bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and genetic findings in a series of Italian children with pure hereditary spastic paraplegia
Authors: Battini R. , Fogli A. , Borghetti D. , Michelucci A. , Perazza S. , Baldinotti F. , Conidi M. E. , Ferreri M. I. , Simi P. , Cioni G. 
Published: June 2010

Background:  Hereditary spastic paraplegias (HSP) are a group of neurodegenerative disorders characterized by progressive lower extremity spastic weakness. SPG7, SPG4 and SPG3A are some of the autosomal genes recently found as mutated in recessive or dominant forms of HSP in childhood. SPG31 is more often associated with a pure spastic paraplegia phenotype, but genotype–phenotype correlation is still unclear. The aims of the current study was: (i) to verify the mutational frequency of SPG4, SPG3A, SPG31 and SPG7 genes in our very‐well‐selected childhood sample, and (ii) to improve our knowledge about the clinical and electrophysiological HSP phenotypes and their possible correlation with a specific mutation.Methods:  A sample of 14 Italian children affected by pure HSP (mean age at diagnosis 5.9 years) was extensively investigated with electrophysiological, neuroradiological and genetic tests.Results:  Three SPG4 mutations were identified in three patients: two novel missense mutations, both sporadic, and one multiexonic deletion already reported. A novel large deletion in SPG31 gene involving exons 2–5 was also detected in one young patient. No mutations in the SPG7 and in the SPG3A genes were found.Conclusions:  Our data confirm that HSP represent a heterogeneous group of genetic neurodegenerative disorders, also in sporadic or autosomal recessive early onset forms. Multiplex Ligation‐dependent Probe Amplification‐based mutation screening for SPG4 and SPG31 genes would be added to sequencing‐based screening of SPG4, SPG31 and SPG3A genes in the routine diagnosis of HSP children.
Acute disseminated encephalomyelitis that meets modified McDonald criteria for dissemination in space is associated with a high probability of conversion to multiple sclerosis in Taiwanese patients
Authors: Liao M. F. , Huang C. C. , Lyu R. K. , Chen C. M. , Chang H. S. , Chu C.‐C. , Hsu W. C. , Wu Y. R. , Kuo H. C. , Cheng M. Y. , Hung P. C. , Chou M. L. , Lin K. L. , Hsieh M. Y. , Ro L. S. 
Published: June 2010

Background:  Patients with acute disseminated encephalomyelitis (ADEM) may relapse and some may ultimately convert to multiple sclerosis (MS); however, no criteria that can predict MS conversion are available to date. Our aim was to describe the clinical and magnetic resonance imaging (MRI) features of patients with an initial ADEM attack and evaluate which MRI criteria can predict conversion to MS.Methods:  We retrospectively reviewed the records of 36 patients diagnosed with ADEM. We determined clinical signs/symptoms, examined the cerebrospinal fluid (CSF), and performed brain MRI scans and compared the findings between patients who did and did not convert to MS.Results:  Clinical signs/symptoms, and CSF analysis show no significant difference between the two groups. The rate of conversion to MS from ADEM in Taiwanese patients is low (11%) after a mean follow‐up period of 28.36 months. Modified McDonald criteria were fulfilled in 19/36 patients: 21% (4/19) of those patients developed MS according to Poser criteria subsequently. Of the other patients (17/36) who did not fulfill these criteria, none converted to MS. (log rank test; P = 0.027).Conclusions:  It is difficult to predict from initial clinical presentations to address which patients with ADEM will convert to MS. Patients with ADEM whose brain MRI findings met the modified McDonald criteria may have clinically isolated syndrome because they have a significantly higher probability of conversion to MS. In contrast, patients whose brain MRI findings did not meeting these criteria may be considered as having classic ADEM because they have a lower probability of conversion to MS.
Towards a mechanistic understanding of dispersal evolution in plants: conservation implications
Authors: Travis Justin M. J. , Smith Hannah S. , Ranwala Sudheera M. W. 
Published: June 2010

