bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treatment of 72 newly diagnosed Waldenström macroglobulinemia cases with oral melphalan, cyclophosphamide, and prednisone
Authors: Annibali Ombretta , Petrucci Maria Teresa , Martini Vincenza , Tirindelli Maria Cristina , Levi Anna , Fossati Carolina , Bianco Patrizia Del , Mandelli Franco , Foa Robin , Avvisati Giuseppe 
Published: December 2004

AbstractBACKGROUNDCurrent treatment regimens for Waldenström macroglobulinemia (WM) are based on the use of oral alkylating agents. Recently, however, other more costly agents have been proposed for the treatment of WM. In the current study, the authors report on results obtained using oral melphalan, cyclophosphamide, and prednisone (MCP) to treat 72 patients with WM, and they compare these results (and the associated costs) with those observed using more aggressive protocols.METHODSBetween July 1973 and April 2002, the authors documented overexpression of the immunoglobulin M paraprotein in 317 consecutive patients. Of these, 100 had newly diagnosed WM, and the 72 who were symptomatic were treated using the MCP protocol. Response rate, overall survival (OS), response duration, freedom from progression (FFP), event‐free survival (EFS) duration, toxicity, and cost per course in Euro and U.S. dollars were evaluated for patients receiving this regimen.RESULTSSeventy‐one of 72 patients (99%) were evaluable. Of these patients, 55 (77%) achieved a response; 7 others (10%) experienced disease stabilization, and the remaining 9 (13%) experienced disease progression. After a median follow‐up of 72 months (range, 3–195 months), the median durations of EFS, FFP, response, and OS were 47, 55, 64, and 66 months, respectively. No World Health Organization Grade III or IV toxicities were observed, and side effects were limited to transient nausea, emesis, and mild neutropenia. The cost per course of the MCP regimen was $16, similar to that of standard protocols involving chlorambucil and much less than that of more aggressive protocols (price range, $91–11091) proposed for the treatment of WM.CONCLUSIONSLike chlorambucil‐based protocols, the MCP regimen is a cost‐effective and safe option for the treatment of patients with WM. Furthermore, the results obtained do not appear to be inferior to those yielded by more expensive, aggressive, and toxic intravenous protocols. Cancer 2005. © 2004 American Cancer Society.
Wallace's unfinished business: The “Other Man” in evolutionary theory
Authors: Smith Charles H. 
Published: December 2004

AbstractAfter a century in the shadows, Alfred Russel Wallace (1823–1913) has recently become the subject of increasing attention. It is suggested here, expanding on observations made by anthropologist Gregory Bateson some years ago, that Wallace's cybernetics‐like view of the operation of natural selection—as a governor‐like principle tending to keep species unvarying—can be expanded to a more complete evolutionary understanding by exploring in modern context Wallace's idea that “more recondite forces” are driving the process. Specifically, when the environment is regarded as a final cause (but not a deterministic force), individual adaptations may be viewed as entropy‐relaying structures (acting in response to, and as a part of, larger scale biogeochemical agenda), whereas negentropy is accumulated by nonrandomly directed organism‐ and population‐level forms of ecological engagement. Thus, range change in particular is viewed as a process that is both driven and nonrandom, and ultimately connected to the derivation of more and more organized individual, population, and community structures. © 2004 Wiley Periodicals, Inc. Complexity 10:25–32, 2004
Non–heparan sulfate‐binding interactions of endostatin/collagen XVIII in murine development
Authors: Rychkova Natalia , Stahl Sonja , Gaetzner Sabine , Felbor Ute 
Published: December 2004

AbstractKnobloch syndrome is characterized by a congenital generalized eye disease and cranial defect. Pathogenic mutations preferentially lead to a deletion or functional alteration of collagen XVIII's most C‐terminal endostatin domain. Endostatin can be released from collagen XVIII and is a potent inhibitor of angiogenesis and tumor growth. We show differential expression of binding partners for endostatin, vascular endothelial growth factor (VEGF), and the collagen XV endostatin homologue in murine embryonal development using a set of alkaline phosphatase fusion proteins. Consistent with the human phenotype, vascular mesenchyme in the developing eye was identified as endostatin's primary target. While endostatin predominantly bound to blood vessels, the VEGF164 affinity probe labeled nonvascular tissues such as forebrain, hindbrain, the optic nerve, and the surface ectoderm of the future cornea. Strikingly increased staining specificity was observed with a non–heparin/heparan sulfate‐binding endostatin probe. In contrast, elimination of the heparan sulfate binding site from VEGF led to complete loss of binding. The collagen XV endostatin homologue showed a highly restricted binding pattern. Oligomerization with endogenous endostatin was ruled out by use of collagen XVIII knockout mice. Our data provide strong evidence that collagen XVIII's C‐terminal endostatin domain harbors a prominent tissue‐binding site and that binding can occur in the absence of heparan sulfates in situ. Developmental Dynamics 232:399–407, 2005. © 2004 Wiley‐Liss, Inc.
Expression of the mouse Fgf15 gene is directly initiated by Sonic hedgehog signaling in the diencephalon and midbrain
Authors: Saitsu Hirotomo , Komada Munekazu , Suzuki Misao , Nakayama Rika , Motoyama Jun , Shiota Kohei , Ishibashi Makoto 
Published: December 2004

