bluuebunny/crossref_metadata_2025_split huggingface dataset:
Darier's Disease: A Clinical Rarity
Authors: Rao Roopa S , Mysorekar Vijaya V , Sumathy TK 
Published: June 2011

ABSTRACT
Darier's disease (DD) is a rare autosomal dominant genodermatosis clinically manifested by greasy hyperkeratotic papules primarily affecting seborrheic areas with less frequent involvement of the oral mucosa. Oral manifestations, if present, are usually asymptomatic, and are discovered in routine dental examination. Acantholysis and dyskeratosis represented by corps ronds and corps grains are the typical histological findings.
We report a case with clinical signs of DD in the palatal mucosa and skin. Skin biopsy and analysis of clinical history helped to diagnose DD. We report this case because of its clinical rarity as the prevalence of the disease has been estimated at 1/50,000-1,00,000. This could be a case of sporadic mutation as there was no family history. This case emphasizes the importance of oral examination among dermatologists, clinicians and pathologists to clinch the diagnosis of DD as it has a wide range of clinical presentations, therefore going undiagnosed for many years.
Role of vitamin K-dependent proteins in the arterial vessel wall
Authors: Chatrou M. L. L. , Reutelingsperger C. P. , Schurgers L. J. 
Published: June 2011

SummaryVitamin K was discovered early last century at the same time as the vitamin K-antagonists. For many years the role of vitamin K was solely ascribed to coagulation and coagulation was thought to be involved only at the venous blood side. This view has dramatically changed with the discovery of vitamin K-dependent proteins outside the coagulation cascade and the role of coagulation factors at the arterial side. Vitamin K-dependent proteins are involved in the regulation of vascular smooth muscle cell migration, apoptosis, and calcification. Vascular calcification has become an important independent predictor of cardiovascular disease. Vitamin K-antagonists induce inactivity of inhibitors of vascular calcification, leading to accelerated calcification. The involvement of vitamin K-dependent proteins such as MGP in vascular calcification make that calcification is amendable for intervention with high intake of vitamin K. This review focuses on the effect of vitamin K-dependent proteins in vascular disease.
Impact of obesity on functioning among women with systemic lupus erythematosus
Authors: Katz Patricia , Yazdany Jinoos , Julian Laura , Trupin Laura , Margaretten Mary , Yelin Edward , Criswell Lindsey A. 
Published: June 2011

AbstractObjectiveObesity is associated with high rates of disability in the general population. The nature of the relationship between obesity and disability in systemic lupus erythematosus (SLE), a condition with a high background rate of disability, is unknown.MethodsData were from 2 interviews, 4 years apart, of a longitudinal cohort of individuals with SLE (n = 716 women). Body mass index (BMI) was calculated from self‐reported height and weight; obesity was classified by usual (BMI ≥30) and revised (BMI ≥26.8) definitions. Three measures of functioning were examined: the Short Form 36 (SF‐36) Health Survey physical function (PF) subscale, Valued Life Activities (VLA) Disability Questionnaire, and employment. Multivariate analyses controlled for demographics, SLE duration and disease activity, glucocorticoid use, depression, and comorbidities. Prospective analyses also controlled for baseline function.ResultsAt a BMI of ≥30, 27.8% of the subjects were obese; at a BMI of ≥26.8, 40.6% of the subjects were obese. Regardless of obesity definition, obese women exhibited poorer baseline function, with decrements ranging from 20–33% depending on the functional measure and obesity definition. With a BMI of ≥26.8, the adjusted SF‐36 PF scores were 4.3 points lower for obese women (P < 0.0001), VLA difficulty was 0.09 higher (P = 0.01), and odds of employment were 80% of nonobese women (odds ratio 0.8, 95% confidence interval 0.5–1.1). At the 4‐year followup, women who were obese at baseline had poorer function and experienced greater functional declines.ConclusionObesity was associated with clinically significant negative effects on function, both concurrently and prospectively. This negative impact occurred at a lower BMI than is often considered problematic clinically. Because of the high rate of SLE‐related disability, addressing preventable risk factors such as obesity may improve long‐term SLE outcomes.
Quadriceps weakness, patella alta, and structural features of patellofemoral osteoarthritis
Authors: Stefanik Joshua J. , Guermazi Ali , Zhu Yanyan , Zumwalt Ann C. , Gross K. Douglas , Clancy Margaret , Lynch John A. , Segal Neil A. , Lewis Cora E. , Roemer Frank W. , Powers Christopher M. , Felson David T. 
Published: June 2011

