bluuebunny/crossref_metadata_2025_split huggingface dataset:
Sorting Stable versus Unstable Hypothetical Compounds: The Case of Multi‐Functional ABX Half‐Heusler Filled Tetrahedral Structures
Authors: Zhang Xiuwen , Yu Liping , Zakutayev Andriy , Zunger Alex 
Published: February 2012

AbstractElectronic structure theory has recently been used to propose hypothetical compounds in presumed crystal structures, seeking new useful functional materials. In some cases, such hypothetical materials are metastable, albeit with technologically useful long lifetimes. Yet, in other cases, suggested hypothetical compounds may be significantly higher in energy than their lowest‐energy crystal structures or competing phases, making their synthesis and eventual device‐stability questionable. By way of example, the focus here is on the family of 1:1:1 compounds ABX called “filled tetrahedral structure” (sometimes called Half‐Heusler) in the four groups with octet electron count: I‐I‐VI (e.g., CuAgSe), I‐II‐V (e.g., AgMgAs), I‐III‐IV (e.g., LiAlSi), and II‐II‐IV (e.g., CaZnSn). First‐principles thermodynamics is used to sort the lowest‐energy structure and the thermodynamic stability of the 488 unreported hypothetical ABX compounds, many of which were previously proposed to be useful technologically. It is found that as many as 235 of the 488 are unstable with respect to decomposition (hence, are unlikely to be viable technologically), whereas other 235 of the unreported compounds are predicted to be thermodynamically stable (hence, potentially interesting new materials). 18 additional materials are too close to determine. The electronic structures of these predicted stable compounds are evaluated, seeking potential new material functionalities.
Functional Silk: Colored and Luminescent
Authors: Tansil Natalia C. , Koh Leng Duei , Han Ming‐Yong 
Published: February 2012

AbstractSilkworm silk is among the most widely used natural fibers for textile and biomedical applications due to its extraordinary mechanical properties and superior biocompatibility. A number of physical and chemical processes have also been developed to reconstruct silk into various forms or to artificially produce silk‐like materials. In addition to the direct use and the delicate replication of silk's natural structure and properties, there is a growing interest to introduce more new functionalities into silk while maintaining its advantageous intrinsic properties. In this review we assess various methods and their merits to produce functional silk, specifically those with color and luminescence, through post‐processing steps as well as biological approaches. There is a highlight on intrinsically colored and luminescent silk produced directly from silkworms for a wide range of applications, and a discussion on the suitable molecular properties for being incorporated effectively into silk while it is being produced in the silk gland. With these understanding, a new generation of silk containing various functional materials (e.g., drugs, antibiotics and stimuli‐sensitive dyes) would be produced for novel applications such as cancer therapy with controlled release feature, wound dressing with monitoring/sensing feature, tissue engineering scaffolds with antibacterial, anticoagulant or anti‐inflammatory feature, and many others.
Implications for genotype–phenotype predictions in Townes–Brocks syndrome: Case report of a novel SALL1 deletion and review of the literature
Authors: Miller Erin M. , Hopkin Robert , Bao Liming , Ware Stephanie M. 
Published: February 2012

AbstractTownes–Brocks syndrome (TBS) is a well‐described genetic syndrome characterized by anal, ear, and thumb anomalies and variable expressivity. Over 60 nonsense and frameshift mutations have been identified in SALL1, the zinc finger transcription factor causing TBS, and are proposed to cause disease via a dominant negative mechanism. In contrast, only four deletions have been described, with mild phenotypes reported as a result of haploinsufficiency. We report on a family with features of TBS in whom a novel 149 kb deletion spanning the SALL1 gene was identified by high resolution cytogenetics SNP microarray. We review the available genotype–phenotype information for all known truncating mutations and deletions. Taken together, they do not support the correlation of SALL1 deletions with a milder TBS phenotype and highlight a need for more robust clinical phenotyping combined with investigation of mutational mechanism. © 2012 Wiley Periodicals, Inc.
Adams–Oliver syndrome and portal hypertension: Fortuitous association or common mechanism?
Authors: Silva Gisela , Braga Alexandre , Leitão Banquart , Mesquita Abel , Reis Aurélio , Duarte Carlos , Barbot José , Silva Ermelinda Santos 
Published: February 2012

