bluuebunny/crossref_metadata_2025 huggingface dataset:
Restless Legs Syndrome Is Common among Female Patients with Fibromyalgia
Authors: Stehlik Romana , Arvidsson Lennart , Ulfberg Jan
Published: December 2008

Background: The prevalence of restless legs syndrome (RLS) among the general population is 2–15%, and with fibromyalgia syndrome (FMS) 2%. Both RLS and FMS are more common among women. The aim of our study was to evaluate the prevalence of RLS in a group of female patients diagnosed with FMS and to compare the occurrence of symptoms of daytime sleepiness and experienced sleep disorders between fibromyalgia patients with or without RLS. Method: Three hundred and thirty-two female patients, 20–60 years old, diagnosed with FMS at Skönviks Rehab between 2002 and 2006, answered a questionnaire mailed to their home address. The questionnaire consisted of the international RLS study group criteria as well as of questions concerning symptoms of insomnia and daytime sleepiness measured according to the Epworth Sleepiness Scale. Results: Nearly 64% of the women were also suffering from RLS. More patients suffering from both RLS and FMS were affected by problems of initiating and maintaining sleep than those suffering from FMS only. More patients suffering from both RLS and FMS did not feel refreshed on awakening compared with those suffering from FMS without RLS. The patients with concomitant RLS and FMS were more often hypersomnolent than those suffering from FMS only. Conclusion: This study shows that 64% of a group of female patients diagnosed with fibromyalgia also concurrently suffered from RLS. Compared with patients who suffer from FMS only, patients with both FMS and RLS more often experience sleep disturbances and pronounced daytime sleepiness.
Sir Samuel Wilks (1824–1911): ‘The Most Philosophical of English Physicians’
Authors: Pearce J.M.S.
Published: December 2008

This paper retells some of the achievements and personal attributes of Sir Samuel Wilks, one of the great Guy’s Hospital physicians and neurologists of the second half of the 19th century. He was a fellow of the Royal Society, president of the Royal College of Physicians, and physician extraordinary to Queen Victoria. A prolific author and original observer of clinical and pathological diseases, he was renowned for his Lectures on Pathological Anatomy, and his original descriptions of syphilis, epilepsy, inflammatory bowel diseases, and myasthenia gravis.
SPECT-Identified Neuroanatomical Predictor of the Cognitive Effects of Donepezil Treatment in Patients with Alzheimer’s Disease
Authors: Hongo Jin , Nakaaki Shutaro , Shinagawa Yoshihiro , Murata Yoshie , Sato Junko , Tatsumi Hiroshi , Tohyama Junko , Soma Tsutomu , Iidaka Tetsuya , Fukui Toshiya , Mimura Masaru , Furukawa Toshiaki A.
Published: December 2008

Background/Objective: We attempted to determine whether the pretreatment regional cerebral blood flow (rCBF) might predict cognitive changes in response to donepezil treatment, as assessed in terms of the Alzheimer Disease Assessment Scale cognitive subscale (ADAS-cog), and in relation to the severity of subcortical hyperintensities (SH). Method: Forty-one patients with Alzheimer’s disease (AD) were treated with donepezil at baseline. All the patients underwent a single photon emission computed tomography examination before donepezil therapy. They also completed the ADAS-cog at baseline and after 24 weeks of donepezil therapy. SH were assessed semiquantitatively using a recently developed visual rating scale. We analyzed the correlation between the baseline rCBF and changes in the ADAS-cog score using statistical parametric mapping, including the severity of the SH as a covariate. Results: Lower pretreatment rCBF levels in the right orbitofrontal cortex (OFC) predicted a better improvement in the ADAS-cog score in response to donepezil therapy. The severity of SH did not appear to influence this correlation. Conclusions: This effect may reflect the choline acetyltransferase activity associated with the OFC. The presence of SH did not appear to influence the effect of donepezil therapy on the cognitive function as assessed by ADAS-cog.
Influence of Conjunctival Suture Removal on the Outcome of Trabeculectomy
Authors: Klink Thomas , Rauch Nicola , Klink Janine , Grehn Franz
Published: December 2008

Background: To evaluate the influence of early conjunctival suture removal following trabeculectomy on postoperative outcome. Methods: In a prospective randomized clinical trial 73 eyes of 69 consecutive patients eligible for primary trabeculectomy were included in the study. Eyes were randomly assigned to a control or intervention group. Trabeculectomy was performed with a fornix-based conjunctival flap and a conjunctival running mattress-suture. In the intervention group, the conjunctival suture was removed 4 weeks postoperatively, in the control group the suture remained in place. Both groups were followed for up to 12 months and best corrected visual acuity (BCVA), refraction, corneal topography, intraocular pressure (IOP), anterior segment and fundus were assessed preoperatively and within a week, 1, 3, 6 and 12 months after surgery. Statistical analysis was performed using SPSS for Windows (t test for independent samples). Results: Trabeculectomy induced similar astigmatism in both groups. Compared to the control group, suture removal seemed to improve BCVA at 6 and 12 months. This was associated with a significantly lower IOP at 6 and 12 months and a significantly higher complete success rate at 12 months (94 vs. 65%, p < 0.001). Astigmatism was not significantly altered by suture removal. Conclusion: Conjunctival suture material appears to hamper trabeculectomy success and postoperative visual acuity. This is independent of postoperative astigmatism. Early removal of conjunctival sutures is advisable.
Di-(2-Ethylhexyl)Phthalate Leakage and Color Changes in Endotracheal Tubes after Application in High-Risk Newborns
Authors: Latini Giuseppe , De Felice Claudio , Del Vecchio Antonio , Barducci Alessandro , Ferri Marcella , Chiellini Federica
Published: December 2008

