewer options are available, and the selection of the preferred form of contraception should be made on an individual basis. Lupus (2007) 16, 600—605.
Review: Neuropsychiatric involvement in pediatric systemic lupus erythematosus
Authors: Benseler S.M. , Silverman E.D.
Published: August 2007

Neuropsychiatric (NP) manifestations are found in approximately 25% of children and adolescents with pediatric SLE (pSLE). In 70% of those, NP involvement will occur within the first year from the time of diagnosis. Headaches (66%), psychosis (36%), cognitive dysfunction (27%) and cerebrovascular disease (24%) are the most common presentations. The support of a psychiatrist is often required. Anti-phospholipid antibodies are associated with distinct NP disease entities and may be implicated in the pathogenesis of several manifestations of NP-pSLE including chorea, cerebrovascular disease and seizures. The role of novel auto-antibodies and imaging modalities is currently explored. The treatment of NP-pSLE is not based on prospective studies; however, an immunosuppressive combination therapy consisting of high doses of prednisone and a second line agent such as cyclophosphamide or azathioprine is commonly suggested for children with NP-pSLE. The role of novel therapies is currently studied. The outcome of children with NP-pSLE is relatively good. The overall survival is 95—97%, 20% of children experience a disease flare during childhood and 25% have evidence of permanent neuropsychiatric damage. Lupus (2007) 16, 564—571.
Review: Systemic lupus erythematosus in the young: the importance of a transition clinic
Authors: Falcini F. , Nacci F.
Published: August 2007

The objective of this report is to focus on the problems of patients with childhood onset systemic lupus erythematosus (SLE) at the age of transition to an adult care Unit. SLE is a multisystem disease characterised by diffuse internal organ involvement and by the presence of antinuclear and anti DNA antibodies. Central nervous system and renal damage are the main complications especially in children. Transition in health-care is a multifaceted, active process that attends to the medical, psychosocial and educational-vocational needs of adolescents when they move from child to adult-oriented lifestyles and systems. Lack of institutional support and difficulty in communicating and in identifying adult specialists are the major concerns in a transition care Unit. Psychosocial matters can make this change dramatic and hard for young people and their families. Patients with juvenile-onset SLE require specialised and multidisciplinary care when entering a transition clinic; physicians need to focus on preventing long-term complications of SLE, including atherosclerosis, obesity, osteoporosis and their treatment. We report on our experience in a cohort of patients with juvenile SLE cared for at our transition clinic over last six years. Lupus (2007) 16: 613—617.
Review: Antiphospholipid antibodies in pediatric systemic lupus erythematosus and the antiphospholipid syndrome
Authors: Avčin T. , Silverman ED
Published: August 2007

The antiphospholipid syndrome (APS) is recognized increasingly as the most common acquired hypercoagulation state of autoimmune etiology and may occur as an isolated clinical entity (primary APS) or in association with an underlying systemic disease, particularly systemic lupus erythematosus (SLE). The major differences between pediatric and adult APS include absence of common acquired risk factors for thrombosis, absence of pregnancy-related morbidity, increased incidence of infection-induced antibodies, differences in cut-off values for determination of aPL and specific factors regarding long-term therapy in children. APS in children has been largely reported in patients with arterial or venous thromboses and less frequently in association with neurological or hematological manifestations. The presence of aPL in pediatric SLE can modify the disease expression and may be an important predictor of the development of irreversible organ damage. Two recently established international registries of neonates and children with APS provide a good opportunity to conduct large, prospective studies on the clinical significance of aPL and long-term outcome of pediatric APS. Lupus (2007) 16, 627—633.
Review: Bone status in juvenile systemic lupus erythematosus
Authors: Lilleby V.
Published: August 2007

Osteoporosis is a well-recognized major health problem in adult patients with systemic lupus erythematosus (SLE). Children and adolescents with SLE, however are at even higher risk of developing osteoporosis later in life, since they develop the disease before achieving peak bone mass, which serves as a `bone bank' for the rest of life. There is still a paucity of studies on bone mass in pediatric SLE, but those studies available provide evidence of reduced bone mass in this age group.

A frequency of osteopenia of 40% measured by dual energy X-ray absorptiometry at one or more skeletal sites has been reported, and the lumbar spine is most seriously affected. Peak bone mass seems to be lower in childhood-onset SLE patients compared to healthy controls, and there are no signs of catch-up of bone mass in young adult patients with a history of pediatric SLE. Glucocorticoid therapy has been found to have a major negative effect on bone mass in these patients, thus the importance of keeping corticosteroid doses down to the lowest possible dose whenever possible.

