bluuebunny/crossref_metadata_2025_split huggingface dataset:
Non‐linkage of familial rhabdoid tumors to SMARCB1 implies a second locus for the rhabdoid tumor predisposition syndrome
Authors: Frühwald Michael C. , Hasselblatt Martin , Wirth Sebastian , Köhler Gabriele , Schneppenheim Reinhard , Subero Jose Igancio Martin , Siebert Reiner , Kordes Uwe , Jürgens Heribert , Vormoor Josef 
Published: October 2005

AbstractBackgroundRhabdoid tumors represent an independent entity among embryonal neoplasms. These tumors affect the kidney (RTK, rhabdoid tumor of kidney) and central nervous system (AT/RT, atypical teratoid, rhabdoid tumor), but may also be found in peripheral soft tissue. Unifying features include immunohistochemical characteristics and inactivation of the putative tumor suppressor gene SMARCB1 (hSNF5/INI1) in chromosome 22q11.2. Several familial cases have been published and summarized under the term rhabdoid tumor predisposition syndrome. In all of the published familial cases, inactivation of SMARCB1 was detected in tumor tissues.Procedure and ResultsWe report on a family with three children, two of which were affected by rhabdoid tumors, one RTK, the other an AT/RT. While both children demonstrated typical morphological and clinical features neither the RTK nor the AT/RT showed evidence for inactivation of SMARCB1 in molecular studies including CGH and array CGH, FISH, gene dosage analysis by dHPLC, and DNA‐sequencing. Immunohistochemistry for SMARCB1 showed normal expression within the nuclei of tumor cells. Furthermore, both children inherited different paternal and maternal SMARCB1 alleles evidenced by haplotype analysis. Conventional cytogenetic, FISH, and mutation analyses lacked evidence for SMARCB1 aberrations or gross chromosomal changes in the parents.ConclusionsWe thus demonstrate a family with rhabdoid tumor predisposition syndrome without linkage to SMARCB1. This finding indicates that other loci than SMARCB1 below the resolution of array CGH are involved in the origin of these tumors. Our data impact on the clinical counseling of affected families and warrant further studies in the molecular biology of these enigmatic tumors. Pediatr Blood Cancer 2006;47:273–278. © 2005 Wiley‐Liss, Inc.
T‐cell acute lymphoblastic leukemia relapsing as acute myelogenous leukemia
Authors: Mantadakis Elpis , Danilatou Vassiliki , Stiakaki Eftichia , Paterakis George , Papadhimitriou Stefanos , Kalmanti Maria 
Published: October 2005

AbstractWe present the unusual case of a 16‐year‐old girl with T‐cell acute lymphoblastic leukemia (ALL) with an early thymocyte immunophenotype without myeloid markers, who after 13 months of complete hematological remission relapsed as acute myelogenous leukemia (AML) with minimal differentiation and died of her disease. Whether the AML represented a relapse with lineage switch of the original immature T‐cell clone or a new secondary malignancy, could not be proven due to the absence of molecular or clonal markers. This report suggests that a subset of CD7+ T‐cell leukemias without mature T‐cell antigens (CD4‐, CD8‐) are minimally differentiated and can relapse as AML. Pediatr Blood Cancer 2007;48:354–357. © 2005 Wiley‐Liss, Inc.
Isolated pulmonary Langerhans cell histiocytosis presenting with recurrent pneumothorax
Authors: Braier Jorge , Latella Antonio , Balancini Blanca , Castaños Claudio , Goldberg Julio 
Published: October 2005