AbstractObjectiveTo determine the relationship between quadriceps weakness and cartilage damage and bone marrow lesions (BMLs) in the patellofemoral joint (PFJ) and if this relationship is modified by patella alta.MethodsThe Multicenter Osteoarthritis Study is a cohort study of persons ages 50–79 years with or at risk of knee osteoarthritis. Concentric knee extensor strength was measured using an isokinetic dynamometer. Patella alta was measured using the Insall‐Salvati ratio (ISR) on the lateral radiograph, and cartilage damage and BMLs were graded on magnetic resonance imaging in the PFJ. We determined the association between quadriceps weakness with cartilage damage and BMLs in the PFJ among knees with (ISR ≥1.2) and without (ISR <1.2) patella alta using multiple binomial regression.ResultsA total of 807 knees were studied (mean age 62 years, body mass index 30 kg/m2, ISR 1.10) and 64% were from women. Compared with the knees in the highest strength tertile, those in the lowest had 10.2% (95% confidence interval [95% CI] 3, 18), 9.1% (95% CI 2, 16), and 7.1% (95% CI 1, 13) higher prevalence of lateral PFJ cartilage damage, medial PFJ cartilage damage, and lateral PFJ BMLs, respectively. The association between quadriceps weakness with cartilage damage and BMLs was not different between knees with and without patella alta in the lateral PFJ.ConclusionQuadriceps weakness was associated with PFJ cartilage damage and BMLs. While both patella alta and quadriceps weakness were associated with PFJ damage, the combination of the two was not associated with more damage than either of these factors alone.
Terpolymers from lactide and bisphenol a derivatives: Scale‐up, properties, and blends
Authors: Singh Akhilesh , Naskar Amit K. , Barden Joel , Drews Michael J. , Smith Dennis W. 
Published: June 2011

AbstractTerpolymerization of L‐lactide (LA) and bisphenol A derivatives was performed on few hundred gram scale, and the resultant terpolymer (TP) was characterized by gel permeation chromatography, infrared spectroscopy (FTIR), differential scanning calorimetry (DSC), dynamic mechanical analysis (DMA), and thermogravimetric analysis. Moderate molecular weight (Mn ∼ 12 kg/mol) TP was achieved with glass transition temperatures about 100°C (DSC and DMA). The TP exhibited improved thermal stability compared with polylactide (PLA), with a thermal degradation temperature of about 80°C higher than PLA. Although the TP exhibited distinctly different surface morphology compared with that of PLA, both showed similar contact angle and surface energy (ca. 40 mN/m) properties. Blends of PLA and TP showed enhanced glass transition (∼ 5°C change in Tg) temperatures compared with PLA homopolymer. This is due to the compatibility of PLA and TP. Thus, TP could be used as an additive for PLA‐based blends to enhance compatibility with phenolic‐based resins. TP electrospun fiber morphology is also reported. © 2011 Wiley Periodicals, Inc. J Appl Polym Sci, 2011
Performance of melamine modified urea–formaldehyde microcapsules in a dental host material
Authors: Then Sonja , Neon Gan Seng , Abu Kasim Noor Hayaty 
Published: June 2011

AbstractUrea–formaldehyde (UF) microcapsules filled with dicyclopentadiene (DCPD) show potential for making self‐healing dental restorative materials. To enhance the physical properties of the capsules, the urea was partially replaced with 0–5% melamine. The microcapsules were analyzed by different microscopic techniques. DSC was used to examine the capsule shell, and the core content was confirmed by 1H NMR spectroscopy. Capsules in the range of 50–300 μm were then embedded in a dental composite matrix consisting of bisphenol‐A‐glycidyl dimethacrylate (Bis‐GMA) and triethylene‐glycol dimethacrylate (TEGDMA). Flexural strength, microhardness, and nanoindentation hardness measurements were performed on the light‐cured specimens. Optical microscopy (OM) examination showed a random distribution of the microspheres throughout the host material. The incorporation of small amounts of the microcapsules did not affect the performance of the matrix material. Scanning electron microscopy (SEM) analysis revealed excellent bonding of the microcapsules to the host material which is a characteristic of utter importance for maintaining the very good mechanical properties of a dental composite with self‐healing ability. © 2011 Wiley Periodicals, Inc. J Appl Polym Sci, 2011.
High incidence of NLRP3 somatic mosaicism in patients with chronic infantile neurologic, cutaneous, articular syndrome: Results of an international multicenter collaborative study
Authors: Tanaka Naoko , Izawa Kazushi , Saito Megumu K. , Sakuma Mio , Oshima Koichi , Ohara Osamu , Nishikomori Ryuta , Morimoto Takeshi , Kambe Naotomo , Goldbach‐Mansky Raphaela , Aksentijevich Ivona , de Saint Basile Geneviève , Neven Bénédicte , van Gijn Mariëlle , Frenkel Joost , Aróstegui Juan I. , Yagüe Jordi , Merino Rosa , Ibañez Mercedes , Pontillo Alessandra , Takada Hidetoshi , Imagawa Tomoyuki , Kawai Tomoki , Yasumi Takahiro , Nakahata Tatsutoshi , Heike Toshio 
Published: June 2011

