bluuebunny/crossref_metadata_2025 huggingface dataset:
Intracranial Meningiomas of Childhood and Adolescence
Authors: Tufan Kadir , Dogulu Fikret , Kurt Gokhan , Emmez Hakan , Ceviker Necdet , Baykaner M. Kemali
Published: May 2005

Meningiomas are rare intracranial neoplasms in childhood and adolescence, representing 0.4–4.1% of the pediatric-age tumors and 1.5–1.8% of all intracranial meningiomas. The goal of this study was to determine epidemiology, clinical and radiological features, and long-term outcome of childhood and adolescence meningiomas. Patients operated for intracranial meningiomas of childhood and adolescence between 1983 and 2003 at Gazi University School of Medicine, Department of Neurosurgery, were evaluated retrospectively. This study presents 11 cases (6 male, 5 female), ranging in age from 14 months to 17 years. Age and sex distribution, presenting symptoms, neurological examination results, location of meningiomas, radiological and histopathological findings, and prognosis were reviewed. The results were compared with those reported in the existing literature. Atypical and malignant meningiomas seem to be more common in childhood and adolescence with respect to adult meningiomas. Tumor location, completeness of tumor removal, and pathological grade are the most important prognostic factors.
The Gravity-Assisted Paedi-Gav Valve in the Treatment of Pediatric Hydrocephalus
Authors: Meling Torstein R. , Egge Arild , Due-Tønnessen Bernt
Published: May 2005

Objective: A single-center, prospective, nonrandomized pilot study was performed to assess the Paedi-Gav gravity-assisted valve for the treatment of pediatric patients with hydrocephalus. Methods: Participants were pediatric patients (age <16 years) who were candidates for a hydrocephalus shunt system that required a valve insertion at the time of enrollment. The primary outcome event was shunt malfunction; subclassified into shunt obstruction, shunt overdrainage, loculated ventricles, or infection. The shunt obstructions were further subclassified according to site. A total of 32 patients were enrolled onto the study, with 2 undergoing first shunt insertion after failed ventriculostomy and 30 undergoing shunt revisions. On average, the patients had had 3.3 shunt procedures prior to insertion of a Paedi-Gav valve. Results: During a follow-up interval of minimum 52 weeks and a median of 24 months after the first implantation on-study, shunt revisions were required in 17 (53.1%) of the 32 patients. The 12-month shunt-survival rate without revision of any component was 53%, with a median shunt-survival time of 388 days. The most common reasons for shunt revision were shunt obstructions (12/17) and overdrainage (3/17). Shunt obstructions were caused by valve-related failures (9/12) and distal obstructions (3/12). Conclusion: Although the small number of patients enrolled in this study warrants cautious conclusions, the overall results are comparable to those reported for primary shunt insertions with conventional valves in pediatric patients with hydrocephalus. Although this study provides a rationale for examining the Paedi-Gav gravity-assisted shunt valve in a larger prospective randomized controlled trial, the shunt failure pattern, with a rather high frequency of valve-related failures, may indicate potential for further improvements in the valve design and/or manufacturing.
Persistent Cognitive Dysfunction Secondary to Cerebellar Injury in Patients Treated for Posterior Fossa Tumors in Childhood
Authors: Rønning Camilla , Sundet Kjetil , Due-Tønnessen Bernt , Lundar Tryggve , Helseth Eirik
Published: May 2005

Traditionally, the cerebral hemispheres have been regarded as the region of the brain responsible for cognitive functions, while the cerebellum has been considered to be primarily involved in motor functions. Recent studies focus also on the possible involvement of the cerebellum in neurocognitive functions. The aim of this study was to determine the neuropsychological profile of young adults treated for a posterior fossa tumor in childhood and look for possible support for the presence of the so-called ‘cerebellar cognitive affective syndrome’ in these patients. Two groups of young adults were studied. The astrocytoma group (n = 12) had been treated for a low-grade cerebellar astrocytoma with surgery alone (mean age at surgery was 8.6 years and mean age at neuropsychological testing was 23.5 years). The medulloblastoma group (n = 11) had been treated with surgery followed by radiotherapy and chemotherapy (mean age at surgery was 6.1 years and mean age at neuropsychological testing was 23.1 years). The neuropsychological test battery comprised measures of intelligence, motor function, attention, psychomotor speed, verbal memory and visual memory. The medulloblastoma group performed poorer than the astocytoma group on all neuropsychological measures except one. Nonetheless, the astrocytoma group also had impaired scores compared with standard norms on measures of motor speed, attention and executive function. No significant correlation between age at time of treatment and grade of neuropsychological impairment was found in the astrocytoma group, though there was a tendency that young age at time of treatment correlated with better outcome on IQ measures. In the medulloblastoma group, age was significantly correlated with outcome, for both IQ and degree of neuropsychological impairment. For this group, young age at time of treatment indicated a worse outcome. Conclusions: Persistent cognitive dysfunction was detected in patients treated for posterior fossa medulloblastoma and cerebellar astrocytoma. The astrocytoma group was treated with surgery alone, indicating that a cerebellar lesion can result in cognitive dysfunction. Thus, this study gives support to the existence of the cerebellar cognitive affective syndrome. Follow-up of all patients treated for posterior fossa tumor in childhood should include an extensive neuropsychological testing at regular intervals. This may be of benefit for school planning and later work planning.
Benign Intradural Extramedullary Masses in Children of Northern India
Authors: Kumar Raj , Singh Vinita
Published: May 2005

