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Terms modified by Rev Selected AbstractsModeling Thermal and Mechanical Effects on Retention of Thiamin in Extruded FoodsJOURNAL OF FOOD SCIENCE, Issue 8 2003J.Y. Cha ABSTRACT: A model was proposed to predict separate thermal and mechanical effects of extrusion cooking on thiamin retention. Thermal effects were determined by heating small samples of wheat flour mixed with 0.30% (wt/ wt) thiamin hydrochloride isothermally at 140 °C, 151 °C, and 161 °C for different times. The calculated activation energy and rate constants at each temperature were 67.28 kJ/g mol and 0.00869/min, 0.0145/min, and 0.0224/min, respectively. The "extruder constant" was estimated as 27.7/rev, based on a matching-viscosity method. Wheat flour with 0.30% (wt/wt) thiamin was extruded at different screw speeds. Mechanical effects caused 89.7% to 94.4% of total thiamin loss. This research provides a generalized method to "fingerprint" the extrusion process. [source] Pharmacokinetics of Levetiracetam and Its Enantiomer (R)-,-ethyl-2-oxo-pyrrolidine acetamide in DogsEPILEPSIA, Issue 7 2001Nina Isoherranen Summary: ,Purpose: The new antiepileptic drug, levetiracetam (LEV, ucb LO59), is a chiral molecule with one asymmetric carbon atom whose anticonvulsant activity is highly enantioselective. The purpose of this study was to evaluate and compare the pharmacokinetics (PK) of LEV [(S)-,-ethyl-2-oxo-pyrrolidine acetamide] and its enantiomer (R)-,-ethyl-2-oxo-pyrrolidine acetamide (REV) after i.v. administration to dogs. This is the first time that the pharmacokinetics of both enantiomers has been evaluated. Methods: Optically pure LEV and REV were synthesized, and 20 mg/kg of individual enantiomers was administered intravenously to six dogs. Plasma and urine samples were collected until 24 h, and the concentrations of LEV and REV were determined by an enantioselective assay. The levels of 2-pyrrolidone- N -butyric acid, an acid metabolite of LEV and REV, were determined by high-performance liquid chromatography (HPLC). The data were used for PK analysis of LEV and REV. Results: LEV and REV had similar mean ± SD values for clearance; 1.5 ± 0.3 ml/min/kg and volume of distribution; 0.5 ± 0.1 L/kg. The half-life (t1/2) and mean residence time (MRT) of REV (t1/2, 4.3 ± 0.8 h, and MRT, 6.0 ± 1.1 h) were, however, significantly longer than those of LEV (t1/2, 3.6 ± 0.8 h, and MRT, 5.0 ± 1.2 h). The renal clearance and fraction excreted unchanged for LEV and REV were significantly different. Conclusions: In addition to the enantioselective pharmacodynamics, ,-ethyl-2-oxo-pyrrolidine acetamide has enantioselective PK. The enantioselectivity was observed in renal clearance. Because REV has more favorable PK in dogs than LEV, the higher antiepileptic potency of LEV is more likely due to intrinsic pharmacodynamic activity rather than to enantioselective PK. [source] Scaling of water flow through porous media and soilsEUROPEAN JOURNAL OF SOIL SCIENCE, Issue 1 2008K. Roth Summary Scaling of fluid flow in general is outlined and contrasted to the scaling of water flow in porous media. It is then applied to deduce Darcy's law, thereby demonstrating that stationarity of the flow field at the scale of the representative elementary volume (REV) is a critical prerequisite. The focus is on the implications of the requirement of stationarity, or local equilibrium, in particular on the validity of the Richards equation for the description of water movement through soils. Failure to satisfy this essential requirement may occur at the scale of the REV or, particularly in numerical simulations, at the scale of the model discretization. The latter can be alleviated by allocation of more computational resources and by working on a finer-grained representation. In contrast, the former is fundamental and leads to an irrevocable failure of the Richards equation as is observed with infiltration instabilities that lead to fingered flow. [source] Numerical estimation of REV and permeability tensor for fractured rock masses by composite element methodINTERNATIONAL JOURNAL FOR NUMERICAL AND ANALYTICAL METHODS IN GEOMECHANICS, Issue 12 2008S.-H. Chen Abstract The Monte Carlo method is used to generate parent stochastic discrete fracture network, from which a series of fractured rock samples of different sizes and orientations are extracted. The fracture network combined with a regular grid forms composite element mesh of the fractured rock sample, in which each composite element is composed of sub-elements incised by fracture segments. The composite element method (CEM) for the seepage is implemented to obtain the nodal hydraulic potential as well as the seepage flow rates through the fractured rock samples. The application of CEM enables a large quantity of stochastic tests for the fractured rock samples because the pre-process is facilitated greatly. By changing the sizes and orientations of the samples, the analysis of the seepage characteristics is realized to evaluate the variation of the permeability components, the existence of the permeability tensor and the representative element volume. The feasibility and effectiveness are illustrated in a numerical example. Copyright © 2008 John Wiley & Sons, Ltd. [source] Influence of Iron Ore Indirect Reduction on the Transfer Process in Porous MediaASIA-PACIFIC JOURNAL OF CHEMICAL ENGINEERING, Issue 3-4 2006Ming-Chun Li