bluuebunny/crossref_metadata_2025_split huggingface dataset:
Characteristics and Outcomes of Children With the Wilms Tumor-Aniridia Syndrome: A Report From the National Wilms Tumor Study Group
Authors: Breslow Norman E. , Norris Robin , Norkool Patricia A. , Kang Tammy , Beckwith J. Bruce , Perlman Elizabeth J. , Ritchey Michael L. , Green Daniel M. , Nichols Kim E. 
Published: December 2003

Purpose: Children with the rare Wilms tumor (WT)-aniridia (WAGR) syndrome have not had systematic evaluation of their clinical and pathologic features. We compared demographics, disease characteristics, and treatment outcomes in a large cohort of WT patients who did or did not have the WAGR syndrome. Patients and Methods: Clinical and pathology records were reviewed for 8,533 patients enrolled between 1969 and 2002 by the National Wilms Tumor Study Group. Results: Sixty-four patients (0.75%) had the WAGR syndrome. For WAGR and non-WAGR patients, respectively, the average birth weights (2.94 and 3.45 kg), median ages at diagnosis (22 and 39 months), and the percentages with bilateral disease (17% and 6%), metastatic disease (2% and 13%), favorable histology (FH) tumors (100% and 92%), and intralobar nephrogenic rests (ILNR; 77% and 22%) all differed. Survival estimates for WAGR and non-WAGR patients were 95% ± 3% and 92% ± 0.3% at 4 years but 48% ± 17% and 86% ± 1.0%, respectively, at 27 years from diagnosis. Five late deaths in WAGR patients were from end-stage renal disease (ESRD). Conclusion: The excess of bilateral disease, ILNR-associated FH tumors of mixed cell type, and early ages at diagnosis in WAGR patients all fit the known phenotypic spectrum of constitutional deletion of chromosome 11p13. Despite a favorable response of their WT to treatment, WAGR patients have a high risk of ESRD as they approach adulthood. The renal pathology associated with this apparent late manifestation of WT1 deletion, and the explanation for the abnormally low birth weights in patients with del 11p13, have yet to be determined.
Prediction of Response to Preoperative Chemotherapy in Gastric Carcinoma by Metabolic Imaging: Results of a Prospective Trial
Authors: Ott Katja , Fink Ulrich , Becker Karen , Stahl Alexander , Dittler Hans-Joachim , Busch Raymonde , Stein Hubert , Lordick Florian , Link Thomas , Schwaiger Markus , Siewert Jörg-Rüdiger , Weber Wolfgang A. 
Published: December 2003

Purpose: We prospectively evaluated the predictive value of therapy-induced reduction of tumor glucose use for subsequent response and patient survival in patients with gastric cancer treated by preoperative chemotherapy. Patients and Methods: Forty-four consecutive patients with locally advanced gastric carcinomas were studied by positron emission tomography with the glucose analog fluorine-18 fluorodeoxyglucose (FDG-PET) at baseline and 14 days after initiation of cisplatin-based polychemotherapy. On the basis of a previous study, a reduction of tumor FDG uptake by more than 35% was used as a criterion for a metabolic response. The metabolic response in FDG-PET was correlated with histopathologic response after completion of therapy (< 10% viable tumor cells in the resected specimen) and patient survival. Results: Thirty-five (80%) of the 44 tumors were visualized with sufficient contrast for quantitative analysis (two of 19 intestinal and seven of 25 nonintestinal tumors showed only low FDG uptake). In the 35 assessable patients, PET imaging after 14 days of therapy correctly predicted histopathologic response after 3 months of therapy in 10 (77%) of 13 responders and 19 (86%) of 22 nonresponders. Median overall survival for patients with a metabolic response has not been reached (2-year survival rate, 90%); for patients without a metabolic response, median survival was only 18.9 months (2-year survival rate, 25%; P = .002) Conclusion: This study prospectively demonstrates that in patients with gastric cancer, response to preoperative chemotherapy can be predicted by FDG-PET early during the course of therapy. By avoiding the morbidity and costs of ineffective therapy, FDG-PET imaging may markedly facilitate the use of preoperative chemotherapy.
Topoisomerase-IIα Is Upregulated in Malignant Peripheral Nerve Sheath Tumors and Associated With Clinical Outcome
Authors: Skotheim Rolf I. , Kallioniemi Anne , Bjerkhagen Bodil , Mertens Fredrik , Brekke Helge R. , Monni Outi , Mousses Spyro , Mandahl Nils , Sœter Gunnar , Nesland Jahn M. , Smeland Sigbjørn , Kallioniemi Olli-P. , Lothe Ragnhild A. 
Published: December 2003

