bluuebunny/crossref_metadata_2025 huggingface dataset:
Biology, clinical, and hematologic features of acute megakaryoblastic leukemia in children
Authors: Paredes‐Aguilera Rogelio , Romero‐Guzman Lina , Lopez‐Santiago Norma , Trejo Rosa Arana
Published: May 2003

To assess the incidence, clinical features at presentation, hematologic, immunophenotypic, and cytogenetic characteristics of AMKL in children we prospectively studied 834 consecutive non selected children with newly diagnosed acute leukemia (AL) admitted to the Hematology Department at the Instituto Nacional de Pediatría (INP), Mexico, D.F. We found 682 cases (81.8%) with a typical ALL immunophenotype, and the remaining 152 (18.2%) were considered to have AML. In 29 of the 152 patients with AML studied, a diagnosis of AMKL was established. These 29 cases represented 19.1% of the cases of AML and 3.48% of the total cases of AL during the time span covered by the study. Twenty‐four percent of the cases occurred in infants 2 years old or younger and 41.4% occurred in children 41 months of age or younger. In contrast, in only 18.6% of the patients with AML (M0–M6), the diagnosis was established before 42 months of age and in 17% before their second year of life. Clinical presentation was not strikingly different than that observed in patients with other types of AML, and the time interval from onset of symptoms to diagnosis was also similar, though in a small subset of patients, the clinical course was characterized by a chronic slowly progressive disorder extending over weeks or months resembling smoldering leukemia or chronic myelofibrosis with agnogenic myeloid metaplasia. Bone marrow (BM) fibrosis was a constant features in our patients; 75% of the patients studied showed this complication at the time of diagnosis. Some rather unusual findings in this study were intense skeletal pains from multiple osteolytic lesions, the presence of soft‐tissue tumor, and the presence of cohesive scanty clusters of primitive‐looking blast cells in BM aspirates. Several interesting cytogenetic findings in our study were t(1;22)(p13;q13) in a 14‐year‐old boy, t(9;22)(q34;q11) in one patient, and monosomy 7 in two patients. Another important finding in our study was the clinical association with colonic adenocarcinoma in one patient, an association that to our knowledge has not been reported previously. In conclusion, our data suggest that the incidence of AMKL in Mexico might be higher than those reported in Caucasian white pediatric population, and that biologic and cytogenetic profile may differ from those of western countries, but more studies are needed to corroborate cytogenetic heterogeneity, ethnic and geographic diversity. Early onset of the disease, low WBC counts, slight thrombocytopenia or normal platelet counts, and BM fibrosis were characteristic distinctive features of at least half of the patients with this subtype of AML. Am. J. Hematol. 73:71–80, 2003. © 2003 Wiley‐Liss, Inc.
Intravenous immunoglobulin therapy results in post‐infusional hyperproteinemia, increased serum viscosity, and pseudohyponatremia
Authors: Steinberger Beth A. , Ford Stephen M. , Coleman Teresa A.
Published: May 2003

Intravenous immunoglobulin (IVIG) therapy is associated with rare reports of thromboembolic events and severe hyponatremia. We hypothesized that IVIG therapy may result in hyperproteinemia, increased serum viscosity, and pseudohyponatremia. We conducted a prospective observational study to evaluate the incidence of hyperproteinemia occurring after IVIG therapy and its relationship to serum sodium, viscosity, osmolality, and the serum osmolar gap. Eighteen IVIG infusions at a standard dose of 2 g/kg administered over 2–5 days were evaluated. Serum glucose, sodium, protein, viscosity, osmolality, and a calculated osmolar gap were obtained prior to therapy, 6 hr after the initiation of therapy, 24 hr after the conclusion of therapy, and on post‐treatment day 10. Paired t‐testing revealed a statistically significant increase in serum protein and viscosity and decrease in serum sodium and calculated osmolality 24 hr after the completion of IVIG therapy. The calculated serum osmolar gap increased insignificantly. In multivariate analysis, hyperproteinemia at the 6‐hr time point predicted hyponatremia (P < 0.000), and hyperproteinemia at the 24‐hr time point predicted both hyponatremia and increased serum viscosity (P = 0.024). These data demonstrate that increased serum viscosity occurs following IVIG therapy due to hyperproteinemia, and the rare hyponatremia reported is a pseudohyponatremia also due to hyperproteinemia. Am. J. Hematol. 73:97–100, 2003. Published 2003 Wiley‐Liss, Inc.
Factor V leiden and prothrombin gene G20210A mutation in children with cerebral thromboembolism
Authors: Bonduel Mariana , Sciuccati Gabriela , Hepner Mirta , Pieroni Graciela , Torres Aurora Feliú , Mardaraz Claudia , Frontroth Juan Pablo
Published: May 2003

We investigated whether there is an association between factor V Leiden (FVL) and/or prothrombin gene G20210A mutation (PT20210A) and cerebral thromboembolism in a pediatric Argentinean population. From May 1992 to January 2002, 44 consecutive children with arterial ischemic stroke (AIS) and 23 children with cerebral sinovenous thrombosis (SVT) were prospectively studied at a single center. The prevalence of both mutations was compared with a 102 age‐matched controls. In children with AIS, the frequencies (patients vs. controls), odds ratio (OR), and 95% confidence interval (95% CI) for the presence of FVL were as follows: 2.3% vs. 2%, OR/95% CI, 1.16/0.2 to 13.2; P value = 0.99. No cases of PT20210A were found in this group. In children with SVT, the frequencies (patients vs. controls), OR, and 95% CI were as follows: FVL (4.3% vs. 2%, OR/95% CI, 2.27/0.22 to 6.2; P value = 0.99) and PT20210A (4.3% vs. 1%; OR/95% CI, 4.6/0.3 to 76.3; P value = 0.3354). One child with PT20210A also had an inherited protein C deficiency. In 12 (18%) out of the 67 children with cerebral thromboembolism, without the aforementioned mutations, other prothrombotic disorders were detected. Although a multi‐center prospective study with a large number of Argentinean pediatric patients is needed to obtain considerable evidence, no association between factor V Leiden and/or prothrombin gene G20210A mutation and cerebral thromboembolism was found in this pediatric series. Am. J. Hematol. 73:81–86, 2003. © 2003 Wiley‐Liss, Inc.
Health status and health‐related quality of life associated with von Willebrand disease
Authors: Barr Ronald D. , Sek Julia , Horsman John , Furlong William , Saleh Mahassen , Pai Mohan , Walker Irwin
Published: May 2003

