bluuebunny/crossref_metadata_2025 huggingface dataset:
Helping the cognitive system learn: exaggerating distinctiveness and uniqueness
Authors: Dror Itiel E. , Stevenage Sarah V. , Ashworth Alan R. S.
Published: July 2007

The caricature advantage demonstrates that performance is better when exaggerated stimuli are presented rather than a faithful image. This can be understood with respect to a theoretical framework in which caricaturing maximises the distinctiveness and thus minimises any perceptual or representational confusion. In this study we examine the possibility to harness caricatures to enhance learning. Thus, during learning the caricatures help the cognitive system pick up the unique and distinctive features of the learned material. This in turn helps to construct representations that correctly direct attention to the critical information. We trained 113 participants to identify aircraft across any orientation and found that the use of caricature is advantageous. However, the caricature advantage was most effective in complex learning where it is difficult to differentiate among different aircraft. Furthermore, the caricature advantage for subsequent recognition is attenuated when over‐learning has been achieved. These results are discussed in terms of the learning situations in which caricatures can be most effective in enhancing learning. Copyright © 2007 John Wiley & Sons, Ltd.
A novel mutation of the β‐globin gene promoter (−102 C>A) and pitfalls in family screening
Authors: Aguilar‐Martinez Patricia , Jourdan Eric , Brun Sophie , Cunat Séverine , Giansily‐Blaizot Muriel , Pissard Serge , Schved Jean‐François
Published: July 2007

We describe a family with β‐thalassemia in which several pitfalls of genetic diagnoses were present. These include coherent family phenotypes with discrepancies in molecular findings because of nonpaternity, and a false β‐globin gene homozygous genotype due to a large deletion in the second locus. These findings underline the difficulties of family genetic studies and the need for tight relationship between professionals involved in laboratory studies and those in‐charge of the clinical follow‐up and genetic counselling. In this family, we also report a new silent β‐thalassemia mutation, −102 (C>A), in the distal CACCC box of the β‐globin gene promoter. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.
Long‐term outcome of Leigh syndrome caused by the NARP‐T8993C mtDNA mutation
Authors: Debray François‐Guillaume , Lambert Marie , Lortie Anne , Vanasse Michel , Mitchell Grant A.
Published: July 2007

Mutations at mitochondrial DNA (mtDNA) nucleotide 8993 can cause neurogenic weakness, ataxia and retinitis pigmentosa (NARP syndrome), or maternally inherited Leigh syndrome (LS), with a correlation between the amount of mutant mtDNA and the severity of the neurological disease. The T8993C mutation is generally considered to be clinically milder than the T8993G mutation but when the level of heteroplasmy exceeds 90%, progressive neurodegeneration has been found. We report on a long‐term follow‐up of a patient who presented at 4 years of age with typical LS but showed an unexpected resolution of his symptoms and a favorable outcome. At 18 years of age, his neurological examination was near normal, with neither peripheral neuropathy nor retinopathy. mtDNA analysis identified the presence of T8993C mutation at high level (>95%) in the patient's blood leukocytes. This case report and literature review emphasizes the variability of the phenotypic expression of the T8993C mutation and the need for caution in predictive counseling in such patients. © 2007 Wiley‐Liss, Inc.
Four siblings with distal renal tubular acidosis and nephrocalcinosis, neurobehavioral impairment, short stature, and distinctive facial appearance: A possible new autosomal recessive syndrome
Authors: Faqeih Eissa , Al‐Akash Samhar I. , Sakati Nadia , Teebi Prof. Ahmad S.
Published: July 2007

We report on four siblings (three males, one female) born to first cousin Arab parents with the constellation of distal renal tubular acidosis (RTA), small kidneys, nephrocalcinosis, neurobehavioral impairment, short stature, and distinctive facial features. They presented with early developmental delay with subsequent severe mental, behavioral and social impairment and autistic‐like features. Their facial features are unique with prominent cheeks, well‐defined philtrum, large bulbous nose, V‐shaped upper lip border, full lower lip, open mouth with protruded tongue, and pits on the ear lobule. All had proteinuria, hypercalciuria, hypercalcemia, and normal anion‐gap metabolic acidosis. Renal ultrasound examinations revealed small kidneys, with varying degrees of hyperechogenicity and nephrocalcinosis. Additional findings included dilated ventricles and cerebral demyelination on brain imaging studies. Other than distal RTA, common causes of nephrocalcinosis were excluded. The constellation of features in this family currently likely represents a possibly new autosomal recessive syndrome providing further evidence of heterogeneity of nephrocalcinosis syndromes. © 2007 Wiley‐Liss, Inc.
Association study between gilles de la tourette syndrome and two genes in the robo‐slit pathway located in the chromosome 11q24 linked/associated region
Authors: Miranda D.M. , Wigg K. , Feng Y. , Sandor P. , Barr C.L.
Published: August 2007

Gilles de la Tourette Syndrome (GTS) is an inherited neuropsychiatric disorder characterized by the presence of motor and phonic tics. Previous genetic studies have identified linkage and association between GTS and the 11q24 chromosomal region. We selected for study, within this region, two possible susceptibility genes for GTS, the ROBO3 and ROBO4 genes. These two genes were selected because of the recent identification of SLITRK1 as a potential susceptibility gene for GTS based on a translocation breakpoint and the further finding of two mutations in the SLITRK1 gene in three patients with GTS. While thus far, the SLITRK1 gene appears to account for only a few cases of GTS, these findings, if confirmed, point to other genes in these pathways that may contribute to GTS. Based on this, we examined two genes in the Slit‐Robo pathway involved in cell migration, axonal pathfinding, and/or neuronal differentiation because of their location in 11q24, a region previously identified as linked and associated with GTS. We selected six haplotype tagging single nucleotide polymorphisms (SNPs) for ROBO3 and four for ROBO4 and genotyped them in our sample of trios and sibpair families diagnosed with GTS. Based on 155 nuclear families with 255 affected children, we did not find evidence for association between GTS and either the ROBO3 or ROBO4 genes. Thus, these two genes are unlikely to be the susceptibility genes contributing to GTS on 11q24. © 2007 Wiley‐Liss, Inc.
Azobisisobutyronitrile‐initiated free‐radical copolymerization of limonene with vinyl acetate: Synthesis and characterization
Authors: Sharma Saroj , Srivastava A. K.
Published: August 2007

