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- Eckhardt, CL, et al.
(author)
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Factor VIII gene (F8) mutation and risk of inhibitor development in nonsevere hemophilia A
- 2013
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In: Blood. - : American Society of Hematology. - 1528-0020 .- 0006-4971. ; 122:11, s. 1954-1962
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Journal article (peer-reviewed)abstract
- The inhibitor incidence in nonsevere hemophilia A patients with certain F8 mutations approaches the inhibitor incidence in severe patients. These findings are highly relevant for clinical practice, as they facilitate identification of high-risk patients based on F8 genotype.
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3. |
- Richard, C., et al.
(author)
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New section of the HITRAN database: Collision-induced absorption (CIA)
- 2012
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In: Journal of Quantitative Spectroscopy & Radiative Transfer. - : Elsevier BV. - 0022-4073 .- 1879-1352. ; 113:11, s. 1276-1285
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Journal article (peer-reviewed)abstract
- This paper describes the addition of Collision-Induced Absorption (CIA) into the HITRAN compilation. The data from different experimental and theoretical sources have been cast into a consistent format and formalism. The implementation of these new spectral data into the HITRAN database is invaluable for modeling and interpreting spectra of telluric and other planetary atmospheres as well as stellar atmospheres. In this implementation for HITRAN, CIAs of N-2, H-2, O-2, CO2, and CH4 due to various collisionally interacting atoms or molecules are presented. Some CIA spectra are given over an extended range of frequencies, including several H-2 overtone bands that are dipole-forbidden in the non-interacting molecules. Temperatures from tens to thousands of Kelvin are considered, as required, for example, in astrophysical analyses of objects, including cool white dwarfs, brown dwarfs. M dwarfs, cool main sequence stars, solar and extra-solar planets, and the formation of so-called first stars. (C) 2011 Elsevier Ltd. All rights reserved.
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4. |
- Amirthalingam, M., et al.
(author)
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Synchrotron diffraction analysis of retained austenite in welded transformation induced plasticity (TRIP) steels
- 2012
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In: Science and Technology of Welding and Joining. - 1362-1718. ; 17:2, s. 146-154
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Journal article (peer-reviewed)abstract
- A quantitative analysis of the retained austenite (RA) fractions in gas tungsten arc welded silicon and aluminium containing transformation induced plasticity steels was carried out by synchrotron X-ray diffraction measurements. The variation in RA transverse to the weld line was measured to study the effect of weld thermal cycles on the stabilisation of austenite in the heat affected zone (HAZ) and the fusion zone (FZ). The results showed that the FZ of silicon based steels contained a higher amount of RA (similar to 7%) than aluminium based steels, which contained only similar to 4%. During the solidification of the weld pool, aluminium was found to partition to solidifying delta-ferrite and to stabilise the soft delta-ferrite grains at the fusion boundaries. Owing to this partitioning, the HAZ was enriched in carbon and the RA content was found to increase with distance from the fusion boundaries. In contrast, this partitioning behaviour was not present in silicon based transformation induced plasticity steels and a lesser amount of RA was found in the coarse grained HAZ than in the FZ.
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5. |
- DE Moerloose, P, et al.
(author)
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Recommendations for assessment, monitoring and follow-up of patients with haemophilia.
- 2011
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In: Haemophilia. - : Wiley. - 1351-8216.
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Journal article (peer-reviewed)abstract
- Summary. Over the last few decades, clinical follow-up of patients with haemophilia has become more complex as a result of the introduction of new treatment strategies, the presence of comorbidities related to haemophilia or ageing, as well as the emergence of new tools to evaluate the medical and social consequences of haemophilia. This publication describes the parameters and information that should be documented and the tests, examinations and interventions required for optimal follow-up of a patient with haemophilia. In the absence of formal studies, the present recommendations have been established as result of a series of consensus meetings in the frame of the European Haemophilia Therapy Standardization Board (EHTSB). The following 11 domains were identified: Baseline information, Current status, Treatment, Inhibitor status, Bleeding, Joint status and pain, Comorbidities, Dental care, Physical activities, Social participation and Quality of life. For each domain, details are proposed for the relevant parameters to be captured and monitored as well as the relevant tools that facilitate data collection. Adopting these recommendations should help the individual care of patients and, even though this is not the primary objective of this article, it should also help at national and international level to shape a new approach to haemophilia by working towards a more standardized outcome assessment. Greater standardization should have implications for data collection, improvements in treatment evaluation and optimizing resources.
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6. |
- de Vries, Stefan P W, et al.
(author)
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Genome analysis of Moraxella catarrhalis strain RH4, a Human Respiratory Tract Pathogen.
