bluuebunny/crossref_metadata_2025_split huggingface dataset:
Metabolic disorders and mental retardation
Authors: Kahler Stephen G. , Fahey Michael C. 
Published: January 2003

AbstractThe metabolic and anatomical substrate of most forms of mental retardation is not known. Because the basis of normal brain function is not sufficiently understood, the basis of abnormal function is understood poorly. Even in disorders where the fundamental biochemical defect is known, such as phenylketonuria (PKU) and other enzyme defects, the exact basis for brain dysfunction is uncertain. The outcome for treated PKU, galactosemia, homocystinuria, and lysosomal disorders is not yet optimal. The various forms of nonketotic hyperglycinemia often respond poorly to current therapy. Less familiar disorders, with or without seizures, such as deficient synthesis of serine or creatine and impaired glucose transport into the brain, and disorders with variable malformations, such as Smith‐Lemli‐Opitz (SLO) syndrome and the congenital disorders of glycosylation (CDGs), may initially be thought to be a nonspecific form of developmental delay.Less familiar disorders, with or without seizures and disorders with variable malformations may initially be thought to be a nonspecific form of developmental delay.Simple tests of urine, blood, and cerebrospinal fluid may lead to a diagnosis, accurate genetic counseling, and better treatment. Metabolic brain imaging (magnetic resonance spectroscopy (MRS)) has also helped to reveal biochemical abnormalities within the brain. © 2003 Wiley‐Liss, Inc.
Costello syndrome: An overview
Authors: Hennekam Raoul C.M. 
Published: January 2003

AbstractThe Costello syndrome is characterized by prenatally increased growth, postnatal growth retardation, coarse face, loose skin resembling cutis laxa, nonprogressive cardiomyopathy, developmental delay, and a outgoing, friendly behavior. Patients can develop papillomata, especially around the mouth, and have a predisposition for malignancies (mainly abdominal and pelvic rhabdomyosarcoma in childhood). Costello syndrome is likely to be an autosomal dominant disorder. The pathogenesis is unclear, but there are many clues for a disturbed elastogenesis, possibly through a disturbed elastin‐binding protein reuse by chondroitin sulfate‐bearing proteoglycans accumulation. A review of the findings in the 73 patients that have been described in sufficient detail is provided. © 2003 Wiley‐Liss, Inc.
Kabuki make‐up syndrome: A review
Authors: Matsumoto Naomichi , Niikawa Norio 
Published: January 2003

AbstractKabuki make‐up syndrome (KMS, OMIM 147920) is an MCA/MR syndrome of unknown cause. It is characterized by a dysmorphic face, postnatal growth retardation, skeletal abnormalities, mental retardation, and unusual dermatoglyphic patterns. Approximately more than 350 cases have been reported from all over the world. Besides these five cardinal manifestations, joint laxity (74%), dental abnormalities (68%), and susceptibility to infections including recurrent otitis media (63%) were well recognized as other frequent features. A variety of visceral anomalies such as caidiovascular anomalies (42%), renal and/or urinary tract anomalies (28%), biliary atresia, diaphragmatic hernia, and anorectal anomaly were also reported. Some patients were said to have normal intelligence (16%) and normal heights, suggesting that they may have reproductive fitness to have their children. At least eight patients had lower lip pits with or without cleft palate, known as a feature of van der Woude syndrome. There have been 13 chromosomal abnormalities associated with KMS. However, no common abnormalities or breakpoints that possibly contribute to positional cloning of the putative KMS gene(s) are known. Although clinical manifestations of KMS are well established, its natural history, useful for genetic counseling, remains to be studied. © 2003 Wiley‐Liss, Inc.
Genetics, mental retardation, and the forging of new alliances
Authors: Finucane Brenda , Haas‐Givler Barbara , Simon Elliott W. 
Published: January 2003

AbstractThe lives of people with intellectual disabilities are tightly intermeshed with the philosophies and practices of special educators, therapists, psychologists, social workers, and others who make up the mental retardation field. These nonmedical workers represent a kind of extended family, which can strongly influence the decisions made and services received by the people they serve. By contrast, most individuals with mental retardation do not have extraordinary medical needs, and healthcare professionals play only a minor role in their human services family. There are deeply rooted historical differences between the medical model of mental retardation with which genetics professionals are familiar and the philosophies embraced by nonmedical workers who dominate the mental retardation field. These contrasts have an important impact on the genetic diagnostic process, as well as a more global effect on mental retardation research. Without the full participation of teachers, therapists, psychologists, and others outside the medical setting, many practical applications of genetic research can neither be implemented nor expanded upon, and an important opportunity for cross‐fertilization is lost. It is in the interest of the genetics community to take advantage of recent overtures by the mental retardation field to forge new alliances and broaden its perspectives on intellectual disability. © 2003 Wiley‐Liss, Inc.
Estimating the length of incomplete long bones: Forensic standards from Guatemala
Authors: Wright Lori E. , Vásquez Mario A. 
Published: February 2003