AbstractAim  A species’ dispersal characteristics will play a key role in determining its likely fate during a period of environmental change. However, these characteristics are not constant within a species – instead, there is often both considerable interpopulation and interindividual variability. Also changes in selection pressures can result in the evolution of dispersal characteristics, with knock‐on consequences for a species’ population dynamics. Our aim here is to make our theoretical understanding of dispersal evolution more conservation‐relevant by moving beyond the rather abstract, phenomenological models that have dominated the literature towards a more mechanism‐based approach.Methods  We introduce a continuous‐space, individual‐based model for wind‐dispersed plants where release height is determined by an individual’s ‘genotype’. A mechanistic wind dispersal model is used to simulate seed dispersal. Selection acts on variation in release height that is generated through mutation.Results  We confirm that, when habitat is fragmented, both evolutionary rescue and evolutionary suicide remain possible outcomes when a mechanistic dispersal model is used. We also demonstrate the potential for what we term evolutionary entrapment. A population that under some conditions can evolve to be sufficiently dispersive that it expands rapidly across a fragmented landscape can, under different conditions, become trapped by a combination of limited dispersal and a large gap between patches.Conclusions  While developing evolutionary models to be used as conservation tools is undoubtedly a challenge, we believe that, with a concerted collaborative effort linking the knowledge and methods of ecologists, evolutionary biologists and geneticists, it is an achievable aim.
ON THE ROBUSTNESS OF RANGE‐BASED VOLATILITY ESTIMATORS
Authors: Akay Ozgur (Ozzy) , Griffiths Mark D. , Winters Drew B. 
Published: June 2010

AbstractWe empirically examine Parkinson's range‐based volatility estimate in the federal funds market, which is unique because institutional regulations create a predictable pattern in interday volatility. We find that range‐based volatility estimates and standard deviations produce the expected volatility pattern. We also find that at trading pressure points where microstructure noise should be greatest, range‐based estimates are less than the standard deviations. Thus, we support the argument that range‐based volatility estimates remove the upward bias created by microstructure noise. We find that the Parkinson method is the most efficient range‐based volatility measure among a set of alternates in this market.
Leg Symptom Categories and Rates of Mobility Decline in Peripheral Arterial Disease
Authors: McDermott Mary M. , Ferrucci Luigi , Liu Kiang , Guralnik Jack M. , Tian Lu , Liao Yihua , Criqui Michael H. 
Published: June 2010

OBJECTIVES: To determine whether asymptomatic lower extremity peripheral arterial disease (PAD) and leg symptoms other than intermittent claudication (IC) in PAD are associated with faster functional decline than in people with both PAD and IC.DESIGN: Prospective, observational study.SETTING: Chicago‐area medical center.PARTICIPANTS: Four hundred fifteen people with PAD followed annually for up to 7 years.MEASUREMENTS: At baseline, patients with PAD were categorized into symptom categories, including IC; leg pain on exertion and rest; participants who could walk through exertional leg pain (pain/carry on); and participants who never experienced exertional leg pain, even during the 6‐minute walk (always asymptomatic). Outcomes included mobility loss (becoming unable to walk one‐quarter of a mile or walk up and down one flight of stairs without assistance) and becoming unable to complete the 6‐minute walk without stopping. Analyses adjusted for age, sex, comorbidities, ankle brachial index, and other confounders.RESULTS: Always‐asymptomatic participants (hazard ratio (HR)=2.94, 95% confidence interval (CI)=1.39–6.19, P=.005) and those with leg pain on exertion and rest (HR=2.89, 95% CI=1.47–5.68, P=.002) had greater mobility loss than participants with IC. Participants with PAD with leg pain/carry on were less likely (P=.047) to become unable to walk for 6 minutes continuously without stopping than participants with IC.CONCLUSION: The ABI identifies patients with asymptomatic PAD and those with atypical leg symptoms who are at risk for greater mobility decline than participants without PAD and participants with PAD with IC.
Modeling fertilizer externalities around Palo Verde National Park, Costa Rica
Authors: Arriagada Rodrigo A. , Sills Erin O. , Pattanayak Subhrendu K. , Cubbage Frederick W. , González Eugenio 
Published: June 2010

AbstractIrrigated rice farming in Costa Rica involves use of agrochemicals that pollute important wetlands ecosystems, such as the Palo Verde National Park in the northeastern province of Guanacaste. We characterize rice farming in this region, apply duality theory to estimate conditional factor demand for fertilizer, and then simulate the impacts of alternative policies on fertilizer use. Using a normalized profit function, we also estimate policy impacts on farmer profits. As expected, prices of rice seeds and fertilizer significantly affect use of fertilizer. Price incentives or taxes could encourage farming practices that reduce the threat to downstream ecosystems.
Twist is inversely associated with claudins in germ cell tumors of the testis
Authors: VÄRE PÄIVI , SOINI YLERMI 
Published: June 2010