Background: Polyvinylchloride (PVC) is often adopted for making medical devices. Objective: Testing the hypothesis that materials degradation occurs in PVC endotracheal tubes during infant ventilation, thus releasing the known toxic plasticizer di-(2-ethylhexyl)phthalate (DEHP). Materials and Methods: Endotracheal tubes degradation was assessed by: (1) analysis of color and spectral changes in endotracheal tubes after use in the 400- to 700-nm range and compared to virgin samples. Color changes were expressed as euclidean distances in the Commission International de l’Eclairage Laboratory and Lightness-Chroma-Hue (Munsell’s) color spaces (i.e., ΔE, ΔL, Δa, Δb, ΔC, and ΔH units); (2) DEHP leakage was assessed by thermal characterization by differential scanning calorimetry (DSC) and thermal gravimetric analysis (TGA). Data from used tubes were compared with those from either virgin devices or submitted to artificial aging, including O2-induced oxidation, washing, UV photodegradation and exposition to acid solutions. Results: Significant color differences of 13.33 ± 0.85 ΔE were evidenced in used endotracheal tubes, as compared to virgin samples (p < 0.0001). Chromatic changes were independent from intubation duration. DSC and TGA analyses showed a loss of DEHP from used tubes, as indicated by increase of the glass transition temperature (Tg) and DEHP weight loss. In vivo application of tubes was associated with an aging process whose effects on the optical and spectral properties were similar to those observed following artificial aging processes. Conclusions: Our findings indicate that significant spectrocolorimetric, DSC and TGA changes occur in endotracheal tubes after application, thus demonstrating for the first time the occurrence of in vivomaterials degradation and DEHP leakage from medical devices worldwide used for neonatal ventilation.
In vivo Dilatation of the Ductus Arteriosus by Rho Kinase Inhibition in the Rat
Authors: Momma Kazuo , Toyoshima Katsuaki , Sun Fang , Nakanishi Toshio
Published: December 2008

Background: Fasudil hydrochloride, a Rho kinase inhibitor, was reported to dilate the constricted ductus arteriosus in vitro, and was suggested as a neonatal bridge to heart surgery. Objectives: To clarify the in vivo effectiveness of fasudil to dilate the neonatal ductus arteriosus, and its usefulness as a bridge to heart surgery. Methods: We studied the dose and timing of administration of fasudil in the neonatal rat. Postnatal ductal closure was studied on the 21st gestational day following cesarean section and incubation for 30–480 min in room air at 33°C, with a rapid whole-body freezing method. In control rats, the ductus closed rapidly after birth, and the ductal diameter was 0.80 and 0.12 mm at 0 and 30 min after birth. Fasudil was injected peritoneally into the neonate, and the ductus was studied 30 min after injection and 60 min after birth. Results: Fasudil, 10 mg/kg, injected within 5 min after birth, dilated the ductus completely to 0.8 mm, and the dose used clinically, 1 mg/kg, dilated the ductus to 0.4 mm. The ductus-dilating effect of fasudil decreased rapidly as the neonatal ductus constricted. Fasudil, 100 mg/kg, dilated the ductus completely, but induced severe respiratory depression and frequent death in 1-hour-old rats. Conclusions: Fasudil dilates the neonatal ductus arteriosus dose-dependently in the rat. Fasudil in large doses dilates the neonatal ductus completely, but is associated with fatal side effects, including respiratory depression.
Pharmacogenetic Predictors of Statin-Mediated Low-Density Lipoprotein Cholesterol Reduction and Dose Response
Authors: Voora Deepak , Shah Svati H. , Reed Carol R. , Zhai Jun , Crosslin David R. , Messer Chad , Salisbury Benjamin A. , Ginsburg Geoffrey S.
Published: December 2008

Background—

There is interindividual variation in low-density lipoprotein cholesterol (LDLc) lowering by statins and limited study into the genetic associations of the dose dependant LDLc lowering by statins.

