bluuebunny/crossref_metadata_2025_split huggingface dataset:
Orotic aciduria and uridine monophosphate synthase: A reappraisal
Authors: Bailey C. J. 
Published: June 2009

AbstractThree subtypes of hereditary orotic aciduria are described in the literature, all related to deficiencies in uridine monophosphate synthase, the multifunctional enzyme that contains both orotate: pyrophosphoryl transferase and orotidine monophosphate decarboxylase activities. The type of enzyme defect present in the subtypes has been re‐examined by steady‐state modelling of the relative outputs of the three enzymic products, uridine monophosphate, urinary orotic acid and urinary orotidine. It is shown that the ratio of urinary outputs of orotidine to orotate provides a means of testing for particular forms of enzyme defect. It is confirmed that the type I defect is caused by loss of uridine monophosphate synthase activity. Cells and tissue of type I cases have a residual amount of activity that is qualitatively unchanged: the relative rates of the transferase and decarboxylase do not differ from those of wild‐type enzyme. The single claimed case of type II, thought to be due to specific inactivation of orotidine monophosphate decarboxylase, is shown to have a product spectrum inconsistent with that claim. It is proposed that this type II form does not differ sufficiently to be accepted as separate from type I. The third subtype, hereditary orotic aciduria without megaloblastic anaemia, occurs in two cases. It has the product spectrum expected of a defect in orotidine monophosphate decarboxylase. This form is the only one that appears to have a qualitatively different uridine monophosphate synthase. The possibility that orotidine monophosphate may control flux through the pyrimidine biosynthesis pathway in hereditary orotic aciduria is discussed.
IgM monoclonal component associated with type I Gaucher disease resolved after enzyme replacement therapy: A case report
Authors: Martinez‐Redondo C. , Ortuño F. J. , Lozano M. L. , Jerez A. , del Mar Osma M. , Giraldo P. , Vicente V. 
Published: June 2009

SummaryThe frequency of monoclonal gammopathy of undetermined significance (MGUS) is higher in patients with type I Gaucher disease (GD I) than in the general population. Although enzyme replacement therapy is effective in the control of the disease, its effect on MGUS is still controversial. We present the case of a 65‐year‐old woman with extensive GD I associated with IgM MGUS, in whom enzyme replacement therapy succeeded in eradicating the monoclonal component. This observation further supports the idea that enzyme replacement therapy decreases the chronic antigenic stimulation responsible for gammopathies in Gaucher disease.
A Facile Strategy for In Situ Core-Template-Functionalizing Siliceous Hollow Nanospheres for Guest Species Entrapment
Authors: Wang Jun , Gao Xin , Yang Xianyan , Gan Yilai , Weng Wenjian , Gou Zhongru 
Published: June 2009

Abstract
The shell wall-functionalized siliceous hollow nanospheres (SHNs) with functional molecules represent an important class of nanocarriers for a rich range of potential applications. Herein, a self-templated approach has been developed for the synthesis of in situ functionalized SHNs, in which the biocompatible long-chain polycarboxylates (i.e., polyacrylate, polyaspartate, gelatin) provide the framework for silica precursor deposition by simply controlling chain conformation with divalent metal ions (i.e., Ca2+, Sr2+), without the intervention of any external templates. Metal ions play crucial roles in the formation of organic vesicle templates by modulating the long chains of polymers and preventing them from separation by washing process. We also show that, by in situ functionalizing the shell wall of SHNs, it is capable of entrapping nearly an eightfold quantity of vitamin Bc in comparison to the bare bulk silica nanospheres. These results confirm the feasibility of guest species entrapment in the functionalized shell wall, and SHNs are effective carriers of guest (bio-)molecules potentially for a variety of biomedical applications. By rationally choosing the functional (self-templating) molecules, this concept may represent a general strategy for the production of functionalized silica hollow structures.
Early improvement as a predictor of remission and response in schizophrenia: Results from a naturalistic study
Authors: Jäger M. , Schmauß M. , Laux G. , Pfeiffer H. , Naber D. , G. Schmidt L. , Gaebel W. , Klosterkötter J. , Heuser I. , Maier W. , Lemke M.R. , Degner D. , Buchkremer G. , Gastpar M. , Möller H.-J. , Riedel M. 
Published: June 2009