AbstractWe report on new standards for estimating long bone length from incomplete bones for use in forensic and archaeological contexts in Central America. The measurements we use closely follow those defined by Steele ([1970] Personal Identification in Mass Disasters; Washington, DC: Smithsonian Institution), but we add several new landmarks. We measured the femur, humerus, tibia, and fibula of 100 Maya skeletons (68 males, 32 females) recovered from forensic exhumations. We derived the equations by regressing bone segment length on bone length, and solved for bone length to maximize the utility of the equations for taller populations. We generated equations for all segments that were significantly correlated with bone length for males, for females, and for both sexes combined, but accepted only regressions with r2 > 0.85 as reliable. Landmarks defined by muscle attachment sites were more variable in location than landmarks on articular architecture; thus we retained few equations that use these landmarks. We tested the male and combined sex equations on 36 males of unknown ethnicity exhumed from a military base in Guatemala, and found that the equations performed satisfactorily. We also evaluated the performance of equations by Steele ([1970] Personal Identification in Mass Disasters; Washington, DC: Smithsonian Institution) and Jacobs ([1992] Am J Phys Anthropol 89:333–345) on the Maya bones, and conclude that significant population variation in long bone proportions hinders their application in Central America. Am J Phys Anthropol 120:233–251, 2003. © 2003 Wiley‐Liss, Inc.
Variations in cortical material properties throughout the human dentate mandible
Authors: Schwartz‐Dabney C.L. , Dechow P.C. 
Published: February 2003

AbstractMaterial properties and their variations in individual bone organs are important for understanding bone adaptation and quality at a tissue level, and are essential for accurate mechanical models. Yet material property variations have received little systematic study. Like all other material property studies in individual bone organs, studies of the human mandible are limited by a low number of both specimens and sampled regions. The aims of this study were to determine: 1) regional variability in mandibular material properties, 2) the effect of this variability on the modeling of mandibular function, and 3) the relationship of this variability to mandibular structure and function. We removed 31 samples on both facial and lingual cortices of 10 fresh adult dentate mandibles, measured cortical thickness and density, determined the directions of maximum stiffness with a pulse transmission ultrasonic technique, and calculated elastic properties from measured ultrasonic velocities. Results showed that each of these elastic properties in the dentate human mandible demonstrates unique regional variation. The direction of maximum stiffness was near parallel to the occlusal plane within the corpus. On the facial ramus, the direction of maximum stiffness was more vertically oriented. Several sites in the mandible did not show a consistent direction of maximum stiffness among specimens, although all specimens exhibited significant orthotropy. Mandibular cortical thickness varied significantly (P < 0.001) between sites, and decreased from 3.7 mm (SD = 0.9) anteriorly to 1.4 mm posteriorly (SD = 0.1). The cortical plate was also significantly thicker (P < 0.003) on the facial side than on the lingual side. Bone was 50–100% stiffer in the longitudinal direction (E3, 20–30 GPa) than in the circumferential or tangential directions (E2 or E1; P < 0.001). The results suggest that material properties and directional variations have an important impact on mandibular mechanics. The accuracy of stresses calculated from strains and average material properties varies regionally, depending on variations in the direction of maximum stiffness and anisotropy. Stresses in some parts of the mandible can be more accurately calculated than in other regions. Limited evidence suggests that the orientations and anisotropies of cortical elastic properties correspond with features of cortical bone microstructure, although the relationship with functional stresses and strains is not clear. Am J Phys Anthropol 120:252–277, 2003. © 2003 Wiley‐Liss, Inc.
Peripartum cortisol levels and mother‐infant interactions in Japanese macaques
Authors: Bardi Massimo , Shimizu Keiko , Barrett Gordon M. , Borgognini‐Tarli Silvana M. , Huffman Michael A. 
Published: February 2003

