bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long-term remission of Philadelphia chromosome–positive acute lymphoblastic leukemia after allogeneic hematopoietic cell transplantation from matched sibling donors: a 20-year experience with the fractionated total body irradiation–etoposide regimen
Authors: Laport Ginna G. , Alvarnas Joseph C. , Palmer Joycelynne M. , Snyder David S. , Slovak Marilyn L. , Cherry Athena M. , Wong Ruby M. , Negrin Robert S. , Blume Karl G. , Forman Stephen J. 
Published: June 2008

AbstractAllogeneic hematopoietic cell transplantation (HCT) is the only known curative modality for patients with Philadelphia chromosome–positive acute lymphoblastic leukemia (Ph+ ALL). Sixty-seven patients with HLA-matched sibling donors received fractionated total body irradiation (FTBI) and high-dose VP16, whereas 11 patients received FTBI/VP16/cyclophosphamide, and 1 patient received FTBI/VP16/busulfan. The median age was 36 years. At the time of HCT, 49 patients (62%) were in first complete remission (CR1) and 30 patients (38%) were beyond CR1 (> CR1). The median follow-up was 75 months (range, 14-245 months). The 10-year overall survival for the CR1 and beyond CR1 patients was 54% and 29% (P = .01), respectively, and event-free survival was 48% and 26% (P = .02), respectively. There was no significant difference in relapse incidence (28% vs 41%, P = .28), but nonrelapse mortality was significantly higher in the beyond CR1 patients, (31% vs 54%, P = .03, respectively). By univariate analysis, factors affecting event-free and overall survival were white blood cell count at diagnosis (< 30 × 109/L vs > 30 × 109/L) and disease status (CR1 vs > CR1). The median time to relapse for CR1 and for beyond CR1 patients was 12 months and 9 months, respectively. Our results indicate that FTBI/VP16 with or without cyclophosphamide confers long-term survival in Ph+ ALL patients and that disease status at the time of HCT is an important predictor of outcome.
The GraRS regulatory system controls Staphylococcus aureus susceptibility to antimicrobial host defenses
Authors: Kraus Dirk , Herbert Silvia , Kristian Sascha A , Khosravi Arya , Nizet Victor , Götz Friedrich , Peschel Andreas 
Published: June 2008

Abstract

Background
Modification of teichoic acids with D-alanine by the products of the dlt operon protects Gram-positive bacteria against major antimicrobial host defense molecules such as defensins, cathelicidins, myeloperoxidase or phospholipase. The gra RS regulatory genes have recently been implicated in the control of D-alanylation in Staphylococcus aureus.

Results
To determine the impact of the GraRS regulatory system on resistance to antimicrobial host defense mechanisms and virulence of S. aureus, we compared inactivation of S. aureus SA113 wild type and its isogenic gra RS deletion mutant by the human cathelicidin LL-37 or human neutrophil granulocytes in vitro, and the ability to cause infection in vivo. We show here that gra RS deletion considerably alters bacterial surface charge, increases susceptibility to killing by human neutrophils or the defense peptide LL-37, and attenuates virulence of S. aureus in a mouse infection model.

Conclusion
Our results indicate that S. aureus can regulate its surface properties in order to overcome innate host defenses.
Variation in WNT7A is unlikely to be a cause of familial Congenital Talipes Equinovarus
Authors: Liu Guoqing , Inglis Julie , Cardy Amanda , Shaw Duncan , Sahota Sukhy , Hennekam Raoul , Sharp Linda , Miedzybrodzka Zosia 
Published: June 2008

Abstract

Background
Genetic factors make an important contribution to the aetiology of congenital talipes equinovarus (CTEV), the most common developmental disorder of the lower limb. WNT7A was suggested as a candidate gene for CTEV on the basis of a genome-wide scan for linkage in a large multi-case family. WNT7A is a plausible candidate gene for CTEV as it provides a signal for pattern formation during limb development, and mutation in WNT7A has been reported in a number of limb malformation syndromes.

Methods
We investigated the role of WNT7A using a family-based linkage approach in our large series of European multi-case CTEV families. Three microsatellite markers were used, of which one (D3S2385) is intragenic, and the other two (D3S2403, D3S1252) are 700 kb 5' to the start and 20 kb from the 3' end of the gene, respectively. Ninety-one CTEV families, comprising 476 individuals of whom 211 were affected, were genotyped. LOD scores using recessive and incomplete-dominant inheritance models, and non-parametric linkage scores, excluded linkage.