The solution copolymerization of limonene (Lim) and vinyl acetate (VA) with azobisisobutyronitrile (AIBN) as an initiator in dioxane at 65 ± 0.1°C for 2 h under a nitrogen blanket has been carried out. The kinetic expression is [AIBN]0.5[VA]1.0[Lim]−1.0. The overall energy of activation has been computed to be 30 kJ/mol. Fourier transform infrared spectroscopy has shown bands at 1731 cm−1 due to acetoxy protons of VA and at 1689 cm−1 due to trisubstituted olefinic protons (CH CHCH2) of Lim. 1H‐NMR spectroscopy of the copolymer has shown peaks at δ = 1.2–1.5 and δ = 5.3–5.6 due to acetoxy and trisubstituted olefinic protons of VA and Lim, respectively. The reactivity ratios have been calculated to be 0.05 for VA and 0.011 for Lim with the Kelen–Tüdos method. The glass‐transition temperature of the copolymer, determined by differential scanning calorimetry, is 32.4°C. The copolymer is likely biodegradative in nature. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Preparation, characterization, and utilization of cationized hemp as anion exchanger for removing permanganates and dichromate anions
Authors: Fahmy H. M. , Kohler R.
Published: July 2007

Using a software experimental design strategy and through 25 experiments, steam exploded hemp bast fibers (HBF) as well as their shives were cationized by the exhaustion method using 3‐chloro‐2‐hydroxypropyl trimethyl ammonium chloride solution. The results obtained revealed that at pH 13, raising the temperature of a bath containing 100 g/L cationizing agent aqueous solution at liquor to substrate ratio 1/15 to 80°C for 90 min produces the cationized forms of HBFs as well as their shives with percent nitrogen content 0.2240 and 0.4893 respectively. The statistical analysis for the results indicated that the percent nitrogen content is significantly influenced by factors of time, pH, and primarily the cationizing agent concentration. The cationized forms of HBFs as well as their shives, having amino contents 76 and 104 mequiv/100 g respectively, were characterized by studying their potentiometric titration and were analyzed by thermogravimetric analysis. Both of these cationized substrates were utilized as anion exchangers for adsorption of permanganate and dichromate anions from their acidic aqueous solutions. Adsorption dynamics of the removal of such anions were first‐order reactions whereas the adsorption isotherms showed that the adsorption of those anions on all the aforementioned anion exchangers was favorable. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Effects of cashew nut shell liquid as a plasticizer on cure characteristics, processability, and mechanical properties of 50 : 50 NR/EPDM blends: A comparison with paraffin oil
Authors: Arayapranee Wanvimon , Rempel Garry L.
Published: August 2007

50 : 50 natural rubber (NR) and ethylene–propylene–diene monomer rubber (EPDM) blends were prepared with different contents of cashew nut shell liquid (CNSL), a natural product obtained from the shells of the cashew nut, as a plasticizer. For comparison, a commercial paraffin oil plasticizer was also used. The effect of plasticizer content on the cure characteristics, processability, and mechanical properties such as tensile strength, elongation at break, and Young's modulus before and after ageing was investigated. Scanning electron microscopy (SEM) was used to observe the blend morphology. The results indicated that the CNSL plasticizer resulted in lower Mooney viscosity and lower cure time of the 50 : 50 NR/EPDM blends. The incorporation of CNSL into 50 : 50 NR/EPDM blends improved tensile strength and elongation at break but decreased Young's modulus. On addition of CNSL the resistance of the blends to heat and weathering ageing improved. Scanning electron micrographs revealed that the morphology of the blend plasticized with CNSL is finer and more homogeneous compared with the blend plasticized with paraffin oil. Overall results indicate that CNSL can be used as a cheaper plasticizer to replace paraffin oil in NR/EPDM blends with improved processability and mechanical properties. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Curing and thermal behaviour of epoxy resin in the presence of pyromellitic dianhydride and imidazole
Authors: Jain Ritu , Choudhary Veena , Narula A. K.
Published: July 2007

The curing behaviour of DGEBA was investigated by differential scanning calorimetry in the presence of varying amounts of PMDA. The molar ratio of DGEBA : PMDA was varied as 1 : 0.8, 1 : 1, 1 : 1.5, 1 : 2.0, and 1 : 2.5. The heat of polymerization (ΔH) was found to be maximum at a molar ratio of 1 : 0.8 (DGEBA : PMDA). To study the effect of imidazole content on the curing behaviour, varying amounts of imidazole, i.e., 0.1, 0.15, 0.2, and 0.3% (w/w) were used keeping the ratio of DGEBA : PMDA (1 : 0.8) constant. A broad exotherm was observed in all the samples. A significant decrease in the peak exotherm was observed when 0.1% imidazole was used. Further increase in the imidazole concentration up to 0.2% resulted in a marginal decrease in the peak exotherm temperature. Thermal stability of epoxy resin, cured isothermally, was evaluated by recording thermogravimetric traces in nitrogen atmosphere. The percent char yield was highest in case of resin sample, IP1‐30 (i.e., DGEBA (1 mole) cured using 0.8 mole of PMDA in the presence of 0.3 (w/w) of imidazole). © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Modification of polysulfide sealant with polysulfide polythio‐urethane‐urea
Authors: Quan Yiwu , He Peng , Zhou Baijie , Chen Qingmin
Published: July 2007