AbstractSonic hedgehog (Shh) is a secreted molecule that is thought to regulate tissue growth and patterning in vertebrate embryos. Although it has been reported that Gli transcription factors mediate Shh signaling to the nucleus, little is known about developmental target genes of Gli. In the previous genetic study, we showed that Shh is required for Fgf15 expression in the diencephalon and midbrain. Here, we examined whether Fgf15 is a direct target of Shh signaling through Gli. Shh was expressed in the midline cells and Fgf15 in the medial region of the diencephalon/midbrain by the seven‐somite stage. The Fgf15 expression domain coincided with that of Gli1 and overlapped with that of Gli2 at this stage. Fgf15 expression in the diencephalon/midbrain was greatly reduced in the seven‐somite Shh mutant embryos. Transgenic analysis showed that the 3.6‐kb 5′‐flanking region of the Fgf15 gene is sufficient for induction of Fgf15 in the medial/ventral diencephalon/midbrain. Luciferase assay showed that the 3.6‐kb Fgf15 enhancer/promoter was activated by Gli2. A Gli‐binding site was located 1 kb upstream of the transcription start site and was required for expression in the medial/ventral diencephalon/midbrain in transgenic embryos and for activation in luciferase assay. These findings indicate that Fgf15 is directly regulated by Shh signaling through Gli proteins. Developmental Dynamics 232:282–292, 2005. © 2004 Wiley‐Liss, Inc.
Oligonucleosides with a Nucleobase‐Including Backbone. Part 12
Authors: Eppacher Simon , Bhardwaj Punit Kumar , Bernet Bruno , Bravo Gala José Luis , Knöpfel Thomas , Vasella Andrea 
Published: December 2004

AbstractIn contradistinction to the corresponding Grignard reagent, bis[(trimethylsilyl)ethynyl]zinc reacted with the 5′‐oxoadenosine 3 diastereoselectively to the β‐D‐allo‐hept‐6‐ynofuranosyladenine 5. Lithiation/iodination of the monomeric propargyl alcohol 5 and of the dimeric propargyl alcohol 22 provided the 8‐iodoadenosines 7 and 18, respectively, considerably shortening the synthesis of the dimeric O‐silylated 8‐iodoadenosine 25. The mixed uridine‐ and adenosine‐derived tetramers 21 and 32 were synthesised. The tetramer 21 was prepared by a linear sequence. Sonogashira coupling of 9 and 13 yielded the trimer 16 that was C‐desilylated to 17. A second Sonogashira coupling of 17 and 19 yielded the tetramer 21. Tetramer 32 was prepared in higher yields by a convergent route, coupling the acetylene 29 and the iodide 30. The uridine‐derived iodides proved more reactive than the adenosine‐derived analogues, and the N6‐unprotected adenosine‐derived alkynes were more reactive than their N6‐benzoylated analogues.
Photooxygenation of Pregnanes
Authors: Ponce María A. , Erra‐Balsells Rosa , Bruttomesso Andrea C. , Gros Eduardo G. 
Published: December 2004