AbstractAs evidence accumulates regarding the influence of hormones and stress‐related conditions on maternal behavior, it becomes critical to better understand the relationship between physiological stress and the ability to cope with infants. Eight Japanese macaque females were observed 3 hr per week during the first 12 weeks after parturition; fecal samples were collected twice a week from each mother, starting 4 weeks before parturition and ending 4 weeks after parturition. Time spent in contact, maternal responsiveness, latency of response, and maternal rejection were measured and correlated with peripartum excreted cortisol and estradiol metabolite levels. Two indices of peripartum hormonal status were also tested against behavior: the postpartum stress index, and the postpartum cortisol/prepartum estradiol ratio (F/E). Postpartum cortisol levels showed a positive correlation with maternal rejection. The cortisol/estradiol ratio was positively correlated with rejection and latency of response, and negatively correlated with maternal responsiveness. Prepartum cortisol levels and the postpartum stress index did not correlate with any aspect of maternal behavior. Our findings suggest that hypothalamic‐pituitary‐adrenal (HPA) axis activity per se is not enough to predict the quality of interaction between mother and infant. Only when cortisol is high relative to estradiol could it be symptomatic of a possible negative feedback response involving stress, adrenal activity, and the ability of mothers to cope with the additional problems imposed by newborns. Am J Phys Anthropol 120:298–304, 2003. © 2003 Wiley‐Liss, Inc.
Patterns of joint size dimorphism in the elbow and knee of catarrhine primates
Authors: Lague Michael R. 
Published: February 2003

AbstractDifferences in body size between conspecific sexes may incur differences in the relative size and/or shape of load‐bearing joints, potentially confounding our understanding of variation in the fossil record. More specifically, larger males may experience relatively greater limb joint stress levels than females, unless an increase in weight‐related forces is compensated for by positive allometry of articular surface areas. This study examines variation in limb joint size dimorphism (JSD) among extant catarrhines to: 1) determine whether taxa exhibit JSD beyond that expected to simply maintain geometric similarity between sexes, and 2) test whether taxa differ in JSD (relative to body size dimorphism) with respect to differences in limb use and/or phylogeny. “Joint size” was quantified for the distal humerus and distal femur of 25 taxa. Analysis of variance was used to test for differences between sexes (in joint size ratios) and among taxa (in patterns of dimorphism). Multiple regression was used to examine differences in JSD among taxa after accounting for variation in body size dimorphism (BSD) and body size. Although degrees of humeral and femoral JSD tend to be the same within species, interspecific variation exists in the extent to which both joints are dimorphic relative to BSD. While most cercopithecoids exhibit relatively high degrees of JSD (i.e., positive allometry), nonhuman hominoids exhibit degrees of JSD closer to isometry. These results may reflect a fundamental distinction between cercopithecoids and hominoids in joint design. Overall, the results make more sense (from a mechanical standpoint) when adjustments to BSD are made to account for the larger effective female body mass associated with bearing offspring. In contrast to other hominoids, modern humans exhibit relatively high JSD in both the knee and elbow (despite lack of forelimb use in weight support). Estimates of BSD based on fossil limb bones will vary according to the extant analogue chosen for comparison. Am J Phys Anthropol 120:278–297, 2003. © 2003 Wiley‐Liss, Inc.
Crystallographic report: Chlorobis(pyrrolinedithiocarbamato)antimony(III)
Authors: Lai Chian Sing , Tiekink Edward R. T. 
Published: January 2003

AbstractThe X‐ray crystal structure of Sb(S2CN(CH2)4)2Cl features a five‐coordinate geometry for antimony within a ClS4 donor set, provides evidence for a stereochemical influence exerted by the lone pair of electrons on antimony, and shows no evidence for molecular aggregation. Copyright © 2003 John Wiley & Sons, Ltd.
Crystallographic report: (4,7‐Diphenyl‐1,10‐phenanthroline) bis(pyrrolinedithiocarbamato)zinc(II) chloroform solvate
Authors: Lai Chian Sing , Tiekink Edward R. T. 
Published: January 2003

AbstractThe mononuclear structure of Zn(S2CN(CH2)4)2(4,7‐Ph2‐1,10‐phenanthroline) shows the zinc atom in each of the two independent molecules comprising the asymmetric unit to exist in a distorted octahedral geometry defined by an N2S4 donor set. Copyright © 2003 John Wiley & Sons, Ltd.
Crystallographic report: Phenyl(N,N‐di‐n‐propyldithiocarbamato)mercury(II)
Authors: Lai Chian Sing , Tiekink Edward R. T. 
Published: January 2003