Väre P, Soini Y. Twist is inversely associated with claudins in germ cell tumors of the testis. APMIS 2010; 118: 640–7.We investigated the expression of claudins 1, 3–7 and transcriptional factor twist in a set of testicular germ cell tumors. The material consisted of 17 seminomas, 13 teratomas, 9 teratocarcinomas, 20 embryonal carcinomas and 9 mixed germ cell tumors. They were immunostained with antibodies to claudins 1, 3–7 and twist. As expected, all claudins were variably present in germ cell tumors with epithelial elements or differentiation, but the intensity of expression varied depending on the claudin type. Mesenchymal elements in teratomatous tumors remained negative for claudins. Expression of different claudins was less intense and inconsistent in other types of germ cell tumors. Choriocarcinomatous elements in germ cell tumors expressed relatively strongly claudin 4 and weaker positivity for claudins 5–7, while claudins 1 and 3 were negative. Seminomas showed expression only for claudins 5 and 7. The transcriptional factor twist was most strongly expressed in seminoma followed by embryonal carcinoma. Twist expression was inversely associated with several claudins (claudins 1, 3, 4 and 6). Germ cell tumors vary in their expression of claudins 1–7. Twist expression was inversely associated with several claudins, suggesting that it takes part in the downregulation of claudins in testicular tumors.
EFFECTS OF HIGH PRESSURE PROCESSING ON SOYBEAN BETA‐CONGLYCININ
Authors: ZHANG HONGKANG , LI LITE , MITTAL G.S. 
Published: June 2010

ABSTRACT Effects of high pressure processing on conformational changes of soybean β‐conglycinin was studied by means of sulfhydryl groups detection, spectrofluorimetry, ultraviolet difference spectra, circular dichroism and electrophoresis. Significantly more sulflhydryl groups as well as hydrophobic regions and amino acid residues, which had ultraviolet absorbance had been found after high pressure processing (≥300 MPa). The CD analysis indicated that some of the ordered structures ofα‐helix andβ‐structure were destroyed and converted to random coil after processing at 500 MPa for 10 min. Electrophoresis analysis revealed that β‐conglycinin could be denatured and might be dissociated into subunits after high pressure processing (≥300 MPa).PRACTICAL APPLICATIONSThe use of high pressure for food processing is now getting an increasing interest in the food industry because of the consumer's demand for convenience foods of the highest quality in terms of natural flavor and taste, and which are free from additives and preservatives. It is a possible alternative to temperature treatment. Soybean proteins play an important role in food consumption worldwide. The objectives of this study were to reveal the conformational changes of soybean β‐conglycinin after high pressure processing. The results will help the application of high pressure technology in soybean protein processing.
Dynamic Cardiovascular Risk Assessment in Elderly People
Authors: Harris Scott , Tepper David , Ip Randy 
Published: June 2010

Abstract.  Objectives. This study sought to determine whether serial measurement of N‐terminal pro‐B‐type natriuretic peptide (NT‐proBNP) in com‐munity‐dwelling elderly people would provide additional prognostic information to that from traditional risk factors. Background. Accurate cardiovascular risk stratification is challenging in elderly people. Methods. NT‐proBNP was measured at baseline and 2 to 3 years later in 2975 community‐dwelling older adults free of heart failure in the longitudinal Cardiovascular Health Study (CHS). This investigation examined the risk of new‐onset heart failure (HF) and death from cardiovascular causes associated with baseline NT‐proBNP and changes in NT‐proBNP levels, adjusting for potential confounders. Results. NT‐proBNP levels in the highest quintile (>267.7 pg/mL) were independently associated with greater risks of HF (hazard ratio [HR], 3.05; 95% confidence interval [CI], 2.46–3.78) and cardiovascular death (HR, 3.02; 95% CI, 2.36–3.86) compared with the lowest quintile (<47.5 pg/mL). The inflection point for elevated risk occurred at NT‐proBNP 190 pg/mL. Among participants with initially low NT‐proBNP (<190 pg/mL), those with a >25% increase on follow‐up to >190 pg/mL (21%) were at greater adjusted risk for HF (HR, 2.13; 95% CI, 1.68–2.71) and cardiovascular death (HR, 1.91; 95% CI, 1.43–2.53) compared with those with sustained low levels. Among participants with initially high NT‐proBNP, those with a >25% increase (40%) were at higher risk for HF (HR, 2.06; 95% CI, 1.56–2.72) and cardiovascular death (HR, 1.88; 95% CI, 1.37–2.57), whereas those with a >25% decrease to ≤190 pg/mL (15%) were at lower risk for HF (HR, 0.58; 95% CI, 0.36–0.93) and cardiovascular death (HR, 0.57; 95% CI, 0.32–1.01) compared with those with unchanged high values. Conclusions. NT‐proBNP levels independently predict HF and cardiovascular death in older adults. NT‐proBNP levels frequently change over time, and these fluctuations reflect dynamic changes in cardiovascular risk.deFilippi CR, Christenson RH, Gottdiener JS, et al. Dynamic cardiovascular risk assessment in elderly people. The role of repeated N‐terminal pro‐B‐type natriuretic testing. J Am Coll Cardiol. 2010; 55: 441‐450.
Sources of Suspended‐Sediment Flux in Streams of the Chesapeake Bay Watershed: A Regional Application of the SPARROW Model1
Authors: Brakebill John W. , Ator Scott W. , Schwarz Gregory E. 
Published: June 2010

