bluuebunny/crossref_metadata_2025_split huggingface dataset:
Congenital Heart Disease Linked to Maternal Autoimmunity against Cardiac Myosin
Authors: Cole Charles R. , Yutzey Katherine E. , Brar Anoop K. , Goessling Lisa S. , VanVickle-Chavez Sarah J. , Cunningham Madeleine W. , Eghtesady Pirooz 
Published: March 2014

Abstract
Structural congenital heart disease (CHD) has not previously been linked to autoimmunity. In our study, we developed an autoimmune model of structural CHD that resembles hypoplastic left heart syndrome (HLHS), a life-threatening CHD primarily affecting the left ventricle. Because cardiac myosin (CM) is a dominant autoantigen in autoimmune heart disease, we hypothesized that immunization with CM might lead to transplacental passage of maternal autoantibodies and a prenatal HLHS phenotype in exposed fetuses. Elevated anti-CM autoantibodies in maternal and fetal sera, as well as IgG reactivity in fetal myocardium, were correlated with structural CHD that included diminished left ventricular cavity dimensions in the affected progeny. Further, fetuses that developed a marked HLHS phenotype had elevated serum titers of anti–β-adrenergic receptor Abs, as well as increased protein kinase A activity, suggesting a potential mechanism for the observed pathological changes. Our maternal–fetal model presents a new concept linking autoimmunity against CM and cardiomyocyte proliferation with cardinal features of HLHS. To our knowledge, this report shows the first evidence in support of a novel immune-mediated mechanism for pathogenesis of structural CHD that may have implications in its future diagnosis and treatment.
Age-Enhanced Endoplasmic Reticulum Stress Contributes to Increased Atg9A Inhibition of STING-Mediated IFN-β Production during Streptococcus pneumoniae Infection
Authors: Mitzel Dana N. , Lowry Virginia , Shirali Anushree C. , Liu Yushi , Stout-Delgado Heather W. 
Published: March 2014

Abstract
Pneumococcal infections remain a leading cause of death in persons ≥65 y of age. Recent reports have illustrated detrimental changes in the endoplasmic reticulum stress response or unfolded protein response in aging and age-related diseases; however, the relationship between aging, the unfolded protein response, and innate immune responses to Streptococcus pneumoniae has not been fully elucidated. Our results illustrate that stimulator of IFN genes–mediated production of IFN-β during S. pneumoniae infection is decreased in aged hosts. Enhanced endoplasmic reticulum stress in response to S. pneumoniae augmented inositol-requiring protein 1/X-box binding protein 1–mediated production of autophagy-related gene 9 (Atg9a). Knockdown of Atg9a or treatment with gemcitabine HCl resulted in enhanced stimulator of IFN genes–mediated production of IFN-β by aged macrophages. Consecutive treatments with gemcitabine during in vivo S. pneumoniae infection decreased morbidity and mortality in aged hosts, which was associated with decreased Atg9a expression, increased IFN-β production, and improved bacterial clearance from lung tissue. Taken together, data presented in this study provide new evidence as to why older persons are more susceptible to S. pneumoniae, and provide a possible mechanism to enhance these responses, thereby decreasing morbidity and mortality in this population.
‘Unexplained intermittent hemoptysis with normal chest radiograph necessitates bronchoscopy’— mucoepidermoid carcinoma of lung: Case report
Authors: Patil Shital , Ayachit Rujuta 
Published: March 2014

Abstract
Hemoptysis is the predominant symptom of tuberculosis and is considered as important clue toward evaluation of tuberculosis and treatment accordingly, especially in high burden countries like India where disease is endemic. 8-25% cases of pulmonary tuberculosis are having hemoptysis during course of their illness. Unexplained hemoptysis with normal chest radiograph needs further evaluation including CT thorax and bronchoscopy. Bronchoscopy would be ideal choice for confirming diagnosis and to localize exact site of bleeding as well. In this case report, we describe a case of a young male who presented with cough and intermittant hemoptysis. During fiberoptic video-bronchoscopy we noted exophytic endobronchial growth in right main stem bronchus. Histopathological evaluation suggests mucoepidermoid carcinoma (MEC) of salivary gland type originating from main stem bronchus right side. PAS and mucicarmine staining differentiated MEC from Adenocarcinoma in our case.
Internal carotid cavernous fistula: One case report and literature review
Authors: Guo Yan-fang , Dong Mei , Mao Zhuo-feng , Zhang Mei-yue , Chen Xin , Zuo Xiao-jie 
Published: March 2014