AbstractThe course of the singlet‐oxygen reaction with pregn‐17(20)‐enes and pregn‐5,17(20)‐dienes was studied to compare the reactivity of the two alkene moieties present in some steroid families. Thus, from commercially available (3β,5α)‐hydroxy‐androstan‐17‐one and (3β)‐3‐hydroxyandrost‐5‐en‐17‐one, the following 3‐{[(tert‐butyl)dimethylsilyl]oxy}‐substituted, 17(20)‐unsaturated pregnanes were prepared (see Fig. 1): (3β,5α)‐21‐norpregn‐17(20)‐ene 1; (3β,5α,17Z)‐pregn‐17(20)‐ene 2, (3β,5α,16α,17E)‐pregn‐17(20)‐en‐16‐ol 3, (16β,5α,17E)‐pregn‐17(20)‐en‐16‐ol 4, (3β,5α,16β,17E)‐pregn‐17(20)‐en‐16‐ol acetate 5, (3β,16α)‐21‐norpregna‐5,17(20)‐dien‐16‐ol 6, (3β,16α,17E)‐pregna‐5,17(20)‐dien‐16‐ol 7, (3β,17Z)‐pregna‐5,17(20)‐diene 8, (3β,17E)‐pregna‐5,17(20)‐dien‐21‐ol 9 and (3β,17E)‐5,17(20)‐dien‐21‐ol acetate 10. The oxygenated products (see Fig. 2) obtained from 1–10 and 1O2, generated by irradiation of Rose Bengal in 3O2‐saturated pyridine solution, were characterized by 1H‐, 13C‐NMR, and MS (EI, FAB, HR‐EI, ESI‐ and UV‐MALDI‐TOF) data. Major products were those formed by the ene reaction involving as intermediates the corresponding hydroperoxides and the cyclic tautomers of the allylic hydroperoxides, i.e., the corresponding oxiranium oxide‐like intermediate (Scheme 5).
Heteropolynuclear Sodium(I) Lead(II) Complex: Crystal and Molecular Structure of a Novel 3‐D Polymer, [(en)Pb(μ3‐ONO)2Na(μ3‐ONO)2Na(μ‐O2ClO2)Na]n
Authors: Morsali Ali , Chen Xiao‐Ming 
Published: December 2004

AbstractA novel 3D polymeric heteropolynuclear sodium(I) lead(II) complex containing different ligands, [NaPb(ClO4)(en)(NO2)2] was synthesized and characterized by elemental analysis and IR, and 1H‐, 13C‐, and 207Pb‐NMR spectroscopy. The single‐crystal X‐ray data of [NaPb(ClO4)(en)(NO2)2]n established that the complex is a three‐dimensional polymer, [(en)Pb(μ3‐ONO)2Na(μ3‐ONO)2Na(μ‐O2ClO2)Na]n. The Pb and Na atoms have four‐ and eight‐coordinate geometry, respectively. The lone pair of electrons at the PbII atom is ‘stereochemically active’.
Synthesis of Cyclohexapeptides Containing Pro and Aib Residues
Authors: Jeremic Tatjana , Linden Anthony , Heimgartner Heinz 
Published: December 2004

AbstractCyclization reactions on hexapeptides containing several α‐aminoisobutyric acid (=2‐amino‐2‐methylpropanoic acid; Aib) residues and the turn‐promoting glycine (Gly) and proline (Pro) residues were investigated. Eight linear hexapeptides were synthesized, and their cyclization was attempted with various coupling reagents. The macrolactamization step proved to be difficult since only three hexapeptides could be cyclized. Two of these latter peptides were the linear precursors of the same cyclic hexapeptide, cyclo(Aib‐Aib‐Phe‐Pro‐Aib‐Gly) (1). Surprisingly, they gave 1 in almost the same yield. Thus, 1 was obtained in 35% yield upon ring closure at the Phe/Pro site by using DEPBT as the coupling reagent, whereas the cyclization at the Aib/Phe site led to 1 in 28 and 34% yield by using PyAOP and DEPC, respectively (DEPBT=3‐[(diethoxyphosphoryl)oxy]‐1,2,3‐benzotriazin‐4(3H)‐one, PyAOP=(1H‐7‐azabenzotriazol‐1‐yloxy)tripyrrolidin‐1‐ylphosphonium hexafluorophosphate, DEPC=diethyl phosphorocyanidate). Another cyclic hexapeptide, cyclo(Aib‐Aib‐Gly‐Aib‐Pro‐Gly) (2) was prepared in 34% yield when DEPC was used in the cyclization step. The solid‐state conformation of 1 was established by X‐ray crystallography.
Efficient Coupling Reactions of Arylalkynes and Aldehydes Leading to the Synthesis of Enones
Authors: Xu Li‐Wen , Li Lyi , Xia Chun‐Gu , Zhao Pei‐Qing 
Published: December 2004

AbstractIonic liquids such as 1‐butyl‐3‐methyl‐1H‐imidazolium 4‐methylbenzenesulfonate (BmimOTs) and its 1‐ethyl analogue EmimOTs are novel effective and recyclable media for acid‐catalyzed coupling reactions of aromatic alkynes with aldehydes affording (E)‐enones (Scheme 1, Table) with simple recovery and easy workup due to the absence of volatile organic solvents. This transformation avoids the use of moisture‐sensitive and heavy‐metal Lewis acids.
Immunosuppressive ent‐Kaurene Diterpenoids from Isodon serra
Authors: Zhao Ai‐Hua , Zhang Yan , Xu Zhao‐Hui , Liu Jian‐Wen , Jia Wei 
Published: December 2004