Twenty-five children with benign intradural extramedullary tumors/lesions were retrospectively analyzed to see the frequency of different histotypes in this location and their variation from the Western world. The duration of symptoms, clinical profile and surgical outcome of these cases were evaluated. The age of children ranged from 1.5 to 18 years, with a mean age of 7.5 years. Thirteen amongst them were male. Follow-up ranged from 3 months to 5 years with a mean of 19 months. McCormick functional grading was used retrospectively to find out the functional outcome in these children. Sixty-eight percent of these cases presented with functional grade IV or V. The mean duration from the appearance of the first symptoms to admission to our hospital in these children was 13 months. Improvement in the functional grade following surgical excision was noted amongst all, except one, who presented with schwannoma, and did not improve from grade III even after a 14-month follow-up. Reoperation/two-staged operation was required in 3 children. The following conclusions were drawn from this study: (1) approximately half (48%) of intradural extramedullary benign tumors are nerve sheath tumors, i.e. neurofibromas, neurilemomas and schwannomas. Giant neurofibromas with giant extraforaminal extension may rarely manifest with a large extraspinal mass; (2) meningioma is an infrequent tumor in this location; (3) developmental cystic dysraphic lesions (neurenteric cyst, arachnoid cysts), unassociated with the stigmata of spinal dysraphism, are significantly more frequent (28%) than reported in the literature; (4) the possibility of infective lesions (granuloma, abscess) causing compressive features cannot be denied even in intradural extramedullary tumors; (5) long-standing lesions, with a long segment involvement, also have a good outcome following a good microsurgical excision, and (6) more than half of the children (68%) present with a severe neurological compromise, either because of ignorance and poor education or due to the scanty diagnostic facilities available at the peripheral hospitals of the developing milieu.
Human Tail with Noncontiguous Intraspinal Lipoma and Spinal Cord Tethering:Case Report and Embryologic Discussion
Authors: Donovan Daniel J. , Pedersen Robert C.
Published: May 2005

Children born with a tail-like appendage have a rare malformation that is frequently associated with abnormalities of the spine and spinal cord. A contiguous fibrolipoma is usually seen extending from the subcutaneous portion of the tail into the inferior spinal cord, resulting in tethered cord syndrome. We present the case of a child born with a tail and intraspinal lipoma that were not contiguous with each other, and were separated by an intact layer of lumbosacral fascia. The tail and lipoma were removed and the spinal cord untethered, and the child is neurologically normal 2 years after surgery. The absence of a contiguous lipoma from the tail to the spinal cord suggests that this condition may be principally caused by a disorder of secondary neurulation and/or regression of the normal embryonic tail bud. The embryology of the lower spine is reviewed and possible etiologies discussed.
Melanin Aggregation and Polymerization: Possible Implications in Age-Related Macular Degeneration
Authors: Sarangarajan Rangaprasad , Apte Shireesh. P.
Published: May 2005

The state of aggregation of the polymer melanin may determine its propensity to act either as an antioxidant or as a pro-oxidant. Age-related alterations in its state of aggregation are suggested to alter the degree of polymerization so as to confer increased pro-oxidant propensity to the melanin polymer. Degradative processes in/of melanosomes and lysosomes in the retinal pigment epithelium (RPE) appear to be intimately connected so that they may involve exchange of contents between these two organelles. An increased pro-oxidant environment inside lysosomes has been associated with preventing the digestion of cellular components including photoreceptor outer rod segments partly by altering function of lysosomal hydrolases. It is speculated that age-related accumulation of low-molecular-weight phototoxic pro-oxidant melanin oligomers within lysosomes in the RPE may be partly responsible for decreasing the digestive rate of incorporated cellular components (including photoreceptor outer rod segments) which may lead to lipofuscin formation. More work is required to definitively refute or support such a hypothesis.
Expectation Maximization Algorithm Based Haplotype Relative Risk (EM-HRR): Test of Linkage Disequilibrium Using Incomplete Case-Parents Trios
Authors: Guo Chao-Yu , DeStefano Anita L. , Lunetta Kathryn L. , Dupuis Josée , Cupples L. Adrienne
Published: May 2005

The Haplotype Relative Risk (HRR) was first proposed [Falk et al., Ann Hum Genet 1987] to test for Linkage Disequilibrium (LD) between a marker and a putative disease locus using case-parent trios. Spurious association does not appear in such family-based studies under population admixture. In this paper, we extend the HRR to accommodate incomplete trios via the Expectation-Maximization (EM) algorithm [Dempster et al., J R Stat Soc Ser B, 1977]. In addition to triads and dyads (parent-offspring pair), the EM-HRR easily incorporates individuals with no parental genotype information available, which is excluded from the one parent Transmission/Disequilibrium Test (1-TDT) [Sun et al., Am J Epidemiol 1999]. Due to the data structure of EM-HRR, transmitted alleles are always available regardless of the number of missing parental genotypes. As a result of having a larger sample size, computer simulations reveal that the EM-HRR is more powerful in detecting LD than the 1-TDT in a population under Hardy-Weinberg Equilibirum (HWE). If admixture is not extreme, the EM-HRR remains more powerful. When a large degree of admixture exists, the EM-HRR performs better the 1-TDT when the association is strong, though not as well when the association is weak. We illustrate the proposed method with an application to the Framingham Heart Study.
Effects of Venlafaxine Treatment on Clozapine Plasma Levels in Schizophrenic Patients
Authors: Repo-Tiihonen Eila , Eloranta Anne , Hallikainen Tero , Tiihonen Jari
Published: May 2005