Von Willebrand disease (VWD) is the commonest inherited disorder of hemostasis and the majority of women with this disorder experience excessive uterine bleeding. Yet very little information is available on the health‐related quality of life (HRQL) in individuals with VWD. To test the a priori hypotheses that these individuals will have poorer HRQL than members of the general population, and that this burden of morbidity will correlate with the severity of VWD, a cross‐sectional study was undertaken of a population‐based cohort in a regional hemophilia program in Ontario, Canada. A survey was made of individuals over 13 years of age with VWD who self‐reported their health status using a standard 15 item questionnaire. The responses were converted to levels in the Health Utilities Index Mark 2 (HUI2) and Mark 3 (HUI3) health status classification systems to form multi‐element vectors from which single attribute morbidity and overall HRQL utility scores were determined. As a group, individuals with VWD were shown to have poorer HRQL than members of the general population and those with Type 2 disease carried a greater burden of overall morbidity than those with Type 1 disorder. Morbidity was evident mainly in the attributes of emotion, cognition with pain. A striking difference was observed between males and females, with the latter having overall HRQL utility scores similar to those reported previously for HIV positive, severe hemophiliacs. It is possible that this remarkable burden of morbidity reflects chronic iron deficiency associated with menorrhagia. A national study has been proposed to address this likelihood as it offers an opportunity for effective therapeutic intervention (iron supplementation) with a concomitant gain in health status and HRQL. Am. J. Hematol. 73:108–114, 2003. © 2003 Wiley‐Liss, Inc.
Complete recovery from refractory immune thrombocytopenic purpura in three patients treated with etanercept
Authors: McMinn J.R. , Cohen Stephen , Moore Joseph , Lilly Scott , Parkhurst Joan , Tarantino Michael D. , Terrell Deirdra R. , George James N.
Published: May 2003

Management of patients with immune thrombocytopenic purpura (ITP) who have persistent, severe, and symptomatic thrombocytopenia following splenectomy is difficult and empirical. No single agent or regimen provides long‐term success for most patients, and for most treatments it is difficult to assess whether benefits outweigh risks. We report three consecutive patients with critical chronic refractory ITP, who responded promptly and completely following treatment with etanercept, an inhibitor of tumor necrosis factor‐alpha. These patients had failed 6–11 previous treatments. In the first patient, etanercept was given for its approved indication: a flare of co‐existing rheumatoid arthritis. The next two patients were treated with etanercept because of successful outcomes in the previous patients. Although etanercept appeared to be effective treatment for ITP in these 3 patients, the experimental nature of this treatment and the potential risks must be emphasized. On the basis of these case reports, a clinical trial has been initiated to systematically evaluate the efficacy and risks of etanercept in the management of children and adults with chronic ITP. Am. J. Hematol. 73:135–140, 2003. © 2003 Wiley‐Liss, Inc.
Clinicopathologic analysis of ocular adnexal lymphomas: Extranodal marginal zone b‐cell lymphoma constitutes the vast majority of ocular lymphomas among Koreans and affects younger patients
Authors: Cho Eun Yoon , Han Jae Joon , Ree Howe Jung , Ko Young Hyeh , Kang Yoon‐koo , Ahn Hyo Sook , Ahn Seung Do , Park Chan Jeoung , Huh Jooryung
Published: May 2003

The majority of ocular adnexal lymphomas (OAL) are primary marginal zone B‐cell lymphomas (MALT lymphomas). The present study correlated the clinicopathological variables with the histologic subtypes by World Health Organization (WHO) classification with emphasis on MALT lymphomas in OALs of Koreans. There were 68 cases (31 males and 37 females), with a mean age of 45.9 years (range 7–89 years). Histologically, 61 MALT‐type, 2 diffuse large B‐cell (DLBCL), 2 mantle cell type (MCL), 1 anaplastic large‐cell (ALCL), and 2 NK/T‐cell lymphomas (NK/T‐L) were counted among them. Fifty‐seven were primary cases (P‐OAL), and 11 were secondary cases (S‐OAL). Nearly all P‐OALs were MALT lymphomas (n = 56, 98%), with an exception of 1 MCL. Eleven S‐OALs included 5 MALT type, 2 DLBCL, 1 ALCL, 1 MCL, and 2 NK/T‐L. All MALT lymphoma patients were alive (n = 59) except for 2 after a mean duration of follow‐up of 27.6 months (range: 0–108 months): one died of an unrelated cause and one died of recurrence. One non‐MALT type P‐OAL was alive with no evidence of disease (42 months). Of the 11 S‐OAL, 4 had marrow involvement and 5 had progression or relapse outside the orbit. Compared with the other subtypes, MALT lymphoma was more likely to present with local disease (P = 0.001), achieve complete remission (CR) (0.022), and be alive at last follow‐up (0.197), and less likely to experience recurrence (P = 0.06). In conclusion, OALs in Koreans are characterized by a preponderance of primary lymphomas over systemic lymphomas, striking predominance of MALT type lymphomas, and young age of occurrence. Histologic subtype by WHO classification has a significant correlation with the final outcome, with the most favorable outcome associated with OALs of the MALT type. Am. J. Hematol. 73:87–96, 2003. © 2003 Wiley‐Liss, Inc.
Prognostic significance of bcl‐2, bax, and p53 expression in diffuse large B‐cell lymphoma
Authors: Sohn Sang Kyun , Jung Jin Tae , Kim Dong Hwan , Kim Jong Gwang , Kwak Eun Kyung , Park Tae in , Shin Dong Gun , Sohn Kyung Rak , Lee Kyu Bo
Published: May 2003