With a small amount of isocyanate‐terminated polysulfide (NCO‐PSF‐NCO) as additive, the commercial polysulfide‐based sealant had significant improvement in mechanical properties. The test results showed that 5% of NCO‐PSF‐NCO could help to rise 50% in the tensile strength and 27% in the ultimate elongation of the polysulfide sealant. The adhesive strength to substrates such as steel, epoxy‐coated or polyurethane‐coated surface also increased significantly. Dynamic mechanical thermal analysis and differential scanning calorimetry analysis revealed that formed polysulfide polythio‐urethane‐urea (polysulfide‐urea) contributed to a slight increase in the Tg of the polysulfide sealant. It might be due to the domains of polysulfide‐urea, which had effects of physical crosslinking and chain‐extending on the polysulfide sealant. However, the introduction of polysulfide‐urea structures had no appreciable influence on oil resistance of the polysulfide sealant. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci, 2007
Mechanical and thermal properties of polycarbonate. II. Influence of titanium dioxide content and quenching on pigmented polycarbonate
Authors: Rouabah F. , Fois M. , Ibos L. , Boudenne A. , Dadache D. , Haddaoui N. , Ausset P.
Published: August 2007

The effects of quenching temperature including different thermal histories on mechanical, physical, and thermal properties of pigmented polycarbonate (PC/TiO2) were investigated. Tensile test, Izod impact strength and heat distortion temperature (HDT) were performed on specimens of 3 mm thickness. Pigment content and quenching temperature are two key factors that affect the properties of the materials. A higher content of pigments results in an increase of modulus of elasticity and a decrease of unotched and notched Izod impact strength, as well as elongation at break. A maximum of yield stress and HDT is obtained at 3% of TiO2, which was considered as the optimum level of pigment. An additional second quenching at 40°C has allowed to improve Izod impact strength and elongation at break of specimens with 3% of TiO2; whereas modulus of elasticity, density, yield stress, and HDT were minimum at this quenching temperature. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Crystallization behavior of dry‐brush PEO‐PS block copolymer and PEO homopolymer blend
Authors: Gao Ying , Liu Honglai
Published: August 2007

The crystallization behavior of semicrystalline PEO homopolymer/triblock PS‐PEO‐PS copolymer blend system, which exhibited “Dry‐Brush” in the melt. A symmetric polystyrene–poly(ethylene oxide)–polystyrene triblock copolymer was blended with PEO homopolymer (h‐PEO) having the same molecular weight as that of the PEO block in the copolymer. Considering the composition of the blend (Wps≥ 0.8), PEO spheres were formed in the blend. Because of the dry‐brush phase behavior of this blend, h‐PEO added was localized in the PEO microdomains, which increases the domain size without changing the microdomain morphology. The crystallization of PEO block was confined within the microdomains and the crystallization temperature was about 60°C lower than normal. Self‐seeding tests were performed to clarify the nucleation mechanism of the blend. Because the droplets size varies greatly, multicrystallization peaks were witnessed in the self‐seeding process. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Photopolymerization of clay/polyurethane nanocomposites induced by intercalated initiator
Authors: Tan Hailin , Nie Jun
Published: July 2007

An intercalated initiator was synthesized and used for preparation of clay/polyurethane nanocomposites by UV irradiation. Organoclays containing initiator groups were prepared by cationic exchange process which acted as both suitable intercalant and photoinitiator. These modified clays were then dispersed in the mixture of urethane acrylate and hexanediol diacrylate in different loading, then situ photopolymerized. Intercalated and exfoliated nanocomposite structure were evidenced by both X‐ray diffraction spectroscopy and Transmission Electron Microscope. Thermal properties and morphologies of the resultant nanocomposites were also investigated. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Photocatalytic functional cotton fabrics containing benzophenone chromophoric groups
Authors: Hong Kyung Hwa , Sun Gang
Published: July 2007

In preparation of self‐decontamination clothing materials in high reactivities against toxic agents, photoactive benzophenone chromophoric groups were incorporated into cotton fabrics. The cotton fabrics were treated by using 4‐hydroxybenzophenone as a reagent, 1,2,3,4,‐butanetetra carboxylic acid (BTCA) as a crosslinker, and sodium hypophosphite as a catalyst. The fabric treatment was conducted by a pad‐dry‐cure method. The benzophenone chromophoric group incorporated cotton fabrics were characterized by FTIR, SEM, TGA, and so on. The results confirmed the expected structures of the benzophenone chromophoric group modified and BTCA crosslinked cotton fabrics. The treated cotton fabrics demonstrated radical reactivities and antibacterial activity under UV irradiation. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci 2007
Kinetics and mechanism of the decay of methyl cellulose‐manganate(VI) polysaccharide transient species–novel spectrophotometric kinetic trace of methyl cellulose hypomangate(V) gel intermediate polysaccharide
Authors: Shaker Ali M. , El‐Khatib Rafat M. , Mahran Hossnia S.
Published: July 2007

Kinetics of the decay of methyl cellulose‐manganate(VI) intermediate polysaccharide [MC‐MnO] have been followed spectrophotometrically in different strong basic solutions at different temperatures, as well as, in the presence of different concentrations of perchlorate salt. The reaction is base‐catalyzed. Salt never affects the reactivity. The activation parameters have been evaluated and discussed in connection with the proposal mechanism. © 2007 Wiley Periodicals, Inc. J Appl Polym Sci, 2007
Hand vascular involvement assessed by magnetic resonance angiography in systemic sclerosis
Authors: Allanore Y. , Seror R. , Chevrot A. , Kahan A. , Drapé J. L.
Published: July 2007

Objective

Impairment of the microcirculation is a cardinal feature of systemic sclerosis (SSc). Magnetic resonance angiography (MRA) has improved the assessment of vascular lesions of the hand. The aim of this study was to evaluate vascular abnormalities in the hands of patients with SSc, using MRA.