AbstractAdams–Oliver syndrome (AOS) is a rare condition defined by combination of cutis aplasia and transverse limb abnormalities. Some authors have described a possible association between this syndrome and portal hypertension (PH) due to hepatoportal sclerosis (HPS). We present a boy with AOS who developed a progressive splenomegaly and hypersplenism at the age of 2 months, and was admitted for acute gastrointestinal bleeding (GI) at the age of 9 months. Subsequently, we documented an extrahepatic portal vein obstruction and esophageal varices. After several episodes of cataclysmic upper GI bleeding a mesentero‐portal shunt (MPS) was performed at 10 months. The shunt thrombosed, and after three failed attempts of thrombectomy, it was removed. One month later a splenorenal shunt was performed, and this closed spontaneously by 3 years. The patient suffered from ischemic stroke after placing the first shunt, and has spastic diplegia, left frontal lobe epilepsy, hyperactivity and attention deficit disorder, and severe psychomotor delay. At 11 years and he presented with chronic liver failure and hyperammonemia and coagulopathy. We hypothesize that there may be an early embryonic vascular abnormality (vascular disruption) that may explain these vascular phenomena. © 2012 Wiley Periodicals, Inc.
Array CGH on unstimulated blood does not detect all cases of Pallister–Killian syndrome: A skin biopsy should remain the diagnostic gold standard
Authors: Hodge Jennelle C. , Hulshizer Rachael L. , Seger Pam , St Antoine Angelique , Bair Jennifer , Kirmani Salman 
Published: February 2012

AbstractA child whose features are consistent with Pallister–Killian syndrome (PKS) did not have detectable tetrasomy 12p due to an additional isochromosome 12p in an unstimulated blood specimen by interphase FISH or array CGH analysis. The diagnosis of PKS was made through these methods solely in a skin biopsy specimen. To determine the sensitivity of our array CGH platform to tetrasomy 12p mosaicism, dilutions of DNA from both the child's skin fibroblasts and a PKS cell line were analyzed. Tetrasomy 12p at 10% mosaicism was identifiable but 5% was below the limit of detection. This result suggests through extrapolation that the tetrasomy 12p is present in <10% of cells in our patient's blood, confirming the tissue‐limited mosaicism of PKS. Multiple recent studies show that array CGH provides greater sensitivity than chromosome analysis to detect mosaic abnormalities including that of tetrasomy 12p in blood specimens. However, our case demonstrates that the biology of PKS precludes the exclusive use of array CGH on blood for diagnosis. A tissue sample should continue to be the diagnostic gold standard for PKS. © 2012 Wiley Periodicals, Inc.
Further characterization of Shwachman–Diamond syndrome: Psychological functioning and quality of life in adult and young patients
Authors: Perobelli Sandra , Nicolis Elena , Assael Baroukh Maurice , Cipolli Marco 
Published: February 2012

AbstractTo assess psychosocial functioning and quality of life in a representative group of adult and young patients with Shwachman–Diamond syndrome (SDS), all patients 3 years old and over included in the Italian SDS Registry were investigated using an ad‐hoc questionnaire for information about demography, education, socialization, rehabilitation therapy, and standardized questionnaires [SF‐36, Child Behavior Check‐List (CBCL)] for quality of life and behavior. Results were compared with those of a Cystic Fibrosis (CF) patient group, matched for age and sex. Eighty‐one percent of patients answered. All but one adult patient lived with their parents, 24% had independent income, and 57% had a driver's license. Different levels (from mild to severe) of cognitive impairment were reported by 76% of the adults and by 65% of the young patients. These data are significantly lower than those of the CF group. Both groups present low scores in the emotional and mental health evaluations at SF‐36, but SDS patients reported significantly more limitations in physical functioning (PF) and more body pain (BP) experiences. As reported by parents at CBCL, young SDS patients show more “social problems” (in the clinical area 31% SDS vs. 6% CF), “attention deficits disorder” (29% SDS vs. 0%CF), and “somatic complaints” (24% SDS vs. 12% CF). Psychosocial functioning is impaired in the majority of SDS patients, significantly more than in patients affected by CF. © 2012 Wiley Periodicals, Inc.
Complex relationship between meiotic recombination frequency and autosomal synaptonemal complex length per cell in normal human males
Authors: Pan Zhenzhen , Yang Qingling , Ye Nan , Wang Liu , Li Jianhua , Yu Dexin , Cooke Howard J. , Shi Qinghua 
Published: February 2012