Purpose: To identify target genes of clinical significance for patients with malignant peripheral-nerve sheath tumor (MPNST), an aggressive cancer for which no consensus therapy exists. Materials and Methods: Biopsies and clinical data from 51 patients with MPNST were included in this study. Based on our previous research implicating chromosome arm 17q amplification in MPNST, we performed gene expression analyses of 14 MPNSTs using chromosome 17–specific cDNA microarrays. Copy numbers of selected gene probes and centromere probes were then determined by interphase fluorescence in situ hybridization in 16 MPNSTs. Finally, we generated a tissue microarray containing 79 samples from 44 MPNSTs, on which in situ protein expressions of candidate genes were examined and related to clinical end points. Results: Among several deregulated genes found by cDNA microarray analyses, topoisomerase IIα (TOP2A) was the most overexpressed gene in MPNSTs compared with benign neurofibromas. Excess copies of the TOP2A were also seen at the DNA level in 10 of 16 cases, and high expression of the TOP2A protein was seen in 83% of the tumors on the tissue microarray. The TOP2A-expressing tumors were associated with poor cancer-specific survival and presence of metastases. Conclusion: We have identified TOP2A as a target gene in MPNST, using a focused gene expression profiling followed by a DNA copy number evaluation and clinical validation of the encoded protein using a tissue microarray. This study is the first to suggest that TOP2A expression may be a predictive factor for adverse outcome in MPNST.
TP53, BRCA1, and BRCA2 Tumor Suppressor Genes Are Not Commonly Mutated in Survivors of Hodgkin’s Disease With Second Primary Neoplasms
Authors: Nichols Kim E. , Heath John A. , Friedman Debra , Biegel Jaclyn A. , Ganguly Arupa , Mauch Peter , Diller Lisa 
Published: December 2003

Purpose: Despite recognition that second malignant neoplasms (SMNs) contribute significantly to mortality after the successful treatment of Hodgkin’s disease (HD), little is known about the molecular events leading to secondary tumors. Factors contributing to second cancer risk include the carcinogenic effects of ionizing radiation and chemotherapy, in combination with possible host susceptibility. To clarify whether host genetic factors contribute to secondary tumorigenesis, we performed mutational analyses of the TP53, BRCA1, and BRCA2 tumor suppressor genes in a cohort of 44 HD patients developing one or more SMN. Patients and Methods: Family cancer histories and constitutional DNA samples were obtained from 44 HD patients with SMNs identified. Using DNA-based sequencing, we evaluated the TP53 gene in all 44 patients. Nineteen female patients developing one or more secondary breast cancer were also analyzed for mutations in the BRCA1 and BRCA2 breast cancer–susceptibility genes. Results: Nineteen patients (43%) had more than one SMN, and 12 patients (27%) had a positive family history of cancer. One of 44 patients tested for TP53 harbored a novel homozygous germline abnormality. One of 19 patients tested for BRCA2 carried a previously described heterozygous inactivating mutation. We identified no germline BRCA1 mutations. Conclusion: Despite features suggestive of genetic predisposition, the TP53, BRCA1, and BRCA2 genes were not frequently mutated in this cohort of HD patients developing SMNs. Larger studies of these genes or investigations of other genes involved in cellular DNA damage response pathways may identify host genetic factors that contribute to secondary tumorigenesis.
Mass Suicide: Historical and Psychodynamic Considerations
Authors: Mancinelli Iginia , Comparelli Anna , Girardi Paolo , Tatarelli Roberto 
Published: December 2003

Mass suicide can be defined as the simultaneous suicide of all the members of a social group and is closely linked to the human dimension of existence, although the social and cultural context may vary. In fact, the term mass suicide can also be used to describe situations in which a particular population has reacted to oppression by denying all normal activities of sustenance, with the intention of bringing about a traumatic metamorphosis in a cultural context (colonization, exploitation by other populations), thus transforming a catastrophe in which a passive role is played into one constructed actively.Therefore, mass suicides can be subdivided into two categories: (a) hetero‐induced, typical of defeated and colonized populations forced to escape from a reality that does not acknowledge their human dignity and (b) self‐induced, in which the motivation is related to a distorted evaluation of reality, without there being either an intolerable situation or a real risk of death. The mass suicides that have taken place in the last 20 years are all related to the establishment of religious sects; the mystic delirium created within the sect leads to the self‐destruction of the group as being interpreted as an act of self‐assertiveness.
Alkaline Degradation of Dissolved Organic Matter
Authors: Brinkmann Thomas , Abbt‐Braun Gudrun , Frimmel Fritz H. 
Published: December 2003