AbstractWe describe the outcome of a 20‐month‐old female and a 6‐year‐old male, both of whom had acutely developed severe respiratory distress with tachypnea, cyanosis and, in Patient 2, thoracic pain. Chest X‐ray and CT scan showed interstitial pulmonary involvement and a bullous process with bilateral pneumothoraces for both children. Pulmonary biopsy confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Laboratory testing and skeletal radiography did not reveal any other involvement of LCH. The patients received chemotherapy (prednisone, vinblastine, 6‐mercaptopurine). They had recurrent episodes of pneumothorax during follow‐up and placement of chest tubes was the treatment chosen. They were asymptomatic, with regression of bullae and disappearance of pneumothorax at 58 and 63 months of follow‐up, respectively. Pulmonary function tests done during follow‐up were normal in both patients. Despite severe pulmonary involvement, conservative surgical treatment and moderate chemotherapy produced good results in these two rare cases. Pediatr Blood Cancer 2007;48:241–244. © 2006 Wiley‐Liss, Inc.
CGH evaluation of two de novo synchronous tumors in a child with a germline p53 mutation
Authors: Roque Lúcia , Lacerda Ana , Rodrigues Raquel , Almeida Odete , Salgueiro João , Pinto Carla 
Published: October 2005

AbstractWe report the case of a child who developed two de novo synchronous tumors: an osteosarcoma and an embryonal rhabdomyosarcoma. The patient was determined to be a de novo carrier of a P53 germline mutation. Comparative genomic hybridization (CGH) analysis revealed that each of the neoplasms was characterized by a specific set of chromosomal imbalances and high‐level amplification (HLA) regions. Our CGH findings provide evidence that cancer development is a cellular/organ specific event. Pediatr Blood Cancer © 2005 Wiley‐Liss, Inc.
The impact of monitoring Epstein–Barr virus PCR in paediatric bone marrow transplant patients: Can it successfully predict outcome and guide intervention?
Authors: Greenfield Hayley M. , Gharib Maged I. , Turner Andrew J.L. , Guiver Malcolm , Carr Trevor , Will Andrew M. , Wynn Robert F. 
Published: October 2005

AbstractBackgroundEpstein–Barr virus (EBV) associated lymphoproliferative disease is a complication of haemopoietic stem cell transplantation (HSCT). In certain groups (unrelated and mismatched donor transplants, T‐cell depleted) the risk may be as high as 25% with significant morbidity and mortality. Strategies to predict the impending development of this disorder and allow early intervention have therefore assumed importance. We routinely screen the peripheral blood of all recipients of allogeneic HSCT to detect EBV DNA by quantitative polymerase chain reaction (PCR) technology and report here how this correlates with clinical disease and management.ProcedureData on 28 successive patients who underwent HSCT at our institution were reviewed. The relationship between EBV reactivation demonstrated by quantitative PCR and development of post transplant lymphoproliferative disease (PTLD) was determined.ResultsEBV reactivation occurred in 68% of patients, however only 7% developed clinical PTLD. Patients with high level reactivation (n = 9) had more frequent episodes of reactivation and all patients who progressed to overt PTLD were found in this group. In contrast none of those patients with low level reactivation (n = 10) or persistently negative results (n = 9) showed any signs of clinical disease. Anti‐CD20 monoclonal antibody (Rituximab) therapy was instigated in both cases of proven PTLD and three cases of high level reactivation with successful outcomes. Response to treatment was associated with a prompt decline in viral copy number.ConclusionsOur results indicate that EBV reactivation is a common occurrence in the paediatric allogeneic transplant setting and that only a proportion of patients will progress to PTLD. Frequent monitoring may help to predict those at highest risk and guide intervention. Pediatr Blood Cancer © 2005 Wiley‐Liss, Inc.
How well does the Canadian haemophilia Outcomes‐Kids' Life Assessment Tool (CHO‐KLAT) measure the quality of life of boys with haemophilia?
Authors: Young Nancy L. , Bradley Catharine S. , Wakefield Cindy D. , Barnard Dorothy , Blanchette Victor S. , McCusker Patricia J. 
Published: October 2005