Abstract
Spontaneous carotid cavernous fistula is usually caused by lesions in the internal carotid cavernous sinus and its branches. We report a young male patient with no history of trauma, in whom the fistula was located at the cavernous sinus of the internal carotid artery. The postoperative angiography found a pseudoaneurysm at the origin of the cavernous sinus of internal carotid artery, which suggested that cavernous sinus fistulas were caused by bleeding from rupture of thin-walled vessels due to the local abnormalities of the vascular wall.
Genotype frequencies of polymorphic MDR1 variants in the Kazakhstani population
Authors: Kozhakhmetov Samat , Supiyev Adil , Kushugulova Almagul , Tynybayeva Indira , Kossumov Alibek , Utepova Leila , Saduakhasova Saule , Shakhabayeva Gulnara , Nurgozhin Talgat , Zhumadilov Zhaxybay 
Published: March 2014

Introduction: Statins appear to be handled by an ATP-dependent membrane transporter and three SNPs (C1236T (rs1128503), G2677T (rs2032582), and C3435T (rs1045642), which capture the common genetic variation at this locus. Individuals, who carry the T allele at each SNP (i.e., the T-T-T haplotype), have higher systemic exposure to simvastatin.A triallelic thymine (T) - guanine (G) - adenine (A), which is a point mutation at nucleotide 2677 in exon 22, leads to ABCB1 in a non-synonymous codons (GCT alanine, TCT serine, threonine ACT) at position 893 in a cytoplasmic loop of ATP-dependent membrane transporters.Methods: Blood samples from healthy individuals were collected in the Republican Diagnostic Center, Astana, Kazakhstan. The research samples included 461 healthy people. Genomic DNA was extracted from peripheral blood using the ‘salting out’ procedure. For the MDR1 exon 21, 2677G˃T/A (Ala893Ser/Thr) polymorphism was genotyped by PCR sequencing by the use of dye-terminator (ABI 3730xl sequencer). Results: The GG allele appeared in 23% of samples, the GA in 6.7%, the GT in 44%, the non-G heterozygote in 4.5%, and the non-G homozygote in 18%. These results are consistent with previously published data. Importantly, the frequency of 2677T alleles in our group was 15.4%. This represents the lowest frequency of this allele compared to published data in different populations. The frequency of the 2677T allele in Asians and Caucasians varies from 38 to 62%, and is 15% for African Americans. On the other hand, the 2677A allele frequency in the Japanese varies from 15 to 22%, and in Caucasians from 2% and 4%. The 2677A allele frequency has been found in 4.6% of samples. Conclusions: Our study further emphasizes differences between various Asian populations and the importance of repeating this genetic study in different ethnic groups.
Nietzsche as Kant's True Heir?
Authors: Louden Robert B. 
Published: March 2014

ABSTRACT
In this short article, I present several challenges to Maudemarie Clark and David Dudrick's bold claim that one of Nietzsche's main goals in Beyond Good and Evil is to establish himself as “Kant's true heir.” First, I critique their argument that the prefaces to the Critique of Pure Reason and BGE bear a “striking similarity” to each other. Second, I try to refute their claim that Nietzsche in BGE 11 is “positioning himself … as the true successor to Kant.” Nietzsche does not exhibit the positive interest in the a priori that one expects from even the most minimal Kantian, and his norms are hardly Kant's. Finally, in my conclusion, I draw some qualified connections between Nietzsche's normative project and a more naturalistic option within the history of philosophy—namely, American pragmatism.
Factors Associated with Health-Related Quality of Life Among Older People with Multiple Sclerosis
Authors: Buhse Marijean , Banker Wendy M. , Clement Lynn M. 
Published: March 2014