AbstractThe degradation of dissolved organic matter (DOM) was studied in alkaline solution. The products were characterised using UV/vis spectroscopy, size‐exclusion chromatography (SEC), and by the analysis of low‐molecular‐weight organic acids (LMWOA). The degradation experiments were performed with water from a brown water lake or its isolated fulvic acid fraction and sodium hydroxide at different reaction times and temperatures. Depending on the wavelength and the reaction time, the UV/vis absorbance between 230 nm and 600 nm increased or decreased. The behaviour of model compounds during reactions in alkaline media was compared to the UV/vis spectroscopic behaviour of DOM. The release of LMWOA was described by kinetic data and compared to the data of model reactions. Evidence was given for the carboxylic esters playing a significant role in the release of LMWOA only during the beginning of the alkaline degradation. The results gained by SEC with on‐line UV and DOC detection showed that the average size of DOM was decreasing, and that a major part of the degradation products consisted of low‐molecular‐weight mono‐ and dicarboxylic acids.
Isochromosome 22 in trisomy 22 mosaic with five cell lines
Authors: Guzé Carol , Qin Naigeng , Kelly JoAnn , Yang Xiaojing , Bruni Roberta , Baker Debra , Hassan Rosetta 
Published: December 2003

AbstractThis report describes a full‐term male infant with trisomy 22 due to an isochromosome 22. Prenatal diagnosis with amniotic fluid showed two cell lines, one with an isochromosome 22 and the other with a deleted isochromosome 22. Subsequent cytogenetic analyses of cord blood, umbilical cord tissue, and placenta revealed additional cell lines. A normal cell line was found in umbilical cord tissue and two of three placental sites. The newborn had numerous dysmorphic features and died within 48 hrs of birth. © 2003 Wiley‐Liss, Inc.
Infantile spinal muscular atrophy with respiratory distress type 1 (SMARD1)
Authors: Grohmann Katja , Varon Raymonda , Stolz Piroschka , Schuelke Markus , Janetzki Catrin , Bertini Enrico , Bushby Kate , Muntoni Francesco , Ouvrier Robert , Van Maldergem Lionel , Goemans Nathalie M. L. A. , Lochmüller Hanns , Eichholz Stephan , Adams Coleen , Bosch Friedrich , Grattan‐Smith Padraic , Navarro Carmen , Neitzel Heidemarie , Polster Tilman , Topaloğlu Haluk , Steglich Christina , Guenther Ulf P. , Zerres Klaus , Rudnik‐Schöneborn Sabine , Hübner Christoph 
Published: December 2003

AbstractAutosomal recessive spinal muscular atrophy with respiratory distress type 1 (SMARD1) is the second anterior horn cell disease in infants in which the genetic defect has been defined. SMARD1 results from mutations in the gene encoding the immunoglobulin μ‐binding protein 2 (IGHMBP2) on chromosome 11q13. Our aim was to review the clinical features of 29 infants affected with SMARD1 and report on 26 novel IGHMBP2 mutations. Intrauterine growth retardation, weak cry, and foot deformities were the earliest symptoms of SMARD1. Most patients presented at the age of 1 to 6 months with respiratory distress due to diaphragmatic paralysis and progressive muscle weakness with predominantly distal lower limb muscle involvement. Sensory and autonomic nerves are also affected. Because of the poor prognosis, there is a demand for prenatal diagnosis, and clear diagnostic criteria for infantile SMARD1 are needed. The diagnosis of SMARD1 should be considered in infants with non‐5q spinal muscular atrophy, neuropathy, and muscle weakness and/or respiratory distress of unclear cause. Furthermore, consanguineous parents of a child with sudden infant death syndrome should be examined for IGHMBP2 mutations.
Febrile seizures impair memory and cAMP response‐element binding protein activation
Authors: Chang Ying‐Chao , Huang A‐Min , Kuo Yu‐Min , Wang Shan‐Tair , Chang Yung‐Yee , Huang Chao‐Ching 
Published: December 2003