AbstractBackgroundIt is important to measure the quality of life (QoL) of boys with haemophilia, because the diagnosis has a significant impact on their lives and this impact fluctuates over time. A disease‐specific measure of QoL is required because the aspects of life that are affected by haemophilia may differ from those assessed by generic QoL measures. This paper describes the final phase of development of a disease‐specific measure of QoL for boys with haemophilia: the Canadian Haemophilia Outcomes‐Kids Life Assessment Tool (CHO‐KLAT).ProcedureA 79‐item version of the CHO‐KLAT was administered to 52 children. A detailed item analysis was conducted to shorten the CHO‐KLAT. The reliability of the revised version was assessed using intraclass correlation coefficients. Validity was assessed by comparing it to the PedsQL and the HaemoQoL.ResultsThe item analysis resulted in the retention of 35 strongly performing items (CHO‐KLAT35). These items were aggregated into the CHO‐KLAT35 summary score. Repeated measures reliability of the CHO‐KLAT35 was 0.74 for children and 0.83 for parents, and the child–parent concordance was 0.75. The validity of the CHO‐KLAT35 was confirmed by a correlation of 0.78 with the Haemo‐QoL and of 0.59 with the PedsQL.ConclusionsThe CHO‐KLAT35 is a reliable and valid measure of QoL for boys with haemophilia. Pediatr Blood Cancer 2006;47:305–311. © 2005 Wiley‐Liss, Inc.
Gefitinib is effective against juvenile pilocytic astrocytoma in vitro
Authors: Foreman Nicholas K. , Gore Lia , Wells Daniel , Straessle Jennifer , Heideman Richard , Donson Andrew M. 
Published: October 2005

AbstractBackgroundJuvenile pilocytic astrocytomas (JPAs) are the most common central nervous system tumors in children. If completely resected, JPAs are associated with an excellent outcome. However, there is need for additional therapeutic approaches for those JPAs which are incompletely resected and fail subsequent standard chemotherapy/radiation. To explore the possibility for a novel therapeutic approach we measured the effect of the epidermal growth factor receptor (EGFR) small molecule tyrosine kinase inhibitor gefitinib on five JPA primary cell‐cultures.ProcedureDue to a lack of established cell‐lines of JPA very few in vitro drug sensitivity assays have been performed. In this study we have succeeded in propagating short‐term primary cell‐cultures established from surgical specimens. The effect of gefitinib on proliferation in JPA derived primary cell‐cultures was measured by a standard tritiated thymidine incorporation assay. The level of expression of EGFR, the intended target of gefitinib, was measured by immunohistochemistry, flow cytometry and RT‐PCR.ResultsGefitinib was shown to inhibit proliferation in all five JPA cell‐cultures tested, with IC‐50's between 1.6 and 9.6 µM. However, EGFR protein and mRNA expression was undetectable. Further studies with cetuximab, an EGFR‐specific inhibitory monoclonal antibody, showed no effect on proliferation in JPA.ConclusionsBased on these preclinical data, gefitinib may be a suitable salvage chemotherapy drug to explore further in those patients with JPA who have recurred after primary chemotherapy. Of interest, it appears that the anti‐tumor effect of gefitinib in JPA cell‐cultures may be mediated through a pathway other than EGFR inhibition. Pediatr Blood Cancer 2006;47:293–298. © 2005 Wiley‐Liss, Inc.
Treatment of infantile choriocarcinoma of the liver
Authors: Yoon Janet M. , Burns R. Cartland , Malogolowkin Marcio H. , Mascarenhas Leo 
Published: October 2005

AbstractPrimary choriocarcinoma of the liver is an extremely rare childhood malignancy frequently associated with clinical instability at initial presentation. It often mimics other benign and malignant childhood liver tumors. Prompt diagnosis and initiation of treatment are necessary to attain a successful outcome. We describe a critically ill infant with metastatic choriocarcinoma whose diagnosis was based on radiographic and tumor marker findings, without an initial biopsy, and her successful management with neo‐adjuvant chemotherapy and delayed surgery. She is currently in continuous remission 24 months from diagnosis. Pediatr Blood Cancer 2007;49:99–102. © 2005 Wiley‐Liss, Inc.
Ovarian germ cell tumors in children: A clinical study of 66 patients
Authors: De Backer Antoine , Madern Gerard C. , Oosterhuis J. Wolter , Hakvoort‐Cammel Friederike G.A.J. , Hazebroek Frans W.J. 
Published: October 2005