AbstractAlthough the relationship between meiotic recombination frequency and synaptonemal complex (SC) length has been of interest for a long time, how recombination frequency is related to SC length has not been carefully explored. To address this question, we have measured the meiotic recombination frequency as represented by MLH1 foci in 889 pachytene spermatocytes and measured the length of 19,558 autosomal SCs from 10 human males. A complex relationship between the number of MLH1 foci and total autosomal SC length per cell was observed. A positive correlation with significant correlation coefficients between the two variables was found in eight of the ten donors examined, with three donors showing weak correlation, and five showing moderate correlation. Two donors who did not show any correlation between the two variables were identified for the first time. Moreover, most cells with similar total autosomal SC length showed very different numbers of MLH1 foci both between individuals and even within an individual, and vice versa. Our data provide the first evidence for a complex relationship between the recombination frequency and total length of autosomal SCs per cell in human males. © 2012 Wiley Periodicals, Inc.
Hypertension and longitudinal changes in cerebral blood flow: The SMART‐MR study
Authors: Muller Majon , van der Graaf Yolanda , Visseren Frank L. , Mali Willem P. Th. M. , Geerlings Mirjam I. ,   for the SMART Study Group
Published: February 2012

AbstractObjective:Cerebral hypoperfusion is among the mechanisms that may explain the association of high blood pressure (BP) with dementia. However, few data are available on the longitudinal association of hypertension and cerebral perfusion.Methods:We examined the longitudinal association of hypertension, BP, and antihypertensive drugs with change in parenchymal cerebral blood flow (pCBF) in 575 patients with manifest atherosclerotic disease (mean age, 57 ± 10 years) from the SMART‐MR study. Total CBF was measured at baseline and at follow‐up with magnetic resonance (MR) angiography and was expressed per 100ml brain volume as an indicator of cerebral perfusion. Automated brain segmentation was used to quantify brain tissue volumes and cerebrospinal fluid on MR imaging.Results:Mean (standard deviation [SD]) baseline pCBF was 52.3 (9.8) ml/min/100ml and after 3.9 years (range, 3.0–5.8 years) of follow‐up declined to 50.7 (10.3) ml/min/100ml. Regression analyses adjusted for age, sex, follow‐up time, and vascular risk showed that untreated and poorly controlled hypertension and higher levels of systolic and diastolic BP (per SD) were significantly associated with a decline in pCBF; mean differences in decline (95% confidence interval) were −2.2 (−4.4 to 0.0), −1.0 (−1.8 to −0.1), and −1.0 (−1.8 to −0.2) ml/min/100ml. In addition, within hypertensive patients (n = 469), patients using angiotensin receptor blockers (ARBs) did not show a decline in pCBF, whereas patients using other antihypertensive drugs did show a decline in pCBF.Interpretation:Untreated hypertension, poorly controlled hypertension, and high BP levels are associated with a decline in pCBF. In addition, treatment with ARBs might result in less decline in pCBF than other antihypertensive treatment.ANN NEUROL 2012
Allene in molekularen Materialien
Authors: Rivera‐Fuentes Pablo , Diederich François 
Published: February 2012

AbstractDieser Kurzaufsatz liefert eine kritische Übersicht über die Entwicklung allenhaltiger fortschrittlicher Funktionsmaterialien. Ausgehend von Entwurf und Synthese stabiler, enantiomerenreiner Bausteine wird eine Vielfalt von Systemen – wie formstabile Makrocyclen, Foldamere, Polymere, Charge‐Transfer‐Chromophore, Dendrimere, Flüssigkristalle und redoxschaltbare chirale Chromophore – bezüglich ihrer Herstellung, Eigenschaften und möglichen Anwendungen diskutiert. Ziel der Zusammenstellung ist es, einen Anreiz zur verstärkten Anwendung enantiomerenreiner Allene beim rationalen Entwurf neuer Funktionswerkstoffe zu liefern.
Improvements in barrier properties of poly(lactic acid) films coated with chitosan or chitosan/clay nanocomposite
Authors: Park Seok‐Hoon , Lee Hyun Soo , Choi Jae Hoon , Jeong Chang Myeong , Sung Myoung Hwan , Park Hyun Jin 
Published: February 2012