Methods

Thirty‐eight patients with SSc were compared with 7 healthy subjects and 7 patients with rheumatoid arthritis. Among patients with SSc, the mean ± SD age was 52 ± 14 years, the mean ± SD Health Assessment Questionnaire (HAQ) score was 0.9 ± 0.8, and the mean ± SD systolic pulmonary artery pressure (PAP) was 32.2 ± 8.4 mm Hg. Ten patients had a history of digital ulcers. The MRA protocol consisted of 4 successive acquisitions, each lasting 52 seconds, of 3‐dimensional coronal cross‐sectional images after gadolinium injection. The primary criteria were distality and quality of arterial opacification, avascular areas, and venous return.

Results

Thirty‐five of the patients with SSc (92%) had at least 1 true digital artery that did not reach the first phalanx at the initial arterial analysis, and 23 patients (61%) had ≥4 damaged arteries. Twenty‐eight patients (74%) had thin arteries, and 20 patients (53%) had >1 avascular area. Nearly all patients (35 of 38 [92%]) had abnormal venous return, and a lack of visible venous return was observed in 16 patients (42%). Results for all of the control subjects were considered normal. Digital ulcers were more frequently observed in patients with SSc who had ≥4 damaged proper digital arteries compared with other patients (P = 0.003), the HAQ score was associated with thin‐caliber arteries (P = 0.04), and systolic PAP was associated with tissue enhancement secondary to ischemia (P = 0.04).

Conclusion

These results show the substantial vascular involvement in SSc. Lesions were diffuse and involved both arterial and venous vessels of small caliber as well as the microcirculation.
An unexpectedly high frequency of MEFV mutations in patients with anti–citrullinated protein antibody–negative palindromic rheumatism
Authors: Cañete Juan D. , Arostegui Juan I. , Queiró Rubén , Gratacós Jordi , Hernández M. Victoria , Larrosa Marta , Alperí Mercedes , Moll Conchita , Rius Josefa , Sanmartí Raimon , Yagüe Jordi
Published: July 2007

Objective

To investigate whether the MEFV gene, which is involved in the regulation of the inflammatory response and has been associated with familial Mediterranean fever (FMF) and intermittent hydrarthrosis, is implicated in the pathogenesis of palindromic rheumatism (PR) and to examine its clinical presentation and its evolution in a Spanish cohort of PR patients.

Methods

Family histories, demographic clinical data, and laboratory characteristics of 75 patients diagnosed as having PR were collected from medical records and personal interviews. The healthy control group included 325 blood bank donors. The FMF control group was made up of 84 Spanish FMF patients. Genomic DNA was isolated, and MEFV gene mutation analysis was performed by polymerase chain reaction amplification and sequence analysis.

Results

Sixty‐five unrelated PR patients were finally included in the study. MEFV gene mutation analysis identified 8 of these 65 patients (12.3%) as carriers of at least 1 mutated MEFV allele. Patients with MEFV mutations had higher mean age and age at disease onset, but lower mean serum levels of anti–citrullinated protein antibodies (ACPAs). No other significant differences were observed between patients with and those without mutations. The frequency of MEFV mutations in ACPA‐negative PR patients was 22.2%, compared with 5.3% in ACPA‐positive PR patients (P = 0.058).

Conclusion

This study shows a previously unreported high prevalence of mutations of the MEFV gene in patients with ACPA‐negative PR. This supports the hypothesis that it might be a susceptibility gene. Our findings also support the hypothesis that the MEFV gene might participate in the pathogenesis of other undifferentiated relapsing inflammatory rheumatic disorders.
Infliximab inhibits progression of radiographic damage in patients with active psoriatic arthritis through one year of treatment: Results from the induction and maintenance psoriatic arthritis clinical trial 2
Authors: van der Heijde D. , Kavanaugh A. , Gladman D. D. , Antoni C. , Krueger G. G. , Guzzo C. , Zhou B. , Dooley L. T. , de Vlam K. , Geusens P. , Birbara C. , Halter D. , Beutler A. ,   IMPACT 2 Study Group
Published: July 2007

Objective

To evaluate the effect of infliximab on progression of structural damage over 1 year in patients with active psoriatic arthritis (PsA) enrolled in the Induction and Maintenance Psoriatic Arthritis Clinical Trial 2.

Methods

In this double‐blind, placebo‐controlled study, 200 patients with active PsA were randomly assigned (1:1 ratio) to receive infusions of infliximab (5 mg/kg) or placebo at weeks 0, 2, and 6, and every 8 weeks thereafter through week 54. At week 24, patients initially assigned to receive placebo crossed over to receive infliximab (5 mg/kg). Based on predefined criteria, patients randomized to receive placebo could enter early escape by receiving infliximab (5 mg/kg) starting at week 16, and patients randomized to receive infliximab could have the dose increased to 10 mg/kg starting at week 38. Patients were analyzed according to the treatment they were randomized to receive. Radiographs of hands and feet were obtained at baseline and at weeks 24 and 54. Two readers blinded to treatment assignment and radiograph sequence independently evaluated erosions and joint space narrowing using the Sharp/van der Heijde scoring method modified for PsA.