AbstractBackgroundOvarian germ cell tumors are rare in childhood. The aim of this study is to review clinical presentation, management, and outcome in a two‐center series of girls with ovarian germ cell tumor.ProcedureThe records of 66 patients (median age 9 years) with histologically proven ovarian germ cell tumor (either benign or malignant), treated over a 44‐year‐span, were reviewed.ResultsPain and an abdominal mass were the most frequent symptoms. The tumors were right‐sided in 35, left‐sided in 28, and bilateral in 3. Most patients (52) were stage I, 4 were stage II, 6 stage III, and 1, with liver metastases, stage IV. Sixteen patients had an emergency operation for tumor torsion. Unilateral salpingo‐oophorectomy was the most frequently performed procedure (n = 46), and ovarian‐sparing tumorectomy was performed in 9 patients (one bilaterally). Histologically, teratomas were found most frequently (mature: 45, immature: 9), followed by mixed tumors (n = 7), yolk sac tumors (n = 3), dysgerminoma (n = 2), gonadoblastoma (n = 2), and embryonal carcinoma (n = 1). Surgical removal of the tumor with or without the ovary and/or adnex was the sole treatment in 55 patients, chemotherapy was administered in 10 and radiotherapy + chemotherapy in one. Intra‐operative spillage of tumoral fluid occurred in six; this did not influence outcome in five. Recurrence was observed in three patients. Two patients, with malignant disease, died. The 64 survivors are now between 8 months and 44 years after treatment.ConclusionsWith a recurrence rate of 4.5% and a mortality rate of 3%, this series confirms the excellent prognosis for girls with ovarian germ cell tumor (GCT). Pediatr Blood Cancer 2006, 46:459–464. © 2005 Wiley‐Liss, Inc.
Vinylic and ring‐opening metathesis polymerization of norbornene with bis(β‐ketoamine) cobalt complexes
Authors: Bao Feng , Lü Xing‐Qiang , Gao Haiyang , Gui Guoqiu , Wu Qing 
Published: October 2005

AbstractCobalt complexes 1–4 bearing N,O‐chelate ligands based on condensation products of 1‐phenyl‐3‐methyl‐4‐benzoyl‐5‐pyrazolone with aniline, o‐methylaniline, α‐naphthylamine, and p‐nitroaniline, respectively, were synthesized, and the structures of 1 and 4 were characterized by single‐crystal X‐ray diffraction analyses. The bis(β‐ketoamine) cobalt complexes could act as moderately active catalyst precursors for norbornene polymerization with the activation of methylaluminoxane. This catalytic reaction proceeded mainly through a vinyl‐type polymerization mechanism. 1H NMR and IR showed that in all cases, a small amount of double bonds raised from ring‐opening metathesis polymerization (ROMP) was present in the polymerization products. The variation of the polymerization conditions affected the ROMP unit ratio in the polynorbornenes. © 2005 Wiley Periodicals, Inc. J Polym Sci Part A: Polym Chem 43: 5535–5544, 2005
Structural/compositional nanoheterogeneity and glass‐transition plurality in amorphous polycyanurate–poly(tetramethylene glycol) hybrid networks
Authors: Bershtein V. A. , David L. , Egorov V. M. , Fainleib A. M. , Grigorieva O. , Bey I. , Yakushev P. N. 
Published: October 2005