AbstractThree new enmein‐type ent‐kaurenoids, i.e., the two pairs 1 and 2 of 20‐epimers and the (20R)‐isomer 3, besides the seven known diterpenoids 4–10, were isolated from the aerial parts of Isodon serra. Their structures were elucidated by spectroscopic techniques and X‐ray diffraction. The immunosuppressive effect for T‐lymphocytes proliferation induced by Con A in BALB/c mouse was evaluated for the isolates 1–10. They all displayed a remarkable inhibitory effect, with multi‐glycosides of Tripterygium wilfordii as positive reference substance (Table 3).
Theoretical influences on research on language development and intervention in individuals with mental retardation
Authors: Abbeduto Leonard , Boudreau Donna 
Published: December 2004

AbstractIn this article, we consider the theoretical debates and frameworks that have shaped research on language development and intervention in persons with mental retardation over the past four decades. Our starting point is the nativist theory, which has been espoused most forcefully by Chomsky. We also consider more recent alternatives to the nativist approach, including the social‐interactionist and emergentist approaches, which have been developed largely within the field of child language research. We also consider the implications for language development and intervention of the genetic syndrome‐based approach to behavioral research advocated by Dykens and others. We briefly review the impact and status of the debates spurred by the nativist approach in research on the course of language development in individuals with mental retardation. In addition, we characterize some of the achievements in language intervention that have been made possible by the debates spurred by nativism and the various alternatives to it. The evidence we consider provides support for all three alternatives to the nativist approach. Moreover, successful interventions appear to embody elements of several of these approaches as well as other theoretical approaches (e.g., behaviorism). We conclude that language intervention must be theoretically eclectic in its approach, with different strategies appropriate for teaching different features of language, at different points in development, and for children displaying different characteristics or learning histories. © 2004 Wiley‐Liss, Inc. MRDD Research Reviews 2004;10:184–192.
Elimination of Hot Cracking in Laser Beam Welding
Authors: Petzet Verena , Büskens Christof , Pesch Hans Josef , Prikhodovsky Andrey , Karkhin Victor , Ploshikhin Vasily 
Published: December 2004

AbstractHot cracking is one of the big problems in laser beam welding. By multi‐beam welding, first suggested in the 70‐s (cf. [1], [4]), hot cracking can be avoided. Hereby two additional laser beams are employed. These beams generate a compression which compensate for the critical tensile strain in the solid‐liquid region of the weld induced by the main laser beam. However, hot cracking can only be prevented if the positions, sizes, and powers of the additional laser beams are suitably chosen, i.e. are optimized. Non‐optimal values can even enhance hot cracking. Until now these quantities have been found either by trial and error or by prescribing them intuitively. In the present paper a constrained nonlinear programming problem is formulated to solve the problem of hot crack initiation by minimizing the accumulated transverse strain, i.e. the opening displacement, in the solid‐liquid region. This approach is based on the so‐called strip expansion technique, cf. [2]. (© 2004 WILEY‐VCH Verlag GmbH & Co. KGaA, Weinheim)
Profile of β‐thalassemia in eastern India and its prenatal diagnosis
Authors: Bandyopadhyay Aditi , Bandyopadhyay Sanmay , Basak Jayasri , Mondal Bama Charan , Sarkar Anjali Angelika , Majumdar Sunipa , Das Mani Kanchan , Chandra Sharmila , Mukhopadhyay Ashis , Sanghamita Mamtaj , Ghosh Kusagradhi , Dasgupta Uma B. 
Published: December 2004

AbstractObjectiveTo control the birth of thalassemic children in India.MethodsMutations present in the population of eastern India and in carrier parents seeking prenatal diagnosis were detected by the PCR‐based technique of ARMS (amplification refractory mutation system) or gap‐PCR. To screen for maternal tissue contamination in CVS, haplotypes associated with the β‐globin gene clusters were constructed using six polymorphic restriction sites. Prenatal diagnosis was accomplished by checking presence of parental mutation in the DNA from chorionic villus sampling (CVS) collected at 8 to 10 weeks' gestation by appropriate technique.ResultsSix hundred and fifty (650) unrelated β‐thalassemia chromosomes were screened for 11 common mutations to characterize the mutation distribution in this population. Starting from early 2000, 63 families from different parts of West Bengal and from surrounding areas have been offered prenatal counseling for β‐thalassemia.ConclusionThe population of this region is conscious and willing to accept prenatal diagnosis as a means of control of thalassemia. Copyright © 2004 John Wiley & Sons, Ltd.
Does nuchal translucency have a role in fetal cardiac screening?
Authors: Hyett Jon 
Published: December 2004