Diffuse large B‐cell lymphoma (DLCL) exhibits heterogeneous clinical features and varies markedly in response to treatment and prognosis. Because apoptosis‐related proteins may play an important role in predicting the prognosis of DLCL, the current study investigated the prognostic significance of a high level of bcl‐2, bax, and p53 expression in relation to clinical characteristics in patients with DLCL. Paraffin‐embedded specimens from 94 patients with de novo DLCL were analyzed immunohistochemically for bcl‐2, bax, and p53 gene expression. Cases with a positive immunohistological stain in more than 50% of the tumor cells were considered to have DLCL‐positive expression. Patients were treated optimally, i.e., with radiotherapy including brief cycles of CHOP or CHOP‐like regimens for patients with stage 1–2A diseases and with at least 6 cycles of CHOP or CHOP‐like regimens for stage 2B–4 diseases. The responses to therapy and survival were then analyzed in 94 uniformly staged patients. bcl‐2 expression was identified in 24 patients (26.4%), bax expression in 35 patients (37.6 %), and p53 expression in 21 patients (22.6%). bax expression proved to be a statistically significant prognostic factor in predicting the overall survival (OS) (P = 0.0015) and disease‐free survival (DFS) (P = 0.0052), regardless of other clinical factors or immunohistological results. There was no significant difference in the OS (P = 0.0682) or DFS (P = 0.088) between the bcl‐2‐positive (n = 24) and bcl‐2‐negative (n = 67) groups. However, bcl‐2 expression was found to be unfavorably associated with the OS (P = 0.0054) in a confined group with low (n = 51) or low intermediate (n = 22) IPI scores. The expression of p53 exhibited no statistical correlation with the OS or DFS. A multivariate analysis revealed that IPI score, bulky mass, and bax expression were all significantly associated with the DFS or OS. bax and bcl‐2 should be considered as independent biologic prognostic parameters in DLCL, thereby aiding in the identification of patient risk groups. As such, bcl‐2‐positive patients with a low or low intermediate IPI score, or without a high level of bax expression could be candidates for more intensive therapy or alternative therapeutic approaches. Am. J. Hematol. 73:101–107, 2003. © 2003 Wiley‐Liss, Inc.
Isolated noncompaction of the left ventricular myocardium in the adult is an autosomal dominant disorder in the majority of patients
Authors: Sasse‐Klaassen Sabine , Gerull Brenda , Oechslin Erwin , Jenni Rolf , Thierfelder Ludwig
Published: May 2003

Isolated noncompaction of the ventricular myocardium (INVM, MIM 300183 and 604169) is a congenital unclassified cardiomyopathy with numerous prominent trabeculations and deep intertrabecular recesses in a hypertrophied and hypokinetic myocardium. Mutations in the G4.5 gene result in a wide spectrum of severe infantile X‐linked cardiomyopathic phenotypes including Barth syndrome with dilated cardiomyopathy and INVM. Molecular genetic analysis of INVM has only been performed in pediatric patients. Although adult INVM patients show similar cardiac abnormalities, the influence of genetic factors, especially of mutations in G4.5, is unknown. We analyzed 25 adult INVM patients for the presence of mutations in the G4.5 gene and performed a pedigree analysis of probands. Mutations were not found in the coding sequence or splice sites of G4.5. Systematic analysis of relatives from seven of nine probands showed multiple affected members consistent with an autosomal dominant pattern of inheritance in the majority of cases. We conclude that INVM in the adult is an autosomal dominant disorder rarely caused by mutations in G4.5 and therefore genetically distinct from infantile X‐linked cases. © 2003 Wiley‐Liss, Inc.
Malignant hematological disorders in children with Wolf–Hirschhorn syndrome
Authors: Sharathkumar Anjali , Kirby Melanie , Freedman Melvin , Abdelhaleem Mohamed , Chitayat David , Teshima Ikuko E. , Dror Yigal
Published: May 2003

Wolf–Hirschhorn syndrome (WHS) is a rare chromosomal disorder attributable to a deletion at the short arm of chromosome 4. This syndrome is associated with characteristic facial appearance, multiple congenital abnormalities, mental retardation, feeding difficulties and failure to thrive. We report two girls with WHS who developed myelodysplastic syndrome (MDS). According to the “Category, Cytology, Cytogenetic (CCC)”classification of childhood MDS, patient 1 had refractory cytopenia with ring sideroblasts at the age of 6 years, while patient 2 had refractory cytopenia with dysplasia at the age of 5½ years. Patient 1 progressed to refractory cytopenia with excess blasts within a year, while patient 2 progressed to acute lymphoblastic leukemia within 1 month of presentation. It is possible that allelic loss of a tumor suppressor gene such as WHSC1 and/or FGFR3 from the deleted segment 4p16.3 plays a critical role in the process of malignant transformation. To our knowledge, this is the first report of severe hematological complications like MDS and leukemia in children with WHS and may be an important genetic model for understanding malignant hematological transformation. This report also underscores the importance of evaluating children with WHS for hematopoietic dysfunction. © 2003 Wiley‐Liss, Inc.
Subcortical band heterotopia with simplified gyral pattern and syndactyly
Authors: Sicca Federico , Silengo Margherita , Parrini Elena , Ferrero Giovanni B. , Guerrini Renzo
Published: May 2003