- 2010
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In: Journal of Bacteriology. - 0021-9193. ; 192:14, s. 3574-3583
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Journal article (peer-reviewed)abstract
- Moraxella catarrhalis is an emerging human-restricted respiratory tract pathogen that is a common cause of childhood otitis media and exacerbations of chronic obstructive pulmonary disease in adults. Here, we report the first completely assembled and annotated genome sequence of an isolate of M. catarrhalis: strain RH4, originally isolated from blood of an infected patient. The RH4 genome consists of 1,863,286 nucleotides harboring 1,886 protein-encoding genes. Comparison of the RH4 genome to the ATCC 43617 contigs demonstrated that the gene content of both strains is highly conserved. In silico phylogenetic analyses based on both 16S rRNA and multilocus sequence typing revealed that RH4 belongs to the seroresistant lineage. We were able to identify close to the entire repertoire of known M. catarrhalis virulence factors, and mapped the members of the biosynthetic pathways for lipooligosaccharide, peptidoglycan, and type IV pili. A reconstruction of the central metabolic pathways suggests that RH4 relies on fatty acid and acetate metabolism, as the genes encoding the enzymes required for the glyoxylate pathway, tricarboxylic acid cycle, gluconeogenic pathway, non-oxidative branch of the pentose phosphate pathway, beta-oxidation pathway of fatty acids, and acetate metabolism were present. Moreover, pathways important for survival under in vivo challenging conditions such as iron-acquisition pathways, nitrogen metabolism, and oxidative stress responses were identified. Finally, we showed by microarray expression profiling that approximately 88% of the predicted coding sequences are transcribed under in vitro conditions. Overall, these results provide a foundation for future research into the mechanisms of M. catarrhalis pathogenesis and vaccine development.
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7. |
- Derde, Sarah, et al.
(author)
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Muscle atrophy and preferential loss of myosin in prolonged critically ill patients
- 2012
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In: Critical Care Medicine. - 0090-3493 .- 1530-0293. ; 40:1, s. 79-89
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Journal article (peer-reviewed)abstract
- OBJECTIVE: Muscle weakness contributes to prolonged rehabilitation and adverse outcome of critically ill patients. Distinction between a neurogenic and/or myogenic underlying problem is difficult using routine diagnostic tools. Preferential loss of myosin has been suggested to point to a myogenic component. We evaluated markers of muscle atrophy and denervation, and the myosin/actin ratio in limb and abdominal wall skeletal muscle, of prolonged critically ill patients and matched controls in relation to insulin therapy and known risk factors for intensive care unit-acquired weakness. DESIGN: Secondary analysis of two large, prospective, single-center randomized clinical studies. SETTING: University hospital surgical and medical intensive care unit. PATIENTS: Critically ill patients and matched controls. INTERVENTIONS: Intensive care unit patients had been randomized to blood glucose control to 80-110 mg/dL with insulin infusion or conventional glucose management, where insulin was only administered when glucose levels rose above 215 mg/dL. MEASUREMENTS AND MAIN RESULTS: As compared with controls, rectus abdominis and vastus lateralis muscle of critically ill patients showed smaller myofiber size, decreased mRNA levels for myofibrillar proteins, increased proteolytic enzyme activities, and a lower myosin/actin ratio, virtually irrespective of insulin therapy. Increased forkhead box protein O1 action may have played a role. Most alterations were more severe in patients treated with corticosteroids. Duration of corticosteroid treatment, independent of duration of intensive care unit stay or other risk factors, was a dominant risk factor for a low myosin/actin ratio. The immature acetylcholine receptor subunit γ mRNA expression was elevated in vastus lateralis, independent of the myosin/actin ratio. CONCLUSIONS: Both limb and abdominal wall skeletal muscles of prolonged critically ill patients showed downregulation of protein synthesis at the gene expression level as well as increased proteolysis. This affected myosin to a greater extent than actin, resulting in a decreased myosin/actin ratio. Muscle atrophy was not ameliorated by intensive insulin therapy, but possibly aggravated by corticosteroids.
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8. |
- Haaker, J, et al.