AbstractObjectiveTo examine the predictive validity of early improvement in a naturalistic sample of inpatients and to identify the criterion that best defines early improvement.MethodsTwo hundred and forty-seven inpatients who fulfilled ICD-10 criteria for schizophrenia were assessed with the Positive And Negative Syndrome Scale (PANSS) at admission and at biweekly intervals until discharge from hospital. Remission was defined according to the recently proposed consensus criteria, response as a reduction of at least 40% in the PANNS total score from admission to discharge.ResultsReceiver operating characteristic (ROC) analyses showed that early improvement (reduction of the PANSS total score within the first 2 weeks of treatment) predicts remission (AUC = 0.659) and response (AUC = 0.737) at discharge. A 20% reduction in the PANSS total score within the first 2 weeks was the most accurate cut-off for the prediction of remission (total accuracy: 65%; sensitivity: 53%; specificity: 76%), and a 30% reduction the most accurate cut-off for the prediction of response (total accuracy: 76%; sensitivity: 47%; specificity: 90%).ConclusionThe findings of clinical drug trials that early improvement is a predictor of subsequent treatment response were replicated in a naturalistic sample. Further studies should examine whether patients without early improvement benefit from an early change of antipsychotic medication.
Clinicopathologic analysis of uterine sarcomas from a single institution in the Canary Islands
Authors: Benito Virginia , Lubrano Amina , Arencibia Octavio , Andújar Miguel , Álvarez Eva , Medina Norberto , Falcón Juan Miguel , Falcón Orlando 
Published: June 2009

AbstractObjectiveTo evaluate the clinicopathologic data and prognostic factors for patients with uterine sarcomas treated at a single institution, with special emphasis on malignant mixed müllerian tumors (MMMT).MethodsMedical and anatomic pathology records were reviewed. Survival rates were analyzed using the Kaplan‐Meier method.ResultsThe study included 89 patients: 48.4% with MMMT; 22.4% with leiomyosarcomas; 20.2% with endometrial stromal sarcomas; and 9% with adenosarcomas. FIGO stages I, II, III, and IV were identified in 57.3%, 9.0%, 22.5%, and 7.8% of patients respectively. Event‐free survival rates after 2, 5, and 10 years were 70%, 61%, and 55% respectively, with a median time of 90 months (95% CI, 41–140 months). Overall survival rates after 2, 5, and 10 years were 50%, 45%, and 39% respectively, with a median time of 43 months (95% CI, 3–83 months). Multivariate analysis showed that stage, histology, tumor size, and parity had an independent influence on overall survival.ConclusionsMMMT are the most aggressive tumors and their behavior strongly resembles that of high‐grade endometrial adenocarcinoma. Prognostic factors affecting survival were stage, histology, tumor size, and parity.
Elevated C‐reactive protein levels are associated with prevalent dementia in the oldest‐old
Authors: Kravitz B. Adar , Corrada Maria M. , Kawas Claudia H. 
Published: June 2009

BackgroundC‐reactive protein (CRP) is a nonspecific marker of inflammation that is increased in the brain and serum of patients with Alzheimer's disease (AD), and has been associated with increased risk of developing dementia. Inflammation increases with age, and the number of people reaching age 90 years and older is growing, making the association between inflammation and dementia increasingly relevant. Using a cross‐sectional design, we examined whether high levels of serum CRP are associated with increased odds of prevalent dementia in the oldest‐old.MethodsSerum CRP levels of 305 participants (mean age ± standard deviation, 94.3 ± 2.9 years) from the 90+ Study, a longitudinal cohort study of people aged 90 years and older, were evaluated with respect to all‐cause dementia. Levels of CRP were divided into three categories: undetectable (<0.5 mg/dL), detectable (0.5–0.7 mg/dL), and elevated (>0.8 mg/dL). Odds ratios (ORs) were calculated using logistic regression, and were adjusted for covariates.ResultsRelative to participants with undetectable CRP levels, participants with detectable or elevated CRP levels had increased odds of all‐cause dementia (detectable: OR, 3.0; 95% confidence interval, 1.2–7.3; elevated: OR, 5.0; 95% confidence interval, 1.9–12.9). When participants were subdivided by gender, significantly increased ORs were seen only in women.ConclusionsIn the oldest‐old, high CRP levels are associated with increased odds of all‐cause dementia, particularly in women. Prospective studies are necessary to confirm whether increased CRP levels are associated with an increased risk of developing dementia in this age group.
When are moods most likely to influence consumers' product preferences? The role of mood focus and perceived relevance of moods
Authors: White Katherine , McFarland Cathy 
Published: June 2009