Results

At week 24, patients randomized to receive infliximab 5 mg/kg had significantly less radiographic progression compared with patients randomized to receive placebo, with mean ± SD changes from baseline in the total Sharp/van der Heijde score of −0.70 ± 2.53 and 0.82 ± 2.62, respectively (P< 0.001). At week 54, mean ± SD changes from baseline in the total Sharp/van der Heijde score were −0.94 ± 3.40 in patients randomized to receive infliximab and 0.53 ± 2.60 in those receiving placebo/infliximab (P= 0.001).

Conclusion

Infliximab significantly inhibits radiographic progression in patients with PsA as early as 6 months after starting treatment, and the beneficial effect continues through 1 year of infliximab therapy.
Modulation of interleukin‐6 and matrix metalloproteinase 2 expression in human fibroblast‐like synoviocytes by functional ionotropic glutamate receptors
Authors: Flood Sophie , Parri Rheinallt , Williams Anwen , Duance Victor , Mason Deborah
Published: July 2007

Objective

Patients with rheumatoid arthritis (RA) have increased concentrations of the amino acid glutamate in synovial fluid. This study was undertaken to determine whether glutamate receptors are expressed in the synovial joint, and to determine whether activation of glutamate receptors on human synoviocytes contributes to RA disease pathology.

Methods

Glutamate receptor expression was examined in tissue samples from rat knee joints and in human fibroblast‐like synoviocytes (FLS). FLS from 5 RA patients and 1 normal control were used to determine whether a range of glutamate receptor antagonists influenced expression of the proinflammatory cytokine interleukin‐6 (IL‐6), enzymes involved in matrix degradation and cytokine processing (matrix metalloproteinase 2 [MMP‐2] and MMP‐9), and the inhibitors of these enzymes (tissue inhibitor of metalloproteinases 1 [TIMP‐1] and TIMP‐2). IL‐6 concentrations were determined by enzyme‐linked immunosorbent assay, MMP activity was measured by gelatin zymography, and TIMP activity was determined by reverse zymography. Fluorescence imaging of intracellular calcium concentrations in live RA FLS stimulated with specific antagonists was used to reveal functional activation of glutamate receptors that modulated IL‐6 or MMP‐2.

Results

Ionotropic and metabotropic glutamate receptor subunit mRNA were expressed in the patella, fat pad, and meniscus of the rat knee and in human articular cartilage. Inhibition ofN‐methyl‐D‐aspartate (NMDA) receptors in RA FLS increased proMMP‐2 release, whereas non‐NMDA ionotropic glutamate receptor antagonists reduced IL‐6 production by these cells. Stimulation with glutamate, NMDA, or kainate (KA) increased intracellular calcium concentrations in RA FLS, demonstrating functional activation of specific ionotropic glutamate receptors.

Conclusion

Our findings indicate that activation of NMDA and KA glutamate receptors on human synoviocytes may contribute to joint destruction by increasing IL‐6 expression.
Aerobic and anaerobic exercise capacity in children with juvenile idiopathic arthritis
Authors: van Brussel M. , Lelieveld O. T. H. M. , van der Net J. , Engelbert R. H. H. , Helders P. J. M. , Takken T.
Published: July 2007

Objective

To compare the aerobic and anaerobic exercise capacity of children with juvenile idiopathic arthritis (JIA) with healthy controls, to determine if there were differences based on disease onset type, and to examine the relationship between aerobic and anaerobic exercise capacity in children with JIA.

Methods

Sixty‐two patients with JIA (mean ± SD age 11.9 ± 2.2 years, range 6.7–15.9) participated in this study. Aerobic exercise capacity was measured using a cardiopulmonary exercise test. Anaerobic exercise capacity was measured using the Wingate Anaerobic Exercise Test (WAnT).

Results

All patients were able to perform the cardiopulmonary exercise test and WAnT without adverse events. On average, the maximal oxygen uptake (VO2peak) and VO2peak/kg were 69.8% and 74.8%, respectively, of that predicted compared with healthy controls. Mean ± SD power was 66.7% ± 37.2% of that predicted compared with healthy children. Mean ± SD peak power was 65.5% ± 43.1% of that predicted compared with healthy children. There were significant differences between subgroups of JIA; the oligoarticular‐onset group values did not significantly differ from healthy control values; the polyarticular rheumatoid factor positive–onset subgroup had the greatest impairment in both aerobic and anaerobic exercise capacity. The correlations of mean power and peak power with VO2peak were r = 0.884 and r = 0.697, respectively (P < 0.05).

Conclusion

This study demonstrates that both the aerobic and anaerobic exercise capacity in children with JIA are significantly decreased. The WAnT might be a valuable adjunct to other assessment tools in the followup of patients with JIA.
Minimal disease activity, remission, and the long‐term outcomes of rheumatoid arthritis
Authors: Wolfe Frederick , Rasker Johannes J. , Boers Maarten , Wells George A. , Michaud Kaleb
Published: July 2007

Objective

To determine the prevalence of minimal disease activity (MDA) and remission in patients with rheumatoid arthritis (RA), to assess the effect of anti–tumor necrosis factor (anti‐TNF) therapy on MDA, and to determine the extent to which MDA status improves long‐term outcomes.

Methods

Using the Patient Activity Scale (PAS) as a surrogate, we assessed the prevalence of MDA and remission in 18,062 patients with RA using the newly developed Outcome Measures in Rheumatology Clinical Trials (OMERACT) criteria for MDA.