Depressive symptoms are found at any stage of schizophrenia, and antidepressant medication may be beneficial. Selective serotonin reuptake inhibitor antidepressants have been considered safe in schizophrenia but in combination with clozapine, that is widely used in chronic treatment-resistant schizophrenia, remarkable pharmacokinetic interactions can occur causing an elevation in clozapine plasma levels. To investigate this further, the plasma levels of clozapine were measured in 11 schizophrenic male patients with depressive symptoms who were administered both clozapine and venlafaxine. Low to moderate doses of venlafaxine did not seem to have any significant effect on clozapine plasma levels.
Relationship between Class I and II HLA Antigens in Schizophrenia and Eye Movement Disturbances: A Preliminary Study
Authors: Bogacki Przemysław A. , Borkowska Alina , Wojtanowska-Bogacka Małgorzata , Rybakowski Janusz K.
Published: May 2005

The region coding human leukocyte antigen (HLA) on chromosome 6q21 was shown to be associated with both the vulnerability to schizophrenia and presence of eye movement disturbances (EMD). The aim of this preliminary study was to investigate how individual class I and II HLA antigens in schizophrenic patients may be related to schizophrenia and to the intensity of two kinds of EMD: fixation and smooth pursuit. The incidence of HLA antigens was compared between 40 schizophrenic patients (17 male, 23 female) and 198 healthy control subjects (112 male, 86 female). In schizophrenic patients, the intensity of EMD assessed by infrared reflectometry and quantified on a scale from 0 to 3 was correlated with the incidence of HLA antigens. A number of differences regarding HLA antigens were found between schizophrenic patients and healthy subjects. Significant correlation was also obtained between some EMD and a number of HLA antigens. Antigens A24 and A28 were found to occur in different frequencies in schizophrenic patients and healthy control subjects. They also correlated with EMD on the fixation and smooth pursuit tests. The results obtained show an association between HLA antigens and EMD as an endophenotypic marker of schizophrenia, and may add to other findings on susceptibility loci for schizophrenia on chromosome 6p21. A limitation of this study is a small number of investigated patients with schizophrenia.
Characterization of Fluoroquinolone and Carbapenem Susceptibilities in Clinical Isolates of Levofloxacin-Resistant Pseudomonas aeruginosa
Authors: Muramatsu Hideaki , Horii Toshinobu , Takeshita Akihiro , Hashimoto Hisakuni , Maekawa Masato
Published: May 2005

Background:Pseudomonas aeruginosa can rapidly acquire resistance to antibiotics, including fluoroquinolones and carbapenems. Methods: We characterized fluoroquinolone, carbapenem and other β-lactam susceptibilities and analyzed fluoroquinolone and carbapenem resistance in 16 clinical isolates of levofloxacin-resistant P. aeruginosa. Results: All levofloxacin-resistant isolates showed high MICs (≧32 µg/ml) for fluoroquinolones including norfloxacin, levofloxacin, sparfloxacin, gatifloxacin and pazufloxacin, whereas the MICs for sitafloxacin were between 2 and 16 µg/ml. These isolates had both a Thr83Ile mutation in GyrA and a Ser87Leu mutation in ParC. An additional mutation, Glu469Asp in GyrB, was detected in 3 isolates. Three of 16 isolates found during antibiotic therapy showed resistance to carbapenems (MIC, 16–32 µg/ml) because of a reduced production of OprD. Fluoroquinolones, β-lactams and sulfamethoxazole-trimethoprim were used for 3 months before the isolation of levofloxacin-resistant P.aeruginosa. Conclusions: Emergence of resistant isolates to both fluoroquinolones and carbapenems during antibiotic therapy is a serious clinical problem. Our results suggest that susceptibilities to fluoroquinolones as well as carbapenems should be monitored during a prolonged course of antibiotic therapy against P. aeruginosa infection.
Calcaneal Osteopenia Is a New Marker for Arterial Stiffness in Chronic Hemodialysis Patients
Authors: Joki Nobuhiko , Hase Hiroki , Shiratake Maiko , Kishi Nobuaki , Tochigi Sawako , Imamura Yoshihiko
Published: May 2005

Background: A high frequency of simultaneous low estimated bone mineral density (BMD) and atherosclerotic vascular disease is found in chronic dialysis patients. The degree of BMD may be closely related to the severity of atherosclerosis in dialysis patients. The purpose of this study was to determine the association of estimated BMD with severity of atherosclerosis using popular economical non-invasive strategies in chronic dialysis patients. Methods: This cross-sectional design study included 58 subjects receiving maintenance hemodialysis (HD) therapy. The mean age was 68 ± 12 years (range 39–93), 22 subjects were female, and 26 subjects (45%) had diabetic nephropathy. The brachial-ankle pulse wave velocity (baPWV) and estimated BMD, using calcaneal quantitative ultrasound measurements of the osteo-sono index (OSI) were measured in all of the subjects. Results: The mean levels of OSI and baPWV were 2.3 ± 0.3 and 21 ± 5 m/s, respectively. Based on single variable regression analysis, the C-reactive protein concentration correlated with baPWV (r = 0.272, p = 0.039), and the serum albumin concentration was inversely correlated with baPWV (r = –0.318, p = 0.015). In addition, OSI had a strong negative correlation with baPWV (r = –0.470, p = 0.0002). To identify the independent factors correlating with baPWV, multivariate regression analysis was performed using confounding variables which had significant association in univariate analysis. OSI and diabetic nephropathy were found to be significant independent parameters. Conclusion: Calcaneal OSI, a surrogate marker for estimated BMD, has a strong ability to predict the severity of arterial stiffness independent of classical risk factors and markers of inflammation and malnutrition.
Requirement of Nuclear Factor-κB in Angiotensin II– and Isoproterenol-Induced Cardiac Hypertrophy In Vivo
Authors: Freund Christian , Schmidt-Ullrich Ruth , Baurand Anthony , Dunger Sandra , Schneider Wolfgang , Loser Peter , El-Jamali Amina , Dietz Rainer , Scheidereit Claus , Bergmann Martin W.
Published: May 2005