AbstractThis program of research examined the hypothesis that moods are most likely to be used as a source of information in making evaluations and choices when consumers both focus on their moods (i.e., acknowledge and pay attention to their feelings) and perceive that moods are a relevant source of information for forming judgments. Support for this prediction was obtained when the relevance of moods was examined by manipulating relevance instructions, varying advertising cues, and manipulating product type. Further, the boundary conditions under which the combination of mood focus and perceived relevance is most influential on product preferences are identified.
Persistent pediatric obstructive sleep apnea and lingual tonsillectomy
Authors: Lin Aaron C. , Koltai Peter J. 
Published: June 2009

ObjectiveTo describe a new method and the indications for lingual tonsillectomy with endoscopy and coblation, and to document its utility for treating children with persistent obstructive sleep apnea after previous tonsillectomy and adenoidectomy.Study Design and SettingCase series with chart review in a tertiary pediatric medical center.Subjects and MethodsTwenty‐six patients aged 3 to 20 met the inclusion criteria of polysomnography‐proven persistent obstructive sleep apnea after tonsillectomy and adenoidectomy, as well as diagnosis of lingual tonsillar hypertrophy made by flexible fiberoptic sleep endoscopy. Endoscopic‐assisted coblation lingual tonsillectomies were performed between June 2005 and January 2008. Preoperative and postoperative nocturnal polysomnogram data were paired and analyzed statistically.ResultsStatistically significant reductions in the respiratory distress index (RDI) were seen when preoperative and postoperative data were compared (mean, 14.7 vs 8.1). There were similar reductions in the number of obstructive apneas and hypopneas. The mean minimum O2 saturation did not change. Two patients in this series developed adhesions between the epiglottis and tongue base; there appeared to be no consequence for airway or feeding issues.ConclusionEndoscopic‐assisted coblation lingual tonsillectomy is an effective technique for the treatment of lingual tonsillar hypertrophy causing persistent obstructive sleep apnea in some children.
Sensorineural hearing loss in patients with cystic fibrosis
Authors: Cheng Alan G. , Johnston Patrick R. , Luz Jennifer , Uluer Ahmet , Fligor Brian , Licameli Greg R. , Kenna Margaret A. , Jones Dwight T. 
Published: June 2009

ObjectiveTo determine the prevalence of sensorineural hearing loss (SNHL) in cystic fibrosis (CF) patients and its relationship to antibiotic use.Study DesignCase series with chart review.SettingTertiary care pediatric hospital.Subjects and MethodsWe reviewed the medical records of CF patients seen in our children's hospital between March 1994 and December 2007. Data collected included patient demographics, audiograms, tympanograms, genotype, and use of potentially ototoxic antibiotics.ResultsSeven of 50 (14%) patients had SNHL. Three percent of patients who received ≤10 courses of intravenous aminoglycosides had SNHL versus 43 percent of those who received >10 courses (P < 0.01). No patients who received five or fewer courses of nasal irrigation with aminoglycosides had SNHL versus 23 percent of those who received more than five courses (P < 0.05). Nine percent of patients who received five or fewer courses of macrolides had SNHL versus 60 percent of those who received more than five courses (P = 0.079).ConclusionCF patients receiving aminoglycosides are at high risk for developing SNHL.
Immunological investigation in the adenoid tissues from children with chronic rhinosinusitis
Authors: Shin Seung‐Youp , Choi Gil‐Soon , Park Hae‐Sim , Lee Kun‐Hee , Kim Sung‐Wan , Cho Joong‐Saeng 
Published: June 2009