AbstractIncreased nuchal translucency is the strongest single marker for chromosomal abnormality. Consequently, it is currently becoming established as the foundation of most early screening programmes for Down syndrome. In the absence of chromosomal abnormality, increased nuchal translucency has been shown to be associated with other congenital anomalies including cardiac defects. Several datasets have now reported this association and here these are reviewed to assess the effectiveness of nuchal translucency measurement as a screening tool for the prenatal detection of congenital heart disease. Copyright © 2004 John Wiley & Sons, Ltd.
Counselling following a diagnosis of congenital heart disease
Authors: Allan Lindsey D. , Huggon Ian C. 
Published: December 2004

AbstractCounselling the parents following a diagnosis of fetal congenital heart disease (CHD) is as important a task for the fetal cardiologist, as the skill involved in achieving an accurate diagnosis. The counsellor will base prognosis not only on the diagnosis itself but also on the security of diagnosis, the stage in gestation and potential for change, the association with extracardiac malformations and the known results of treatment. Depending on the gestational age and legal situation the counsellor is operating in, termination of pregnancy may be one of the options to consider and one that should always be raised in discussion. Thus, the parents may be in the position of making a crucial decision concerning the management of the pregnancy on the basis of the information received, so it is vital that the counsellor is truly able to communicate with them, whatever be their level of understanding. Copyright © 2004 John Wiley & Sons, Ltd.
The influence of image magnification in first‐trimester screening for Down syndrome by fetal nuchal translucency in Asians
Authors: Hsu J. J. , Chiang C. H. , Hsieh C. C. , Hsieh T. T. 
Published: December 2004

AbstractObjectiveThe aim of this prospective study was to evaluate the impact of image magnification in the measurements of crown‐rump length (CRL) and nuchal translucency (NT) thickness for first‐trimester Down syndrome screening in Asians.MethodsUltrasound measurements of NT and CRL were performed in 561 consecutive Taiwanese unaffected fetuses and 11 cases of Down syndrome fetuses between 12 and 14 weeks of gestation. All sonographic images were measured by one qualified examiner to prospectively undergo first‐trimester NT screening for Down syndrome. Fetal CRL and NT thickness were measured on three separated images including the original image, regular image, and the magnified image.ResultsA significant mean difference (0.59 ± 4.24 mm) of CRL was found between measurements on the original and regular image (p < 0.001). There was a significant mean difference of NT thickness measurements between the regular and magnified image (0.12 ± 0.25 mm, p < 0.001). Seven out of the 11 cases (63.6%) of Down syndrome with NT thickness ≥2.5 mm was measured on three separated images. A significantly reduced incidence of NT thickness ≥2.5 mm on the magnified image was noted than those of the original and regular image measurements in unaffected cases (p < 0.001). Either using the assessing method by the 95th centile cutoff value of NT thickness or combined risk, our results could achieve observed detection rate of 63.6% measured on three separated images.ConclusionsOur data indicate that the image magnification could reduce the false‐positive rate by using a fixed cutoff value of NT thickness, but would have no influence on the results when using the assessing method either by the 95th centile cutoff value of NT thickness or the combined risk. In order to place the caliper more accurately, a magnified image should be recommended as a standard image in the measurements of the NT thickness. Copyright © 2004 John Wiley & Sons, Ltd.
Cytomegalovirus (CMV) glycoprotein B genotype and CMV DNA load in the amniotic fluid of infected fetuses
Authors: Picone Olivier , Costa Jean‐Marc , Leruez‐Ville Marianne , Ernault Pauline , Olivi Martine , Ville Yves 
Published: December 2004

AbstractObjectiveTo assess the value of both cytomegalovirus (CMV) DNA quantification in amniotic fluid (AF) and CMV glycoprotein B (gB) genotype as prognostic factors in CMV congenital infection.MethodsQuantification of CMV DNA was performed prospectively by real‐time PCR and gB genotypes were analysed by gene sequencing analysis in the amniotic fluid of 42 fetuses infected by CMV. These were correlated with clinical data on fetal anomalies and outcome.ResultsThe proportion of severely symptomatic fetuses was similar in each gB genotype group. Median CMV DNA load was higher in the group of symptomatic fetuses but this difference was not significant and high and low viral loads were found in both groups.ConclusionNeither gB genotype nor CMV DNA load in AF correlate with the severity of CMV congenital infection and these markers are unlikely to prove useful for the management of congenital infection. Copyright © 2004 John Wiley & Sons, Ltd.