Methods and Results—

Five hundred nine patients with hyperlipidemia were randomly assigned atorvastatin 10 mg, simvastatin 20 mg, or pravastatin 10 mg (low-dose phase) followed by 80 mg, 80 mg, and 40 mg (high-dose phase), respectively. Thirty-one genes in statin, cholesterol, and lipoprotein metabolism were sequenced and 489 single nucleotide polymorphisms with minor allele frequencies >2% were tested for associations with percentage LDLc lowering at low doses using multivariable adjusted general linear regression. Significant associations from the analysis at low dose were then repeated at high-dose statins. At low doses, only 1 single nucleotide polymorphism met our experiment-wide significance level,
ABCA1
rs12003906. Twenty-six subjects carried the minor allele of rs12003906, which was associated with an attenuated LDLc reduction (LDLc reduction in carriers versus noncarriers −24.1�2.6% versus −32.2�1.5%;
P
=0.0001). In addition, we replicated the association with the
APOE
ε3 allele and a reduced LDLc reduction. At high doses, carriers of the minor allele of
ABCA1
rs12003906 and the
APOE
ε3 allele improved their LDLc reduction but continued to have a diminished LDLc reduction compared with noncarriers (−30.5�4.0% versus −42.0�2.4%;
P
=0.005) and (−38.5�1.9% versus −45.3�2.8%;
P
=0.009), respectively.

Conclusions—

An intronic single nucleotide polymorphism in
ABCA1
and the
APOE
ε3 allele are associated with reduced LDLc lowering by statins and identify individuals who may be resistant to maximal LDLc lowering by statins.
Response to a Steady Poleward Outflow. Part II: Oscillations and Eddies
Authors: Durland Theodore S. , Spall Michael A. , Pedlosky Joseph
Published: December 2008

A conceptually simple model is presented for predicting the amplitude and periodicity of eddies generated by a steady poleward outflow in a 1½-layer β-plane formulation. The prediction model is rooted in linear quasigeostrophic dynamics but is capable of predicting the amplitude of the β plume generated by outflows in the nonlinear range. Oscillations in the plume amplitude are seen to represent a near-zero group velocity response to an adjustment process that can be traced back to linear dynamics. When the plume-amplitude oscillations become large enough so that the coherent β plume is replaced by a robust eddy field, the eddy amplitude is still constrained by the plume-amplitude prediction model. The eddy periodicity remains close to that of the predictable, near-zero group-velocity linear oscillations.

Striking similarities between the patterns of variability in the model and observations south of Indonesia’s Lombok Strait suggest that the processes investigated in this study may play an important role in the generation of the observed eddy field of the Indo-Australian Basin.
Dynamic Assessment and Response to Intervention
Authors: Grigorenko Elena L.
Published: December 2008

This article compares and contrasts the main features of dynamic testing and assessment (DT/A) and response to intervention (RTI). The comparison is carried out along the following lines: (a) historical and empirical roots of both concepts, (b) premises underlying DT/A and RTI, (c) terms used in these concepts, (d) use of these concepts, (e) evidence in support of DT/A and RTI, and (f) expectations associated with each of the concepts. The main outcome of this comparison is a conclusion that both approaches belong to one family of methodologies in psychology and education whose key feature is in blending assessment and intervention in one holistic activity. Because DT/A has been around much longer than RTI, it makes sense for the proponents of RTI to consider both the accomplishments and frustrations that have accumulated in the field of DT/A.
Does Community Resource Fit Matter to Fathers?
Authors: Barnett Rosalind Chait , Gareis Karen C.
Published: December 2008

Several scholars have noted that community resources might facilitate or hinder employees' ability to meet their many work and family demands, thereby affecting their psychological well-being. However, this is the first study to estimate these relationships using a newly developed quantitative measure of community resource fit that assesses the satisfaction of employed parents of school-aged children with key community resources. In this analysis, the authors focus on the relationships linking one aspect of community resource fit—specifically, child's school and school activity schedules, or school resource fit—as a contextual variable influencing well-being (i.e., job-role quality, psychological distress) in a sample of 53 employed married fathers. Among these fathers, having school and school activity schedules that meet their needs is associated with low psychological distress and high job-role quality. Importantly, fathers with fewer resources in terms of income or job flexibility benefit most in terms of their job-role quality.
Occultism, Race and Politics in German-speaking Europe, 1880—1940: A Survey of the Historical Literature
Authors: Staudenmaier Peter
Published: December 2008

In recognition of a recent renewal of scholarly interest in nineteenth- and twentieth-century German occultism, this review essay surveys the variety of existing historical treatments of the topic and points to possible directions for further research. Sources examined range from the 1960s to the present, in German and English, with attention to broader European parallels. Among other debates, a central focus is the nature and extent of the historical relationship between occultism and the völkisch milieu as well as National Socialism. In addition to a detailed overview of the available literature, the essay appraises the strengths and weaknesses of current scholarship on the modern German occult revival and argues for several specific interpretive possibilities regarding contentious issues of racial theory and the political affiliations of occult and esoteric thought.
Clinical dilemmas in children with life-limiting illnesses: decision making and the law
Authors: Wright B , Aldridge J , Wurr K , Sloper T , Tomlinson H , Miller M
Published: December 2008