The solid material used in most metallurgical reactions can be considered as porous media consisting of the accumulated pellets. When the fluid flows through the porous media, various chemical reactions may occur between the fluid and solid matrix which have a significant effect on the transfer process in the porous media. Based on derivation of the overall rate formulation of the representative elementary volume (REV), a mathematical model describing the transfer process in the porous media was established. The model was tested numerically by applying the implicit formulation finite volume method. The predictions were validated by comparison with the experimental results and literature data. For the indirect reduction of iron ores, the influence of the flow rate, the particle size, reaction rate, the dimension of the reactor, and the ratio of the Thielet number to the Peclet number on the distribution of gas concentration and the solid conversion degree were analyzed. [source] A Dinuclear Double-Stranded Oxido Complex of ReV with a Bis(benzene- o -dithiolato) LigandEUROPEAN JOURNAL OF INORGANIC CHEMISTRY, Issue 27 2009Jorge S. Gancheff Abstract The reaction of [ReOCl3(PPh3)2] with 1,2-bis(2,3-dimercaptobenzamido)ethane (H4 - 1) in the presence of Na2CO3 in methanol under anaerobic conditions affords the dinuclear ReV oxido complex [PPh4]2[ReO(1)]2 containing two distorted square-pyramidal {ReVOS4} units bridged by the ligand strands in a double-stranded fashion. The coordinationgeometry around the metal centers is similar to the one observed for [ReO(bdt)2],. The ReS4 planes are arranged in a coplanar fashion and are not twisted around the metal,metal vector, which prevents the complex to adopt a helical structure. Luminescence studies show the presence of emission bands, which are assigned to singlet-singlet transitions exhibiting very fast decays (ca. 10 ns). Theoretical Density Functional (DFT) studies on geometry and electronic properties were performed employing the hybrid B3LYP and PBE1PBE functionals. While the general trends observed in the experimental data are well reproduced in all cases, a good agreement was obtained using PBE1PBE, in particular for the Re,S bonds. Natural Bond Orbitals (NBO) analysis indicates the presence of polarized Re,O and Re,S bonds, both of them polarized toward the non-metal. The calculation show that the molecular orbitals of the ReV are doubly degenerated, the occupied 5d orbital of rhenium lying beneath occupied sulfur-based MOs due to the rigid geometry imposed by the C,C backbone of the bis(benzene- o -dithiolato) ligands. The origin of all absorption bands is ascribed to a ligand-to-metal charge transfer (LMCT), in which occupied sulfur-based orbitals and unoccupied rhenium-centered orbitals are involved.(© Wiley-VCH Verlag GmbH & Co. KGaA, 69451 Weinheim, Germany, 2009) [source] Streamflow estimation using optimal regional dependency functionHYDROLOGICAL PROCESSES, Issue 25 2009Abdüsselam Altunkaynak Abstract The determination of spatial dependency of regionalized variable (ReV) is important in engineering studies. Regional dependency function that leads to calculation of weighting coefficients is required in order to make regional or point-wise estimations. After obtaining this dependency function, it is possible to complete missing records in the time series and locate new measurement station. Also determination of regional dependency function is also useful to understand the regional variation of ReV. Point Cumulative Semi-Variogram (PCSV) is another methodology to understand the regional dependency of ReV related to the magnitude and the location. However, this methodology is not useful to determine the weighting coefficient, which is required to make regional and point-wise estimations. However, in Point Semi-Variogram (PSV) proposed here, weighting coefficient depends on both magnitude and location. Although the regional dependency function has a fluctuating structure in PSV approach, this function gradually increases with distance in PCSV. The study area is selected in Mississippi river basin with 38 streamflow stations used for PCSV application before. It is aimed to compare two different geostatistical models for the same data set. PSV method has an ability to determine the value of variable along with optimum number of neighbour stations and influence radius. PSV and slope PSV approaches are compared with the PCSV. It was shown that slope slope point semi-variogram (SPSV) approaches had relative error below 5%, and PSV and PCSV methods revealed relative errors below 10%. Copyright © 2009 John Wiley & Sons, Ltd. [source] Bioenergetics and the epigenome: Interface between the environment and genes in common diseasesDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 2 2010Douglas C. Wallace Abstract Extensive efforts have been directed at using genome-wide association studies (GWAS) to identify the genes responsible for common metabolic and degenerative diseases, cancer, and aging, but with limited success. While environmental factors have been evoked to explain this conundrum, the nature of these environmental factors remains unexplained. The availability of and demands for energy constitute one of the most important aspects of the environment. The flow of energy through the cell is primarily mediated by the mitochondrion, which oxidizes reducing equivalents from hydrocarbons via acetyl-CoA, NADH + H+, and FADH2 to generate ATP through oxidative phosphorylation (OXPHOS). The mitochondrial genome encompasses hundreds of nuclear DNA (nDNA)-encoded genes plus 37 mitochondrial DNA (mtDNA)-encoded genes. Although the mtDNA has a high mutation rate, only milder, potentially adaptive mutations are introduced into the population through female oocytes. In contrast, nDNA-encoded bioenergetic genes have a low mutation rate. However, their expression is modulated by histone phosphorylation and acetylation using mitochondrially-generated ATP and acetyl-CoA, which permits increased gene expression, growth, and reproduction when calories are abundant. Phosphorylation, acetylaton, and cellular redox state also regulate most signal transduction pathways and activities of multiple transcription factors. Thus, mtDNA mutations provide heritable and stable adaptation to regional differences while mitochondrially-mediated changes in the epigenome permit reversible modulation of gene expression in response to fluctuations in the energy environment. The most common genomic changes that interface with the environment and cause complex disease must, therefore, be mitochondrial and epigenomic in origin. © 2010 Wiley-Liss, Inc. Dev Disabil Res Rev 2010;16:114,119. [source] The neurologic manifestations of mitochondrial diseaseDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 2 2010Sumit Parikh Abstract The nervous system contains some of the body's most metabolically demanding cells that are highly dependent on ATP produced via mitochondrial oxidative phosphorylation. Thus, the neurological system is consistently involved in patients with mitochondrial disease. Symptoms differ depending on the part of the nervous system affected. Although almost any neurological symptom can be due to mitochondrial disease, there are select symptoms that are more suggestive of a mitochondrial problem. Certain symptoms that have become sine qua non with underlying mitochondrial cytopathies can serve as diagnostic "red-flags." Here, the typical and atypical presentations of mitochondrial disease in the nervous system are reviewed, focusing on "red flag" neurological symptoms as well as associated symptoms that can occur in, but are not specific to, mitochondrial disease. The multitudes of mitochondrial syndromes are not reviewed in-depth, though a select few are discussed in some detail. © 2010 Wiley-Liss, Inc. Dev Disabil Res Rev 2010;16:120,128. [source] The use of neuroimaging in the diagnosis of mitochondrial diseaseDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 2 2010Seth D. Friedman Abstract Mutations in nuclear and mitochondrial DNA impacting mitochondrial function result in disease manifestations ranging from early death to abnormalities in all major organ systems and to symptoms that can be largely confined to muscle fatigue. The definitive diagnosis of a mitochondrial disorder can be difficult to establish. When the constellation of symptoms is suggestive of mitochondrial disease, neuroimaging features may be diagnostic and suggestive, can help direct further workup, and can help to further characterize the underlying brain abnormalities. Magnetic resonance imaging changes may be nonspecific, such as atrophy (both general and involving specific structures, such as cerebellum), more suggestive of particular disorders such as focal and often bilateral lesions confined to deep brain nuclei, or clearly characteristic of a given disorder such as stroke-like lesions that do not respect vascular boundaries in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode (MELAS). White matter hyperintensities with or without associated gray matter involvement may also be observed. Across patients and discrete disease subtypes (e.g., MELAS, Leigh syndrome, etc.), patterns of these features are helpful for diagnosis. However, it is also true that marked variability in expression occurs in all mitochondrial disease subtypes, illustrative of the complexity of the disease process. The present review summarizes the role of neuroimaging in the diagnosis and characterization of patients with suspected mitochondrial disease. © 2010 Wiley-Liss, Inc. Dev Disabil Res Rev 2010;16:129,135. [source] Anomalous development of brain structure and function in spina bifida myelomeningoceleDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 1 2010Jenifer Juranek Abstract Spina bifida myelomeningocele (SBM) is a specific type of neural tube defect whereby the open neural tube at the level of the spinal cord alters brain development during early stages of gestation. Some structural anomalies are virtually unique to individuals with SBM, including a complex pattern of cerebellar dysplasia known as the Chiari II malformation. Other structural anomalies are not necessarily unique to SBM, including altered development of the corpus callosum and posterior fossa. Within SBM, tremendous heterogeneity is reflected in the degree to which brain structures are atypical in qualitative appearance and quantitative measures of morphometry. Hallmark structural features of SBM include overall reductions in posterior fossa and cerebellum size and volume. Studies of the corpus callosum have shown complex patterns of agenesis or hypoplasia along its rostral-caudal axis, with rostrum and splenium regions particularly susceptible to agenesis. Studies of cortical regions have demonstrated complex patterns of