AbstractObjectiveChronic infantile neurologic, cutaneous, articular (CINCA) syndrome, also known as neonatal‐onset multisystem inflammatory disease (NOMID), is a dominantly inherited systemic autoinflammatory disease. Although heterozygous germline gain‐of‐function NLRP3 mutations are a known cause of this disease, conventional genetic analyses fail to detect disease‐causing mutations in ∼40% of patients. Since somatic NLRP3 mosaicism has been detected in several mutation‐negative NOMID/CINCA syndrome patients, we undertook this study to determine the precise contribution of somatic NLRP3 mosaicism to the etiology of NOMID/CINCA syndrome.MethodsAn international case–control study was performed to detect somatic NLRP3 mosaicism in NOMID/CINCA syndrome patients who had shown no mutation during conventional sequencing. Subcloning and sequencing of NLRP3 was performed in these mutation‐negative NOMID/CINCA syndrome patients and their healthy relatives. Clinical features were analyzed to identify potential genotype–phenotype associations.ResultsSomatic NLRP3 mosaicism was identified in 18 of the 26 patients (69.2%). Estimates of the level of mosaicism ranged from 4.2% to 35.8% (mean ± SD 12.1 ± 7.9%). Mosaicism was not detected in any of the 19 healthy relatives (18 of 26 patients versus 0 of 19 relatives; P < 0.0001). In vitro functional assays indicated that the detected somatic NLRP3 mutations had disease‐causing functional effects. No differences in NLRP3 mosaicism were detected between different cell lineages. Among nondescript clinical features, a lower incidence of mental retardation was noted in patients with somatic mosaicism. Genotype‐matched comparison confirmed that patients with somatic NLRP3 mosaicism presented with milder neurologic symptoms.ConclusionSomatic NLRP3 mutations were identified in 69.2% of patients with mutation‐negative NOMID/CINCA syndrome. This indicates that somatic NLRP3 mosaicism is a major cause of NOMID/CINCA syndrome.
Post‐epidemic eosinophilia–myalgia syndrome associated with L‐tryptophan
Authors: Allen Jeffrey A. , Peterson Alicia , Sufit Robert , Hinchcliff Monique E. , Mahoney J. Matthew , Wood Tammara A. , Miller Frederick W. , Whitfield Michael L. , Varga John 
Published: June 2011

AbstractEosinophilia–myalgia syndrome (EMS) is characterized by subacute onset of myalgias and peripheral eosinophilia, followed by chronic neuropathy and skin induration. An epidemic of EMS in 1989 was linked to consumption of L‐tryptophan that had originated from a single source. Following the ban by the Food and Drug Administration (FDA) on the sale of L‐tryptophan, the incidence of EMS declined rapidly. Moreover, no new cases have been described since the FDA ban was lifted in 2005. We report the clinical, histopathologic, and immunogenetic features of a new case of L‐tryptophan–associated EMS, along with evidence of activated transforming growth factor β and interleukin‐4 signaling in the lesional skin.
Effects of gadolinium chelate on the evolution of the nanoscale structure in peptide hydrogels
Authors: Taraban Marc B. , Weerasekare Mahika , Trewhella Jill , Shi Xianfeng , Jeong Eun‐Kee , Yu Yihua Bruce 
Published: June 2011

AbstractSmall‐angle X‐ray scattering (SAXS) was used to monitor peptide hydrogelation with and without the MRI contrast agent gadolinium chelate (Gd(III)‐chelate). The gelators are a pair of decapeptides that are self‐repulsive but mutually attractive. Gd(III)‐chelate was either free or covalently bound to one of the decapeptides. Free and conjugated Gd(III)‐chelate have the opposite effects on peptide gelation: free Gd(III)‐chelate slows down gelation while having little effect on the cross‐sectional area of peptide fibers; covalently conjugated Gd(III)‐chelate speeds up gelation and results in peptide fibers with significantly greater cross‐sectional area. After 24 h of gelation, the cross‐sectional areas of hydrogels with no Gd(III)‐chelate, with free Gd(III)‐chelate and with conjugated Gd(III) chelate are 3700, 3800, and 6300 Å2, respectively. Free Gd(III)‐chelate is not incorporated into peptide fibers and remains in solution with little effect on MRI intensity upon gelation. In contrast, covalently conjugated Gd(III)‐chelate is not only incorporated into peptide fibers, but further aggregates toward the center of the peptide fibers. In conclusion, to visualize hydrogelation using 1H MRI, it is necessary to conjugate Gd(III)‐chelate to the material covalently and use T2‐weighted imaging. © 2011 Wiley Periodicals, Inc. Biopolymers (Pept Sci) 98: 50–58, 2012.
Effect of sulfur oxyanions on lignocellulose‐derived fermentation inhibitors
Authors: Cavka Adnan , Alriksson Björn , Ahnlund Maria , Jönsson Leif J. 
Published: June 2011