We describe a girl with an unusual form of subcortical band heterotopia (SBH) and a complex malformation syndrome. SBH had an irregular inner margin, organized in contiguous fascicles of migrating neurons, sometimes giving the appearance of many small contiguous gyri. The true cortex had decreased thickness and showed a simplified gyral pattern with decreased number of gyri, which were usually of increased width, and shallow sulci. The cerebellum was hypoplastic. Additional features included epicanthal folds, hypertelorism, small nose with hypoplastic nares, bilateral syndactyly of the toes, pulmonary valve stenosis, atrial and ventricular septal defects. At the age of 1 year the patient had severe developmental delay and epilepsy. Chromosome studies and mutation analysis of the DCX and LIS1 genes gave negative results. This observation delineates a new multiple congenital abnormalities mental retardation syndrome and confirms genetic heterogeneity of SBH. © 2003 Wiley‐Liss, Inc.
Unusual congenital abdominal wall defect and review
Authors: Carrera Ignacio Arroyo , Pitarch Vicente , García María Jesús , Barrio Ana Raquel , Martínez‐Frías María Luisa
Published: May 2003

Abdominal wall defects distant from the umbilicus are very unusual. We describe a patient with a congenital defect adjacent and external to the lateral border of the recti muscles, rounded in shape, bilateral and symmetrical, but affecting different structures in each side, musculocutaneous agenesis in the left side and only muscle agenesis in the right one. The morphologic characteristics of the defect and the absence of associated anomalies, suggest that this anomaly may be the result of an event acting during phenogenesis. We consider that this defect is different from other structural abnormalities of the anterior abdominal wall which occur much earlier at certain periods of the embryogenesis. Though the etiology is speculative, we could postulate a disruptive origin of the defect of unknown cause. © 2003 Wiley‐Liss, Inc.
What does “A gene for heart disease” mean? A focus group study of public understandings of genetic risk factors
Authors: Bates Benjamin R. , Templeton Alan , Achter Paul J. , Harris Tina M. , Condit Celeste M.
Published: May 2003

There is growing concern in the medical community about potential genetic determinism in the patient population. Limited information about the public understanding of genetic factors in disease formation is available. To access public perceptions of potentially deterministic phrasing of genetic risk factors, we sought to establish interpretations of the phrase, “a gene for heart disease.” Focus groups in urban, suburban, and rural communities were conducted from July through October, 2001 in Georgia. A total of 108 participants were recruited. Participants were recruited to balance sex and racial representation. We used three outcome measures for participants understandings of the phrase: (1) participants' statements of the meaning of the phrase; (2) the level of determinism assigned to genetic factors by participants; and (3) participant reports of the health consequences of having “a gene for heart disease.” Participants did not report a single interpretation of the phrase. There were dominant participant interpretations under each outcome measure: (1) “a gene for heart disease” was interpreted as meaning genetic and environmental factors both played roles in disease formation; (2) genetic predisposition was perceived as heightened, not absolute, risk; (3) the perceived health impact was a greater risk of becoming sick. Minority interpretations were found under each measure. Overall, naming “a gene for heart disease” does not appear to have a deterministic impact on a plurality of participants' perceptions of risks associated with genetic factors. Genetic fatalism in patient populations may be confined to a sizable minority. Important considerations for provider intervention and patient education are indicated. © 2003 Wiley‐Liss, Inc.
Polysyndactyly, complex heart malformations cardiopathy, and hepatic ductal plate anomalies: An autosomal recessive syndrome diagnosed antenatally
Authors: Stoll Claude , Gasser B.
Published: May 2003

A distinct syndrome was ascertained in a 3‐year‐old girl and her brother. The proband was the first child of first cousin parents. She was born after an uneventful pregnancy. At birth, multiple congenital anomalies were noted: ptosis of the left eyelid, hypertelorism, anteverted nares, large fontanel, long philtrum, ungueal hypoplasia, polysyndactyly, single transverse crease, complex cardiopathy, and hepatic cysts. During another pregnancy of the mother, fetal ultrasonographic examination showed an hypertrophy of the right ventricle and atria, a dextroposition of the aorta, a bilateral renal pelvis dilatation, and a club foot. After termination of the pregnancy, necropsy showed facial anomalies, a small penis, a polysyndactyly, a ventricular septum defect, and a malformation of the ductal plate. Bonneau et al. [1983: J Genet Hum 2:93–105] described a family in which three sibs had a complex cardiac malformation, hexadactyly of the first toe, and syndactyly of the third and fourth fingers. Rajab [1997: Clin Dysmorphol 6:85–88] described two sibs with similar features in an Omani family. The sibs described in this report had anomalies of the ductal plate which were not reported in the two other families. These new findings are in favor of autosomal inheritance of this condition which is amenable to antenatal diagnosis. © 2003 Wiley‐Liss, Inc.
Synthesis and structure of [K(12‐crown‐4)2]2[K(12‐crown‐4)]2[Sn9]·4en
Authors: Ugrinov Anger , Sevov Slavi C.
Published: May 2003