Background: This study was conducted to determine which factors (clinical and demographic) are associated with mental and physical health-related quality of life (HRQOL) among people with multiple sclerosis (MS) aged 60 years and older.
Methods: Data were collected at four MS centers on Long Island, New York, from a total of 211 patients. Three surveys were administered that collected demographic information and included validated questionnaires measuring quality of life (QOL), cognition, depression, and disability. Multivariate linear regression analyses examined the relationship between patient demographics and scores on standardized scales measuring mental and physical HRQOL (Multiple Sclerosis Quality of Life–54). Variables included in the regression models were selected on the basis of the Andersen Healthcare Utilization model. This framework encompasses the multiple influences on health status, including predisposing characteristics, enabling resources, need, and health behavior.
Results: We found that mental HRQOL was negatively associated with having a high school education or less, risk of neurologic impairment, physical disability, and depression. No variables were positively associated with mental HRQOL. Physical HRQOL was negatively associated with risk of neurologic impairment, physical disability, depression, and the comorbidity of thyroid disease. However, patient employment and, surprisingly, being widowed were positively associated with physical HRQOL. These findings are consistent with those of similar studies among younger patients in which lower HRQOL was associated with increased disability, depression, risk of neurologic impairment, and lower levels of education.
Conclusions: The findings that patient employment and being widowed were associated with better physical HRQOL suggest that older patients have the ability to adapt and adjust to the challenges of MS over time. Clinicians should regularly screen for HRQOL in older patients with MS.
Psychological characteristics of internationally adopted post-institutionalized children with Fetal Alcohol Spectrum Disorders
Authors: Gindis Boris 
Published: March 2014

Gindis, B. (2014). Psychological characteristics of internationally adopted post-institutionalized children with Fetal Alcohol Spectrum Disorders. The International Journal Of Alcohol And Drug Research, 3(1), 35-42. doi:10.7895/ijadr.v3i1.133Aims: Fetal Alcohol Syndrome Disorder (FASD) is widely observed in internationally adopted (IA) post-institutionalized children. The specificity of FASD in IA children has significant practical implications and necessitates a modified methodology for identification and remediation.Design methods, and participants: Clinical case study with statistical analysis (simple frequency recorded in an Excel spreadsheet); quantitative and qualitative data was obtained through individual medical, neuropsychological, and educational assessments of 63 children, ages five to sixteen, adopted from Eastern Europe to the United States.Findings: FASD in international adoptees presents amplified characteristics typical for this condition, with the following specificities revealed in our research: rapid first-language loss and a particular pattern of English language learning; profound complex childhood trauma related to extreme deprivation and institutional upbringing; “mixed maturity” evident in impaired executive functions; low predictive accuracy during a pre-adoption screening for FASD conditions; general cognitive ability (IQ) being in the Low Average to Borderline range, with processing speed, attention, and working memory as the weakest cognitive skills; and academic achievements being higher than could be predicted based on cognitive abilities.Conclusions: FASD must be recognized as an educational handicap in our school system in order to change the outcomes for afflicted children. Educational remediation and cognitive-behavioral therapeutic intervention are the most effective remedial methods for IA children with FASD. Practical recommendations for adoptive parents include early identification and specialized remediation of “secondary” disabilities through concerted efforts of the school and family.
Photodissociation of water and O(3PJ) formation on a lunar impact melt breccia
Authors: DeSimone Alice J. , Orlando Thomas M. 
Published: March 2014

AbstractPhotodissociation of water deposited on an impact melt breccia collected during Apollo 16 was studied by measuring O(3PJ = 2,1,0) photoproducts detected with resonance‐enhanced multiphoton ionization. For each spin‐orbit state, the oxygen atom time‐of‐flight (TOF) spectrum was measured as a function of H2O exposure and 157 nm irradiation time. Four Maxwell‐Boltzmann distributions with translational temperatures of 10,000 K, 1800 K, 400 K, and 102 K were required to fit the data. The most likely formation mechanisms are molecular hydrogen elimination following ion‐electron recombination, secondary recombination of hydroxyl radicals, and photodissociation of adsorbed hydroxyls. The irradiation time required to reach maximum oxygen signal suggests that water clusters into islands when adsorbing on the lunar impact melt breccia. After enough irradiation for the oxygen atom yield to reach its maximum, the slowly decreasing signal was fit with an exponential curve to obtain a cross section that represents the rate of surface hydroxyl depletion. For 0.1, 1, and 5 Langmuir (1 L = 10−6 Torr s) H2O exposure, respectively, the measured O(3P) depletion cross sections were 4.9 × 10−20, 6.6 × 10−20, and 4.6 × 10−20 cm2. These results imply that photodissociation of water on the lunar surface cannot account for the large mass‐16 (±1 amu) signal observed in the lunar atmosphere. Unless another significant source of oxygen atoms is present, this unexpectedly large signal is likely due to CH4 or OH.
Crustal structure in the area of the 2002 Molise earthquake: Clues for the evolution of the southern Apennines
Authors: Chiarabba C. , De Gori P. , Latorre D. , Amato A. 
Published: March 2014