Background—

In vitro experiments have proposed a role of nuclear factor-κB (NF-κB), a transcription factor, in cardiomyocyte hypertrophy and protection against apoptosis. Currently, the net effect on cardiac remodeling in vivo under common stress stimuli is unclear.

Methods and Results—

We have generated mice with cardiomyocyte-restricted expression of the NF-κB super-repressor IκBαΔN (ΔN
MHC
) using the Cre/lox technique. ΔN
MHC
mice displayed an attenuated hypertrophic response compared with control mice on infusion of angiotensin II (Ang II) or isoproterenol by micro-osmotic pumps, as determined by echocardiography (left ventricular wall dimensions: control plus Ang II, ×1.5±0.1 versus sham; ΔN
MHC
plus Ang II, ×1.1±0.1 versus sham;
P
<0.05; n≥9), heart weight, and histological analysis. Real-time reverse-transcriptase polymerase chain reaction showed significantly reduced expression of hypertrophy markers β-myosin heavy chain and atrial natriuretic peptide in Ang II–treated ΔN
MHC
mice (
P
<0.05 versus control plus Ang II; n=4). Neither cardiomyocyte apoptosis nor left ventricular dilatation was observed. In cultured adult rat cardiomyocytes, NF-κB DNA binding activity was increased by both Ang II– and interleukin-6–related cytokines. The latter are known to be released by cardiac fibroblasts on Ang II stimulation and thus could locally increase the NF-κB response of cardiomyocytes. Finally, results from in vitro and in vivo experiments suggest a role for NF-κB in the regulation of prohypertrophic interleukin-6 receptor gp130 on mRNA levels.

Conclusions—

These results indicate that targeted inhibition of NF-κB in cardiomyocytes in vivo is sufficient to impair Ang II– and isoproterenol-induced hypertrophy without increasing the susceptibility to apoptosis.
Experience overrides colour in lizard contests
Authors: Stuart-Fox Devi M. , Johnston Gregory R.
Published: May 2005

We examined the role of conspicuous coloration in male-male contests for two species of Australian dragon lizards, Ctenophorus decresii and C. vadnappa, in which conspicuous coloration has a demonstrated predation cost. We conducted contests in which the overall conspicuousness of male coloration was manipulated using paints that matched the spectral reflectance of the lizards, as well as natural (control) contests. There was little evidence for an influence of colour on contest outcome or aggression levels for either species when all experiments were considered. However, we found a significant effect of trial order and experience on contest outcome and aggression levels (the same pair of males was used for both types of contest), despite a 2-3 week interval between contests. When we examined only the first trial between unfamiliar males, we found that male C. vadnappa that had been painted to appear more conspicuous consistently won. Comparison with the natural trials suggests that the aspect of colour manipulation that was responsible for this result was the 'hue' of the throat: males with yellower throats consistently beat males with bluer throats in both natural and painted trials. The difference in coloration of flank markings also predicted the difference in aggression scores between contestants in the natural trials. These results suggest that although colour is important in opponent assessment and in determining contest outcome in C. vadnappa, previous agonistic experience can override the effects of colour and have a long-lasting influence on aggressive behaviour.
State Regulation of Nonprofit Organizations: Accountability Regardless of Outcome
Authors: Irvin Renée A.
Published: May 2005

Although recent calls for increased accountability of the nonprofit sector imply that the role of state regulatory agencies should expand, the costs of expanded regulation to the nonprofit sector—and indirectly to the general public and charitable beneficiaries—are significant, and should not be ignored. This article examines the motivations that state regulators have in promulgating registration and reporting requirements for nonprofit organizations and professional fund-raisers. Examination of six states with no annual requirements for nonprofit organizations or professional fund-raiser registration and financial reporting revealed no obvious accountability pathologies such as unusually high fraudulent activity or abnormally low donations to nonprofit organizations. Because nonprofit accountability in states without annual registration appears as robust as that in other states, the author proposes removal of state regulations requiring nonprofit annual registration and financial reporting. At the very least, the author urges caution in the face of increasing calls form ore stringent state regulation of nonprofit organizations.
Government Funding and the Nonprofit Sector: Exploring a New Census Bureau Data Source—The Federal Audit Clearinghouse
Authors: Jalandoni Nadine T. , Petrescu Claudia , Green Gordon W.
Published: May 2005