AbstractThe structure of PhHg(S2CNPr2) shows a distorted linear geometry about mercury defined by a sulfur and a carbon atom. Centrosymmetric molecules aggregate via HgS interactions to form loosely associated dimers. Copyright © 2003 John Wiley & Sons, Ltd.
Alumina‐supported nickel catalyst for liquid‐phase reactions: an expedient and efficient heterogeneous catalyst for hydrogenation reactions
Authors: Jurvilliers Xavier , Schneider Raphaël , Fort Yves , Ghanbaja Jaafar 
Published: January 2003

AbstractThe preparation of a new nickel(0)/Al2O3 catalyst for hydrogenation reactions is described. The nickel(0)/Al2O3 catalysts were prepared by impregnation of alumina with a solution of a nickel(II) salt. After drying, the nickel(II) salt was reduced under mild conditions into nickel(0) using t‐BuONa‐activated sodium hydride in tetrahydrofuran at 65 °C. The nickel(0)/Al2O3 catalysts obtained were characterized by transmission electron microscopy and energy‐dispersive X‐ray spectroscopy. The supported catalysts were successfully used in solution‐phase hydrogenation of double and triple bonds. Although the activity of the nickel(0)/Al2O3 is comparable to non‐supported nickel(0) reagents, it has the advantage of being reusable more than ten times with only a slight decrease of reactivity. Copyright © 2003 John Wiley & Sons, Ltd.
Structure of azo dye organotin(IV) compounds containing a C,N‐chelating ligand
Authors: Růžička Aleš , Lyčka Antonín , Jambor Roman , Novák Petr , Čísařová Ivana , Holčapek Michal , Erben Milan , Holeček Jaroslav 
Published: January 2003

AbstractTwo complexes derived from simple azo dyes (methyl orange and para‐methyl red) and the [(2‐dimethylaminomethyl)phenyl](diphenyl)tin(IV) moiety have been prepared and their NMR, MS, IR, Raman and UV–VIS spectra were measured and X‐ray structures determined. Both compounds reveal the same structure in chloroform and in the solid state. The central tin atoms exist in a slightly distorted trans‐trigonal bipyramidal geometry. Copyright © 2003 John Wiley & Sons, Ltd.
In vitro antitumour activity of some organogermanium radioprotectors
Authors: Célariès Benoît , Gielen Marcel , de Vos Dick , Rima Ghassoub 
Published: January 2003

AbstractFour germanium derivatives of 2,2′‐oxydiethanethiol and 2,2′‐thiodiethanethiol have been synthesized and characterized by 1H and 13C NMR, mass spectroscopy and elemental analysis. The antitumour activity of one of them is comparable to those of cis‐platin and etoposide. Copyright © 2003 John Wiley & Sons, Ltd.
N‐Germyl derivatives of sulfacetamide and ortho‐(sulfonamido)phenylamines: characterization of N‐[o‐(N′,N′‐dimethylsulfonamido)phenyl]‐N‐dimesitylgermaimine
Authors: Chazalette C. , Khallaayoun A. , Rivière‐Baudet M. , El Baz F. 
Published: January 2003

AbstractTriethylgermylation of sulfacetamide occurs on the sulfonamido nitrogen in competition with the 1,2 addition of the starting triethylgermyl dimethylamine on the carbonyl group. Thermal decomposition in the presence of dimethylamine yields N‐triethylgermylsulfanilamide. Stable 1:1 sulfacetamide–DBU and 1:1 sulfacetamide–Et3N complexes were isolated and fully characterized in the course of dehydrochlorination reactions. o‐Sulfonamidophenylamine yields N,N′‐bis‐triethylgermylated derivatives, whereas o‐(N,N‐dimethylsulfonamido)phenylamine leads to monogermylated compounds. The N‐dimethylaminodimesitylgermyl derivative is thermally stable. Dehydrohalogenation of the N‐dimesitylfluorogermyl compound leads to the thermally stable but water sensitive N‐[o‐(N′,N′‐dimethylsulfonamido)phenyl]‐N‐dimesitylgermaimine. Copyright © 2003 John Wiley & Sons, Ltd.
Synthesis of two novel cobalt complexes and their crystal structures
Authors: Shi Min , Duan Wei‐Liang 
Published: January 2003