AbstractThe Apennines is a mountain belt with paired extension and compression that are explained by the subduction and delamination of the Ionian/Adria lithosphere. During the belt formation, the Adria crust is flexed downward and then incorporated into the wedge after peeling off along some weak level whose origin is still discussed. In southern Apennines, this process seems to have stopped at about 0.7 Ma by the development of a tear in the subducting lithosphere and consequent extension that spread along the mountain range. Seismological data acquired after the 2002 Mw 6.0 Molise strike‐slip earthquake yield modeling the structure of the Adria crust as congealed close to the leading edge of the belt. Tomography images show inverted normal faults within the sedimentary cover (Apulian units). Fault inversion, incomplete close to the compressional front, testifies for a final squeezing of the entire, previously flexed, Apulian continental margin. Pronounced high Vp and low Vp/Vs anomalies are observed at depth greater than 12 km and undoubtfully attributed to heterogeneities of the basement, solving the long‐lasting contention on the nature of similar high‐speed bodies observed beneath the Apennines. Beneath these bodies, we observe a strong reflectivity, suggesting the presence of a fluid‐filled layer in the middle lower crust that may act as a decoupling level in the Apennines tectonics. The deep fluids are also favoring the rupture of strike‐slip earthquakes, like those that occurred in 2002, and the intermediate‐depth seismicity diffused in the Apulian region.
High Degree of Polymerization in a Fullerene‐Containing Supramolecular Polymer
Authors: Isla Helena , Pérez Emilio M. , Martín Nazario 
Published: March 2014

AbstractSupramolecular polymers based on dispersion forces typically show lower molecular weights (MW) than those based on hydrogen bonding or metal–ligand coordination. We present the synthesis and self‐assembling properties of a monomer featuring two complementary units, a C60 derivative and an exTTF‐based macrocycle, that interact mainly through π–π, charge‐transfer, and van der Waals interactions. Thanks to the preorganization in the host part, a remarkable log Ka=5.1±0.5 in CHCl3 at room temperature is determined for the host–guest couple. In accordance with the large binding constant, the monomer self‐assembles in the gas phase, in solution, and in the solid state to form linear supramolecular polymers with a very high degree of polymerization. A MW above 150 kDa has been found experimentally in solution, while in the solid state the monomer forms extraordinarily long, straight, and uniform fibers with lengths reaching several microns.
A2: Rapid and Sustained Response in Patients Suffering From Severe Cryopyrin‐Associated Periodic Syndromes Treated With Anakinra (Kineret®)
Authors: Leinonen Mika , Hallén Bengt , Olivecrona Hans 
Published: March 2014

Background/Purpose:Cryopyrin‐Associated Periodic Syndromes (CAPS) are a group of rare inherited autoinflammatory diseases consisting of a spectrum of three conditions: FCAS, Muckle‐Wells Syndrome and the most severe form, NOMID. Anakinra was recently approved in the US for treatment of NOMID and in the European Union for treatment of CAPS. The objective of the present analysis is to assess the response rate to anakinra treatment and time to changes in symptom severity after initiation, withdrawal and re‐institution of the treatment in patients with severe CAPS.Methods:A prospective, open‐label withdrawal design study of anakinra treatment with long‐term extension including 43 patients was conducted at the National Institutes of Health (Goldbach‐Mansky et al 2006, Sibley et al 2012). The primary efficacy endpoint, the Diary Symptom Sum Score (DSSS) collected daily up to 60 months included 5 symptoms (fever, rash, joint pain, vomiting, headache); each scored from 0 (no symptoms) to 4 (severe symptoms). Earlier data demonstrated that rash and conjunctivitis disappeared in 3 days in all patients, the DSSS was decreased at 3 months and all patients except one met the criteria of a flare during the 7‐day withdrawal phase (Goldbach‐Mansky et al 2006). In the present analysis, we defined the response as at least 50% improvement from baseline in the weekly average DSSS. The mean changes in DSSS were estimated with Mixed Model for Repeated Measures (MMRM).Results:Twenty nine out of the 43 enrolled patients provided valid DSSS data for the analysis. A majority of the patients were children (17% <2 years, 55% 2–11 years, 7% 12–17 years, 21% 18 years or more). All 29 patients responded to the treatment during the first 10 days based on DSSS. The average DSSS decreased from 4.5 to 0.8 in 2 days. This response was maintained throughout the study up to 60 months and at each post‐baseline visit >80% of the patients met the response criteria. Eleven consecutive patients participated in the withdrawal phase at 3 months of treatment. After treatment withdrawal the average DSSS increased from 0.6 to 5.2 (p<0.0001 compared to continuously treated patients). After re‐institution of treatment the DSSS returned to pre‐withdrawal levels within 3 days.Conclusion:All patients experienced a rapid response to the anakinra treatment, both initially and after a flare caused by the withdrawal phase. The response was maintained up to 60 months.
Supramolecular Organogels Based on Dendrons and Dendrimers
Authors: Feng Yu , He Yan‐Mei , Fan Qing‐Hua 
Published: March 2014