Using the information provided by the Federal Audit Clearinghouse, maintained by the U.S. Census Bureau, this research attempts to improve our understanding of the nature, scale, extent, and sources of federal funding to social service and educational nonprofit organizations. Using 2001 as the base year, this research is divided into two parts. The first part looks into federal funding provided to social service organizations by laying out the amounts of grants awarded by 57 different federal agencies and looking into its geographic and per capita distribution. The second part traces federal funding provided to universities and provides information on the institutions and states receiving the most grants and the federal agencies that provided them. The data’s utility, limitations, and potential uses to academicians and practitioners are also discussed.
Cohesion as Shared Beliefs in Exercise Classes
Authors: Burke Shauna M. , Carron Albert V. , Patterson Michelle M. , Estabrooks Paul A. , Hill Jennie L. , Loughead Todd M. , Rosenkranz Sara R. , Spink Kevin S.
Published: May 2005

The purpose of the study was to determine if perceptions of cohesion in exercise classes demonstrated sufficiently high consensus and between-group variance to support a conclusion that exercise classes are groups. Participants (N = 1,700) in 130classes were tested on either the Group Environment Questionnaire (GEQ) or the Physical Activity Group Environment Questionnaire (PAGEQ). Results showed that exercise classes satisfied the statistical criteria necessary to support a conclusion that they are true groups; that is, they exhibited acceptable levels of consensus about cohesion within classes and acceptable differences in cohesion between classes. In addition, index-of-agreement values were significantly greater for participants completing the PAGEQ than for participants completing the GEQ. Finally, consensus was greatest when participants evaluated how the exercise class satisfied their own personal task needs (i.e., individual attractions to the group-task), and second greatest when participants evaluated the collective unity around the task objectives (i.e., group integration-task).
Small Group Negotiation
Authors: Schei Vidar , Rognes Jørn K.
Published: May 2005

Members of small groups often disagree when making decisions. Therefore, they need to negotiate to reach a joint decision. Small group negotiations will often be difficult because group members approach the task with different motivational orientations, some members being individualistic (i.e., focusing only on their own interests) and others being more cooperative (i.e., focusing both on their own interests and those of the group as a whole). Examining 3-person groups in simulated negotiations, it was found that the differences in group members’ motivational orientations affected both (a) how resources were distributed within the groups (individualistic members exploited cooperative members and got the lion’s share of the resources, even when the cooperators were in a majority) and (b) how fairness was perceived across group compositions (members of groups with only cooperative members perceived the negotiations to be more fair than did members of groups that had one or more individualistic members).
Communication Medium and Member Familiarity
Authors: Adams Susan J. , Roch Sylvia G. , Ayman Roya
Published: May 2005

The effects of communication medium and member familiarity on decision time, member satisfaction, and decision accuracy were explored. The study represents the first known attempt to examine the role of member familiarity on the study variables in face-to-face (FTF) and computer-mediated (CM) mediums. The results of 71 three-person groups revealed familiarity affected decision time and accuracy within the CM condition. For CM groups only, as member familiarity among group members increased, decision time decreased, and unexpectedly decision accuracy decreased. Another finding for CM group members was an indication that familiarity increased satisfaction with group process. Last, satisfaction with communication medium was only influenced by the communication medium (CM group members were less satisfied). It appears that familiarity plays a larger role in CM groups than in FTF groups. Implications are discussed.
A Competency-Based Model for Developing Human Resource Professionals
Authors: McEvoy Glenn M. , Hayton James C. , Warnick Alan P. , Mumford Troy V. , Hanks Steven H. , Blahna Mary Jo
Published: May 2005

This article describes a framework for the design and implementation of a competency-based curriculum for graduate management education. The article also outlines how this model has been implemented at one university in the context of a graduate degree in human resource management. Among the significant challenges discussed are the identification of competencies required for professional success; the identification of the specific traits, knowledge, and skills that combine to create competence; and the creation of a curriculum and the associated assessment tools necessary to promote the acquisition of competencies. Student orientation, student development, and student assessment are discussed.
Achieving Buy-In
Authors: Kramer Desre M. , Wells Richard P.
Published: May 2005

This article offers an overview and an evaluation of the process of transferring a complex body of knowledge from a research institute to workplace parties. It includes practical insights into the “how” of building knowledge transfer networks. It also describes the development of a network-based strategy to transfer knowledge about workplace safety/ergonomics to a group of practitioner-based associations within Ontario’s Health & Safety Prevention system. The purpose of the practitioner network was to have them become knowledge brokers of the research linking to multiple workplaces in many different sectors. This strategy builds on the theoretical frameworks of knowledge transfer and network theory. Through multiple group interactions, the practitioners became familiar with the research, identified matching concepts between the research and their experiences, saw the research as relevant, adopted the principles of the research, and went on to apply it with their client workplaces.
Reporting the Future—Journalism Meets Emerging Science
Authors: Bielak Alex T.
Published: May 2005

The 4th World Conference of Science Journalists that was held in Montreal in October 2004 was a major success, with more than 600 participants from nearly sixty countries. Jointly organized by the national Canadian Science Writers’ Association and the Association des Communicateurs Scientifiques du Québec, the meeting featured thirty-two sessions and more than 100 speakers. With thirty presidents of regional, national, and international science writer associations present, the meeting provided the venue for the formal launch of the World Federation of Science Journalists, the goals of which include promoting excellence in science and technology reporting.
The Etiology of Tax Complexity: Evidence from U.S. State Income Tax Systems
Authors: Slemrod Joel
Published: May 2005