Decision making about interventions for children and young people with life-limiting illnesses is fraught with difficulties but faced regularly by staff in children’s hospices and paediatric wards. The perspectives of the child, various family members and professionals may all be different. The process of discussion and negotiation and the mechanism by which a decision is arrived at is complex. Various laws have recently changed in the UK that have an impact on this process. This article discusses several clinical scenarios to better understand these decisions and the effects of changes in the law. It also discusses how multidisciplinary teams in children’s hospices (and other supportive clinical systems) can best support young people and families with and without recourse to the law.
A profile of the belief system and attitudes to end-of-life decisions of senior clinicians working in a National Health Service Hospital in the United Kingdom
Authors: Pugh EJ , Song R , Whittaker V , Blenkinsopp J
Published: December 2008

There is evidence from outside the United Kingdom to show that physicians’ religious beliefs influence their decision making at the end of life. This UK study explores the belief system of consultants, nurse key workers and specialist registrars and their attitudes to decisions which commonly must be taken when caring for individuals who are dying. All consultants ( N = 119), nurse key workers ( N = 36) and specialist registrars ( N = 44) working in an acute hospital in the north-east of England were asked to complete a postal questionnaire. In all, 65% of consultants, 67% of nurse key workers and 41% of specialist registrars responded. Results showed that consultants’ religion and belief systems differed from those of nurses and the population they served. Consultants and nurses had statistically significant differences in their attitudes to common end of life decisions with consultants more likely to continue hydration and not withdraw treatment. Nurses were more sympathetic to the idea of physician-assisted suicide for unbearable suffering. This study shows the variability in belief system and attitudes to end of life decision making both within and between clinical groups. This may have practical implications for the clinical care given and the place of care. The personal belief system of consultants was not shown to affect their overall attitudes to withdrawing life-sustaining treatment or physician-assisted suicide.
Public Service Motivation in Undergraduate Giving and
Authors: Clerkin Richard M. , Paynter Sharon R. , Taylor Jami Kathleen
Published: December 2008

Most public service motivation (PSM) research compares government and business employees. This article fits into an emerging body of research that links PSM to volunteer activity. PSM is a needs-based approach to motivation. People may sate this need in ways other than direct government service. In this article, the authors investigate the relationship between PSM and charitable decisions. They surveyed undergraduate students at North Carolina State University using Perry’s PSM instrument and antecedent questions. To further investigate students’ motivations toward public service, they asked an additional series of questions focused on volunteering and donating choices. The authors find that students with higher levels of PSM are more likely to choose to engage in charitable activity. Individual characteristics such as family income, political identity, sex, religiosity, family socialization, and high school volunteering experiences are also significantly related to the choices students make about engaging in charitable activities.
County Limits
Authors: Choi Sang Ok , Bae Sang-Seok , Kwon Sung-Wook , Feiock Richard
Published: December 2008

This study extends Peterson’s city limits perspective to counties to empirically examine how economic, political, institutional, and demographic factors influence overall county spending and spending priorities across three core policy arenas. Pooled cross-sectional time-series analysis of Florida counties reveals that although population density and economic conditions influence spending, politics and institutions matter. Political ideology as indicated by presidential and gubernatorial Democratic vote share has a positive influence on all three spending categories. The findings also reveal that county adoption of a home rule charter leads to greater emphasis on developmental and redistributive rather than allocational functions.
Music, movement and marimba: an investigation of the role of movement and gesture in communicating musical expression to an audience
Authors: Broughton Mary , Stevens Catherine
Published: December 2008

The experiment reported in this article investigated the assumption that visual movement plays a role in musician-to-audience communication in marimba performance. Body movement is of particular relevance here as the expressive capabilities of the marimba are relatively restricted, and the movements required to play it are visible. Twenty-four musically trained and 24 musically untrained observers rated auditory-only and auditory-visual presentations of 20th-century solo marimba excerpts for perceived expressiveness and interest. Performances were given by a male and a female professional musician in projected (public performance expression) and deadpan (minimized expressive features) performance manners. As hypothesized, higher ratings were recorded in response to projected performances than to deadpan. The hypothesized interaction between modality and performance manner was observed. Musically trained participants recorded higher ratings than musically untrained observers, upholding the final hypothesis. Expressive body movement plays an important role in the communication between marimba performer and audience — a role relevant for both performers and educators.
What radio can do to increase a song's appeal: a study of Canadian music presented to American college students
Authors: Silva Kathleen M. , Silva Francisco J.
Published: December 2008