Results
No significant evidence for linkage was observed using either parametric or non-parametric models. LOD scores for the parametric models remained strongly negative in the regions between the markers, and in the 0.5 cM intervals outside the marker map. No significant lod scores were obtained when the data were analysed allowing for heterogeneity.

Conclusion
Our evidence suggests that the WNT7A gene is unlikely to be a major contributor to the aetiology of familial CTEV.
The diagnostic work up of growth failure in secondary health care; An evaluation of consensus guidelines
Authors: Grote Floor K , Oostdijk Wilma , De Muinck Keizer-Schrama Sabine MPF , van Dommelen Paula , van Buuren Stef , Dekker Friedo W , Ketel Arnoldus G , Moll Henriette A , Wit Jan M 
Published: June 2008

AbstractBackgroundAs abnormal growth might be the first manifestation of undetected diseases, it is important to have accurate referral criteria and a proper diagnostic work-up. In the present paper we evaluate the diagnostic work-up in secondary health care according to existing consensus guidelines and study the frequency of underlying medical disorders.MethodsData on growth and additional diagnostic procedures were collected from medical records of new patients referred for short stature to the outpatient clinics of the general paediatric departments of two hospitals (Erasmus MC – Sophia Children's Hospital, Rotterdam and Spaarne Hospital, Haarlem) between January 1998 and December 2002. As the Dutch Consensus Guideline (DCG) is the only guideline addressing referral criteria as well as diagnostic work-up, the analyses were based on its seven auxological referral criteria to determine the characteristics of children who are incorrectly referred and the adequacy of workup of those who are referred.ResultsTwenty four percent of children older than 3 years were inappropriately referred (NCR). Of the correctly referred children 74–88% were short corrected for parental height, 40–61% had a height SDS <-2.5 and 21% showed height deflection (Δ HSDS < -0.25/yr or Δ HSDS < -1). In none of the children a complete detailed routine diagnostic work up was performed and in more than 30% no routine laboratory examination was done at all. Pathologic causes of short stature were found in 27 children (5%).ConclusionExisting guidelines for workup of children with suspected growth failure are poorly implemented. Although poorly implemented the DCG detects at least 5% pathologic causes of growth failure in children referred for short stature. New guidelines for referral are required with a better sensitivity and specificity, wherein distance to target height should get more attention. The general diagnostic work up for short stature should include testing for celiac disease in all children and for Turner syndrome in girls.
Bacterial-based systems for expression and purification of recombinant Lassa virus proteins of immunological relevance
Authors: Branco Luis M , Matschiner Alex , Fair Joseph N , Goba Augustine , Sampey Darryl B , Ferro Philip J , Cashman Kathleen A , Schoepp Randal J , Tesh Robert B , Bausch Daniel G , Garry Robert F , Guttieri Mary C 
Published: June 2008

Abstract

Background
There is a significant requirement for the development and acquisition of reagents that will facilitate effective diagnosis, treatment, and prevention of Lassa fever. In this regard, recombinant Lassa virus (LASV) proteins may serve as valuable tools in diverse antiviral applications. Bacterial-based systems were engineered for expression and purification of recombinant LASV nucleoprotein (NP), glycoprotein 1 (GP1), and glycoprotein 2 (GP2).

Results
Full-length NP and the ectodomains of GP1 and GP2 were generated as maltose-binding protein (MBP) fusions in the Rosetta strains of Escherichia coli (E. coli) using pMAL-c2x vectors. Average fusion protein yields per liter of culture for MBP-NP, MBP-GP1, and MBP-GP2 were 10 mg, 9 mg, and 9 mg, respectively. Each protein was captured from cell lysates using amylose resin, cleaved with Factor Xa, and purified using size-exclusion chromatography (SEC). Fermentation cultures resulted in average yields per liter of 1.6 mg, 1.5 mg, and 0.7 mg of purified NP, GP1 and GP2, respectively. LASV-specific antibodies in human convalescent sera specifically detected each of the purified recombinant LASV proteins, highlighting their utility in diagnostic applications. In addition, mouse hyperimmune ascitic fluids (MHAF) against a panel of Old and New World arenaviruses demonstrated selective cross reactivity with LASV proteins in Western blot and enzyme-linked immunosorbent assay (ELISA).