Results

MDA was noted in 20.2% of 18,062 patients and persistent MDA, operationally defined as having MDA during ≥2 consecutive 6‐month observation periods, occurred in 13.5% of 7,433 patients followed longitudinally. Disease activity at remission levels was noted in 7%. Among patients with MDA, 82% received disease‐modifying antirheumatic drugs or biologic agents. Following anti‐TNF initiation, the cumulative probability of achieving MDA at 2 and 6 years was 4.1% and 7.6%, respectively, and persistent MDA probabilities were 2.7% and 4.5%, respectively. Regardless of RA duration, patients with MDA had substantially better outcomes, including a 10‐fold reduction in work disability and an approximately 2‐fold reduction in total joint replacement and mortality.

Conclusion

Remission remains uncommon in RA, and the prevalence of new remission in community practice is substantially lower than noted in published trials of biologic therapy. On average, persons with MDA appear to have persistently mild RA. This might be the effect of milder RA and/or more effective treatment in early RA. The PAS had satisfactory levels of agreement with the full MDA criteria and appears suitable for use in clinical and epidemiologic research.
Development and validation of a new short and simple measure of physical function for juvenile idiopathic arthritis
Authors: Filocamo Giovanni , Sztajnbok Flavio , Cespedes‐cruz Adriana , Magni‐Manzoni Silvia , Pistorio Angela , Viola Stefania , Ruperto Nicolino , Buoncompagni Antonella , Loy Anna , Martini Alberto , Ravelli Angelo
Published: July 2007

Objective

To develop and validate a new short and simple measure of physical function in children with juvenile idiopathic arthritis (JIA).

Methods

The Juvenile Arthritis Functionality Scale (JAFS) is a 15‐item questionnaire that explores physical function in 3 body areas (lower limbs, hand/wrist, and upper segment). Validation of the Italian version of the instrument was accomplished by evaluating 211 consecutive JIA patients ages 2.2–18 years. The instrument's feasibility, face and content validity, construct and discriminative ability, internal consistency, interrater reliability, and responsiveness to clinical change were examined. JAFS psychometric properties were compared with those of the Childhood Health Assessment Questionnaire (C‐HAQ).

Results

The JAFS was found to be feasible and to possess both face and content validity. The JAFS score correlated with most of the other JIA outcome measures in the range predicted, thereby demonstrating good construct validity, and discriminated well among different levels of disability. The internal consistency (Cronbach's alpha) was 0.82. The intraclass correlation coefficients between raters (mothers, fathers, and children) and between reported and observed level of function ranged from 0.65 to 0.84. The JAFS revealed fair responsiveness, with a standardized response mean ranging from 0.42 to 0.56. Comparison with the C‐HAQ indicated that the JAFS may be superior in terms of construct validity and reliability, and at least as good in terms of discriminant validity and responsiveness.

Conclusion

The JAFS exhibited good reliability, construct validity, and discriminative ability and fair responsiveness, and is therefore a valid instrument for the assessment of physical function in children with JIA.
Predictors of exercise and effects of exercise on symptoms, function, aerobic fitness, and disease outcomes of rheumatoid arthritis
Authors: Neuberger Geri B. , Aaronson Lauren S. , Gajewski Byron , Embretson Susan E. , Cagle Perri E. , Loudon Janice K. , Miller Peggy A.
Published: July 2007

Objective

To determine the effects of participation in a low‐impact aerobic exercise program on fatigue, pain, and depression; to examine whether intervention groups compared with a control group differed on functional (grip strength and walk time) and disease activity (total joint count, erythrocyte sedimentation rate, and C‐reactive protein) measures and aerobic fitness at the end of the intervention; and to test which factors predicted exercise participation.

Methods

A convenience sample of 220 adults with rheumatoid arthritis (RA), ages 40–70, was randomized to 1 of 3 groups: class exercise, home exercise using a videotape, and control group. Measures were obtained at baseline (T1), after 6 weeks of exercise (T2), and after 12 weeks of exercise (T3).

Results

Using structural equation modeling, overall symptoms (latent variable for pain, fatigue, and depression) decreased significantly at T3 (P < 0.04) for the class exercise group compared with the control group. There were significant interaction effects of time and group for the functional measures of walk time and grip strength: the treatment groups improved more than the control group (P ≤ 0.005). There were no significant increases in measures of disease activity. Fatigue and perceptions of benefits and barriers to exercise affected participants' amount of exercise, supporting previous research.

Conclusion

This study supported the positive effects of exercise on walk time and grip strength, and demonstrated that fatigue and perceived benefits/barriers to exercise influenced exercise participation. Furthermore, overall symptoms of fatigue, pain, and depression were positively influenced in this selective group of patients with RA ages 40–70 years.
Racial/ethnic differences in activities of daily living disability in older adults with arthritis: A longitudinal study
Authors: Song Jing , Chang Huan J. , Tirodkar Manasi , Chang Rowland W. , Manheim Larry M. , Dunlop Dorothy D.
Published: July 2007

Objective

To investigate racial/ethnic differences in disability onset among older Americans with arthritis. Factors amenable to clinical and public health intervention that may explain racial/ethnic differences in incident disability were examined.

Methods

We analyzed longitudinal data (1998–2004) from a national representative sample of 5,818 non‐Hispanic whites, 1,001 African Americans, 228 Hispanics interviewed in Spanish (Hispanic/Spanish), and 210 Hispanics interviewed in English (Hispanic/English), with arthritis and age ≥51 years who did not have baseline disability. Disability in activities of daily living (ADL) was identified from report of inability, avoidance, or needing assistance to perform ≥1 ADL task.