AbstractRecent results show that treatments with reducing agents, including the sulfur oxyanions dithionite and hydrogen sulfite, efficiently improve the fermentability of inhibitory lignocellulose hydrolysates, and that the treatments are effective when the reducing agents are added in situ into the fermentation vessel at low temperature. In the present investigation, dithionite was added to medium with model inhibitors (coniferyl aldehyde, furfural, 5‐hydroxymethylfurfural, or acetic acid) and the effects on the fermentability with yeast were studied. Addition of 10 mM dithionite to medium containing 2.5 mM coniferyl aldehyde resulted in a nine‐fold increase in the glucose consumption rate and a three‐fold increase in the ethanol yield. To investigate the mechanism behind the positive effects of adding sulfur oxyanions, mixtures containing 2.5 mM of a model inhibitor (an aromatic compound, a furan aldehyde, or an aliphatic acid) and 15 mM dithionite or hydrogen sulfite were analyzed using mass spectrometry (MS). The results of the analyses, which were performed by using UHPLC‐ESI‐TOF‐MS and UHPLC‐LTQ/Orbitrap‐MS/MS, indicate that the positive effects of sulfur oxyanions are primarily due to their capability to react with and sulfonate inhibitory aromatic compounds and furan aldehydes at low temperature and slightly acidic pH (such as 25°C and pH 5.5). Biotechnol. Bioeng. 2011;108: 2592–2599. © 2011 Wiley Periodicals, Inc.
Evaluation of nanoparticle‐immobilized cellulase for improved ethanol yield in simultaneous saccharification and fermentation reactions
Authors: Lupoi Jason S. , Smith Emily A. 
Published: June 2011

AbstractEthanol yields were 2.1 (P = 0.06) to 2.3 (P = 0.01) times higher in simultaneous saccharification and fermentation (SSF) reactions of microcrystalline cellulose when cellulase was physisorbed on silica nanoparticles compared to enzyme in solution. In SSF reactions, cellulose is hydrolyzed to glucose by cellulase while yeast simultaneously ferments glucose to ethanol. The 35°C temperature and the presence of ethanol in SSF reactions are not optimal conditions for cellulase. Immobilization onto solid supports can stabilize the enzyme and promote activity at non‐optimum reaction conditions. Mock SSF reactions that did not contain yeast were used to measure saccharification products and identify the mechanism for the improved ethanol yield using immobilized cellulase. Cellulase adsorbed to 40 nm silica nanoparticles produced 1.6 times (P = 0.01) more glucose than cellulase in solution in 96 h at pH 4.8 and 35°C. There was no significant accumulation (<250 µg) of soluble cellooligomers in either the solution or immobilized enzyme reactions. This suggests that the mechanism for the immobilized enzyme's improved glucose yield compared to solution enzyme is the increased conversion of insoluble cellulose hydrolysis products to soluble cellooligomers at 35°C and in the presence of ethanol. The results show that silica‐immobilized cellulase can be used to produce increased ethanol yields in the conversion of lignocellulosic materials by SSF. Biotechnol. Bioeng. 2011;108: 2835–2843. © 2011 Wiley Periodicals, Inc.
One‐step production of unacetylated sophorolipids by an acetyltransferase negative Candida bombicola
Authors: Saerens Karen M.J. , Saey Lien , Soetaert Wim 
Published: June 2011