Brakebill, John W., Scott W. Ator, and Gregory E. Schwarz, 2010. Sources of Suspended‐Sediment Flux in Streams of the Chesapeake Bay Watershed: A Regional Application of the SPARROW Model. Journal of the American Water Resources Association (JAWRA) 46(4): 757‐776. DOI: 10.1111/j.1752‐1688.2010.00450.xAbstract:  We describe the sources and transport of fluvial suspended sediment in nontidal streams of the Chesapeake Bay watershed and vicinity. We applied SPAtially Referenced Regressions on Watershed attributes, which spatially correlates estimated mean annual flux of suspended sediment in nontidal streams with sources of suspended sediment and transport factors. According to our model, urban development generates on average the greatest amount of suspended sediment per unit area (3,928 Mg/km2/year), although agriculture is much more widespread and is the greatest overall source of suspended sediment (57 Mg/km2/year). Factors affecting sediment transport from uplands to streams include mean basin slope, reservoirs, physiography, and soil permeability. On average, 59% of upland suspended sediment generated is temporarily stored along large rivers draining the Coastal Plain or in reservoirs throughout the watershed. Applying erosion and sediment controls from agriculture and urban development in areas of the northern Piedmont close to the upper Bay, where the combined effects of watershed characteristics on sediment transport have the greatest influence may be most helpful in mitigating sedimentation in the bay and its tributaries. Stream restoration efforts addressing floodplain and bank stabilization and incision may be more effective in smaller, headwater streams outside of the Coastal Plain.
Radiation‐inducible human tumor necrosis factor‐related apoptosis‐inducing ligand (TRAIL) gene therapy: a novel treatment for radioresistant uveal melanoma
Authors: Zhou Yixiong , Song Xin , Jia Renbin , Wang Haibo , Dai Liyan , Xu Xiaofang , Gu Ping , Ge Shengfang , Fan Xianqun 
Published: June 2010

SummaryUveal melanoma (UM) is one of the most therapy‐resistant cancers. Radiotherapy is the preferred treatment for most cases of UM. However, some UM cells, such as the SP6.5 or OM431 cell lines, are relatively radioresistant. In this study, we attempted to improve the current UM therapy using an adenovirus radio‐inducible gene therapy system. The antitumor adenovirus was constructed by inclusion of the radiation‐inducible early growth response gene 1 (EGR1) promoter and the anticancer tumor necrosis factor‐related apoptosis‐inducing ligand (TRAIL) gene. We demonstrated that the UM SP6.5 and OM431 cell lines were susceptible to the TRAIL‐induced antitumor effect. TRAIL expression was enhanced in the adenovirus containing EGR1/TRAIL (Ad‐ET) treatment group by radiotherapy, whereas Ad‐ET significantly increased cell death and apoptosis caused by radiotherapy. In mice bearing xenograft tumors, apoptotic cells were detected in pathological tumor sections. Adenovirus Ad‐ET combined with radiation therapy significantly inhibited tumor growth compared with the other treatment groups (P < 0.01). Our findings indicate that radioresponsive gene therapy has the potential to be a more effective and specific therapy for UM because the therapeutic gene can be spatially or temporally controlled by exogenous radiation.
Prehypertension: the Rationale for Early Drug Therapy
Authors: Fuchs Flávio Danni 
Published: June 2010