This article uses the variation in U.S. state income tax systems to examine what engenders tax complexity. An analysis of simple measures of the variation in complexity, based on the number of lines in the tax forms and the number of pages in the instruction booklets, reveals some intriguing patterns. States with more professional legislatures, as measured by the salaries paid, tend to have more complex tax systems, as do states with a less active voting population.
Tax Amnesties and Political Participation
Authors: Torgler Benno , Schaltegger Christoph A.
Published: May 2005

In many countries, thinking about a (new) tax amnesty is currently in vogue. However, international experience shows that the financial success of such a tax amnesty is not guaranteed, in part because it is thought that tax amnesties may over time undermine tax compliance. This article conducts experiments in two different countries (Switzerland and Costa Rica) to examine the effects of amnesties on compliance. In contrast to other experiments, these experiments analyze the relationship between tax compliance and subjects’ possibility to vote for or against an amnesty. The results suggest that tax compliance only increases after voting, when people get the opportunity to discuss the amnesty prior to voting. Thus, voting with discussion may induce a kind of civic duty, as taxpayers become aware of the importance of contributing to the provision of public goods.
Haploinsufficiency, rather than the effect of an excessive production of soluble CD95 (CD95ΔTM), is the basis for ALPS Ia in a family with duplicated 3′ splice site AG in CD95 intron 5 on one allele
Authors: Roesler Joachim , Izquierdo Jose-Maria , Ryser Martin , Rösen-Wolff Angela , Gahr Manfred , Valcarcel Juan , Lenardo Michael J. , Zheng Lixin
Published: May 2005

Autoimmune lymphoproliferative syndrome type Ia (ALPS Ia) is caused by mutations in the CD95/APO1/FAS (TN-FRSF6) gene, which lead to a defective CD95 ligand (CD95L)–induced apoptosis. Soluble CD95 (sCD95) has been suggested to play an important role in the pathogenesis of diverse autoimmune and malignant diseases by antagonizing CD95L. Here we evaluate a family with 4 of its 5 members harboring an ex-6–3C→G mutation that affects the splice cis regulatory region (cctacag/ex-6→cctagag/ex-6) of the CD95 gene. The mutation causes skipping of exon-6, which encodes the transmembrane region of CD95, and thereby leads to an excessive production of sCD95 in all 4 affected individuals. The mutation is associated with a low penetrance of disease phenotype and caused mild and transient ALPS in one male patient whereas all other family members are completely healthy. In all family members with the mutation we found that the cell surface expression of CD95 was low and the activated T cells were resistant to CD95-induced apoptosis. Unexpectedly, excessive production or addition of sCD95 had no effect on the CD95-induced apoptosis in diverse cells. In contrast, increasing the surface expression of CD95 was able to correct the defect in apoptosis. Thus we conclude that the ALPS in the one male patient was caused by haploinsufficiency of membrane CD95 expression. Our data challenge the hypothesis that sCD95 causes autoimmunity.
High-dose chemotherapy and autologous stem cell transplantation for primary refractory or relapsed Hodgkin lymphoma: long-term outcome in the first 100 patients treated in Vancouver
Authors: Lavoie Julye C. , Connors Joseph M. , Phillips Gordon L. , Reece Donna E. , Barnett Michael J. , Forrest Donna L. , Gascoyne Randy D. , Hogge Donna E. , Nantel Stephen H. , Shepherd John D. , Smith Clayton A. , Song Kevin W. , Sutherland Heather J. , Toze Cynthia L. , Voss Nicholas J. S. , Nevill Thomas J.
Published: May 2005

Beginning in 1985, patients in British Columbia with Hodgkin lymphoma (HL) that was not controlled by conventional chemotherapy routinely underwent high-dose chemotherapy and autologous stem cell transplantation (HD-ASCT). Long-term complications of HD-ASCT have become apparent as more patients survive without recurrence of HL. Data were obtained retrospectively on the first 100 patients that underwent HD-ASCT for HL in Vancouver, focusing on relapse, treatment-related complications, and the occurrence of late events. Fifty-three patients remain alive (median follow-up, 11.4 years [range, 10.0-17.4 years]) with an overall survival (OAS) of 54% at 15 years. OAS was significantly better in patients in first relapse (67%) than in patients with primary refractory-induction failure (39%) and advanced disease (29%) (P = .002). The major cause of death was progression of HL (32% at 15 years). Treatment-related mortality, including death from second malignancy, was 17% at 15 years. Cumulative risk of a second malignancy was 9% at 15 years. Karnofsky performance status was at least 90% in 47 patients although hypogonadism (20 patients), hypothyroidism (12 patients), unusual infections (10 patients), anxiety or depression (7 patients), and cardiac disease (5 patients) were not uncommon in survivors. HD-ASCT can lead to durable remissions in relapsed or refractory HL with acceptable but definite late toxicity. The occurrence of late events necessitates lifelong medical surveillance. (Blood. 2005;106:1473-1478)
Critical variables in the conversion of marrow cells to skeletal muscle
Authors: Abedi Mehrdad , Greer Deborah A. , Foster Bethany M. , Colvin Gerald A. , Harpel Joshua A. , Demers Delia A. , Pimentel Jeffery , Dooner Mark S. , Quesenberry Peter J.
Published: May 2005