AbstractDendrons and dendrimers have well‐defined, discrete structures that can be precisely controlled at the molecular lever. Owing to their unique architectures and multiple functionalities, dendritic molecules have shown intensive self‐assembly behavior and functional performance. In particular, they have been shown to be promising candidates for applications in the assembly of gel‐phase materials. Furthermore, the introduction of suitable functional moieties into the core, the branches, and/or the periphery of the dendritic gelators enables the construction of smart and functional supramolecular gel materials. Over the past decade, a number of dendritic organogelators that are based on poly(amino acid), poly(amide), and poly(aryl ether) dendrons, or together with multiple alkyl chains on the periphery, have been reported. This review describes the important developments in dendritic organogelators, with an emphasis on new strategies for the molecular design of dendritic gelators, understanding of driving forces for gel formation, and their evolution for potential applications in smart soft materials.
Rollover‐Assisted C(sp2)C(sp3) Bond Formation
Authors: Zucca Antonio , Maidich Luca , Canu Laura , Petretto Giacomo L. , Stoccoro Sergio , Cinellu Maria Agostina , Clarkson Guy J. , Rourke Jonathan P. 
Published: March 2014

AbstractRollover cyclometalation involves bidentate heterocyclic donors, unusually acting as cyclometalated ligands. The resulting products, possessing a free donor atom, react differently from the classical cyclometalated complexes. Taking advantage of a “rollover”/“retro‐rollover” reaction sequence, a succession of oxidative addition and reductive elimination in a series of platinum(II) complexes [Pt(N,C)(Me)(PR3)] resulted in a rare C(sp2)C(sp3) bond formation to give the bidentate nitrogen ligands 3‐methyl‐2,2′‐bipyridine, 3,6‐dimethyl‐2,2′‐bipyridine, and 3‐methyl‐2‐(2′‐pyridyl)‐quinoline, which were isolated and characterized. The nature of the phosphane PR3is essential to the outcome of the reaction. This route constitutes a new method for the activation and functionalization of CH bond in the C(3) position of bidentate heterocyclic compounds, a position usually difficult to functionalize.
Access to the Aeruginosin Serine Protease Inhibitors through the Nucleophilic Opening of an Oxabicyclo[2.2.1]heptane: Total Synthesis of Microcin SF608
Authors: Diethelm Stefan , Schindler Corinna S. , Carreira Erick M. 
Published: March 2014

AbstractSerine proteases play key roles in many biological processes and are associated with several human diseases such as thrombosis or cancer. During the search for selective inhibitors of serine proteases, a family of linear peptides named the aeruginosins was discovered in marine cyanobacteria. We herein report an entry route into the synthetically challenging core fragment of these natural products. Starting from the common oxabicyclic building block 11, we accessed the octahydroindole core of the aeruginosins, exemplified by the total synthesis of microcin SF608 (2). Key to the synthetic strategy is a highly efficient nucleophilic opening of an oxabicyclo[2.2.1]heptane producing the hydroindole motif of microcin SF608. Moreover, during the synthetic efforts we have observed an unusual regioselective epoxide reduction. Detailed experimental studies of this reaction led us to propose a mechanistic rationale involving intramolecular hydrogen atom delivery by a carbamate NH group to control the regioselectivity of the homolytic epoxide cleavage.