Interestingly, studies of oral alendronate therapy in children with rheumatic childhood diseases have shown promising results with increases of 15—33% during one year of treatment with no major side effects reported. Finally, there is a hope that new biologic therapies, which are more specific and steroid-sparing, will also have a beneficial effect on bone health in SLE in the future. Lupus (2007) 16, 580—586.
Review: Macrophage activation syndrome in juvenile systemic lupus erythematosus: an under-recognized complication?
Authors: Pringe A. , Trail L. , Ruperto N. , Buoncompagni A. , Loy A. , Breda L. , Martini A. , Ravelli A.
Published: August 2007

Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases that is thought to be caused by the activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to widespread haemophagocytosis and cytokine overproduction. It is seen most commonly in systemic juvenile idiopathic arthritis, but is increasingly recognized also in juvenile systemic lupus erythematosus (J-SLE). Recognition of MAS in patients with J-SLE is often challenging because it may mimic the clinical features of the underlying disease or be confused with an infectious complication. This review summarizes the characteristics of patients with J-SLEassociated MAS reported in the literature or seen by the authors and analyses the distinctive clinical, diagnostic and therapeutic issues that the occurrence of MAS may raise in patients with J-SLE. Lupus (2007) 16, 587—592.
Review: The Paediatric Rheumatology International Trials Organization (PRINTO)
Authors: Ruperto N. , Bazso A. , Ravelli A. , Malattia C. , Filocamo G. , Pistorio A. , Rodriguez Lozano A.L. , Viola S. , Martini A.
Published: August 2007

In this review we describe an international project, conducted by the Paediatric Rheumatology International Trials Organization (PRINTO) that was aimed to identify, validate and promulgate core sets of measures and a definition of improvement for the evaluation of response to therapy in clinical trials and in daily clinical practice in patients with juvenile systemic lupus erythematosus (JSLE).

The following clinical measures were included in the PRINTO core set of outcome measure for the evaluation of response to therapy: 1) physician's global assessment of disease activity; 2) global disease activity measure; 3) 24-hour proteinuria; 4) parent's global assessment of the overall patient's well-being; 5) health-related quality of life assessment. The measures included in the core set were found to be feasible and not redundant, to have good construct validity, discriminative ability, internal consistency, with fair responsiveness to clinically important change in disease activity, and to be associated strongly with treatment outcome.

In order to be classified as responder to a given treatment, a patient should demonstrate at least 50% improvement from baseline in any two of the five core set measures with no more than one of the remaining worsening by more than 30%. This definition is now known as the `PRINTO/American College of Rheumatology (ACR) provisional criteria for JSLE'.

The proposed core set and definition of improvement incorporate clinically meaningful change in a composite endpoint for the evaluation of global response to therapy in JSLE. The definition is now proposed for use in JSLE clinical trials, and may help physicians to decide if a child with SLE has responded adequately to therapy. Lupus (2007) 16, 670—676.
Immune function in children born to mothers with autoimmune diseases and exposed in utero to immunosuppressants
Authors: Biggioggero M. , Borghi MO , Gerosa M. , Trespidi L. , Cimaz R. , Meroni PL
Published: August 2007

The administration of immunosuppressive drugs during pregnancy is often necessary in women with autoimmune diseases. Teratogenicity of immunosuppressives during pregnancy has been evaluated, only few data exist about the effects on immune systems. We therefore performed a pilot study on the influence of foetal exposure to immunosuppressives on immune function of babies born to mothers with autoimmune disorders. We investigated serological and cellular parameters as indicators of immune system status. We included in the study 14 babies (mean age 11 months, range 1—24) born to mothers with autoimmune diseases and exposed in utero to different immunosuppressants and, as controls, 14 babies whose mothers had autoimmune manifestations but did not receive immunosuppressive therapy. We evaluated: (i) complete blood count, (ii) immunoglobulin levels and IgG subclasses, (iii) antibody response to hepatitis B vaccine, (iv) leukocyte subpopulations and (v) interleukin-2 and interferon γ in vitro production by resting or activated peripheral blood mononuclear cells. We did not find statistically significant differences between exposed and not exposed babies or among treatments for the tested parameters. Immunosuppressive regimens currently in use for controlling maternal autoimmune disorders do not significantly affect the immune status of the offspring. Lupus (2007) 16, 651—656.
Review: New treatment strategies for proliferative lupus nephritis: keep children in mind!
Authors: Ranchin B. , Fargue S.
Published: August 2007

Renal involvement is frequent in children with systemic lupus erythematosus (SLE) and carries significant short and long-term morbidity. Treatment strategy in proliferative glomerulonephritis relies mainly on studies in adult patients where conventional treatment regimens including high doses of cyclophosphamide (CYC) and steroids may cause severe side effects. New strategies including sequential therapies of various combinations of low dose CYC, calcineurine inhibitors (cyclosporine or tacrolimus), mycophenolate mofetil, azathioprine, rituximab are now under investigation in adult patients with very few data in children. Organization of international registries and controlled trials in children with lupus nephritis is mandatory to determine long term prognosis and to validate less toxic therapy regimens in childhood. Lupus (2007) 16, 684—691.
Unusual neurologic manifestations (II): posterior reversible encephalopathy syndrome (PRES) in the context of juvenile systemic lupus erythematosus
Authors: Punaro M. , Abou-Jaoude P. , Cimaz R. , Ranchin B.
Published: August 2007