ObjectiveChronic rhinosinusitis (CRS) is characterized by persistent inflammation and tissue remodeling of the nasal mucosa. Adenoidectomy is an effective surgical treatment in pediatric CRS. To evaluate the effect of pediatric CRS on the severity and characteristics of adenoid inflammation, the authors evaluated the expressions of inflammatory cell activation markers and tissue remodeling in adenoid tissues associated with cytokines tissue‐remodeling‐associated cytokines in adenoid tissues.Study Design and SettingA prospective controlled study on 40 pediatric patients admitting for adenotonsillectomy.Subjects and MethodsImmunoassays were performed on adenoid tissues homogenates from 16 children with CRS and from 24 children without CRS to quantify the levels of inflammatory cell activation markers, such as soluble interleukin (IL)‐2 receptor (sIL‐2R), soluble CD23 (sCD23), IL‐6, eosinophilic cationic protein (ECP), and tryptase, and the levels of cytokines associated with tissue remodeling, such as transforming growth factor (TGF)‐β1, matrix metalloproteinase (MMP) 2 and 9, and tissue inhibitor of metalloproteinase (TIMP)‐1.ResultsThe mean levels (the ratio to albumin level) of sIL‐2R, TGF‐β1, MMP‐2, MMP‐9, and TIMP‐1 were significantly higher in adenoid tissues of patients with CRS (27.31 ± 30.32, 4894.65 ± 2388.77, 500.13 ± 604.59, and 23.06 ± 10.37, respectively) than those without it (16.27 ± 10.93, 2635.51 ± 1448.63, 120.87 ± 321.50, 16.74 ± 11.10, and 7.39 ± 3.12, respectively; all P < 0.05). Regarding the severity of CRS, ECP level was significantly higher in patients with severe CRS than in those with mild to moderate CRS (P = 0.033).ConclusionsAdenoid tissues in pediatric CRS patients had higher levels of tissue‐remodeling–associated cytokines, which may explain the relationship between pediatric CRS and adenoid inflammation.
Minimally invasive radioguided parathyroidectomy performed for primary hyperparathyroidism
Authors: Adil Eelam , Adil Tabrez , Fedok Fred , Kauffman Gordon , Goldenberg David 
Published: June 2009

ObjectiveParathyroid surgery is often challenging due to considerable variability in anatomy. Minimally invasive radioguided parathyroidectomy (MIRP) is a directed surgical approach that has been made possible by the advent of new imaging techniques, specifically sestamibi scanning. In this study, we review our experience and outcomes with MIRP in one of the largest case series reported in the literature.Study DesignCase series with chart review.SettingTertiary care center.Subjects and MethodsSubjects were 305 patients who underwent MIRP in our institution between 1997 and 2007. Data including symptoms, preoperative and postoperative calcium levels, and PTH levels were collected. Analyses were performed using Excel AnalysisPak.ResultsMIRP in this series had a 100 percent rate of success in removing a hyperfunctional parathyroid gland. The mean preoperative calcium was 10.9 whereas the mean postoperative level was 9.8. There was a significant difference between preoperative and postoperative calcium levels (both ionized and total) (P < 0.01). Mean preoperative and postoperative serum PTH levels were 138 and 50, respectively. PTH levels were therefore significantly lower postoperatively (P < 0.01). Rapid PTH testing showed a similar pattern. There were no cases of recurrent laryngeal nerve palsy.ConclusionMIRP is an effective method for removal of image‐localized hyperfunctional parathyroid glands.
Prevalence of depression and antidepressant use in an otolaryngology patient population
Authors: Chandra Rakesh K. , Epstein Victoria A. , Fishman Andrew J. 
Published: June 2009

ObjectiveTo determine the prevalence of depressive diagnoses and antidepressant use in various subsets of otolaryngology patients.Study DesignAcademic practice EMR database query.Subjects and MethodsOver 12,000 consecutive otolaryngology patients were analyzed by primary diagnosis and in three groups: Group 1 (tinnitus, vertigo), Group 2 (rhinosinusitis), and Group 3 (sleep apnea). The number with depressive diagnoses and/or those taking antidepressants was determined.ResultsThe prevalence of any depressive diagnosis was 11 percent, and 18 percent had been prescribed antidepressants. The prevalence of depression was 13 percent, 10 percent, and 14 percent for Groups 1, 2, and 3, respectively. Antidepressants had been prescribed in 21 percent, 15 percent, and 28 percent, respectively. The primary‐encounter diagnosis with the highest incidence of depression (21%) and antidepressant use (46%) was sleep apnea with insomnia.ConclusionOtolaryngology patients may exhibit greater prevalence of depression than is observed in the general population (7.3%). Highest prevalence was found in patients with inner ear disease and sleep apnea. Depression may be a cause of significant comorbidity in patients with chronic otolaryngic conditions.
Climate and vegetation history from a 14,000-year peatland record, Kenai Peninsula, Alaska
Authors: Jones Miriam C. , Peteet Dorothy M. , Kurdyla Dorothy , Guilderson Thomas 
Published: June 2009