We have studied conversion of marrow cells to skeletal muscle in cardiotoxin-injured anterior tibialis muscle in a green fluorescent protein (GFP) to C57BL/6 transplantation model and ascertained that total body irradiation (TBI) with establishment of chimerism is a critical factor. Local irradiation has little effect in lower doses and was detrimental at higher doses. Whole body (1000 cGy) with shielding of the leg or a combination of 500 cGy TBI and 500 cGy local radiations was found to give the best results. In non-obese diabetic-severe combined immunodeficient (NOD-SCID) recipients, we were able to show that conversion could occur without radiation, albeit at relatively lower levels. Within 3 days of cardiotoxin injury, GFP-positive mononuclear cells were seen in the muscle, and within 2 weeks GFP-positive muscle fibers were identified. Conversion rates were increased by increasing donor-cell dose. Timing of the cardiotoxin injury relative to the transplantation was critical. These studies show that variables in transplantation and injury are critical features of marrow-to-muscle conversions. Irradiation primarily effects conversion by promoting chimerism. These data may explain the differences in the literature for the frequency of marrow-to-skeletal muscle conversion and can set a platform for future models and perhaps clinical protocols. (Blood. 2005;106:1488-1494)
Antiphospholipid syndrome in patients with systemic lupus erythematosus treated by autologous hematopoietic stem cell transplantation
Authors: Statkute Laisvyde , Traynor Ann , Oyama Yu , Yaung Kim , Verda Larissa , Krosnjar Nela , Burt Richard K.
Published: May 2005

Systemic lupus erythematosus (SLE) is the most common disease associated with antiphospholipid syndrome (APS). We, therefore, evaluated 46 patients with refractory SLE treated by autologous hematopoietic stem cell transplantation (HSCT) for a history of APS prior to transplantation. The prevalence of SLE-related APS in our patient population was 61% (28 of 46 patients with refractory SLE). Nineteen of 28 patients with APS had lupus anticoagulant (LA) or high titers of anticardiolipin antibodies (ACLAs), either immunoglobulin (Ig)G or IgM, when evaluated at study entry. Six of 8 evaluable LA+ patients became and remained LA–; 5 of 7 initially ACLA IgG+ patients and 9 of 11 ACLA IgM+ patients demonstrated normalization of ACLA titers when followed after HSCT. Eighteen of 22 patients refractory to chronic anticoagulation discontinued anticoagulation therapy a median of 4 months after transplantation; 78% of them remained free of thrombotic events and in complete SLE remission for up to 78 months (median, 15 months) after HSCT. There was no treatment-related mortality. Autologous HSCT may be performed safely in patients with APS and appears to be effective therapy for eliminating ALPAs and preventing thrombotic complications in patients with SLE.
2B4 (CD244)-CD48 interactions provide a novel MHC class I-independent system for NK-cell self-tolerance in mice
Authors: McNerney Megan E. , Guzior Dustin , Kumar Vinay
Published: May 2005

Natural killer (NK) cells must be able to eliminate infected and transformed cells while remaining tolerant of normal cells. NK-cell self-tolerance is thought to be maintained by self-major histocompatibility complex (MHC) class I recognition; however, there are examples where NK cells are not regulated by MHC class I and yet remain self-tolerant. Here, we show that 2B4 (CD244) and CD48 represent a second system for murine NK-cell self-recognition. 2B4 and MHC class I receptors act nonredundantly to inhibit NK lysis of syngeneic tumor cells. NK cells from β2 microglobulin (β2m)-deficient mice and NK cells that lack expression of self-MHC-binding inhibitory receptors are inhibited by 2B4. Moreover, we provide the first in vivo evidence for MHC-independent NK self-recognition in a bone marrow rejection assay. These data suggest that NK-cell self-tolerance can be mediated by molecules other than MHC. (Blood. 2005;106:1337-1340)
Nucleophosmin mutations in childhood acute myelogenous leukemia with normal karyotype
Authors: Cazzaniga Giovanni , Dell'Oro Maria Grazia , Mecucci Cristina , Giarin Emanuela , Masetti Riccardo , Rossi Vincenzo , Locatelli Franco , Martelli Massimo F. , Basso Giuseppe , Pession Andrea , Biondi Andrea , Falini Brunangelo
Published: May 2005

Nucleophosmin (NPM) is a nucleocytoplasmic shuttling protein involved in leukemia-associated chromosomal translocations, and it regulates the alternate reading frame (ARF)-p53 tumorsuppressor pathway. Recently, it has been demonstrated that mutations of the NPM1 gene alter the protein at its C-terminal, causing its cytoplasmic localization. Cytoplasmic NPM was detected in 35% of adult patients with primary non-French-American-British (FAB) classification M3 acute myeloid leukemia (AML), associated mainly with normal karyotype. We evaluated the prevalence of the NPM1 gene mutation in non-M3 childhood AML patients enrolled in the ongoing Associazione Italiana di Ematologia e Oncologia Pediatrica (AIEOP-AML02) protocol in Italy. NPM1 mutations were found in 7 (6.5%) of 107 successfully analyzed patients. NPM1- mutated patients carried a normal karyotype (7/26, 27.1%) and were older in age. Thus, the NPM1 mutation is a frequent abnormality in AML patients without known genetic marker; the mutation may represent a new target to monitor minimal residual disease in AML and a potential candidate for alternative and targeted treatments. (Blood. 2005;106:1419-1422)
Comparison of standard exponential and linear techniques to amplify small cDNA samples for microarrays
Authors: Wadenbäck Johan , Clapham David H , Craig Deborah , Sederoff Ronald , Peter Gary F , von Arnold Sara , Egertsdotter Ulrika
Published: May 2005

Background

The need to perform microarray experiments with small amounts of tissue has led to the development of several protocols for amplifying the target transcripts. The use of different amplification protocols could affect the comparability of microarray experiments.