(author)
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A review on human reinstatement studies: an overview and methodological challenges
- 2014
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In: Learning & memory (Cold Spring Harbor, N.Y.). - : Cold Spring Harbor Laboratory. - 1549-5485. ; 21:9, s. 424-440
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Journal article (peer-reviewed)abstract
- In human research, studies of return of fear (ROF) phenomena, and reinstatement in particular, began only a decade ago and recently are more widely used, e.g., as outcome measures for fear/extinction memory manipulations (e.g., reconsolidation). As reinstatement research in humans is still in its infancy, providing an overview of its stability and boundary conditions and summarizing methodological challenges is timely to foster fruitful future research. As a translational endeavor, clarifying the circumstances under which (experimental) reinstatement occurs may offer a first step toward understanding relapse as a clinical phenomenon and pave the way for the development of new pharmacological or behavioral ways to prevent ROF. The current state of research does not yet allow pinpointing these circumstances in detail and we hope this review will aid the research field to advance in this direction. As an introduction, we begin with a synopsis of rodent work on reinstatement and theories that have been proposed to explain the findings. The review however mainly focuses on reinstatement in humans. We first describe details and variations of the experimental setup in reinstatement studies in humans and give a general overview of results. We continue with a compilation of possible experimental boundary conditions and end with the role of individual differences and behavioral and/or pharmacological manipulations. Furthermore, we compile important methodological and design details on the published studies in humans and end with open research questions and some important methodological and design recommendations as a guide for future research.
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9. |
- Hermans, Joen J., et al.
(author)
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The molecular structure of three types of long-chain zinc(II) alkanoates for the study of oil paint degradation
- 2014
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In: Polyhedron. - : Elsevier BV. - 0277-5387 .- 1873-3719. ; 81, s. 335-340
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Journal article (peer-reviewed)abstract
- The molecular structures of three types of zinc(II) alkanoates (Zn(C-n)(2), ZnNa2(C-n)(4) and ZaK(2)(C-n)(4), C-n = CH3(CH2)(n-2)COO-, n = 8-18) were investigated by Fourier transform infrared spectroscopy (FTIR) and powder X-ray diffraction (PXRD) to aid the study of degradation phenomena in oil paint. Each type of complex forms a homologous series in the studied chain length range. For (Zn(C-n)(2) and ZnNa2(C-n)(4), these homologous series indicate that the long-chain complexes have structures highly similar to those published for short-chain single-crystal analogs. Moreover, we introduce a chain tilt factor F-tilt to derive information about alkanoate chain angles from highly accurate PXRD long spacing values. The complex ZnK2(C-n)(4) has not been reported before. It consists of metal sheets containing tetrahedrally coordinated Zn atoms and octahedrally coordinated K atoms bridged by effectively monodentate carboxylate groups, and alkanoate chains that are tilted relative to the long axis.
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10. |
- Langereis, Jeroen D, et al.
(author)
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Modified Lipooligosaccharide Structure Protects Nontypeable Haemophilus influenzae from IgM-Mediated Complement Killing in Experimental Otitis Media
- 2012
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In: mBio. - : American Society for Microbiology: mBio / American Society for Microbiology. - 2161-2129 .- 2150-7511. ; 3:4
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Journal article (peer-reviewed)abstract
- Nontypeable Haemophilus influenzae (NTHi) is a Gram-negative, human-restricted pathogen. Although this bacterium typically colonizes the nasopharynx in the absence of clinical symptoms, it is also one of the major pathogens causing otitis media (OM) in children. Complement represents an important aspect of the host defense against NTHi. In general, NTHi is efficiently killed by complement-mediated killing; however, various resistance mechanisms have also evolved. We measured the complement resistance of NTHi isolates isolated from the nasopharynx and the middle ear fluids of OM patients. Furthermore, we determined the molecular mechanism of NTHi complement resistance. Complement resistance was strongly increased in isolates from the middle ear, which correlated with decreased binding of IgM. We identified a crucial role for the R2866_0112 gene in complement resistance. Deletion of this gene altered the lipooligosaccharide (LOS) composition of the bacterium, which increased IgM binding and complement-mediated lysis. In a novel mouse model of coinfection with influenza virus, we demonstrate decreased virulence for the R2866_0112 deletion mutant. These findings identify a mechanism by which NTHi modifies its LOS structure to prevent recognition by IgM and activation of complement. Importantly, this mechanism plays a crucial role in the ability of NTHi to cause OM. less thanbrgreater than less thanbrgreater thanIMPORTANCE Nontypeable Haemophilus influenzae (NTHi) colonizes the nasopharynx of especially young children without any obvious symptoms. However, NTHi is also a major pathogen in otitis media (OM), one of the most common childhood infections. Although this pathogen is often associated with OM, the mechanism by which this bacterium is able to cause OM is largely unknown. Our study addresses a key biological question that is highly relevant for child health: what is the molecular mechanism that enables NTHi to cause OM? We show that isolates collected from the middle ear fluid exhibit increased complement resistance and that the lipooligosaccharide (LOS) structure determines IgM binding and complement activation. Modification of the LOS structure decreased NTHi virulence in a novel NTHi-influenza A virus coinfection OM mouse model. Our findings may also have important implications for other Gram-negative pathogens harboring LOS, such as Neisseria meningitidis, Moraxella catarrhalis, and Bordetella pertussis.
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