Posterior reversible encepalopathy syndrome (PRES), or reversible posterior leukoencephalopathy, is a neurologic condition characterized by recognizable pattern of altered mental status, headache, visual changes and seizures in association with findings indicating a predominantly posterior leucoencephalopathy on imaging studies. It has rarely been described in children. We report two cases of pediatric systemic lupus erythematosus (SLE) complicated by PRES and review the literature. Lupus (2007) 16, 576—579.
Examining the societal impacts of nanotechnology through simulation: NANO SCENARIO
Authors: Jarmon Leslie , Keating Elizabeth , Toprac Paul
Published: August 2007

This article describes a university-sponsored experiential-based simulation, the NANO SCENARIO, to increase the public's awareness and affect attitudes on the societal implications of nanoscience and nanotechnology by bringing together diverse stakeholders' perspectives in a participatory learning environment. Nanotechnology has the potential for massive societal effects across all sectors, globally. Thus, new forms of education are needed to prepare members of society for making complex decisions about policy, governance, and values. Here, the authors examine the theoretical and conceptual framework of the simulation, which uses improvised role-play and perspective-taking to engage the public in a transformative learning experience. As part of this examination, they describe the uses and constraints of simulations with open-ended goals and without competition and prescribed rules. They believe educational role-play scenarios with active participation of the public can serve as a dynamic method for civic engagement across a range of complex, interdisciplinary topics and new technological dilemmas.
An Intervention for Parents of Children With Epilepsy and Significant Learning Problems: Lessons Learned From a Feasibility Study
Authors: Buelow Janice M.
Published: August 2007

Children with epilepsy often experience significant learning, mental health, and behavior problems that affect their quality of life. Moreover, their parents report high rates of stress. The objectives of this article are to describe the development of a theory-based cognitive behavioral intervention for parents of these children, changes made to the program based on content validity testing, and the lessons learned from implementation of a feasibility trial. The intervention was designed to foster parents' skills and confidence in advocating for their children when interacting with health care professionals and school personnel and to help parents identify community resources. Four mothers participated in the program to establish feasibility and usability. Using qualitative analysis of discussions following each phase of the intervention, we learned that parents needed more time for epilepsy education and interacting with one another and that the intervention should emphasize forming partnerships with health care and school professionals. J Am Psychiatr Nurses Assoc, 2007; 13(3), 146-152. DOI: 10.1177/1078390307303747
Exploring Depression Among a Cohort of African American Women
Authors: Waite Roberta , Killian Priscilla
Published: August 2007

BACKGROUND: Depression is a chronic and disabling illness that occurs more often in women than men and commonly goes undetected and/or undertreated in African American women. Understanding sociocultural factors that may influence detection of depression is important for early intervention and treatment. OBJECTIVE: To improve understanding of how African American women articulate and conceptualize depression as well as how they manage depression in their daily life. This information can assist in detecting depression, which is requisite to accessing treatment. STUDY DESIGN: In accordance with this study's exploratory descriptive design, 36 African American women diagnosed with depression by a licensed mental health provider within the past year participated in five focus groups. RESULTS: Main themes included enduring the experience, revealing distress, and coping. CONCLUSION: Women's accounts of how they shaped their views and words used to communicate their depression were influenced by social and cultural experiences, values, and their knowledge about depression. J Am Psychiatr Nurses Assoc, 2007; 13(3), 161-169. DOI: 10.1177/1078390307304996
APNA and ANCC Collaboration: Achieving Consensus on Future Credentialing for Advanced Practice Psychiatric and Mental Health Nursing
Authors: Rice Michael J. , Moller Mary D. , DePascale Christine , Skinner Lee
Published: August 2007

The American Psychiatric Nurses Association and the American Nurses Credentialing Center conducted a logical job analysis based on three recent role-delineation studies (RDSs) to determine whether there was enough commonality in the practice of psychiatric and mental health nursing by clinical nurse specialists (PMH-CS) and nurse practitioners (PMH-NP) to support the development of a single certification examination. Three hundred seventy-one work tasks gleaned from the three RDSs were presented for systematic review and discussion by the logical job analysis committee of 28 nurses certified in the specialty. Of the 371 work tasks, 332 (90%) were considered important enough to test for both the PMH-CS and PMH-NP. The results of the logical job analysis indicated that there was enough commonality in the practices of the PMH-CS and PMH-NP in psychiatric mental health nursing to warrant the development of one advanced practice examination for both roles. J Am Psychiatr Nurses Assoc, 2007; 13(3), 153-159.
Intracardiac Thrombosis and Acute Right Ventricular Failure Following Complex Reoperative Cardiac Surgery With Aprotinin and Deep Hypothermic Circulatory Arrest
Authors: Stone Marc E. , Silverman Scott H. , Nomoto Koichi
Published: August 2007

Although coagulopathy and bleeding are common in the setting of cardiac surgery, a growing number of case reports in the literature suggest that hypercoagulability may also result in significant morbidity and mortality. We present a case of apparent hypercoagulability with formation of thrombus in the cardiac chambers following reoperative cardiac surgery using cardiopulmonary bypass, aprotinin, and deep hypothermic arrest. A review of those hypercoagulable disorders with reported impact on cardiac surgery and a discussion follow the case presentation.
Answers to 20 Questions About Interrater Reliability and Interrater Agreement
Authors: LeBreton James M. , Senter Jenell L.
Published: August 2007