Conclusion
These results demonstrate the potential for developing broadly reactive immunological assays that employ all three arenaviral proteins individually and in combination.
Germanachandachsoin repair uptake: Resources to sustain or remove epistemic asymmetry
Authors: Golato Andrea , Betz Emma 
Published: June 2008

AbstractUsing conversation analytic methodology, this article examines the use of the German tokensach‘oh’ andachso‘oh I see’ in the uptake of repair. Whileachsohas been studied from a functional-pragmatic perspective, no contrastive study ofachandachsoexists. We investigate two questions: Do different forms ofachoccur in the same sequential position? Are they associated with different interactional functions?The paper shows that in sequentially third position, repairs are receipted withachorachso. The different tokens have different interactional import. While ach receipts new informational content (claiming a change-of-state, similar to Englishoh; Heritage 1984), it does not mark understanding.Achso, by contrast, serves as a receipt for informationandprior action, claiming that speakers now share the same epistemic level and thus marking a successful repair resolution.Achsomakes sequence closure relevant and is followed by a shift in action and/or topic while anach-turn makes more talk relevant, specifically next turn confirmation.The paper concludes by discussing the component tokensachandsoseparately and outlining implications for the study of epistemics through ‘little words.’
Embryonic Stem Cell-Derived Neurons as a Cellular System to Study Gene Function: Lack of Amyloid Precursor Proteins APP and APLP2 Leads to Defective Synaptic Transmission
Authors: Schrenk-Siemens Katrin , Perez-Alcala Siro , Richter Jens , Lacroix Emmanuel , Rahuel Joseph , Korte Martin , Müller Ulrike , Barde Yves-Alain , Bibel Miriam 
Published: June 2008

Abstract
The in vitro generation of uniform populations of neurons from mouse embryonic stem cells (ESCs) provides a novel opportunity to study gene function in neurons. This is of particular interest when mutations lead to lethal in vivo phenotypes. Although the amyloid precursor protein (APP) and its proteolysis are regarded as key elements of the pathology of Alzheimer's disease, the physiological function of APP is not well understood and mice lacking App and the related gene Aplp2 die early postnatally without any obvious histopathological abnormalities. Here we show that glutamatergic neurons differentiated from ESCs lacking both genes reveal a decreased expression of the vesicular glutamate transporter 2 (VGLUT2) both at the mRNA and protein level, as well as a reduced uptake and/or release of glutamate. Blocking γ-secretase cleavage of APP in wild-type neurons resulted in a similar decrease of VGLUT2 expression, whereas VGLUT2 levels could be restored in App−/−Aplp2−/− neurons by a construct encompassing the C-terminal intracellular domain of APP. Electrophysiological recordings of hippocampal organotypic slice cultures prepared from corresponding mutant mice corroborated these observations. Gene expression profiling and pathway analysis of the differentiated App−/−Aplp2−/− neurons identified dysregulation of additional genes involved in synaptic transmission pathways. Our results indicate a significant functional role of APP and amyloid precursor-like protein 2 (APLP2) in the development of synaptic function by the regulation of glutamatergic neurotransmission. Differentiation of ESCs into homogeneous populations thus represents a new opportunity to explore gene function and to dissect signaling pathways in neurons.
Disclosure of potential conflicts of interest is found at the end of this article.
Osteopontin Promotes the Development of Natural Killer Cells from Hematopoietic Stem Cells
Authors: Chung Jin Woong , Kim Mi Sun , Piao Zheng-Hao , Jeong Mira , Yoon Suk Ran , Shin Nara , Kim Sang Yong , Hwang Eun Sook , Yang Young , Lee Young Ho , Kim Young Sang , Choi Inpyo 
Published: June 2008

AbstractThe detailed mechanisms driving the development of natural killer (NK) cells from hematopoietic stem cells remain to be clearly elucidated. Here, we show that osteopontin (OPN) is a key factor for NK development. OPN-deficient mice evidenced severe impairments of NK development in bone marrow (BM) and spleen in which the NK populations that express CD122 and NK cell receptors were reduced. However, the absence of intrinsic OPN expression did not affect NK development, whereas the absence of OPN in the microenvironment caused a significant reduction in NK population. The expression of OPN was induced by interleukin (IL)-15 in BM stromal cells, and the defect in NK differentiation in IL-15−/− hematopoietic precursor cells (HPC) was recovered by addition of recombinant OPN, suggesting that the microenvironmental OPN may be a key factor in IL-15-mediated NK differentiation. In addition, OPN-driven NK maturation was reduced in T-bet-deficient HPC, suggesting that T-bet is required for OPN-mediated NK development. Collectively, these results show that paracrine OPN signaling drives NK-lineage commitment, thus ultimately promoting NK cell development.Disclosure of potential conflicts of interest is found at the end of this article.
Functional Outcomes and Quality of Life After Tumor-Related Hemipelvectomy
Authors: Beck Lisa A , Einertson Marlene J , Winemiller Mark H , DePompolo Robert W , Hoppe Kurtis M , Sim Franklin F 
Published: June 2008

Background and Purpose
Hemipelvectomy is a life-changing treatment for pelvic malignancies. This study compared functional outcomes and quality of life of patients following internal or external hemipelvectomies.