Four experiments examined whether it was possible to quickly and easily increase the appeal of unfamiliar rock songs presented to American college students. In Experiment 1, reading an essay about an artist increased the appeal of the artist's songs, but repeated exposure to the songs did not. In Experiments 2a and 2b, repeatedly following an affectively neutral song with a liked song increased the appeal of the first song. In Experiment 3, listening to a music critic praise a song increased the song's appeal. These results show that intramusical and intrapersonal strategies, evaluative conditioning, and persuasion by authority can be used to increase a song's appeal. It should be possible for radio stations to use these cost-effective techniques to expand their playlists without a net loss of listeners.
Diffuse Onset of Ictal Electroencephalography in a Typical Case of Panayiotopoulos Syndrome and Review of the Literature
Authors: Iannetti Paola , Spalice Alberto , Rocchi Valerio , Verrotti Alberto
Published: December 2008

Panayiotopoulos syndrome is a common and benign childhood autonomic epilepsy of debated localization. Although officially considered as occipital epilepsy, this is most likely of multifocal origin. Ictal electroencephalography is the gold standard of seizure localization, but in Panayiotopoulos syndrome, because patients have single or rare seizures, only 7 cases with ictal electroencephalography have been reported. Ictal onsets show variable anterior and more often posterior locations. We describe an 8-year-old girl with 5 nocturnal autonomic seizures typical of Panayiotopoulos syndrome from age 4. The last seizure was captured with electroencephalography and showed a diffuse onset of the ictal discharge, whereas various interictal electroencephalography had infrequent multifocal spikes. This case contributes to the understanding of the pathophysiology of Panayiotopoulos syndrome in favor of a diffuse and multifocal cortical epileptogenicity that triggers an unstable central autonomic nervous system solely or prior to the focal cortical symptoms
The Role of Public Agencies in the Policy Making Process
Authors: Verschuere Bram
Published: December 2008

This article focuses on the role public agencies play in the policy making process. Based on a single embedded case-study of one Flemish public agency and two policy-programmes in which this agency is involved, the normative practitioner model of the policy/operations divide is tested. This model assumes (or prescribes) that policy making is a political prerogative, while public agencies as administrative actors should stick to implementing policy. The evidence shows that reality is more complex than the rhetoric of the practitioner model. Agencies may be more involved in policy preparation and policy decision-making than assumed, and political actors may in some cases have a large say in policy implementation. The evidence from my case-study shows that in reality the policy/operations divide might not be that clear-cut, and that this normative model should be revised on some points. Next to that, the findings may be discussed in the light of the current administrative reform projects in various countries that propagate a strict labour division between the political sphere (policy) and the administrative sphere (implementing policy). This discussion is particularly relevant for the Flemish public sector that is currently facing a major reform of that kind.
Partnerships in Health and Social Care
Authors: Evans Debbie , Forbes Tom
Published: December 2008

Since 1997 partnership working across the public sector has been a key theme of UK Government and Scottish Executive policy. Both Governments' policy approaches initially converged on this theme. However, while the UK Government has become lukewarm to the use of partnership working to deliver public services, the Scottish Executive has remained true to the partnership ethos. This article compares approaches to partnership working in health and social care between the UK Government and the Scottish Executive using a qualitative methodology based on semi-structured interviews two English and two Scottish health and social care partnerships are examined with regard to policy implementation of both Government's partnership agenda. The UK Government appears to have been confused over its aims and objectives for health and social care, while the Scottish Executive on the other hand has followed a more consistent approach beginning with the integration of primary and secondary health services and desire to integrate health and social services. However, in what appears to be a major flaw in policy, both the UK Government and the Scottish Executive have privileged the NHS as the main player in their health and social care partnership designs at times alienating the Local Authorities. As a result, there has been a missed the opportunity to develop true health and social care partnerships in the UK that are fully inclusive of all partners and instead we have seen the retention of many historical antecedents to effective joint working between the NHS and Local Authorities.
Australia's Regions
Authors: Everingham Jo-Anne
Published: December 2008

The article uses the example of a region in the Australian State of Queensland to illustrate the emergence during the 1990s of a plethora of regional organizations including Catchment Coordinating Committees, Regional Development Organisations and Regional Organisations of Councils. Some have regarded this profusion of sub-national bodies as diminishing the role of the state. Certainly one result was ambiguity in the roles and responsibilities of different bodies and different levels of government. Weaving together these disparate initiatives into a coordinated system of regional governance is a significant challenge. A case study of the Central Queensland region supports recent sociological accounts of changes in governance notably those of Fung and Wright (Empowered Participatory Governance), Healey (Collaborative Planning) and Sorensen and Torfing (Network Governance). The analysis identifies institutional features and normative principles of an emerging, multi-layered and multi-stakeholder, form of governance where a network employing deliberative practices coordinates formal political institutions and other actors from non-state sectors. These offer a potential model of regional governance suggesting the network form (with a role for state actors, but not state-dominated) and deliberative decision-making practices may provide coherence and coordination in policy-formulation and administration at a regional level.
Comparison between familial and sporadic systemic lupus erythematosus in Kuwaiti patients
Authors: Abutiban F , Mokkaddam K , Ameen E , Moussa M , Alsaeid K
Published: December 2008