AbstractThe nanostructure and dynamics over the temperature range of −140 to 300 °C were studied in a series of polycyanurate (PCN)–poly(tetramethylene glycol) (PTMG) hybrid networks by wide‐angle X‐ray diffraction and small‐angle X‐ray scattering, with a synchrotron radiation setup, and by differential scanning calorimetry (DSC) and laser‐interferometric creep rate spectroscopy (CRS) techniques, respectively. The networks were synthesized from the dicyanate ester of bisphenol A and hydroxyl‐terminated PTMG with a number‐average molecular weight of 1.000 g/mol; the PTMG content varied from 0 to 40 wt %, and the degree of its chemical incorporation into the PCN network changed from 78.8 to 97%. The noncrystalline structure and considerable structural nanoheterogeneity of the hybrid networks were shown. CRS/DSC analysis revealed a complicated dynamic behavior, that is, a wide dispersion of glass transitions in the hybrid networks due to the presence of nanodomains with different degrees of rigid crosslinking (i.e., compositional nanoheterogeneity). Besides the physical significance, the plurality of glass transitions found in the PCN–PTMG hybrid networks was also of practical interest because it resulted in increasing mechanical strength of the brittle PCN network due to microplasticity arising at room temperature and moderate temperatures and the retention of some rigidity and creep resistance at temperatures much higher than the basic glass‐transition temperature. © 2005 Wiley Periodicals, Inc. J Polym Sci Part B: Polym Phys 43: 3261–3272, 2005
Use of symbolic algebra in the calculation of hyperspherical harmonics
Authors: Wang Desheng , Kuppermann Aron 
Published: October 2005

AbstractA symbolic algebra method for determining democratic hyperspherical harmonics (HH) for three‐particle and four‐particle systems is given. It is based on a recursion relation that, starting from a complete set of such harmonics for a given value n of the system's grand‐canonical angular momentum quantum number, determines a similarly complete set for n + 1. Using this method, all 2.3 million HH for n up to 40 for the three‐particle case and all 43.8 million for n up to 30 for the four particle case were determined. The method, details, and results of its implementation are described. © 2005 Wiley Periodicals, Inc. Int J Quantum Chem, 2006
Complexes self‐associate by hydrogen bonding and metallophilic attraction: Theoretical study
Authors: Mendizabal Fernando , Reyes Dana , Olea‐Azar Claudio 
Published: October 2005

AbstractHydrogen bonding and metallophilic attractions are studied in the model systems: [(AuNH3Cl)2], [(AuNH(CH3)2Cl)2], [{Au2(μ‐SH)(PH2O)(PH2OH)}2], [(CuNH3Cl)2], and [{Cu(NH3)Cl}4] at the Hartree–Fock (HF) and second‐order Møller–Plesset (MP2) levels. The two interactions are found to be comparable and prevailing in the final structure. It is determined that the aurophilic contact has a same magnitude that the hydrogen bonding, and is stronger than the cuprophilic interaction. The presence of hydrogen bond directs the growth of the crystal. © 2005 Wiley Periodicals, Inc. Int J Quantum Chem, 2006
Tetraalkylammonium halides as chemical standards for positive electrospray ionization with ion mobility spectrometry/mass spectrometry
Authors: Viidanoja Jyrki , Sysoev Alexey , Adamov Alexey , Kotiaho Tapio 
Published: October 2005

AbstractChemical standards for positive ion mode electrospray ionization ion mobility spectrometry/mass spectrometry (ESI(+)‐IMS/MS) are suggested. The low clustering tendency of tetraalkylammonium halides makes them ideal chemical standards for ESI(+)‐IMS/MS. A homologous series of these compounds forms a useful external standard for instrument testing and resolution calibration of an IMS instrument. Selected homologues or a mixture of tetraalkylammonium halides can be used as mobility standards in the analytical runs. Absolute and relative reduced mobilities were calculated for C2C8, C10 and C12 tetraalkylammonium halides. Absolute reduced mobilities in nitrogen were 1.88, 1.56, 1.33, 1.15, 1.02, 0.92, 0.84, 0.73, and 0.67 cm2 V−1 s−1, for C2C8, C10 and C12 tetraalkylammonium halides, respectively. Relative reduced mobilities (relative to 2,6‐di‐tert‐butylpyridine) for the same compounds were 1.20, 1.00, 0.855, 0.743, 0.658, 0.59, 0.54, 0.47, and 0.43, respectively. Copyright © 2005 John Wiley & Sons, Ltd.
Simultaneous determination of carisoprodol and meprobamate in human hair using solid‐phase extraction and gas chromatography/mass spectrometry of the trimethylsilyl derivatives
Authors: Kim Jin Young , In Moon Kyo , Paeng Ki‐Jung , Chung Bong Chul 
Published: October 2005