Subjects
Ninety-seven patients who underwent tumor-related internal (n=39) or external (n=58) hemipelvectomy surgery between January 1, 1988, and December 31, 2004, participated in the study.

Methods
Using a descriptive retrospective cohort study design, functional status was evaluated with the Barthel Index at 3 time points. Quality-of-life parameters were evaluated at follow-up using the Linear Analog Self-Assessment tool (LASA).

Results
Data were collected on all 97 patients at rehabilitation consultation and hospital discharge. Follow-up data were obtained via survey on 44% of the original group at a median of 5.8 years (interquartile range [IRQ]=1.7–10.4) after surgery. Median total Barthel Index scores were similar between the internal and external hemipelvectomy groups at the initial physical medicine and rehabilitation assessment (10 [IQR=10–15] versus 10 [IQR=3.75–15]), at discharge (40 [IQR=30–65] versus 50 [IQR=35–66.25]), and at follow-up (92.5 [IQR=76.25–100] versus 92.5 [IQR=78.75–96.25]). Participants with external hemipelvectomies were less independent in bladder function and experienced greater pain severity at follow-up compared with those with internal hemipelvectomies. Overall quality-of-life parameters were similar between the groups.

Discussion and Conclusion
Despite significant differences in surgical procedures, immediate and long-term functional outcomes and quality-of-life parameters were similar among participants with internal and external hemipelvectomies.
Nonisothermal crystallization kinetics of poly(ethylene terephthalate)/antimony‐doped tin oxide nanocomposites
Authors: Chen Xiaolei , Li Chunzhong , Shao Wei , Liu Tianxi , Wang Lumin 
Published: June 2008

AbstractPoly(ethylene terephthalate) (PET)/antimony‐doped tin oxide (ATO) nanocomposites were prepared by in situ polymerization. The nonisothermal crystallization behaviors of neat PET and PET/ATO nanocomposites were investigated with differential scanning calorimetry. The nonisothermal crystallization data were analyzed with the Avrami analysis modified by Jeziorny, the Ozawa method, and a method developed by Liu et al. The modified Avrami equation could describe only the primary stage of nonisothermal crystallization of PET and PET/ATO nanocomposites. The Ozawa analysis, when applied to the polymer systems studied here, failed to describe their nonisothermal crystallization behavior. The kinetic method developed by Liu et al. was successful in describing the nonisothermal crystallization of neat PET and PET/ATO nanocomposites. According to the Kissinger equation, the activation energies were determined to be −205.3, −220.0, and −243.7 kJ/mol for neat PET and 99/1 and 95/5 PET/ATO nanocomposites, respectively. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
pH‐responsive fibers based on acrylonitrile acrylic acid block copolymers: Effect of spinning conditions and postspinning operations on response and mechanical properties
Authors: Sahoo Anasuya , Jassal Manjeet , Agrawal Ashwini K. 
Published: June 2008

AbstractpH sensitive poly(acrylonitrile‐co‐acrylic acid) having ∼50 mol % acrylic acid with block type structure (AA50B) was synthesized by controlled dosing method of free radical polymerization. The polymer was converted into fibers by wet solution spinning technique in DMF‐water system. The resulting block type copolymer could generate a domain type morphology with segregated domains of acrylonitrile and acrylic acid on heat‐setting. The drawing ratio and heat‐setting temperature had a significant effect on the formation of these domains and their stability. The domain formation was more pronounced when the fibers could be drawn to higher draw ratios during coagulation or heat‐set at higher temperature. The stability of the fibers, which is influenced by domain formation, was lowest (at few cycles of transitions) when the fibers were heat‐set at 100°C, while it is improved significantly to more than 50 cycles as the heat‐setting temperature was increased to 150°C. The coagulation conditions, drawing and the heat‐setting also greatly influenced the mechanical properties, transition behavior, and retractive stresses of the responsive fibers. The tenacity improved by 6.6 times in swollen state and 1.4 times in deswollen state, while the retractive stresses during deswelling were significantly increased to about 4.7 times. However, the increased heat‐setting temperature was also found to have a negative effect on the equilibrium swelling values as well as the response rate. The effect of heat‐setting on chemical structure of the copolymer was also investigated. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
Preparation of phosphor coated with a polymer by emulsion polymerization and its application in low‐density polyethylene
Authors: Chen Tonghua , Luo Yongyue , Zou Jiangpeng , Jiang Long , Dan Yi , Zhang Li , Zhao Kun 
Published: June 2008