The new compound [K(12C4)2]2[K12C4]2[Sn9]·4en (1), where 12C4 is 12‐crown‐4 polyether, was crystallized from an ethylenediamine solution of an intermetallic precursor with nominal composition K4Li2Sn8. The structure of the compound (orthorhombic, Cmca, a = 21.763(4), b = 16.030(3), c = 51.85(1) Å, Z = 8) contains isolated deltahedral clusters of [Sn9]4−. The clusters are of Cs symmetry and are nido according to the number of bonding electrons. The potassium countercations are sequestered by the very small 12C4 ether: half of them are sandwiched between a pair of molecules and the other half are coordinated by a single molecule. The latter potassium cations also bridge edges of the tin clusters, and each cluster has two such coordinated cations. Coordination of potassium cations by such small crown ethers is quite rare. Copyright © 2003 John Wiley & Sons, Ltd.
Synthesis of bipyridine‐linked dimers of azanonaborane: electronic absorption and molecular‐orbital calculations
Authors: El‐Zaria Mohamed E. , Borrmann Tobias , Gabel Detlef
Published: May 2003

A new class of azanonaborane–bipyridine derivatives was synthesized from the reaction of bidentate nitrogen ligands with the azanonaborane cluster [(RNH2)B8H11NHR] in benzene under reflux. The exchange of the exo‐amino ligand by bipyridine derivatives is a convenient route to yield a dimer of the azanonaborane cluster in good yield. Thus, 4,4′‐bipyridine (PyPy) and trans‐1,2‐di‐(4‐pyridyl)‐ethene (PyPy) replace the exo‐NH2R unit to give [(PyPy)B8H11NHR] or [RHNH11B8PyPyB8H11NHR] and [(PyPy)B8H11NHR] or [RHNH11B8PyPyB8H11NHR] respectively as colored products due to electronic interaction between the {B8N} unit and the bonded bipyridine units. This interaction has been investigated by UV–vis spectroscopy and by AM‐1 molecular‐orbital calculations. In the case of [(PyPy)B8H11NHR], the 1H and 13C NMR analysis of the products revealed two isomeric forms (cis and trans). This is attributed to thermal cis–trans isomerization. Copyright © 2003 John Wiley & Sons, Ltd.
Synthesis, characterization, X‐ray crystal structure and in vitro antitumour activity of bis(1,2‐dicarba‐closo‐dodecaborane‐9‐carboxylato)di‐n‐butyltin
Authors: Bregadze Vladimir I. , Glazun Sergey A. , Petrovskii Pavel V. , Starikova Zoya A. , Rochev Valery Ya. , Dalil Hassan , Biesemans Monique , Willem Rudolph , Gielen Marcel , de Vos Dick
Published: May 2003

The 1 : 2 condensation of dibutyltin(IV) oxide with 1,2‐carborane‐9‐carboxylic acid resulted in bis(1,2‐dicarba‐closo‐dodecaborane‐9‐carboxylato)di‐n‐butyltin (1), the first carborane‐based organotin compound where the carborane cage is linked to the carboxylic moiety via a boron atom. The structure of 1, characterized by 1H, 11B, 13C, 119Sn NMR spectroscopy and X‐ray diffraction, was shown to correspond to bis(1,2‐dicarba‐closo‐dodecaborane‐9‐carboxylato)di‐n‐butyltin. Compound 1 was screened in vitro against seven tumour cell lines of human origin and was found to be significantly more active than 5‐fluorouracil, cis‐platin and carboplatin but less active than methotrexate and doxorubicin. Copyright © 2003 John Wiley & Sons, Ltd.
Combination of 1,2‐hydroboration and 1,1‐organoboration: synthesis of novel organo‐substituted 1‐silacyclobutenes
Authors: Wrackmeyer Bernd , Maisel Heidi E. , Molla Elias , Mottalib Abdul , Badshah Amin , Bhatti Moazzam H. , Ali Saqib
Published: May 2003

The reaction of di(alkyn‐1‐yl)silanes Me(R)Si(CCtBu)2 [1; R = Me (a), H (b)] with diethylborane or 9‐borabicyclo[3.3.1]nonane in a 1 : 1 ratio affords the 1‐silacyclobutene derivatives 6a, 7a,b as a result of intermolecular 1,1‐hydroboration followed by intramolecular 1,1‐organoboration. Intermediates, in which both an alkenyl and an alkynyl group are linked to silicon (2a, 3a), were identified and prepared independently by the reaction of the corresponding chlorosilane 5a with the lithium alkynide LiCCtBu. This procedure also opened the way to a stepwise synthesis of 1‐silacyclobutenes with a choice of substituents in the 2,4‐positions (6b and 9a). All reactions were monitored by 29Si NMR spectroscopy, and the structural assignment of the final products was based on multinuclear magnetic resonance data (1H, 11B, 13C and 29Si NMR). Copyright © 2003 John Wiley & Sons, Ltd.
Gas‐phase hydrodechlorination of chlorobenzenes over silica‐supported palladium and palladium–ytterbium
Authors: Jujjuri Satyakrishna , Ding Errun , Shore Sheldon G. , Keane Mark A.
Published: May 2003