The use of interrater reliability (IRR) and interrater agreement (IRA) indices has increased dramatically during the past 20 years. This popularity is, at least in part, because of the increased role of multilevel modeling techniques (e.g., hierarchical linear modeling and multilevel structural equation modeling) in organizational research. IRR and IRA indices are often used to justify aggregating lower-level data used in composition models. The purpose of the current article is to expose researchers to the various issues surrounding the use of IRR and IRA indices often used in conjunction with multilevel models. To achieve this goal, the authors adopt a question-and-answer format and provide a tutorial in the appendices illustrating how these indices may be computed using the SPSS software.
Investigating Faking Using a Multilevel Logistic Regression Approach to Measuring Person Fit
Authors: LaHuis David M. , Copeland Derek
Published: August 2007

This article describes how a multilevel logistic regression (MLR) approach to assessing person fit can be used to test hypotheses concerning faking on personality assessments. Item difficulty and person trait estimates obtained from a two-parameter logistic item response theory model are used to predict the probability of endorsing an item in a MLR equation. The regression slope for item difficulty reflects the extent to which the probability of endorsement decreases as item difficulty increases. Less negative slopes may indicate faking, and slope variance may be modeled with person-level variables using MLR. Two examples are presented. Example 1 models faking on a personality assessment with dichotomous items. Example 2 extends the approach to scales using polytomous items.
Assessing the quality of websites providing information on multiple sclerosis: evaluating tools and comparing sites
Authors: Harland Juliet , Bath Peter
Published: August 2007

The quality of health information available on the Internet has proved difficult to assess objectively. The Internet's growing popularity as a source of health information, accompanied by the lack of regulation of websites, has resulted in research that has developed and tested tools to evaluate health website quality. However, only a few studies have tested the validity and reliability of these tools. There is a lack of consensus about appropriate indicators with which to operationalize the concept of quality health information. This study aimed to contribute to this research by testing the validity and reliability of existing tools, through their application to websites that provided information about multiple sclerosis. Furthermore, a specific tool for evaluating multiple sclerosis information was developed, contributing to the debate about suitable criteria for measuring the `quality' of health information on the web.
How consumers search for health information
Authors: Toms Elaine G. , Latter Celeste
Published: August 2007

To date most of the research concerning consumer health information has focused on trust and quality of health information websites. In this research, we observed 48 consumers searching for four health-related topics (some of their own choosing) using Google. Using transaction logs, video screen capture, retrospective verbal protocols and self-reported questionnaires, we examined holistically the consumer's search process. Results indicate significant problems in query formulation and in making efficient selections from results lists.
Qualitative outcomes of progressive resistance exercise for people with COPD
Authors: O'Shea S.D. , Taylor N.F. , Paratz J.D.
Published: August 2007

The outcomes of quantitative investigations examining the effectiveness of exercise interventions for people with COPD are limited by the small number of measurement tools that can be included. In contrast, qualitative inquiry allows broader exploration of the perceived outcomes of an intervention. The purpose of this investigation is to explore the qualitative outcomes of a progressive resistance exercise (PRE) program for people with COPD. People with COPD, enrolled in a randomized controlled trial of PRE, were invited to participate in two semi-structured interviews conducted at the end (12 weeks) and 12 weeks after the training intervention (24 weeks). Interviews were audiotaped, transcribed and then coded independently by two researchers. Themes relating to training outcomes were then developed and described. Twenty-two participants were interviewed at 12 weeks, and 19 participants at 24 weeks. After PRE, participants reported a range of physical gains, particularly with regard to improved strength and reduced breathlessness during daily activities. Improved control and confidence during activities of daily living were important psychological benefits perceived by people with COPD, as was the social support experienced during group training sessions. At 24 weeks, confidence persisted despite a perceived plateau or dissipation of physical gains. People with COPD reported physical, psychological and social benefits after PRE, which had a positive effect on activity performance. Although the perceived physical benefits of training were not prominent at 24 weeks, feelings of increased confidence and control persisted. Chronic Respiratory Disease 2007 ;4: 135—142
Review Series: Chronic cough: Future directions in chronic cough: mechanisms and antitussives
Authors: Chung KF
Published: August 2007