AbstractThe feasibility of chitosan or/and clay coatings as an alternative oxygen barrier material to poly(lactic acid) (PLA) film was investigated. The coated films were composed of natural materials based on biodegradable and biocompatible polymers, which exhibit excellent oxygen‐barrier properties compared with neat PLA films. Thus, these films can contribute toward effectively reducing environmental pollution. The chitosan/clay nanocomposite solutions were effectively dispersed through the clay using an ultrasonic bath and homogenizer. The barrier and morphology properties of the PLA films were tested by measuring selected film properties such as oxygen transmission rate (OTR), water vapor transmission rate (WVTR), field emission scanning electron microscopy, X‐ray diffraction and transmission electron microscope. OTR values measured using the chitosan and chitosan/clay nanocomposite coatings were significantly lower than those measured for neat PLA film. WVTR values measured using chitosan/clay nanocomposite coatings were also lower than those measured for neat PLA film and PLA film coated with chitosan. © 2012 Wiley Periodicals, Inc. J Appl Polym Sci, 2012
Synthesis and Anti‐Oxidant Activity of (5E,9E)‐16‐(Substituted)‐7,8‐dihydro‐16λ5‐dibenzo[d,l][1,3,7,10,2]dioxadiazaphosphacyclotridecine‐16‐ones
Authors: Reddy Chinta Raveendra , Rani Chinthaparthi Radha , Sekhar Kuruva Chandra , Reddy Gangireddy Chandra Sekhar , Raju Chamarthi Naga 
Published: February 2012

AbstractA new class of biologically active 13‐membered phosphorus‐macroheterocycles (6a–l) were conveniently synthesized from 1,2‐bis(salicylidene amino)‐phenylene (1), by treating with phosporusoxychloride (3) and followed by reacting with various aromatic thiols and amines (5f–l) in one path, and in another path 1 was directly treated with various phosphorodichloridates (2a–e) in the presence of triethylamine at 0–10°C under N2 atmosphere in THF. All the title compounds were confirmed by analytical and spectral data (IR, 1H‐, 13C‐, 31P‐NMR, and mass spectra) and screened for anti‐oxidant activity. Among these compounds, 6k, 6e, and 6l containing nitro, fluoro, and chloro groups as substituents on the phenyl ring exhibited high anti‐oxidant activity with effective inhibitory concentration (IC50) values.
Reactions of N3‐Substituted Amidrazones with cis‐1,2‐Cyclohexanedicarboxylic Anhydride and Biological Activities of the Products
Authors: Modzelewska‐Banachiewicz Bozena , Ucherek Marzena , Zimecki Michał , Kutkowska Jolanta , Kaminska Teresa , Morak‐Młodawska Beata , Paprocka Renata , Szulc Michał , Lewandowski Grzegorz , Marciniak Joanna , Bobkiewicz‐Kozlowska Teresa 
Published: February 2012

AbstractA series of novel compounds were synthesized in reactions of N3‐substituted amidrazones with cis‐1,2‐cyclohexanedicarboxylic anhydride: linear, isoindole, and triazole derivatives. All new structures were confirmed by H1 NMR and IR spectrometry as well as elemental analysis. Potential biological effects of new compounds were predicted with the Prediction of Activity Spectra for Substances (PASS) program. Antiviral, antibacterial, analgesic, and anti‐inflammatory activities were experimentally verified.
Very high levels of anti–citrullinated protein antibodies are associated with HLA–DRB1*15 non–shared epitope allele in patients with rheumatoid arthritis
Authors: Laki Judit , Lundström Emeli , Snir Omri , Rönnelid Johan , Ganji Izabella , Catrina Anca I. , Bengtsson Camilla , Saevarsdottir Saedis , Wick Marius C. , Alfredsson Lars , Klareskog Lars , Padyukov Leonid 
Published: February 2012