A 5% w/w palladium loading on silica has been achieved via impregnation of the support with Pd(C2H3O2)2 and { (DMF)10Yb2[Pd(CN)4]3} ∞ precursors to deliver monometallic (Pd/SiO2) and bimetallic (Yb–Pd/SiO2) catalyst systems respectively. The catalytic action of each has been assessed in the continuous gas‐phase hydrodechlorination (HDC) of chlorobenzene (CB) and 1,2‐dichlorobenzene (1,2‐DCB) (T = 423 K, inlet chlorine/palladium mol ratio of 5 × 103 h−1) and the hydrogenation of benzene (T = 423 K, inlet C6H6/palladium mol ratio of 35 h−1). Activation of both catalysts delivered similar palladium crystallite size distributions with an average palladium diameter of 5–6 nm where the ytterbium component (in Yb–Pd/SiO2) was present as a thin surface coating. The Pd–Yb bimetallic exhibited significantly higher HDC and hydrogenation activities, the former manifested by significantly greater fractional dechlorinations and benzene selectivities/yields. Yb/SiO2 proved inactive in terms of promoting hydrogen scission or addition and the promotional effect of ytterbium in Yb–Pd/SiO2 is discussed in terms of electron donation and hydrogen transfer via surface YbH2. Under identical reaction conditions, a lower HDC activity was recorded for 1,2‐DCB compared with CB, a response that is attributed to steric constraints allied to the deactivating effect of the second chlorine substituent. Both Pd/SiO2 and Yb–Pd/SiO2 exhibited a decline in HDC activity with time‐on‐stream, but the bimetallic was significantly more resistant to deactivation. Copyright © 2003 John Wiley & Sons, Ltd.
Fast solution of problems with multiple load cases by using wavelet‐compressed boundary element matrices
Authors: Bucher Henrique F. , Wrobel Luiz C. , Mansur Webe J. , Magluta Carlos
Published: May 2003

This paper presents a fast approach for rapidly solving problems with multiple load cases using the boundary element method (BEM). The basic idea of this approach is to assemble the BEM matrices separately and to compress them using fast wavelet transforms. Using a technique called ‘virtual assembly’, the matrices are then combined inside an iterative solver according to the boundary conditions of the problem, with no need for recompression each time a new load case is solved. This technique does not change the condition number of the matrices—up to a small variation introduced by compression—so that previous theoretical convergence estimates are still valid. Substantial savings in computer time are obtained with the present technique. Copyright © 2003 John Wiley & Sons, Ltd.
Mathematica solution of Rayleigh equation in non‐linear vibration
Authors: Mikhailov M. D.
Published: May 2003

The stable periodic motion, described by Rayleigh differential equation, is solved by using the Mathematica software system. We define rules computing the periods T, the magnitude A, the displacement u(t), and the velocity v(t) for prescribed perturbation parameter ε and circular frequency ω. These rules have been explored to find the period T, the magnitude A, and the reducing factor of the circular frequency α=2π/T with 10 correct digits after decimal point for ω equal to 1 and the values of ε in the range from 0.1 to 100. The displacement and the velocity are plotted for ε equal to 0.1, 1, 10, and 100. Copyright © 2003 John Wiley & Sons, Ltd.
A critique of claims from Reynolds, Nicolson & Hambly (2003) that DDAT is an effective treatment for children with reading difficulties–‘Lies, damned lies and (inappropriate) statistics?’
Authors: Snowling Margaret J. , Hulme Charles
Published: May 2003

Reynolds, Nicolson and Hambly (Dyslexia 2003; 9: 48) report a study to evaluate the effectiveness of dyslexia dyspraxia attention deficit treatment (DDAT)—an exercise‐based treatment for children with reading difficulties. They claim that DDAT is an effective treatment with positive effects on the cognitive skills underlying literacy and the reading process. We outline the numerous methodological and statistical problems with this study and conclude that it provides no evidence that DDAT is an effective form of treatment for children with reading difficulties. Copyright © 2003 John Wiley & Sons, Ltd.
Distinguishing between deservingness and entitlement: earned outcomes versus lawful outcomes
Authors: Feather N. T.
Published: May 2003

Two studies are described that investigated a proposed distinction between deservingness and entitlement. Deservingness was assumed to relate to the evaluative structure of actions and their contingent outcomes and entitlement to an external framework involving rights, rules and social norms. Study 1 investigated reactions to scenarios in which a student running for election in a national student organization exerted either high or low effort, was either eligible or ineligible for election by virtue of age, and was either elected or not elected. Study 2 investigated reactions to a scenario in which a stimulus person suffering from an illness had to decide how much money to leave in a will to a son, nephew, or friend who provided him with either help or limited help. In both studies, student participants (n = 134 in Study 1, n = 236 in Study 2) completed ratings of deservingness and entitlement, as well as other measures. Results of both studies supported the distinction between deservingness and entitlement. Whether an outcome was deserved depended on amount of effort in Study 1 and on amount of help in Study 2. Results are also reported for other justice variables and for reported affect in Study 1, and for the amount allocated in the will in Study 2. Copyright © 2003 John Wiley & Sons, Ltd.
Leaders and their treatment of subgroups: implications for evaluations of the leader and the superordinate group
Authors: Duck Julie M. , Fielding Kelly S.
Published: May 2003

This experiment examined members' evaluations of a group leader and the group in contexts where a superordinate group comprised two subgroups and the group leader was aligned with one or other subgroup. The design varied group leader (ingroup, outgroup) and leader behavior (ingroup favoring, outgroup favoring) as well as the broader comparative context (intragroup, intergroup). Across a number of measures, results indicated a consistent Group Leader × Leader Behavior interaction that was independent of comparative context. Although group members were most satisfied with an ingroup leader who favored the ingroup, ingroup leaders were perceived positively irrespective of their behavior. Outgroup leaders who unexpectedly favored the other subgroup were also perceived positively. However, outgroup leaders who favored their own subgroup were perceived as less fair and as more biased than other leaders. They also engendered less identification with the superordinate group and a less unified perception of the group. Results demonstrate the importance of social identity concerns to leadership in nested group contexts and emphasize the fact that perceptions of leader fairness and concern for the common group mediate responses to the superordinate category. Copyright © 2003 John Wiley & Sons, Ltd.
Genuine giving or selfish sacrifice? The role of commitment and cost level upon willingness to sacrifice
Authors: Powell Chantal , Van Vugt Mark
Published: May 2003