Cough is an important defensive reflex of the respiratory tract needed to clear and protect the upper airways; however, it may become exaggerated and interfere with quality of life. Although chronic cough may be successfully treated when associated with the common causes such as asthma and eosinophilic bronchitis, gastrooesophageal reflux disease and post-nasal drip syndrome or rhinosinusitis, increasingly, idiopathic cough or cough with no associated cause is recognised. Chronic cough is often associated with an increased response to tussive agents such as capsaicin, used as an index of the cough reflex. Some airway receptors mediate or influence cough through activation of vagal afferent pathways which converge on brain stem respiratory networks and of supramedullary centres. Plastic changes in intrinsic and synaptic excitability at the brain stem, spinal or ganglionic level may be the mechanism by which the cough reflex is enhanced in chronic cough. Subjective and objective measurements of cough in the clinic are now available but a major unmet need in chronic cough is the availability of effective antitussives. Future directions in chronic cough include the pathophysiological mechanisms of the enhanced cough reflex, and the discovery of effective antitussives that can successfully alleviate chronic cough. Chronic Respiratory Disease 2007; 4: 159—165
Review Series: Lung function made easy: Assessing lung size
Authors: Clayton N.
Published: August 2007

Spirometry is available in most GP surgeries and provides an invaluable tool for assessing respiratory function in chronic obstructive pulmonary disease (COPD) and asthma. Spirometry alone may not provide the clinician with an accurate assessment of lung disease as it misses two important measurements of lung volume. By measuring Residual Volume (RV) and Total Lung Capacity (TLC) it is possible to determine true restrictive or hyperinflated disease processes.

Helium dilution, body plethysmography and nitrogen washout are three different methods which may be used to measure lung volume. These tests are normally only provided in the acute setting. Comparing values of RV and TLC to predicted values makes it possible to grade the severity of disease far more accurately than spirometry.

Four case studies of asthma, obesity, COPD and pulmonary fibrosis clearly demonstrate anomalies that may arise when interpreting lung disease from spirometry compared to the interpretation made with additional lung volume data. Chronic Respiratory Disease 2007; 4: 151—157
Progressive Nonfluent Aphasia Associated With a New Mutation V363I in Tau Gene
Authors: Munoz David G. , Ros Raquel , Fatas Marta , Bermejo Felix , de Yebenes Justo García
Published: August 2007

Reported here is a new missense mutation V363I in exon 12 of the microtubule-associated protein tau (MAPT) gene associated with progressive nonfluent aphasia, with onset at the age of 69 years in a woman. Although near mute, she maintained complex activities and had no discernible deficits outside of language until the age of 75 years, when progressive gait and swallowing disturbances appeared. There was a history of late-onset aphasia and apraxia in her father. All of her children were asymptomatic adults, but psycholinguistic abnormalities were detected in those bearing the mutation, consisting of difficulties in comprehension, both reading (symbol discrimination and comprehension of oral spelling) and oral (matching sentences to pictures and comprehension of locative relationships). A mutation-bearing sibling showed no abnormalities at 70 years old, consistent with the limited penetrance expected in late-onset disease. The mutation, corresponding to a highly conserved residue in the fourth tubulin-binding repeat, was not present in 194 normal individuals with the same genetic background.
Apoptotic Gene Expression in Alzheimer's Disease Hippocampal Tissue
Authors: Sajan Farrah D. , Martiniuk Frank , Marcus David L. , Frey William H. , Hite Richard , Bordayo Elizabeth Z. , Freedman Michael L.
Published: August 2007

Alzheimer's disease (AD) is the major cause of dementia, accounting for 50% to 70% of the late-onset patients, with 17 to 20 million affected. It is characterized by neurofibrillary tangles, neuronal loss, and amyloid plaques in tissues of the cortex, hippocampus, and amygdala. Apoptosis or programmed cell death appears in the progression of AD. In this study, we investigated the gene expression of 14 apoptotic genes ( E2F1, p21/WAF, ICE-LAP3, Fas Antigen, CPP-32, GADD153, ICE-β, c-Fos, c-Jun, Bax-α, Bcl-2, Bcl-(x)L, BAK, and p53) in 5 normal and 6 AD human hippocampal tissues, using reverse transcription-polymerase chain reaction. Our results show an upregulation of gene expression in AD patients for c-Fos and BAK. ICE-β, c-Jun, Bax-α, Bcl-x(L), p53, and GADD153 were found to be upregulated in some AD samples but were not detected or downregulated in other AD or normal samples. No gene expression was found for E2F1 , p21/WAF, ICE-LAP3, Fas Antigen, CPP32, or Bcl-2. These results indicate significant increases in c-Fos , c-Jun, and Bak; therefore, we suggest that these genes may be critical in the apoptotic cascades of AD.
A Quick Test for Cognitive Speed: A Measure of Cognitive Speed in Dementia With Lewy Bodies
Authors: Andersson Maria , Wiig Elisabeth H. , Minthon Lennart , Londos Elisabet
Published: August 2007

The purpose of this article is to investigate how patients with dementia with Lewy bodies (DLB) perform on A Quick Test for Cognitive Speed (AQT) compared with patients with Alzheimer's disease (AD) and age- and sex-matched controls and to see whether this test might be helpful in distinguishing DLB from AD at comparable cognitive levels. Twenty-three patients with DLB, 18 patients with AD, and 24 controls were included. The time in seconds to complete the AQT was recorded for the 3 independent study groups according to standard directives. The DLB patients had significantly longer reading times than the AD patients at equivalent and relatively high Mini-Mental State Examination levels. We suggest that slow performance on the AQT at relatively high Mini-Mental State Examination levels could be one way of distinguishing DLB from AD. This may have clinical implications for treatment as well as for understanding the neuropathological properties of the disease.
Job Satisfaction and Career Commitment Among Nursing Assistants Providing Alzheimer's Care
Authors: Coogle Constance L. , Parham Iris A. , Young Katherine A.
Published: August 2007