AbstractObjectiveProduction of anti–citrullinated protein antibodies (ACPAs) is an important biomarker for rheumatoid arthritis (RA). We undertook this study to determine whether genetic factors (HLA—DRB1 alleles) are associated with extreme ACPA levels in individuals with ACPA‐positive RA, and to ascertain whether there are any phenotypic characteristics associated with these subgroups of RA.MethodsHLA–DRB1 allelic groups were genotyped in 1,073 ACPA‐positive RA patients from the Swedish Epidemiological Investigation of Rheumatoid Arthritis study. We found that 283 patients (26.4%) had high ACPA levels (defined as >1,500 units/ml using the Euro‐Diagnostica anti‐CCP2 test), while the rest of the patients had moderate ACPA levels and served as the comparison group. A replication group consisted of 235 RA patients.ResultsNo significant differences in baseline disease activity were observed between patients with high and those with moderate ACPA levels. However, the HLA–DRB1*15 allele was associated with high ACPA levels (P = 0.0002). A similar trend was detected in HLA–DRB1*15–positive patients in the replication cohort, with meta‐analysis of the discovery and replication cohorts demonstrating an overall effect of HLA–DRB1*15 on development of high ACPA levels in both the discovery and replication cohorts (P < 0.0001 by Mantel‐Haenszel test with a fixed‐effects model).ConclusionOur data indicate that HLA–DRB1*15 may promote the production of high ACPA levels. Due to the high value of ACPA level scores in the 2010 American College of Rheumatology/European League Against Rheumatism classification criteria for RA, the presence of HLA–DRB1*15 may, at least in part, contribute to fulfilling the criteria for RA. This illustrates the complex nature of the genetic regulation of ACPA levels. Additional mechanistic studies of the regulation of ACPAs and ACPA‐positive RA are pending.
Associations of varus thrust and alignment with pain in knee osteoarthritis
Authors: Lo Grace H. , Harvey William F. , McAlindon Timothy E. 
Published: February 2012

AbstractObjectiveTo investigate associations of varus thrust and varus static alignment with pain in patients with knee osteoarthritis (OA).MethodsThis was a cross‐sectional study of participants from a randomized controlled trial of vitamin D treatment for knee OA. Participants were video recorded while walking and scored for presence of varus thrust. Static alignment was measured on standard posteroanterior knee radiographs. Pain questions from the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) questionnaire were used to assess symptoms. We calculated means for total WOMAC pain in relation to varus thrust and static varus alignment (i.e., corrected anatomic alignment <178 degrees). Ordinal logistic regressions were performed, with responses on individual WOMAC pain questions as the outcomes and varus thrust and varus alignment as the predictors.ResultsThere were 82 participants, 60% of whom were female. The mean ± SD age was 65.1 ± 8.5 years, and the mean ± SD body mass index was 30.2 ± 5.4 kg/m2. The mean total WOMAC pain score was 6.3 versus 3.9, respectively, in those with versus without definite varus thrust (P = 0.007) and 5.0 versus 4.2 in those with versus without varus alignment (P = 0.36). Odds ratios for pain with walking and standing were 4.7 (95% confidence interval 1.8–11.9) and 5.5 (95% confidence interval 2.2–14.2), respectively, in those with and those without definite varus thrust. There were no significant associations between varus alignment and responses to individual WOMAC pain questions. Sensitivity analyses suggested that varus classified using a more stringent definition might have been associated with pain on walking and standing.ConclusionIn patients with knee OA, varus thrust, and possibly varus static alignment, were associated with pain, specifically during weight‐bearing activities. Treatment of varus thrust (e.g., via bracing or gait modification) may lead to improvement of symptoms.
Necessary and Sufficient Conditions for Consensus of Delayed Fractional‐order Systems
Authors: Shen Jun , Cao Jinde 
Published: February 2012

AbstractIn this paper, we study the consensus problem of fractional‐order systems with input delays. Using Laplace transform method, the stability of the fractional‐order systems is first discussed in the frequency domain. Based on the generalized Nyquist stability criterion, a necessary and sufficient condition is further derived to ensure the consensus of fractional‐order systems with identical input delays over directed interaction topology. Furthermore, when the interaction topology is undirected, the consensus condition of fractional‐order systems with heterogeneous input delays is explicitly given. Finally, some illustrative examples are presented to show the effectiveness and advantages of the theoretical results.
Optimal Performance in Tracking Stochastic Signal Under Disturbance Rejection
Authors: Zhan Xi‐Sheng , Guan Zhi‐Hong , Liao Rui‐Quan , Yuan Fu‐Shun 
Published: February 2012

AbstractThe analysis method of optimal tracking performance is proposed for multiple‐input multiple‐output (MIMO) linear time‐invariant (LTI) systems under disturbance rejection. An H2 criterion of the error signal between the output of the plant and the reference signal is used as a measure for the tracking performance. Spectral factorization is applied to obtain the optimal solution of the system tracking error. The explicit expressions are derived for this minimal tracking error with respect to random reference signals under disturbance rejection. It is shown that the nonminimum phase zeros, the zero direction, the unstable poles, the pole direction of a given plant, statistical characteristics of the reference input signal, and disturbance signal have a negative effect on a feedback system's ability to reduce the system error with disturbance rejection. The results show that the optimal tracking performance will further be damaged because of disturbance rejection. Some typical examples are given to illustrate the theoretical results.