AbstractThe long‐term effects of brief but repetitive febrile seizures (FS) on memory have not been as thoroughly investigated as the impact of single and prolonged seizure in the developing brain. Using a heated‐air FS paradigm, we subjected male rat pups to one, three, or nine episodes of brief FS on days 10 to 12 postpartum. Neither hippocampal neuronal damage nor apoptosis was noted within 72 hours after FS, nor was there significant hippocampal neuronal loss, aberrant mossy fiber sprouting, or altered seizure threshold to pentylenetetrazol in any FS group at adulthood. The adult rats subjected to nine episodes of early‐life FS, however, showed long‐term memory deficits as assessed by the Morris water maze. They also exhibited impaired intermediate and long‐term memory but spared short‐term memory in the inhibitory avoidance task. Three hours after inhibitory avoidance training, phosphorylation of cAMP response‐element binding (CREB) protein in the hippocampus was significantly lower in nine‐FS‐group rats than in controls. Furthermore, rolipram administration, which activated the cAMP–CREB signaling pathway by inhibiting phosphodiesterase type IV, reversed the long‐term memory deficits in nine‐FS‐group rats by enhancing hippocampal CREB phosphorylation. These results raise concerns about the long‐term cognitive consequences of even brief frequently repetitive FS during early brain development. Ann Neurol 2003;54:706–718
Differential scanning calorimetry investigation of formation of poly(ethylene glycol) hydrate with controlled freeze–thawing of aqueous protein solution
Authors: Hillgren Anna , Aldén Maggie 
Published: December 2003

AbstractThe formation of poly(ethylene glycol) (PEG) hydrate during freeze–thawing of dilute lactate dehydrogenase solutions with the addition of 0.05–160 mg/mL PEG 6000 is investigated by differential scanning calorimetry and modulated temperature differential scanning calorimetry. The freeze–thawing process is performed with a controlled temperature history. A moderate cooling rate to a low freezing temperature in combination with a low heating rate seems to create the most stable PEG hydrate. The maximum amount and the most stable hydrate phase are obtained when the freezing temperature is at or below −60°C. The enthalpy of melting for the hydrate at −15°C is dependent on the heating rate but not on the cooling rate if the freezing temperature is −60°C. The effect of the addition of reduced form nicotinamide adenine dinucleotide to the PEG and protein solution indicates that competing interactions with the protein can increase the stability of the PEG hydrate. The amount of bound water in the PEG hydrate can be calculated directly from the melting enthalpy of the hydrate if an adequate temperature history is used. For solutions with >10 mg/mL PEG there are 1.7–2.7 water molecules bound per PEG unit. The PEG protection of the protein at freeze–thawing can be an effect of the amount of available PEG hydrate in relation to the amount of ice surface. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 91: 1626–1634, 2004
Eucalyptus tar pitch pretreatment for carbon material processing
Authors: Prauchner Marcos J. , Pasa Vânya M. D. , Otani Choyu , Otani Satika , de Menezes Sonia M. C. 
Published: December 2003

AbstractEucalyptus tar pitches are generated on a large scale in Brazil as byproducts of the charcoal manufacturing industry. They have a macromolecular structure consisting mainly of phenolic, guaiacylic, and siringylic units substituted and interlinked by aliphatic side chains rich in oxygenated polar groups (such as alcohol, ether, carbonyl, and carboxyl), similar to lignin. Under treatment around 250°C, the pitches undergo polymerization. C—O links are broken up, and, while side chains are released, the heavier fragments rich in aromatic rings react to form larger molecules. The presence of AlCl3 and CH2O makes polymerization at lower temperatures (150°C and 100°C, respectively) possible. AlCl3 acts as a Lewis acid, complexing to oxygen atoms and weakening C—O bonds. In turn, CH2O acts as a polymerizing agent, as in the preparation of resol phenolic resins. These pretreatments have made it possible to adjust the softening point in order to yield spinnable pitches susceptible to post spinning thermal stabilization, besides yielding pitches with improved thermal stability. These possibilities are important because they make Eucalyptus tar pitches potential carbon fiber precursors. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 91: 1604–1611, 2004
Preparation and characterization by radiation of hydrogels of PVA and PVP containing Aloe vera
Authors: Park Kyoung Ran , Nho Young Chang 
Published: December 2003

AbstractIn these studies, hydrogels for wound dressing were made from a mixture of Aloe vera, poly(vinyl alcohol) (PVA) and poly(N‐vinylpyrrolidone) (PVP) by freeze‐thaw, gamma‐ray irradiation, or a two‐step process of freeze‐thaw and gamma‐ray irradiation. Physical properties, such as gelation, water absorptivity, gel strength and degree of water evaporation were examined to evaluate the applicability of these hydrogels to wound dressing. The PVA:PVP ratio was 6:4, and the dry weight of Aloe vera was in the range of 0.4‐1.2 wt %. The solid concentration of PVA/PVP/Aloe vera solution was 15 wt %. Mixtures of PVA/PVP/Aloe vera were exposed to gamma irradiation doses of 25, 35 and 50 kGy to evaluate the effect of irradiation dose on the physical properties of the hydrogels. Gel content and gel strength increased as the concentration of Aloe vera in PVA/PVP/Aloe vera decreased and as irradiation dose increased and freeze‐thaw was repeated. Swelling degree was inversely proportional to gel content and gel strength. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 91: 1612–1618, 2004
Mechanical, surface, and thermal properties of polyamideimide–polydimethylsiloxane nanocomposites fabricated by sol–gel process
Authors: Park Young‐Wook , Lee Dong‐Sung , Kim Sang‐Hyun 
Published: December 2003