Results

Here we compare expression data from Pinus taeda cDNA microarrays using transcripts amplified either exponentially by PCR or linearly by T7 transcription. The amplified transcripts vary significantly in estimated length, GC content and expression depending on amplification technique. Amplification by T7 RNA polymerase gives transcripts with a greater range of lengths, greater estimated mean length, and greater variation of expression levels, but lower average GC content, than those from PCR amplification. For genes with significantly higher expression after T7 transcription than after PCR, the transcripts were 27% longer and had about 2 percentage units lower GC content. The correlation of expression intensities between technical repeats was high for both methods (R2 = 0.98) whereas the correlation of expression intensities using the different methods was considerably lower (R2 = 0.52). Correlation of expression intensities between amplified and unamplified transcripts were intermediate (R2 = 0.68–0.77).

Conclusion

Amplification with T7 transcription better reflects the variation of the unamplified transcriptome than PCR based methods owing to the better representation of long transcripts. If transcripts of particular interest are known to have high GC content and are of limited length, however, PCR-based methods may be preferable.
Brief cognitive assessment in a UK population sample – distributional properties and the relationship between the MMSE and an extended mental state examination
Authors: Huppert Felicia A , Cabelli Sara T , Matthews Fiona E ,   the MRC Cognitive Function and Ageing Study (MRC CFAS)
Published: May 2005

Background

Despite the MMSE's known flaws, it is still used extensively as both a screening instrument for dementia and a population measure of cognitive ability. The aim of this paper is to provide data on the distribution of MMSE scores in a representative sample from the UK population and to compare it with an extended cognitive assessment (EMSE) which covers a wider range of cognitive domains and provides a wider range of difficulty levels.

Methods

The MMSE and the EMSE were administered to over 12,000 participants at the screening stage of the MRC Cognitive Function and Ageing Study (MRC CFAS). MRC CFAS is a multi-centre population-based study in England and Wales with respondents aged 65 years and older.

Results

Normative values on the MMSE and EMSE are presented by age group, sex and level of education. There are very large differences between age groups, with smaller differences seen between the sexes and by level of education. The EMSE extends the scores at the high end of the ability range, but is no better than the MMSE at differentiating between dementia and non-dementia.

Conclusion

Population-derived norms are valuable for comparing an individual's score to the score that would be expected among the general population, given the individual's specific demographic characteristics.
Paradise – not without its plagues: Overwhelming Blastomycosis pneumonia after visit to lakeside cottages in Northeastern Ontario
Authors: Parmar Malvinder S
Published: May 2005

Background

Visiting lakes and cottages is a common leisure activity during summer among most Canadians and paradise for some. Various leisure activities are involved during these visits, including cleaning and 'airing' the cottage after long-winters, activities at the lakes and dock building etc, exposing the Canadians to moist soil and decaying woods – a source of white or tan mould – Blastomyces dermatitidis that may cause a flu-like illness to severe pneumonia that often remains a diagnostic challenge and results in delay in diagnosis and appropriate treatment thereby increasing associated morbidity and mortality.

Case Presentations

Five cases of overwhelming acute blastomycosis pneumonia are presented. Four of the five patients presented within few weeks of their visit to the cottages and surrounding lakes and all were initially treated as "community acquired pneumonia" that resulted in delay in diagnosis and poor outcome in the first patient. The first case, however, taught an important lesson that led to high-index of suspicion in the others with early diagnosis and improved outcomes. Interestingly, all patients were obese and had a shorter incubation period and severe clinical course. The possible mechanism for early and severe disease in association with obesity is speculated and literature is reviewed.

Conclusion

High-index of suspicion is important in the early diagnosis and appropriate management acute blastomycosis pneumonia to improve associated morbidity and mortality.
Hypervascular nodule in a fibrotic liver overloaded with iron: identification of a premalignant area with preserved liver architecture
Authors: Sá Cunha António , Blanc Jean-Frédéric , Trillaud Hervé , De Ledinghen Victor , Balabaud Charles , Bioulac-Sage Paulette
Published: May 2005

Background

The presence of a hypervascular nodule in a patient with cirrhosis is highly suggestive of a hepatocellular carcinoma.

Case presentation

A 55 year old man with idiopathic refractory anaemia was addressed for the cure of a recently appeared 3.3 cm hypervascular liver nodule. The nodule was not visible on the resected fresh specimen, but a paler zone was seen after formalin fixation. The surrounding liver was fibrotic (METAVIR score F3) and overloaded with iron. However, the paler zone, thought to be the nodule, had in fact a normal architecture, was less fibrotic, and contained some "portal tract-like structures" (but with arteries only); moreover, this paler area was devoid of iron, contained less glycogen and was characterized by foci of clear hepatocytes.

Conclusion

In spite of the absence of architectural distortion, and a normal proliferative index, the possibility of premalignancy or malignancy should be considered in this type of hypervascular and hyposiderotic nodule, occurring in the context of an iron overloaded liver.