This research uses interdependence theory to examine the link between commitment, costs, and willingness to sacrifice within interdependence dilemmas occurring in intimate relationships. Advancing prior work, which has demonstrated a positive association between relationship commitment and willingness to sacrifice, we investigated the moderating role of the cost of sacrifice in a scenario‐based survey. Consistent with our hypotheses it was found that in high cost interdependence dilemmas, significantly greater levels of sacrifice were observed from individuals classified as high in commitment than from individuals classified as low in commitment. However in low cost dilemmas the relationship between commitment and sacrifice disappears. Possible differences in motivations for sacrifice are discussed with respect to this finding. Copyright © 2003 John Wiley & Sons, Ltd.
Perceived rationality, morality, and power of social choice as a function of interdependence structure and social value orientation
Authors: Joireman Jeffrey A. , Kuhlman D. Michael , Van Lange Paul A. M. , Doi Toshiaki , Shelley Gregory P.
Published: May 2003

The present study examines how individuals with different social value orientations (i.e. prosocial, individualistic, and competitive) construe the rationality, morality, and power of choices in four distinct interdependence structures which systematically differ in the motives that could underlie the most prosocial or least aggressive choice: (a) altruism only, (b) altruism and cooperation, (c) altruism, cooperation, and individualism, and (d) altruism, cooperation, individualism, and competition. Results revealed that rationality ratings, and to a lesser degree morality and power ratings, increased most when the motives that could underlie a choice were part of the perceiver's social value orientation. Overall, the pattern of rationality ratings provided reasonable support for the Goal Prescribes Rationality Principle. Ratings of morality and power suggested a corresponding Goal Prescribes Morality/Power Principle (for prosocials and individualists), but revealed only mixed support for the Might Over Morality Hypothesis. Copyright © 2003 John Wiley & Sons, Ltd.
MISSING FLOWERS gene controls axillary meristems initiation in sunflower
Authors: Fambrini Marco , Cionini Giuliano , Bertini Daniele , Michelotti Vania , Conti Alessio , Pugliesi Claudio
Published: May 2003

Summary: The initiation and growth of axillary meristems are fundamental components of plant architecture. Here, we describe the mutant missing flowers (mf) of Helianthus annuus characterized by the lack of axillary shoots. Decapitation experiments and histological analysis indicate that this phenotype is the result of a defect in axillary meristem initiation. In addition to shoot branching, mutation affects floral differentiation. The indeterminate inflorescence of sunflower (capitulum) is formed of a large flat meristem which produces floret primordia in multiple spirals. In wildtype plants a bisecting crease divides each primordium in two distinct bumps that adopt different fate. The peripheral (abaxial) part of the primordium becomes a small leaf‐like bract and the adaxial part becomes a flower. In the mf mutant, the formation of flowers at the axil of bracts is precluded. Histological analyses show that in floret primordia of the mutant a clear subdivision in dyads is not established. The primordia progressively bend inside and only large involucral floral bracts are developed. The results suggest that the MISSING FLOWERS gene is essential to provide or perceive an appropriate signal to the initiation of axillary meristems during both vegetative and reproductive phases. genesis 36:25–33, 2003. © 2003 Wiley‐Liss, Inc.
Longitudinal axis of the hippocampus: Both septal and temporal poles of the hippocampus support water maze spatial learning depending on the training protocol
Authors: de Hoz Livia , Knox Jane , Morris Richard G.M.
Published: May 2003

It has been suggested previously that 30% sparing of the hippocampus is enough to support spatial learning of a reference memory task in a water maze provided the spared tissue is located septally (Moser et al. 1995, Proc Natl Acad Sci USA 92:9697–9701). Therefore, the temporal hippocampus may not be involved in spatial memory. Place cells are also found in this part of the structure, and it has been suggested that these place cells have larger, less well‐tuned place fields than are found in the septal hippocampus. We tested the possibility that the temporal hippocampus might be involved in spatial learning when the animals are required to distinguish between different contexts. Experiment 1 was a replication of the findings reported by Moser et al., using their protocol (8 trials/day, 6 days) and the groups with 20–40% hippocampus spared septally or temporally (volume assessed by quantitative volumetric techniques). In experiment 2, rats with also 20–40% sparing of the hippocampus either septally or temporally were trained in two water maze concurrently (four trials/day/water maze, 8 days). Rats with 20–40% hippocampus spared temporally were able to learn the two water maze tasks normally, and no difference was observed between rats with septal and temporal hippocampus spared across different measures of performance. In experiment 3, rats with 20–40% hippocampus spared septally or temporally were trained in one water maze as in experiment 1, but using a spaced training protocol similar to that of experiment 2 (four trials/day, 8 days). Rats with temporal hippocampus spared developed a preference for the training quadrant and acquired levels of performance indistinguishable from those of rats with septal hippocampus spared. The results suggest that the temporal hippocampus can support the learning of two, but also one, spatial water maze reference memory task, provided the training protocol is adequate. Hippocampus 2003;13:587–603. © 2003 Wiley‐Liss, Inc.
Fetal hippocampal CA3 cell grafts enriched with fibroblast growth factor‐2 exhibit enhanced neuronal integration into the lesioned aging rat hippocampus in a kainate model of temporal lobe epilepsy
Authors: Zaman Vandana , Shetty Ashok K.
Published: May 2003