As part of a statewide dementia-specific training collaborative, data were collected from nursing assistants and aides who provide paid Alzheimer's care. This study explored the relevance of previous education and practical experiences to specific constructs associated with worker recruitment and retention. Direct-care providers with prior training in gerontology and geriatrics had lower levels of extrinsic job satisfaction and career resilience than those without this kind of continuing education. Program participants who were currently or previously the primary caregiver for a friend or relative with Alzheimer's disease had higher levels of intrinsic job satisfaction but lower levels of career resilience than those with no informal caregiving experience. Current or previous informal caregiving experience may enhance intrinsic job satisfaction by increasing personal commitment to pursue formal care work and providing a kind of inoculation against the demoralization that is too often suffered in these very challenging jobs.
Preserved Implicit Memory in Dementia: A Potential Model for Care
Authors: Harrison Barbara E. , Son Gwi-Ryung , Kim Jiyoung , Whall Ann L.
Published: August 2007

A growing body of evidence supports the presence of a preserved implicit memory (PIM) system for persons with Alzheimer's disease (AD). This article describes a new approach to dementia care, the PIM model, which translates evidence from implicit memory research into a practice model of dementia care. The PIM model predicts that function can be sustained longer for persons with AD through interventions and environments that activate an individual's PIM. Activation of PIM can occur with perceptual priming of familiar objects and reinforcement of learned motor skill memories within tasks. This practice model provides a new framework for planning and implementing dementia care that may preserve function for persons with Alzheimer's dementia.
Evidence for MPL W515L/K mutations in hematopoietic stem cells in primitive myelofibrosis
Authors: Chaligné Ronan , James Chloé , Tonetti Carole , Besancenot Rodolphe , Le Couédic Jean Pierre , Fava Fanny , Mazurier Fréderic , Godin Isabelle , Maloum Karim , Larbret Frédéric , Lécluse Yann , Vainchenker William , Giraudier Stéphane
Published: August 2007

The MPL (W515L and W515K) mutations have been detected in granulocytes of patients suffering from certain types of primitive myelofibrosis (PMF). It is still unknown whether this molecular event is also present in lymphoid cells and therefore potentially at the hematopoietic stem cell (HSC) level. Toward this goal, we conducted MPL genotyping of mature myeloid and lymphoid cells and of lymphoid/myeloid progenitors isolated from PMF patients carrying the W515 mutations. We detected both MPL mutations in granulocytes, monocytes, and platelets as well as natural killer (NK) cells but not in T cells. B/NK/myeloid and/or NK/myeloid CD34+CD38−-derived clones were found to carry the mutations. Long-term reconstitution of MPL W515 CD34+ cells in nonobese diabetic/severe combined immunodeficient (NOD/SCID) mice was successful for as long as 12 weeks after transplantation, indicating that MPL W515 mutations were present in HSCs. Moreover, the 2 MPL mutations induced a spontaneous megakaryocytic growth in culture with an overall normal response to thrombopoietin (TPO). In contrast, erythroid progenitors remained EPO dependent. These results demonstrate that in PMF, the MPL W515L or K mutation induces a spontaneous megakaryocyte (MK) differentiation and occurs in a multipotent HSCs.
The PRKAR1A gene is fused to RARA in a new variant acute promyelocytic leukemia
Authors: Catalano Alberto , Dawson Mark A. , Somana Karthiga , Opat Stephen , Schwarer Anthony , Campbell Lynda J. , Iland Harry
Published: August 2007

We report the molecular and cytogenetic characterization of a novel variant of acute promyelocytic leukemia (APL). The bone marrow showed 88% hypergranular promyelocytes, and the karyotype was 47,XY,+22 [5]/46,XY[30]. Fluorescence in situ hybridization (FISH) indicated disruption and deletion of the 5′-end of the RARA gene. Treatment with all-trans retinoic acid, idarubicin, and arsenic trioxide induced cytogenetic complete remission without morphologic evidence of residual leukemia. The diagnostic marrow was negative for PML-RARA transcripts by reverse transcription–polymerase chain reaction (RT-PCR), but an atypical product was observed. Sequencing showed partial homology to the PRKAR1A gene, encoding the regulatory subunit type I-α of cyclic adenosine monophosphate–dependent protein kinase. RT-PCR using specific primers for PRKAR1A and RARA amplified 2 transcript splice variants of a PRKAR1A-RARA fusion gene, and PRKAR1A and RARA FISH probes confirmed the fusion. This novel PRKAR1A-RARA gene rearrangement is the fifth variant APL in which the RARA partner gene has been identified and the second known rearrangement of PRKAR1A in a malignant disease. This trial was registered at www.actr.org.au with the Australian Clinical Trials Registry as number 12605000070639.
JAK2 V617F mutational status predicts progression to large splenomegaly and leukemic transformation in primary myelofibrosis
Authors: Barosi Giovanni , Bergamaschi Gaetano , Marchetti Monia , Vannucchi Alessandro M. , Guglielmelli Paola , Antonioli Elisabetta , Massa Margherita , Rosti Vittorio , Campanelli Rita , Villani Laura , Viarengo Gianluca , Gattoni Elisabetta , Gerli Giancarla , Specchia Giorgina , Tinelli Carmine , Rambaldi Alessandro , Barbui Tiziano
Published: August 2007