AbstractThe long‐afterglow phosphor SrAl2O4 : Eu2+, Dy3+ is liable to hydrolyze in water with deterioration of its luminescent properties. In this study, in situ emulsion polymerization was first used to prepare phosphor coated with poly(methyl methacrylate‐co‐butyl acrylate) [P(MMA‐co‐BA)] to improve water resistance. Fourier transform infrared spectra suggested that the polymer attached to the phosphor by chemical bonding. Observation by scanning electron microscopy (SEM) showed that a polymer layer formed on the surface of the phosphor. The resistance to water of the phosphor coated with the polymer layer was much better than that of the uncoated phosphor because the transparent polymer layer could suppress its hydrolysis process. Low‐density polyethylene (LDPE) plastics, doped with long‐afterglow phosphors, were manufactured with an extrusion technique. Through coating with P(MMA‐co‐BA), the compatibility of phosphor with the LDPE matrix was improved, as determined by SEM. The luminous LDPE plastics blended with the phosphor coated with polymer showed long and strong phosphorescence with little loss of persistence phosphorescence compared to the uncoated phosphor. The LDPE plastics still retained their mechanical properties through doping with 3% (mass fraction) of the phosphors. © 2008 Wiley Periodicals, Inc. J Appl Polym Sci, 2008
High‐speed microchip electrophoresis method for the separation of (R,S)‐naproxen
Authors: Guihen Elizabeth , Hogan Anna‐Marie , Glennon Jeremy D. 
Published: June 2008

AbstractIn this research, a capillary electrophoretic method for the fast enantiomeric resolution of (R,S)‐naproxen was investigated. Method development involved variation of applied potential, buffer concentration, buffer pH, and cyclodextrin concentration. The optimum electrophoretic separation conditions were 110 mM sodium acetate run buffer (pH 6.0), 30 mM methyl‐β‐cyclodextrin, 20% (v/v) acetonitrile, 25°C. The total length of capillary was 48 cm, (50 μm I.D.) with ultra violet (UV) detection at 232 nm. Using these conditions, the number of theoretical plates was close to one million (896,000/m). The possibility of achieving a fast chiral separation of (R,S)‐naproxen on a microchip of 2.5 cm in length was investigated. Complete enantiomeric resolution of naproxen was achieved in less than 1 min, on this microchip platform, with linear imaging UV detection. This system had the advantage of real‐time separation monitoring, so that enantiomeric resolution could be visually observed, and high‐speed chiral analysis was realized. The microchip electrophoresis (MCE) separation was compared with the capillary electrophoresis (CE) separation with regards to speed, efficiency, separation platform, and precision. This work highlights the potential of CE and MCE in future chiral separations. Chirality, 2009. © 2008 Wiley‐Liss, Inc.
PERMIS: a modular authorization infrastructure
Authors: Chadwick David , Zhao Gansen , Otenko Sassa , Laborde Romain , Su Linying , Nguyen Tuan Anh 
Published: June 2008

AbstractAuthorization infrastructures manage privileges and render access control decisions, allowing applications to adjust their behavior according to the privileges allocated to users. This paper describes the PERMIS role‐based authorization infrastructure along with its conceptual authorization, access control, and trust models. PERMIS has the novel concept of a credential validation service, which verifies a user's credentials prior to access control decision‐making and enables the distributed management of credentials. PERMIS also supports delegation of authority; thus, credentials can be delegated between users, further decentralizing credential management. Finally, PERMIS supports history‐based decision‐making, which can be used to enforce such aspects as separation of duties and cumulative use of resources. Details of the design and the implementation of PERMIS are presented along with details of its integration with Globus Toolkit, Shibboleth, and GridShib. A comparison of PERMIS with other authorization and access control implementations is given, along with suggestions where future research and development are still needed. Copyright © 2008 John Wiley & Sons, Ltd.