SUMMARYBlood pressure within prehypertensive levels confers higher cardiovascular risk by two means. At first, these levels are associated with higher risk for cardiovascular events, starting at BP values as low as 115/75 mmHg, and doubling at each 20 mmHg for systolic or 10 mmHg for diastolic BP. Prehypertension is also an intermediate stage for full hypertension, which develops in an annual rate of 7 out 100 individuals with 40–50 years of age. The precocious drug intervention in patients with prehypertension is therefore appealing. In individuals with previous cardiovascular disease or diabetes the use of BP‐lowering agents is compulsory, since the 18–42% reduction of major cardiovascular events demonstrated in randomized clinical trials translates in palpable clinical benefit. In the absence of higher baseline risk, the absolute benefit of treatment is presumably small and was not demonstrated to date. These individuals could be candidate to treatment with the aim to prevent the development of full hypertension. The long‐lasting effectiveness of non‐drug therapies is low outside the controlled conditions of randomized clinical trials, and there are evidences that the use of BP‐lowering drugs reduces the incidence of hypertension in individuals with prehypertension by more than 60%. Clinical trials testing the efficacy and safety of BP agents to prevent hypertension in a population‐based perspective are required. In the meantime, it is worthy to present the option to start low doses of BP agents for individuals with prehypertension without co‐morbidities who do not respond to the prescription of lifestyle modification.
Oxidative Stress Impairs Endothelial Function in Nondipper Hypertensive Patients
Authors: Maio Raffaele , Perticone Maria , Sciacqua Angela , Tassone Eliezer J. , Naccarato Paola , Bagnato Chiara , Iannopollo Gianmarco , Sesti Giorgio , Perticone Francesco 
Published: June 2010

SUMMARYAims: Essential hypertension, as well as other established cardiovascular risk factors, is associated with endothelial dysfunction. Hypertensive patients with a nondipper circadian pattern have a greater risk of cerebrovascular and cardiovascular complications in comparison with those with a dipper circadian pattern. In this study, we evaluated the association between nondipper pattern and endothelial function in patients with essential hypertension. Methods: We evaluated the forearm blood flow (FBF) response to intraarterial acetylcholine (ACh), an endothelium‐dependent vasodilator, and sodium nitroprusside (SNP), an endothelium‐independent vasodilator, infusions in 190 hypertensive patients stratified according to dipper and nondipper status. The FBF was measured by strain‐gauge plethysmography. Effects of oxidative stress on FBF were evaluated by intraarterial infusion of vitamin C. Ambulatory BP monitorings were obtained by a validated oscillometric device (SpaceLabs 90207 Monitor Inc., Issaquah, WA, USA). Results: Systolic and diastolic blood pressures were higher during daytime and lower during night‐time in dipper subjects than in nondippers. The peak percent increase in ACh‐stimulated FBF was higher in dippers than in nondippers (473% vs. 228%, P < 0.001). The FBF responses to SNP were similar in dipper and nondipper patients. The FBF response to ACh during coinfusion of vitamin C was higher in nondippers rather than in dipper hypertensives. Conclusions: Present data demonstrate that endothelium‐dependent vasodilation is impaired in patients who have nondipper hypertension. The effects of vitamin C on impaired ACh‐stimulated vasodilation support the hypothesis that oxidative stress contributes to endothelial dysfunction of nondipper hypertensive patients.
REVIEW: An Approach for Neuroprotective Therapies of Secondary Brain Damage after Excitotoxic Retinal Injury in Mice
Authors: Ito Yasushi , Shimazawa Masamitsu , Hara Hideaki 
Published: June 2010

SUMMARY Background: Many current therapeutic strategies for several eye diseases, such as glaucoma, retinal ischemia, and optic neuropathy, are focused on protection of the retinal ganglion cells (RGCs). In fact, loss of visual field, including irreversible blindness, is caused by RGC damage in these diseases. However, recent evidence suggests that the RGC damage extends to visual center in brain: the visual impairment induced by these diseases may result not only from RGC loss, but also from neuronal degeneration within the visual center in brain. Objective: To protect neurons within the visual center in the brain, as well as retinal treatment, for the prevention of visual disorder in these diseases. Methods: Once considered difficult to study the visual center in brain following RGCs loss, because obtaining the human samples that are suitable for the study may be difficult. In addition, the monkey, mainly used as glaucomatous model, is relatively high cost and needs to long experiment‐span. Here, we focused on mice, because of their high degree of availability, relatively low cost, and amenability to experimental and genetic manipulation. Conclusion: In this review, we describe time‐dependent alterations in the visual center in brain following RGCs loss, and whether some drugs prevent the neuronal damage of the visual center in the brain.