To validate the use of multiplex case families in studying the pathogenesis of systemic lupus erythematosus (SLE), we investigated the pattern of familial SLE in relation to sporadic SLE in the highly consanguineous Kuwaiti population. We sought to determine whether familial and sporadic SLEs have the same clinical and serological features. We compared 21 cases of familial SLE in 21 families with 42 non-familial SLE controls matched for age, sex and duration of disease. Twenty-one families, in which the diagnosis of SLE was verified in at least two relatives, were included in the study. The diagnosis was made according to the revised 1982 American College of Rheumatology criteria. There were no significant differences in clinical features or serological manifestations between familial SLE cases and their matched controls. However, our results showed that the frequency of La/SSB antibodies was higher in the sporadic group ( P = 0.048), although this was not significant after application of Bonferroni’s correction for the number of comparisons. Familial cases of SLE were more likely to present at younger age of 20 years and sporadic cases at 26 years ( P = 0.006). The prevalence of familial SLE was 27.4%. We have found that familial and sporadic cases of SLE are broadly similar, and it is justified to include multiple case families in genetic studies.
Introduction
Authors: Lescher Marirose
Published: December 2008

The purpose of this panel is to honor the many contributions of Rosemary Radford Ruether in the field of feminist theology, focusing on her most recent book America, Amerikkka: Elect Nation and Imperial Violence. Rosemary Radford Ruether is the Carpenter Emerita Professor of Feminist Theology at Pacific School of Religion and the GTU, as well as the Georgia Harkness Emerita Professor of Applied Theology at Garrett Evangelical Theological Seminary. She is currently a visiting professor, part-time, at Claremont School of Theology and Claremont Graduate University. Rosemary has enjoyed a long and distinguished career as a scholar, teacher, and activist in the Roman Catholic Church, and is well known as a groundbreaking figure in Christian feminist theology. She has published numerous books, including Sexism and God-Talk, In Our Own Voices: Four Centuries of American Women's Religious Writing (ed. with Rosemary Skinner Keller), and The Wrath of Jonah: The Crisis of Religious Nationalism in the Israeli-Palestinian Conflict (with Herman J. Ruether). Her more recent books include Goddesses and the Divine Feminine: A Western Religious History (May 2005), Integrating Ecofeminism, Globalization, and World Religions (Nature's Meaning) (June 2005), a collaborative multi-volume Encyclopedia of Women in American Religion, with Rosemary Skinner Keller (April 2006), and Interpreting the Postmodern: Responses to `Radical Orthodoxy' (ed. with Marion Grau (January 2006). Rosemary's new book America, Amerikkka was published in 2007.
Parity of Participation and the Politics of Status
Authors: Armstrong Chris , Thompson Simon
Published: December 2008

Over the past decade, Nancy Fraser has developed a sophisticated theory of social justice. At its heart lies the principle of parity of participation, according to which all adult members of society must be in a position to interact with one another as peers. This article examines some obstacles to the implementation of that principle. Concentrating on the contemporary status order, it asks two specific questions. Is it possible to produce a precise account of how the status order might need to be ordered for parity of participation to be realized? And is it possible to derive a detailed and coherent political strategy capable of achieving such parity within the status order? The argument of this article is that, while Fraser has recognized the difficulties that the complexity of the contemporary status order poses for achieving parity of participation, she has nevertheless underestimated those difficulties. If parity of participation requires status equality, important and difficult work remains to be done in delineating the nature, and demands, of equality in the contemporary status order.
The Role of Complement Inhibition in PNH
Authors: Hillmen Peter
Published: December 2008

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, chronic, debilitating, acquired disorder that most frequently presents in early adulthood and usually continues throughout the life of a patient. PNH results in the death of approximately half of affected individuals, mainly through thrombotic complications, and until recently had no specific therapy. In 2007 eculizumab, an anti-complement antibody targeting the C5 complement component was approved for PNH by both the US Food and Drug Administration (FDA) and the European Medicines Agency (EMEA). Eculizumab is very effective in the treatment of intravascular hemolysis and all its sequelae, which include most of the symptoms and complications of PNH. Eculizumab has revolutionized our approach to hemolytic PNH and as it markedly reduces the principal complications of PNH, namely thrombosis and renal failure, should have a significant impact on survival. However, the development of eculizumab presents new challenges in PNH, such as how to avoid complications of therapy, how to overcome some of the problems associated with treatment and who to select for treatment, as only a proportion of patients with a PNH clone will benefit. This article will review the evidence behind the use of eculizumab in PNH, the effect it will have on the complications of the disease, the most appropriate selection of patients for therapy, the optimal management and the potential complications of the therapy.
The Anemia of Inflammation/Malignancy: Mechanisms and Management
Authors: Adamson John W.
Published: December 2008