AbstractSophorolipids from the non‐pathogenic yeast Candida bombicola are applied commercially as biodegradable, eco‐friendly surface active agents. These sophorolipids are produced by cultivation in presence of a hydrophobic carbon source and are always constituted of a mixture of structurally related molecules. For some applications however, certain structural variants perform better than others. Acetylation of the sophorolipid molecule is such a parameter that gains interest because of its influence on water solubility, foaming properties, and biological activity. Fully unacetylated sophorolipids therefore are interesting metabolites but cannot be produced in a pure way by conventional cultivation. Here we report the identification of the acetyltransferase gene AT, responsible for acetylation of de novo synthesized sophorolipids in Candida bombicola. By the creation of a Δat deletion mutant, we could create a yeast strain producing purely unacetylated sophorolipids with a yield of 5 ± 0.7 g/L using rapeseed oil as hydrophobic carbon source. In contrast to the chemical production of unacetylated sophorolipids used nowadays, the microbial production leads to mainly lactonic sophorolipids, in addition to minor amounts of acidic sophorolipids. Biotechnol. Bioeng. 2011;108: 2923–2931. © 2011 Wiley Periodicals, Inc.
A mechanistic model for rational design of optimal cellulase mixtures
Authors: Levine Seth E. , Fox Jerome M. , Clark Douglas S. , Blanch Harvey W. 
Published: June 2011

AbstractA model‐based framework is described that permits the optimal composition of cellulase enzyme mixtures to be found for lignocellulose hydrolysis. The rates of hydrolysis are shown to be dependent on the nature of the substrate. For bacterial microcrystalline cellulose (BMCC) hydrolyzed by a ternary cellulase mixture of EG2, CBHI, and CBHII, the optimal predicted mixture was 1:0:1 EG2:CBHI:CBHII at 24 h and 1:1:0 at 72 h, at loadings of 10 mg enzyme per g substrate. The model was validated with measurements of soluble cello‐oligosaccharide production from BMCC during both single enzyme and mixed enzyme hydrolysis. Three‐dimensional diagrams illustrating cellulose conversion were developed for mixtures of EG2, CBHI, CBHII acting on BMCC and predicted for other substrates with a range of substrate properties. Model predictions agreed well with experimental values of conversion after 24 h for a variety of enzyme mixtures. The predicted mixture performances for substrates with varying properties demonstrated the effects of initial degree of polymerization (DP) and surface area on the performance of cellulase mixtures. For substrates with a higher initial DP, endoglucanase enzymes accounted for a larger fraction of the optimal mixture. Substrates with low surface areas showed significantly reduced hydrolysis rates regardless of mixture composition. These insights, along with the quantitative predictions, demonstrate the utility of this model‐based framework for optimizing cellulase mixtures. Biotechnol. Bioeng. 2011;108: 2561–2570. © 2011 Wiley Periodicals, Inc.
Blushing Propensity and Psychological Distress in People with Rosacea
Authors: Su Daphne , Drummond Peter D. 
Published: June 2011

Rosacea is a chronic skin disorder, characterized by persistent painful facial flushing and often accompanied by papules and pustules. To evaluate the psychological and social impacts of rosacea, 31 individuals with rosacea filled in the Blushing Propensity Scale, the Fear of Negative Evaluation Questionnaire, the Depression Anxiety and Stress Scale, the Social Interaction Anxiety Scale and the Social Phobia Scale. The questionnaires were also completed by 86 controls. Participants with extensive facial papules and pustules had higher blushing propensity, stress and social phobia scores than controls or others without papules or pustules. Childhood blushing was also reported more frequently by participants with rosacea than controls. Cognitive–behavioural therapy appeared to be helpful for managing social anxiety in three individuals with rosacea with a fear of blushing. These findings suggest that people with severe rosacea are anxious about the social consequences of blushing and generally prefer to avoid situations that might involve scrutiny by others. Persistent facial flushing could prime interoceptive cues of blushing or increase anxiety about facial coloration in provocative situations. Treatments that target fear of blushing may help to reduce social anxiety in people with severe rosacea. Copyright © 2011 John Wiley & Sons, Ltd.Key Practitioner Message

Blushing propensity scores are elevated in people with severe rosacea.
Fear of blushing may contribute to social anxiety and avoidance in such cases.
Cognitive–behavioural therapy for fear of blushing may help to reduce social anxiety in people with severe rosacea.
QoS multicast routing in cognitive radio ad hoc networks
Authors: Xie Liming , Jia Xiaohua , Zhou Kunxiao 
Published: June 2011

SUMMARYIn this paper, we discussed the issues of QoS multicast routing in cognitive radio ad hoc networks. The problem of our concern was: given a cognitive radio ad hoc network and a QoS multicast request, how to find a multicast tree so that the total bandwidth consumption of the multicast is minimized while the QoS requirements are met. We proposed two methods to solve it. One is a two‐phase method. In this method, we first employed a minimal spanning tree‐based algorithm to construct a multicast tree and then proposed a slot assignment algorithm to assign timeslots to the tree links such that the bandwidth consumption of the tree is minimized. The other is an integrated method that considers the multicast routing together with the slot assignment. Extensive simulations were conducted to show the performance of our proposed methods. Copyright © 2011 John Wiley & Sons, Ltd.