AbstractAnalysis of pollen, spores, macrofossils, and lithology of an AMS 14C-dated core from a subarctic fen on the Kenai Peninsula, Alaska reveals changes in vegetation and climate beginning 14,200 cal yr BP. Betula expansion and contraction of herb tundra vegetation characterize the Younger Dryas on the Kenai, suggesting increased winter snowfall concurrent with cool, sunny summers. Remarkable Polypodiaceae (fern) abundance between 11,500 and 8500 cal yr BP implies a significant change in climate. Enhanced peat preservation and the occurrence of wet meadow species suggest high moisture from 11,500 to 10,700 cal yr BP, in contrast to drier conditions in southeastern Alaska; this pattern may indicate an intensification and repositioning of the Aleutian Low (AL). Drier conditions on the Kenai Peninsula from 10,700 to 8500 cal yr BP may signify a weaker AL, but elevated fern abundance may have been sustained by high seasonality with substantial snowfall and enhanced glacial melt. Decreased insolation-induced seasonality resulted in climatic cooling after 8500 cal yr BP, with increased humidity from 8000 to 5000 cal yr BP. A dry interval punctuated by volcanic activity occurred between 5000 and 3500 cal yr BP, followed by cool, moist climate, coincident with Neoglaciation. Tsuga mertensiana expanded after ~ 1500 cal yr BP in response to the shift to cooler conditions.
Molecular epidemiology of African sleeping sickness
Authors: HIDE G. , TAIT A. 
Published: June 2009

SUMMARYHuman sleeping sickness in Africa, caused by Trypanosoma brucei spp. raises a number of questions. Despite the widespread distribution of the tsetse vectors and animal trypanosomiasis, human disease is only found in discrete foci which periodically give rise to epidemics followed by periods of endemicity A key to unravelling this puzzle is a detailed knowledge of the aetiological agents responsible for different patterns of disease – knowledge that is difficult to achieve using traditional microscopy. The science of molecular epidemiology has developed a range of tools which have enabled us to accurately identify taxonomic groups at all levels (species, subspecies, populations, strains and isolates). Using these tools, we can now investigate the genetic interactions within and between populations of Trypanosoma brucei and gain an understanding of the distinction between human- and nonhuman-infective subspecies. In this review, we discuss the development of these tools, their advantages and disadvantages and describe how they have been used to understand parasite genetic diversity, the origin of epidemics, the role of reservoir hosts and the population structure. Using the specific case of T.b. rhodesiense in Uganda, we illustrate how molecular epidemiology has enabled us to construct a more detailed understanding of the origins, generation and dynamics of sleeping sickness epidemics.
Internet Voting in Comparative Perspective: The Case of Estonia
Authors: Alvarez R. Michael , Hall Thad E. , Trechsel Alexander H. 
Published: June 2009

ABSTRACTSeveral countries have conducted Internet voting trials in binding public elections over the past decade, including Switzerland, the United Kingdom, and the United States. However, Estonia—a former Soviet republic and now a full member of the European Union—has advanced the farthest in deploying Internet voting. In this article, we focus on how the Estonians have systematically addressed the legal and technical considerations required to make Internet voting a functioning voting platform, as well as the political and cultural framework that promoted this innovation. Using data from our own qualitative and quantitative studies of the Estonian experience, we consider who voted over the Internet in these elections, and the political implications of the voting platform.
Are Two (or Three or Four … or Nine) Heads Better than One? Collaboration, Multidisciplinarity, and Publishability
Authors: Sigelman Lee 
Published: June 2009

ABSTRACTAlthough collaborative research has become much more common in the social sciences, including political science, little is known about the consequences of collaboration. This article uses papers submitted to the American Political Science Review to assess whether the widely acknowledged benefits of collaboration produced papers that were more likely to be accepted for publication. The results indicate that collaboration per se made little or no difference, but that the disciplinary configuration of the authors did result in differences in the success of these submissions.
Tenure Standards in Political Science Departments: Results from a Survey of Department Chairs
Authors: Rothgeb John M. , Burger Betsy 
Published: June 2009

ABSTRACTThis article presents the results from a survey of political science department chairs regarding the tenure procedures and standards at their colleges or universities. The findings reveal that only a small fraction of the colleges and universities in the United States refuse to offer tenure or are attempting to limit tenure. We also find general agreement regarding the standards for evaluating teaching and service and that research expectations vary according to the highest degree offered by a department.
Representation and the Rules of the Game: An Electoral Simulation
Authors: Hoffman Donna R. 
Published: June 2009

ABSTRACTIt is often a difficult proposition for introductory American government students to comprehend different electoral systems and how the rules of the game affect the representation that results. I have developed a simulation in which different proportional-based electoral systems are compared with a single-member plurality electoral system. In addition, the simulation can also serve to illustrate other concepts commonly introduced in introductory courses.