AbstractTwo new cobalt complexes were successfully synthesized from the reaction of binaphthyl Schiff base 2 with Co(OAc)2 in the presence of sodium methoxide at 80 °C for 24 h and Co(acac)3 in toluene under reflux. Their unique crystal structures are unambiguously disclosed by X‐ray analysis. Complex 3 is triclinic, space group P1, unit cell dimensions a = 10.742(2) Å, b = 11.153(2) Å, c = 12.715 Å, α = 79.865(3) °, β = 76.053 °, γ = 72.532(4) °, volume 1401.3(5) Å3, Z = 2. Complex 4 is triclinic, space group P1, unit cell dimensions a = 10.801(2) Å, b = 12.554(3) Å, c = 15.219(3) Å, α = 105.672(4) °, β = 103.048 °, γ = 104.594(4) °, volume 1824.8(7) Å3, Z = 2, calculated density 1.428 Mg m−3. Copyright © 2003 John Wiley & Sons, Ltd.
The analysis of volatile trace compounds in landfill gases, compost heaps and forest air
Authors: Maillefer Sarah , Lehr Corinne R. , Cullen William R. 
Published: January 2003

AbstractLandfill gas, cryotrapped on a loop fashioned from a length of a capillary gas chromatography (GC) column, was examined for volatile organometallic compounds (VOMCs) and for volatile organic compounds (VOCs) by using GC–mass spectrometry (MS). A large number of organic components were present and many were identified, but the only VOMCs present in high enough concentrations to be detected were trimethylstibine and tetramethyltin. The use of inductively coupled plasma (ICP)‐MS as an element‐specific detector allowed the identification of a number of other organometallic species in the landfill gas, including trimethylarsine and trimethylbismuth, and, for the first time, butyltrimethyltin and dibutyldimethyltin. The presence of molybdenum hexacarbonyl was confirmed. Gas from a large‐scale compost heap and from compost incubated in the laboratory contained iodomethane but no common VOMCs (GC–ICP‐MS). Only VOCs were present in forest air (GC–MS). Copyright © 2003 John Wiley & Sons, Ltd.
Preparation, characterization and biological activities of novel ferrocenyl‐substituted azaheterocycle compounds
Authors: Fang Jian‐Xin , Jin Zhong , Li Zeng‐Ming , Liu Wei 
Published: January 2003

Abstractα‐Haloacetylferrocene and α‐triazolylacetylferrocene have been prepared from acetylferrocene and they have proved to be useful building blocks for the synthesis of ferrocenyl propenone. Two new types of ferrocenyl vinyl triazole compound, (Z,E)‐ferrocenyl‐[1‐(1,2,4‐triazol‐1‐yl)‐2‐phenyl]‐vinyl‐ones, have been synthesized and their structures characterized by crystal X‐ray diffraction analysis. It has been shown for the first time that ferrocene, as an organometallic compound, has been introduced into bioactive triazole compounds in search of potent bioactive substances. Their biological activities are also discussed. Copyright © 2003 John Wiley & Sons, Ltd.
Combating prejudice and racism: new interventions from a functional analysis of racist language
Authors: Guerin Bernard 
Published: February 2003

AbstractBased on a conversational model of language‐use‐in‐social‐context, this article focuses on one particular form of racist and prejudiced talking that has not received enough attention—conversations in which racist statements function to maintain groups and relationships rather than seriously promote racism. Despite their casualness, such statements are still pernicious, and a range of potential interventions are proposed for this language function. These involve actively altering a community's discursive resources to include more rejoinders to racist comments. Such rejoinders must be utilized in the most appropriate way for any relationship, and this might mean polite corrections, witty repartee, strong put‐downs to silence someone making racist comments, or as counter‐jokes to racist jokes, depending upon the social context and power relations involved. Copyright © 2003 John Wiley & Sons, Ltd.
The prevention of depressive symptoms in rural Australian women
Authors: Day Chris , Kane Robert T. , Roberts Clare 
Published: February 2003

AbstractTwo interventions aimed at preventing depressive symptoms in women living in Western Australian rural communities were evaluated against a no‐intervention control condition. The standard intervention was based upon traditional cognitive‐behaviour treatments for depression; the experimental intervention was based upon prevention strategies derived from the learned helplessness model of depression (e.g. Peterson, Maier, & Seligman, 1993). Seventy‐six women were randomly assigned to either the standard or the experimental group, and a further 20 women formed a no‐intervention control group. The standard group showed a reduction in depressive symptoms at post‐test, but no effects at 6‐week or 6‐month follow‐ups. In contrast, a reduction in depressive symptoms did not appear for the experimental group until the 6‐week follow‐up at which time a less depressive attributional style was also evident; these effects were even more pronounced at the 6‐month follow‐up. The no‐intervention control group showed no changes across time. It is argued that these results support the applicability of prevention strategies based on the learned helplessness model to this population. Copyright © 2003 John Wiley & Sons, Ltd.