Few investigators have evaluated the usefulness of the JAK2 V617F mutation for explaining the phenotypic variations and for predicting the risk of major clinical events in primary myelofibrosis (PMF). In a transversal survey we assayed by allele-specific polymerase chain reaction (PCR) the JAK2 V617F mutational status in 304 patients with PMF. Multiple DNA samples were collected prospectively from 64 patients, and a highly sensitive quantitative PCR was used as a confirmatory test. In a longitudinal prospective study we determined the progression rate to clinically relevant outcomes in 174 patients who had JAK2 mutation determined at diagnosis. JAK2 V617F was identified in 63.4% of patients. None of the V617F-negative patients who were sequentially genotyped progressed to become V617F positive, whereas progression rate from heterozygous to homozygous mutation was 10 per 100 patient-years. JAK2 V617F mutation contributed to hemoglobin, aquagenic pruritus, and platelet count variability, whereas homozygous mutation was independently associated with higher white blood cell count, larger spleen size, and greater need for cytoreductive therapies. Adjusting for conventional risk factors, V617F mutation independently predicted the evolution toward large splenomegaly, need of splenectomy, and leukemic transformation. We conclude that JAK2 V617F genotype should be considered in any future risk stratification of patients with PMF.
A highly conserved regulatory element controls hematopoietic expression of GATA-2in zebrafish
Authors: Yang Zhongan , Jiang Hong , Zhao Fang , Shankar Deepa B , Sakamoto Kathleen M , Zhang Michael Q , Lin Shuo
Published: August 2007

Background

GATA-2 is a transcription factor required for hematopoietic stem cell survival as well as for neuronal development in vertebrates. It has been shown that specific expression of GATA-2 in blood progenitor cells requires distal cis-acting regulatory elements. Identification and characterization of these elements should help elucidating transcription regulatory mechanisms of GATA-2 expression in hematopoietic lineage.

Results

By pair-wise alignments of the zebrafish genomic sequences flanking GATA-2 to orthologous regions of fugu, mouse, rat and human genomes, we identified three highly conserved non-coding sequences in the genomic region flanking GATA-2, two upstream of GATA-2 and another downstream. Using both transposon and bacterial artificial chromosome mediated germline transgenic zebrafish analyses, one of the sequences was established as necessary and sufficient to direct hematopoietic GFP expression in a manner that recapitulates that of GATA-2. In addition, we demonstrated that this element has enhancer activity in mammalian myeloid leukemia cell lines, thus validating its functional conservation among vertebrate species. Further analysis of potential transcription factor binding sites suggested that integrity of the putative HOXA3 and LMO2 sites is required for regulating GATA-2/GFP hematopoietic expression.

Conclusion

Regulation of GATA-2 expression in hematopoietic cells is likely conserved among vertebrate animals. The integrated approach described here, drawing on embryological, transgenesis and computational methods, should be generally applicable to analyze tissue-specific gene regulation involving distal DNA cis-acting elements.
Patterns and correlates of physical activity: a cross-sectional study in urban Chinese women
Authors: Jurj Adriana L , Wen Wanqing , Gao Yu-Tang , Matthews Charles E , Yang Gong , Li Hong-Lan , Zheng Wei , Shu Xiao-Ou
Published: August 2007

Background

Inactivity is a modifiable risk factor for many diseases. Rapid economic development in China has been associated with changes in lifestyle, including physical activity. The purpose of this study was to investigate the patterns and correlates of physical activity in middle-aged and elderly women from urban Shanghai.

Methods

Study population consisted of 74,942 Chinese women, 40–70 years of age, participating in the baseline survey of the Shanghai Women's Health Study (1997–2000), an ongoing population-based cohort study. A validated, interviewer-administered physical activity questionnaire was used to collect information about several physical activity domains (exercise/sports, walking and cycling for transportation, housework). Correlations between physical activity domains were evaluated by Spearman rank-correlation coefficients. Associations between physical activity and socio-demographic and lifestyle factors were evaluated by odds ratios derived from logistic regression.

Results

While more than a third of study participants engaged in regular exercise, this form of activity contributed only about 10% to daily non-occupational energy expenditure. About two-thirds of women met current recommendations for lifestyle activity. Age was positively associated with participation in exercise/sports and housework. Dietary energy intake was positively associated with all physical activity domains. High socioeconomic status, unemployment (including retirement), history of chronic disease, small household, non-smoking status, alcohol and tea consumption, and ginseng intake were all positively associated with exercise participation. High socioeconomic status and small household were inversely associated with non-exercise activities.