Aging impairs the conduciveness of the lesioned hippocampus for robust survival of neurons derived from homotopic fetal cell grafts (Zaman and Shetty, Neuroscience 109:537–553, 2002), suggesting a need for graft augmentation in fetal graft‐mediated therapeutic strategies for the lesioned aging hippocampus. We hypothesize that pre‐treatment and grafting of donor hippocampal CA3 cells with fibroblast growth factor‐2 (FGF‐2) considerably enhances graft neuronal integration into the lesioned CA3 region of the aging hippocampus. We employed the optical fractionator cell counting method and quantified the number of surviving cells and neurons derived from 5′‐bromodoxyuridine‐labeled embryonic day 19 CA3 cell grafts pre‐treated and transplanted with FGF‐2 into the lesioned CA3 region of the middle‐aged and aged rat hippocampus at 4 days post‐lesion. In both middle‐aged and aged hippocampus, pre‐treatment and transplantation of CA3 cell grafts with FGF‐2 resulted in a robust yield of surviving cells (72–80% of injected cells) and neurons (62–69% of injected cells) from grafts. The overall yield was dramatically greater than the yield observed earlier from standard (untreated) fetal CA3 cell grafts into the lesioned aging hippocampus but was highly comparable to that observed for standard fetal CA3 cell grafts into the lesioned young hippocampus (Zaman and Shetty, Neuroscience 109:537–553, 2002). Thus, a robust neuronal integration from fetal CA3 cell grafts can be achieved into the lesioned CA3 region of the aging hippocampus with a simple pre‐treatment and grafting of donor fetal CA3 cells with FGF‐2. These results have implications toward the development of suitable cell grafting strategies for repair of the lesioned aging hippocampus in neurodegenerative diseases, particularly the temporal lobe epilepsy, stroke, and Alzheimer's disease. Hippocampus 2003;13:618–632. © 2003 Wiley‐Liss, Inc.
Distal landmarks and hippocampal place cells: Effects of relative translation versus rotation
Authors: Knierim James J. , Rao Geeta
Published: May 2003

Hippocampal neurons are selectively active when a rat occupies restricted locations in an environment. These place cells derive their specificity from a multitude of sources, including idiothetic cues and sensory input derived from both distal and local landmarks. Most experiments have attempted to dissociate the relative strengths and roles played by these sources by rotating one set against the other. Few studies have addressed the effects of relative translation of the local cue set versus salient distal landmarks. To address this question, ensembles of place cells were recorded as a rectangular or circular track was moved to different locations in a room with controlled visual landmarks. Place cells primarily maintained their firing fields relative to the track (i.e., occupying new locations relative to the distal landmarks), even though the track could occupy completely nonoverlapping regions of the room. When the distal landmarks were rotated around the circular track, however, the place fields rotated with the landmarks, demonstrating that the cues were perceptible to the rat. These results suggest that, under these conditions, the spatial tuning of place cells may derive from an interaction between local and idiothetic cues, which define the precise firing locations of the cells and the relationships between them, and distal landmarks, which set the orientation of the ensemble representation relative to the external environment. Hippocampus 2003;13:604–617. © 2003 Wiley‐Liss, Inc.
Retracted: Treatment with metallothionein prevents demyelination and axonal damage and increases oligodendrocyte precursors and tissue repair during experimental autoimmune encephalomyelitis
Authors: Penkowa Milena , Hidalgo Juan
Published: May 2003

Experimental autoimmune encephalomyelitis (EAE) is an animal model for the human demyelinating disease multiple sclerosis (MS). EAE and MS are characterized by significant inflammation, demyelination, neuroglial damage, and cell death. Metallothionein‐I and ‐II (MT‐I + II) are antiinflammatory and neuroprotective proteins that are expressed during EAE and MS. We have shown recently that exogenous administration of Zn‐MT‐II to Lewis rats with EAE significantly reduced clinical symptoms and the inflammatory response, oxidative stress, and apoptosis of the infiltrated central nervous system areas. We show for the first time that Zn‐MT‐II treatment during EAE significantly prevents demyelination and axonal damage and transection, and stimulates oligodendroglial regeneration from precursor cells, as well as the expression of the growth factors basic fibroblast growth factor (bFGF), transforming growth factor (TGF)β, neurotrophin‐3 (NT‐3), NT‐4/5, and nerve growth factor (NGF). These beneficial effects of Zn‐MT‐II treatment could not be attributable to its zinc content per se. The present results support further the use of Zn‐MT‐II as a safe and successful therapy for multiple sclerosis. © 2003 Wiley‐Liss, Inc.
Light exposure during daytime modulates expression of Per1 and Per2 clock genes in the suprachiasmatic nuclei of mice
Authors: Challet Etienne , Poirel Vincent‐Joseph , Malan André , Pévet Paul
Published: May 2003

The suprachiasmatic nuclei (SCN) of the hypothalamus contain the master circadian clock in mammals. Nocturnal light pulses that reset the circadian clock also lead to rapid increases in levels of Per1 and Per2 mRNA in the SCN, suggesting that these genes are involved in the synchronization to light. During the day, when light has no phase‐shifting effects in nocturnal rodents, the consequences of light exposure for Per expression have been less thoroughly studied. Therefore, the effects of light exposure during the day were assessed on Per1 and Per2 mRNA in the SCN of mice. Expression of Per1 and Per2 was generally increased by 30‐min light pulses during the subjective day, with more pronounced effects in the morning. One exception was noted for a transient decrease in Per2 expression after a short light pulse applied at midday. Prolonged light exposure (up to 3 hr) starting at midday markedly increased Per2 expression but not that of Per1. Moreover, the amplitude of the daily variations of both Per and the duration of Per1 peak was increased in mice exposed to a light‐dark cycle compared with those transferred to constant darkness. Finally, the amplitude of the daily variations of both Per and the basal level of Per1 were increased in mice under a light‐dark cycle compared with animals synchronized to a skeleton photoperiod (i.e., with daily dawn and dusk 1‐hr exposures to light). Taken together, the results indicate that prolonged light exposure during daytime positively modulates daily levels of Per1 and Per2 mRNA in the SCN of mice. © 2003 Wiley‐Liss, Inc.