AbstractCarisoprodol (CSP) is a musculoskeletal relaxant whose active metabolite is meprobamate (MPB). This drug has recently been noticed to be abused as an inexpensive alternative to illicit drugs in Korea. A method using solid‐phase extraction (SPE) and gas chromatography/mass spectrometry (GC/MS) was developed for the determination of CSP and MPB in human hair. Hair samples (30 mg) were washed with distilled water and acetone, cut into small fragments (<1 mm), incubated in 1.0 M HCl overnight at 50°C, and then adjusted to pH 6.5. The drugs were extracted from the resulting hydrolyzed solutions using a SPE column. The eluents were evaporated to dryness, then derivatized using N,O‐bis(trimethylsilyl)trifluoroacetamide (BSTFA) with 1% trimethylchlorosilane (TMCS) at 120°C for 30 min. The derivatized extract (1 µL) was injected into the GC/MS system. Recoveries were in the range of 91.5–93.1% for CSP and 85.5–93.0% for MPB. The linear ranges were 0.5–10.0 ng/mg for both CSP and MPB with good correlation coefficients (r2 = 0.995). The intra‐day precision and accuracy ranged from 1.5 to 9.3% and −17.5 to 3.6%, respectively, and the inter‐day precision and accuracy ranged from 3.9 to 6.2% and −15.0 to −3.9%, respectively. The limits of detection for CSP and MPB were 0.13 and 0.12 ng/mg, respectively. The applicability of the method was proven by analyzing a hair sample from an authentic abuser. Copyright © 2005 John Wiley & Sons, Ltd.
Binding characteristics of the 5‐HT2A receptor antagonists altanserin and MDL 100907
Authors: Kristiansen Heidi , Elfving Betina , Plenge Per , Pinborg Lars H. , Gillings Nic , Knudsen Gitte M. 
Published: October 2005

AbstractTo study the 5‐HT2A receptors in the living human brain, using positron emission tomography (PET), two selective radiotracers are currently in use: [18F]altanserin and [11C]MDL 100907. It is, however, currently unknown to what extent data obtained with either tracer are directly comparable. The aim of this study was to compare binding characteristics of these two radiotracers in rat brain with respect to affinity (Kd), receptor binding density (Bmax), binding potential (BP), and nonspecific binding. Further, binding kinetics, sensitivity towards competition with the endogenous transmitter serotonin, and the competitive/noncompetitive interaction between the two radioligands were evaluated. In addition, the selectivity of [18F]altanserin for the 5‐HT2A receptor was assessed.The Kd value of [18F]altanserin and [3H]MDL 100907 was in the order of 0.3 nM. Bmax in frontal cortex was 523 and 527 fmol/mg protein, respectively. The binding of [18F]altanserin was not influenced by blocking either the 5‐HT2B/2C or the α1‐adrenergic receptors. At 37°C the association t1/2 was 2.8 and 2.7 min and the dissociation t1/2 was 11 and 13.5 min for [18F]altanserin and [3H]MDL 100907, respectively.Both radioligands were displaced by 5‐HT, only at high concentrations; the Ki value of 5‐HT ranging between 650 and 3,300 nM. This indicates that binding of both radioligands in PET studies is not directly influenced by changes in endogenous 5‐HT.Overall, the binding of [18F]altanserin and [3H]MDL 100907 to the 5‐HT2A receptor was very comparable, showing selective high affinity binding in the subnanomolar range. Synapse 58:249–257, 2005. © 2005 Wiley‐Liss, Inc.