Anemia is a common complication in patients with inflammatory diseases of many kinds, including cancer. The mechanisms that have captured the most attention include cytokine-mediated changes in both the production of and the response to erythropoietin (Epo), as well as important alterations in iron metabolism. The last is brought about by the relatively recently recognized peptide hormone, hepcidin. The availability of recombinant human Epo and its derivatives (known by class as Erythropoietic Stimulating Agents, ESAs) has dramatically changed anemia management in patients with cancer but, in the process, has raised as many issues as have been answered. This chapter reviews the mechanisms resulting in anemia in inflammation, including cancer, and focuses on the controversies around management with the ESAs and the adjuvant use of iron in anemia management.
Thrombophilia in Children: Who to Test, How, When, and Why?
Authors: Raffini Leslie
Published: December 2008

Thrombosis and thrombotic risk factors in children are receiving increased attention, and pediatric hematologists frequently are asked to evaluate children with symptomatic thrombosis, or asymptomatic children who have relatives affected with either thrombosis or thrombophilia. The clinical utility of thrombophilia testing has become increasingly debated, both in adults and children. Children with thrombosis are a heterogeneous group, and it is unlikely that a single approach to testing or treatment is optimal or desirable. A causative role of inherited prothrombotic defects in many pediatric thrombotic events, particularly catheter-related thrombosis, has not been established. Pediatric patients most likely to benefit from thrombophilia testing include adolescents with spontaneous thrombosis and teenage females with a known positive family history who are making choices about contraception. Recent data suggest that some inherited thrombophilic defects are associated with a higher risk of recurrent venous thromboembolism in children, though optimal management of these patients has yet to be determined. The decision to perform thrombophilia testing in asymptomatic patients with a family history should be made on an individual basis after discussion with the family. Given that the field of pediatric thrombosis continues to evolve, and the settings in which many of these events occur are unique to childhood, prospective longitudinal analyses of such patients to determine outcome and response to treatment as well as the impact of known thrombophilic states on these outcomes are clearly needed.
New Anticoagulants in Children
Authors: Young Guy
Published: December 2008

Thromboembolic complications are increasing in children and the use of anticoagulation has seen a dramatic increase despite the lack of randomized clinical trials. The most widely used agents in children are heparin, low-molecular-weight heparins (LMWH), and warfarin. These agents, however, have significant limitations that are exaggerated in children. Novel anticoagulants such as direct thrombin inhibitors and the selective factor Xa inhibitor, fondaparinux, have been approved for use in adults and have properties that suggest they may be safer and more efficacious than the standard agents; however, until recently, publications using these agents in children were limited to case reports. Recently, clinical trials for two direct thrombin inhibitors, bivalirudin and argatroban, have been completed and a clinical trial of fondaparinux is under way. This review will compare the standard agents with the novel agents and briefly review the results of the clinical trials.
Pathobiology and Molecular Profiling of Peripheral T-Cell Lymphomas
Authors: de Leval Laurence , Gaulard Philippe
Published: December 2008

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of rare diseases, usually manifesting clinical aggressiveness. Although important novel insights into the pathobiology of nodal PTCL have been gained recently from molecular profiling studies and clinico-pathological analyses, the pathogenetic molecular lesions remain to be deciphered for most entities. Angioimmunoblastic T-cell lymphoma (AITL) comprises CD4+ CXCL13+ neoplastic cells displaying overlapping immunophenotypical and molecular features with normal follicular helper T cells. This derivation might account for the presence of a prominent non-neoplastic component in AITL tissues and the clinical manifestations of the disease reflective of an immunological dysfunction. ALK+ anaplastic large cell lymphoma (ALCL), defined by ALK gene translocation with various gene partners, is composed of CD30+ ALK+ cells with a cytotoxic phenotype and usually carries a good prognosis. ALK– ALCL, now considered as a distinct disease entity, is morphologically and immunophenotypically similar to ALK+ ALCL, except for ALK expression, but has distinctive molecular features. PTCL, not otherwise specified (PTCL, NOS), the largest PTCL category, which is derived from activated CD4+ (or CD8+) T cells, is markedly heterogeneous, including at the molecular level. Gene expression profiling approaches have identified novel biomarkers of potential therapeutic interest, and suggest the existence of molecularly distinct PTCL, NOS subgroups.
Prognosis and Primary Therapy in Peripheral T-Cell Lymphomas
Authors: Savage Kerry J.
Published: December 2008

Peripheral NK/T-cell neoplasms are an uncommon group of diseases that show distinct racial and geographic variation. The prognostic significance of the T-cell phenotype has been clearly defined in recent studies by using modern lymphoma classification systems. However, within this heterogenous group of neoplasms, some have a more favorable prognosis, such as ALK-positive anaplastic large-cell leukemia (ALCL) and primary cutaneous ALCL, and some have ultimately fatal courses with standard chemotherapy programs (e.g., hepatosplenic γδ T-cell lymphomas). Further, unlike the benefits observed with CHOP chemotherapy in the treatment of diffuse large B-cell lymphoma (DLBCL), peripheral T-cell lymphomas (PTCL), other than ALK-positive ALCL, are relatively chemoresistant to this regimen. Given disease rarity and biological heterogeneity, advances in diagnosis, prognosis and treatment have lagged behind DLBCL. Recently, however, studies are emerging that focus specifically on PTCLs with the ultimate goal of better understanding disease biology and developing more effective therapies.
Challenging Problems: Coincident Pregnancy, HIV Infection, and Older Age
Authors: Connors Joseph M.
Published: December 2008