Conclusion

This study demonstrates that physical activity domains other than sports and exercise are important contributors to total energy expenditure in women. Correlates of physical activity are domain-specific. These findings provide important information for research on the health benefits of physical activity and have public health implications for designing interventions to promote participation in physical activity.
Proteomic analyses of retina of excitatory amino acid carrier 1 deficient mice
Authors: Okumichi Hideaki , Kanamoto Takashi , Souchelnytskyi Nazariy , Tanimoto Seiji , Tanaka Kohichi , Kiuchi Yoshiaki
Published: August 2007

Background

Excitatory amino acid carrier 1 (EAAC1) is a glutamate transporter found in neuronal tissues and is extensively expressed in the retina. EAAC1 plays a role in a variety of neural functions, but its biological functions in the retina has not been fully determined. The purpose of this study was to identify proteins regulated by EAAC1 in the retina of mice. To accomplish this, we used a proteomics-based approach to identify proteins that are up- or down-regulated in EAAC1-deficient (EAAC1-/-) mice.

Results

Proteomic analyses and two-dimensional gel electorphoresis were performed on the retina of EAAC1-/- mice, and the results were compared to that of wild type mice. The protein spots showing significant differences were selected for identification by mass spectrometric analyses. Thirteen proteins were differentially expressed; nine proteins were up-regulated and five proteins were down-regulated in EAAC1-/- retina. Functional clustering showed that identified proteins are involved in various cellular process, e.g. cell cycle, cell death, transport and metabolism.

Conclusion

We identified thirteen proteins whose expression is changed in EAAC-/- mice retinas. These proteins are known to regulate cell proliferation, death, transport, metabolism, cell organization and extracellular matrix.
Coexistence of gastrointestinal stromal tumor (GIST) and colorectal adenocarcinoma: A case report
Authors: Efstathios Papalambros , Athanasios Petrou , Papaconstantinou Ioannis , Alexandros Papalambros , Frangisca Sigala , Sotirios Georgopoulos , Evangelos Felekouras , Athanasios Giannopoulos
Published: August 2007

Background

Gastrointestinal stromal tumors (GIST) represent the most common mesenchymal tumors of the digestive tract. Over the last ten years the management of GISTs has dramatically altered but their coexistence with other gasrointesinal tumors of different histogenesis presents a special interest. The coexistence of GISTs with other primaries is usually discovered incidentally during GI surgery for carcinomas.

Case presentation

We present here, a case of a 66-year-old patient with intestinal GIST and a synchronous colorectal adenocarcinoma discovered incidentally during surgical treatment of the recurrent GIST. Immunohistochemical examination revealed the concurrence of histologically proved GIST (strongly positive staining for c-kit, vimentin, SMA, and focal positive in S-100, while CD-34 was negative) and Dukes Stage C, (T3, N3, M0 according the TNM staging classification of colorectal cancer).

Conclusion

The coexistence of GIST with either synchronous or metachronous colorectal cancer represents a phenomenon with increasing number of relative reports in the literature the last 5 years. In any case of GIST the surgeon should be alert to recognize a possible coexistent tumor with different histological origin and to perform a thorough preoperative and intraoperative control. The correct diagnosis before and at the time of the surgical procedure is the cornerstone that secures the patients' best prognosis.
Circulating Bmi-1 mRNA as a possible prognostic factor for advanced breast cancer patients
Authors: Silva Javier , García Vanesa , García José M , Peña Cristina , Domínguez Gemma , Díaz Raquel , Lorenzo Yolanda , Hurtado Alicia , Sánchez Antonio , Bonilla Félix
Published: August 2007

Introduction

Deregulation of Polycomb member Bmi-1 is involved in cell proliferation and human oncogenesis. Modulation of Bmi-1 is found in several tumor tissues, including primary breast carcinomas; however, analysis of Bmi-1 in plasma of cancer patients has not been reported. This is the first study that evaluates Bmi-1 in plasma by using a large series of primary breast carcinomas to investigate the presence at diagnosis of detectable Bmi-1 mRNA in plasma and possible correlations between this event and a series of clinical-pathological parameters of the tumors.

Methods

Bmi-1 expression levels were quantified in plasma of 111 breast cancer patients and in 20 healthy controls by real-time quantitative polymerase chain reaction.

Results

Cancer patients with the presence of Bmi-1 mRNA in plasma had higher levels of Bmi-1 expression than healthy controls with Bmi-1 mRNA in plasma. The higher expression levels of Bmi-1 correlated with well-established markers of poor clinical outcome in breast cancer such as positive p53 immunostaining and negative progesterone receptors. Moreover, we described for the first time a statistically significant correlation between Bmi-1 expression in plasma of breast cancer patients and disease-free and overall survival in advanced stages.

Conclusion

Our results suggest that levels of Bmi-1 expression may be a surrogate marker of poor prognosis and may become clinically useful as noninvasive diagnostic markers.