Results

Over the period of 6 years, 28.0% of African Americans, 28.5% of Hispanic/Spanish, 19.1% of Hispanic/English, and 16.2% of whites developed disability. The demographic‐adjusted disability hazard ratios (AHR) were significantly greater among African Americans (AHR 1.94, 95% confidence interval [95% CI] 1.51–2.38) and Hispanic/Spanish (AHR 2.03, 95% CI 1.35–2.71), but not significantly increased for Hispanic/English (AHR 1.41, 95% CI 0.82–2.00) compared with whites. Differences in health factors (comorbid conditions, functional limitations, and behaviors) explained over half the excess risk among African Americans and Hispanic/Spanish. Medical access factors (education, income, wealth, and health insurance) were substantial mediators of racial/ethnic differences in all minority groups.

Conclusion

Racial/ethnic differences in the development of disability among older adults with arthritis were largely attenuated by health and medical access factors. Lack of health insurance was particularly problematic. At the clinical level, treatment of comorbid conditions, functional limitations, and promotion of physical activity and weight maintenance should be a priority to prevent the development of disability, especially in minority populations.
Effects of adherence to treatment on short‐term outcomes in children with juvenile idiopathic arthritis
Authors: Feldman Debbie Ehrmann , De Civita Mirella , Dobkin Patricia L. , Malleson Peter N. , Meshefedjian Garbis , Duffy Ciarán M.
Published: July 2007

Objective

To determine the impact of adherence to treatment (medication and prescribed exercise) on outcomes in children with juvenile idiopathic arthritis (JIA).

Methods

In this longitudinal study, we studied parents of patients with JIA at the Montreal Children's Hospital and British Columbia Children's Hospital in Vancouver. Adherence was evaluated on a visual analog scale in the Parent Adherence Report Questionnaire. Outcomes of interest were active joint count, pain, child functional score on the Child Health Assessment Questionnaire, quality of life score on the Juvenile Arthritis Quality of Life Questionnaire, and parental global impression of overall well‐being. The association between adherence to treatment and subsequent outcomes was evaluated using generalized estimating equations and logistic regression.

Results

Mean age and disease duration of our sample of 175 children were 10.2 and 4.1 years, respectively. Moderate adherence to medication was associated with lower active joint count (odds ratio [OR] 0.47, 95% confidence interval [95% CI] 0.22–0.99). Moderate adherence to exercise was associated with better functional score (OR 0.13, 95% CI 0.03–0.54), and lower pain during the last week (OR 0.14, 95% CI 0.04–0.50). Both high and moderate adherence to exercise were associated with parental perception of global improvement.

Conclusion

Improved outcomes in patients who adhered to treatment underscores the need for clinicians to address adherence issues with their patients. Sustaining adherence, particularly to the more time‐consuming treatment of exercise, is a challenge.
Using the Short Form 6D, as an overall measure of health, to predict damage accrual and mortality in patients with systemic lupus erythematosus: XLVII, results from a multiethnic US cohort
Authors: Fernández Mónica , Alarcón Graciela S. , MCgwin Gerald , Sanchez Martha L. , Apte Mandar , Vilá Luis M. , Reveille John D. ,   LUMINA STUDY GROUP
Published: July 2007

Objective

To determine if overall health status as assessed by the Short Form 6D (SF‐6D) index, a preference‐based generic measure of health, is associated with the occurrence of damage accrual and mortality in patients with systemic lupus erythematosus (SLE).

Methods

We studied SLE patients (American College of Rheumatology criteria) from the LUpus in MInorities, NAture versus nurture cohort (LUMINA), a longitudinal multiethnic cohort. The contribution of the SF‐6D as assessed at enrollment to damage accrual at the last visit and mortality was examined. All variables previously shown to be determinants of damage accrual and mortality and corroborated by univariable analyses were adjusted for in multivariable models (Poisson and Cox proportional hazards regressions, respectively). Damage accrual and mortality were the dependent variables. Similar analyses were performed examining the associations of the Short Form 36 summary measures (physical component summary [PCS], mental component summary [MCS]) with these outcomes.

Results

In 552 patients, the SF‐6D was negatively associated with damage accrual and mortality in the univariable analyses; the association with damage was confirmed in the multivariable analyses (χ2 = 9.020, P = 0.002) but the association with mortality was not confirmed (hazard ratio 0.495, 95% confidence interval 0.041–6.038). When the PCS and MCS were evaluated, the PCS, but not the MCS, was found to be associated with damage but not with mortality.

Conclusion

The SF‐6D (and the PCS) as measured early in the disease course were found to independently predict damage accrual at the last visit, but not mortality. Although the SF‐6D was originally conceived as a utility measure, it may be used to accurately assess overall health status in patients with SLE.
Antioxidants and other novel cardiovascular risk factors in subjects with rheumatoid arthritis in a large population sample
Authors: De Pablo Paola , Dietrich Thomas , Karlson Elizabeth W.
Published: July 2007

Objective

To compare antioxidants and other novel and traditional cardiovascular disease (CVD) risk factors in participants with rheumatoid arthritis (RA) and non‐RA controls in a large population sample.

Methods

The Third National Health and Nutrition Examination Survey (NHANES‐III) was a cross‐sectional population survey in which subjects ages ≥60 underwent a musculoskeletal examination. RA subjects were defined as those who met ≥3 of 6 available 1987 American College of Rheumatology (ACR) criteria. Non‐RA subjects were defined as those who met no ACR criteria. We performed univariate and multivariate analyses of the association between RA and each novel and traditional CVD risk factor in RA versus non‐RA subjects.

Results

The sample included 5,302 subjects ages ≥60, with 131 (2.5%) RA and 4,444 (84%) non‐RA participants. A total of 727 subjects were excluded. Plasma levels of antioxidants α‐carotene, β‐cryptoxanthin, lutein/zeaxanthin, and lycopene were significantly lower in RA subjects compared with non‐RA subjects in multivariate analysis adjusting for potential confounders. Compared with non‐RA participants, RA subjects were more likely to have increased C‐reactive protein (CRP) levels in multivariate analysis adjusting for potential confounders. RA and non‐RA participants had similar prevalence of traditional CVD risk factors and previous CVD.