AbstractPolyamideimide (PAI)–epoxysilane (coupling agent) composites were reacted with oligomeric polydimethylsiloxane (PDMS), a condensation product of difunctional silane, by using the sol–gel process, and were then dried into films. After this procedure, the surface, mechanical, and thermal properties were measured. The study showed that PDMS existed in the PAI matrix by the use of FTIR. With respect to mechanical properties, the maximum elongation and toughness were increased in the PAI with silane groups, although the maximum tensile strength was slightly decreased. In this experiment, PAI–30 wt % epoxysilane composite had the best mechanical properties. The intensive dispersion of the silane groups on the surface of PAI was confirmed through XPS measurement. As a function of the siloxane contents, the TGA curve shows less thermal stability in terms of their initial weight loss. However, in an oxygen atmosphere at about 700°C, the series of PAI–siloxane composites indicated a significant increase in char concentration. In the end, PAI with a relevant amount of silane groups was improved in both toughness and surface properties. This experiment showed that PDMS added to PAI had better properties than those of classical materials. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 91: 1774–1783, 2004
Gel swelling properties of alginate fibers
Authors: Qin Yimin 
Published: December 2003

AbstractThis article described a study on the gel‐forming abilities of a number of different types of alginate fibers. The gelling abilities were assessed through the measurement on the release of calcium ions from the fibers and the absorption capacities in water and 0.9% saline solution. The effects of the chemical compositions of the alginate and the calcium and sodium contents of the fibers on the gelling abilities were studied. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 91: 1641–1645, 2004
Kinetics of microwave‐assisted polymerization of ϵ‐caprolactone
Authors: Sivalingam G. , Agarwal Nitin , Madras Giridhar 
Published: December 2003

AbstractThe kinetics of polymerization of ϵ‐caprolactone (CL) in bulk was studied by irradiating with microwave of 350 W and frequency of 2.45 GHz with different cycle‐heating periods (30–50 s). The molecular weight distributions were determined as a function of reaction time by gel permeation chromatography. Because the temperature of the system continuously varied with reaction time, a model based on continuous distribution kinetics with time/temperature‐dependent rate coefficients was proposed. To quantify the effect of microwave on polymerization, experiments were conducted under thermal heating. The polymerization was also investigated with thermal and microwave heating in the presence of zinc catalyst. The activation energies determined from temperature‐dependent rate coefficients for pure thermal heating, thermally aided catalytic polymerization, and microwave‐aided catalytic polymerization were 24.3, 13.4, and 5.7 kcal/mol, respectively. This indicates that microwaves increase the polymerization rate by lowering the activation energy. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 91: 1450–1456, 2004
Ultrasonic devulcanization of carbon black–filled ethylene propylene diene monomer rubber
Authors: Yun J. , Yashin V. V. , Isayev A. I. 
Published: December 2003

AbstractThe recycling of carbon black (CB)–filled ethylene propylene diene monomer (EPDM) rubber was carried out by using a newly built continuous ultrasonic grooved‐barrel reactor. Using this reactor, it was possible to increase by two times the output of devulcanized rubber in comparison with the coaxial reactor built earlier. The dynamic viscoelastic properties of virgin vulcanizates, devulcanized, and revulcanized EPDM rubbers were measured. The cure behavior of virgin and devulcanized rubbers was measured. The gel fraction and crosslink density of virgin vulcanizate and devulcanized EPDM rubbers, and the mechanical properties of virgin vulcanizates and revulcanized rubbers were determined. Similar to our previous findings using the coaxial reactor, the revulcanizates of unfilled EPDM rubbers of the present study showed higher mechanical properties than those of the virgin unfilled vulcanizates. However, a progressive decrease in the mechanical properties of CB‐filled EPDM revulcanizates with an increase of filler concentration, attributed to partial deactivation of filler, was observed. An improvement of mechanical properties of vulcanizates was achieved upon blending the devulcanized and virgin filled rubbers. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 91: 1646–1656, 2004