With the application of modern chemotherapy and radiation techniques, most patients with Hodgkin lymphoma can be cured, regardless of initial extent of disease. However, the lymphoma sometimes presents coincident with certain other major conditions, including pregnancy, infection with human immunodeficiency virus (HIV) or older age, which complicate treatment and make management considerably more challenging. Specially crafted approaches to staging and treatment, including the addition of specific supportive care measures, are required in these situations. Pregnant patients with coincident Hodgkin lymphoma should be staged without the use of imaging requiring radiation and treated with an approach that includes initial treatment deferral when appropriate, single agent vinblastine and reservation of multi-agent chemotherapy for the small minority with very aggressive lymphoma. Patients with Hodgkin lymphoma and HIV infection can be given standard anti-lymphoma treatment but require intensive supportive care with highly active anti-retroviral treatment (HAART) and prophylactic anti-fungal and anti-Pneumocystis antibiotics plus neutrophil growth factors. Standard staging and full dose multi-agent chemotherapy are necessary if older patients with Hodgkin lymphoma are to be afforded the best chance of cure but the final choice of the individual elements of treatment must respect co-morbid conditions and age- or other disease-related organ compromise. If appropriately chosen, these special measures permit delivery of safe, effective treatment and frequent cure of the Hodgkin lymphoma despite complicating pregnancy, HIV infection or older age.
Burkitt Lymphoma in Adults
Authors: Perkins Archibald S. , Friedberg Jonathan W.
Published: December 2008

This review will begin with a detail of the revision of the WHO classification, and pathological definitions of Burkitt lymphoma. Over the past several years, molecular understanding of Burkitt lymphoma has improved significantly. Using gene expression profiling, a genomic “signature” of Burkitt lymphoma may be identified, that has fidelity beyond c-myc expression, and the presence of the classical t(8;14). Then, evaluation and therapy of the adult patient with Burkitt lymphoma will be reviewed. Relatively few data exist on optimal therapy of the adult patient with Burkitt lymphoma. Principles of therapy should include high doses of alkylating agents, frequent administration of chemotherapy, and attention to central nervous system (CNS) prophylaxis with high doses of systemic chemotherapy, intrathecal therapy, or both. The outcome of adult patients with Burkitt lymphoma, particularly those over 40 years of age, is inferior to the outcome of younger patients, but may be improving over the past few years. Results from an international collaborative effort, which are helpful in evaluating results of Burkitt lymphoma therapy in adults, will be presented. HIV-associated Burkitt lymphoma, and elderly patients with Burkitt lymphoma, comprise special clinical situations that will be also covered in this review.
Marginal Zone Lymphomas: Management of Nodal, Splenic, and MALT NHL
Authors: Kahl Brad , Yang David
Published: December 2008

Marginal zone lymphomas are indolent B-cell lymphomas that originate from the marginal zone of B-cell follicles. For several subtypes, the initiation of disease appears to be a consequence of chronic infection and/ or inflammation. While the initial lymphoid hyperplasia is driven by physiologic antigenic stimulation, additional oncogenic events, such as chromosomal translocations leading to constitutive activation of signaling pathways, occur during the progression of disease that ultimately result in antigen-independent lymphoproliferation. Despite having a common origin in the marginal zone of the B-cell follicle, there are distinct clinical and molecular characteristics of marginal zone lymphomas originating at different anatomic sites. As such, marginal zone–derived lymphomas are currently categorized by the World Health Organization (WHO) into those originating in the spleen (splenic marginal zone lymphoma), extranodal mucosa-associated lymphoid tissue (MALT lymphoma), or lymph node (nodal marginal zone lymphoma).
From Palliation to Epigenetic Therapy in Myelofibrosis
Authors: Rambaldi Alessandro , Barbui Tiziano , Barosi Giovanni
Published: December 2008

Myelofibrosis shows a progressive clinical course and usually a poor, lethal prognosis. The molecular pathogenesis of this disease largely remains to be fully understood but the identification of the JAK2V617F mutation in more than half of patients was a major improvement in our understanding of the disease biology and may represent the first biologic marker useful for risk stratification, independently from conventional clinical predictors. After many elusive efforts, new effective treatment strategies are becoming available for this disease. Allogeneic transplantation following reduced-intensity conditioning programs, at least in some patients, may induce not only a hematologic response but also a molecular remission, thus supporting the hope of a possible, definitive eradication of the disease. Moreover, new innovative drugs, targeting either the JAK2V617F mutation or more general oncogenic mechanisms, may provide widely applicable, effective treatments to many patients for whom allogeneic transplantation is not feasible.