Conclusion

In this large population study, RA subjects had similar prevalence of previous CVD and traditional CVD risk factors as controls. Among novel CVD risk factors, plasma carotenoid levels were significantly lower and CRP level was significantly higher in RA compared with non‐RA subjects after adjustment for potential confounders. Further research should evaluate whether these differences account for the observed increased incidence of CVD in individuals with RA.
Applications of isothermal titration calorimetry in RNA biochemistry and biophysics
Authors: Feig Andrew L.
Published: August 2007

Isothermal titration calorimetry (ITC) has been applied to the study of proteins for many years. Its use in the biophysical analysis of RNAs has lagged significantly behind its use in protein biochemistry, however, in part because of the relatively large samples required. As the instrumentation has become more sensitive, the ability to obtain high quality data on RNA folding and RNA ligand interactions has improved dramatically. This review provides an overview of the ITC experiment and describes recent work on RNA systems that have taken advantage of its versatility for the study of small molecule binding, protein binding, and the analysis of RNA folding. © 2007 Wiley Periodicals, Inc. Biopolymers 87: 293–301, 2007.

This article was originally published online as an accepted preprint. The “Published Online” date corresponds to the preprint version. You can request a copy of the preprint by emailing the Biopolymers editorial office at biopolymers@wiley.com
Viren als Werkzeuge der Gentherapie. Wie aus einem Retrovirus ein Gentransfer‐Vektor entsteht
Authors: Thiel Gerald , Rössler Oliver
Published: July 2007

Die somatische Gentherapie basiert auf dem Konzept, monogenetische Erbkrankheiten ursächlich und nicht symptomatisch zu behandeln. Dazu ist ein Transfer genetischen Materials erforderlich, das den genetischen Defekt beheben könnte. In den vergangenen Jahren sind Methoden entwickelt worden, um rekombinante Lentiviren aus dem humanen Immunodefizienzvirus zu erzeugen, weil diese Viren gleichermaßen teilungsfähige und postmitotische Zellen erfolgreich infizieren können. Dieser Artikel beschreibt den Lebenszyklus der Retroviren und erläutert die Methoden zur Erzeugung von rekombinanten Retro‐ und Lentiviren. An Beispielen wird aufgezeigt, wie rekombinante Retroviren in der Gentherapie einsetzt werden und welche Probleme derzeit in diesem Gebiet vorhanden sind.
Nachtflugspezialisten haben auch tagsüber den Durchblick
Authors: Müller Brigitte , Peichl Leo
Published: July 2007

In der Netzhaut der meisten Säugetiere gibt es zwei Typen von Lichtsinneszellen: die Zapfen für das Tageslicht‐ und Farbensehen sowie die empfindlicheren Stäbchen für das Nachtsehen. Bisher nahm man an, dass die nachtaktiven Fledermäuse jedoch nur Stäbchen besitzen. Aktuelle Forschungsergebnisse belegen nun, dass die Netzhaut der ebenfalls nachtaktiven Flughunde, die nahe Verwandte der Fledermäuse sind, auch Zapfen enthält. Flughunde sind also auch für ein Sehen bei Tageslicht ausgestattet. Dies hilft ihnen bei der frühzeitigen Erkennung von Raubvögeln und bei sozialen Interaktionen in der Kolonie.
Philippinen: Meeressäuger schützen und beobachten
Authors: Diaz Gloria C. , Kronberg Inge
Published: July 2007

Sie atmen mit Lungen, haben eine Körpertemperatur von 37° C, gebären lebende Jungen und säugen sie mit Milch – damit sind Wal, Delfin und Dugong uns Menschen näher, als ihr Äußeres vermuten lässt. Obwohl sie als Meerjungfrauen und Sirenen die Phantasie der Seefahrer beflügelten, hat es Generationen gedauert, bevor sie unsere Sympathie und Fürsorge ernteten. Noch immer werden sie gejagt, versehentlich getötet oder ihr Lebensraum wird zerstört. Ein Schutz der Meeressäuger lässt sich nicht allein per Gesetz realisieren, sondern ist von der Zusammenarbeit mit Küstenbewohnern und Fischern abhängig. Das zeigt beispielhaft der integrierte Küstenschutz auf den Philippinen.
Functional results of intersphincteric resection for low rectal cancer
Authors: Yamada K , Ogata S , Saiki Y , Fukunaga M , Tsuji Y , Takano M
Published: August 2007

Background

Intersphincteric resection (ISR) is the ultimate sphincter-preserving operation for very low rectal cancer. The aim of this study was to assess defaecatory function after ISR in relation to the degree of resection of the internal anal sphincter.

Methods

Between 2001 and 2003, 35 consecutive patients with low rectal cancer had curative ISR, categorized as total, subtotal or partial resection of the internal anal sphincter. Defaecatory function was assessed in terms of frequency of bowel movements and continence. Sphincter function was evaluated by manometric study and anorectal sensation testing before surgery and 3, 6 and 12 months afterwards.

Results

Defaecatory function was satisfactory after ISR; 34 of 35 patients were grossly continent. The maximum resting anal canal pressure fell after all three procedures. Patients who had total ISR had reduced anal canal sensation at 3 months, but this had improved by 12 months after surgery.

Conclusion

These functional results suggest that ISR should be considered as an alternative to abdominoperineal resection for low rectal cancer. However, as the outcome for continence is worse after total ISR than subtotal or partial ISR, the indication for total ISR should strictly take into account the preoperative sphincter function.