thickening, thinning, and gyrification. Diffusion tensor imaging studies have reported compromised integrity of some specific white matter pathways. Given equally complex ocular motor, motor, and cognitive phenotypes consisting of relative strengths and weaknesses that seem to align with altered structural development, studies of SBM provide new insights to our current understanding of brain structure,function associations. © 2010 Wiley-Liss, Inc. Dev Disabil Res Rev 2010;16:23,30. [source] The cognitive phenotype of spina bifida meningomyeloceleDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 1 2010Maureen Dennis Abstract A cognitive phenotype is a product of both assets and deficits that specifies what individuals with spina bifida meningomyelocele (SBM) can and cannot do and why they can or cannot do it. In this article, we review the cognitive phenotype of SBM and describe the processing assets and deficits that cut within and across content domains, sensory modality, and material, including studies from our laboratory and other investigations. We discuss some implications of the SBM cognitive phenotype for assessment, rehabilitation, and research. © 2010 Wiley-Liss, Inc. Dev Disabil Res Rev 2010;16:31,39. [source] Turner syndrome: Neuroimaging findings: Structural and functionalDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 4 2009Ronan Mullaney Abstract Neuroimaging studies of Turner syndrome can advance our understanding of the X chromosome in brain development, and the modulatory influence of endocrine factors. There is increasing evidence from neuroimaging studies that TX individuals have significant differences in the anatomy, function, and metabolism of a number of brain regions; including the parietal lobe; cerebellum, amygdala, hippocampus; and basal ganglia; and perhaps differences in "connectivity" between frontal and parieto-occipital regions. Finally, there is preliminary evidence that genomic imprinting, sex hormones and growth hormone have significant modulatory effects on brain maturation in TS. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:279,283. [source] The cognitive phenotype in Klinefelter syndrome: A review of the literature including genetic and hormonal factorsDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 4 2009Richard Boada Abstract Klinefelter syndrome (KS) or 47,XXY occurs in ,1 in 650 males. Individuals with KS often present with physical characteristics including tall stature, hypogonadism, and fertility problems. In addition to medical findings, the presence of the extra X chromosome can lead to characteristic cognitive and language deficits of varying severity. While a small, but significant downward shift in mean overall IQ has been reported, the general cognitive abilities of patients with KS are not typically in the intellectual disability range. Most studies support that males with KS have an increased risk of language disorders and reading disabilities. Results of other studies investigating the relationship between verbal and nonverbal/spatial cognitive abilities have been mixed, with differing results based on the age and ascertainment method of the cohort studied. Executive function deficits have been identified in children and adults with KS, however, the research in this area is limited and further investigation of the neuropsychological profile is needed. In this article, we review the strengths and weaknesses of previous cognitive and neuropsychological studies in males with KS in childhood and adulthood, provide historical perspective of these studies, and review what is known about how hormonal and genetic factors influence cognitive features in 47,XXY/KS. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:284,294. [source] Structural and functional neuroimaging in Klinefelter (47,XXY) syndrome: A review of the literature and preliminary results from a functional magnetic resonance imaging study of languageDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 4 2009Kyle Steinman Abstract Klinefelter (47,XXY) syndrome (KS), the most common form of sex-chromosomal aneuploidy, is characterized by physical, endocrinologic, and reproductive abnormalities. Individuals with KS also exhibit a cognitive/behavioral phenotype characterized by language and language-based learning disabilities and executive and attentional dysfunction in the setting of normal general intelligence. The underlying neurobiologic mechanisms are just now beginning to be elucidated through structural and functional neuroimaging. Here, we review the literature of structural and functional neural findings in KS identified by neuroimaging and present preliminary results from a functional magnetic resonance imaging study examining brain activity during a verb generation task in KS. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:295,308. [source] Prevalence and epidemiologic characteristics of FASD from various research methods with an emphasis on recent in-school studiesDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 3 2009Philip A. May Abstract Researching the epidemiology and estimating the prevalence of fetal alcohol syndrome (FAS) and other fetal alcohol spectrum disorders (FASD) for mainstream populations anywhere in the world has presented a challenge to researchers. Three major approaches have been used in the past: surveillance and record review systems, clinic-based studies, and active case ascertainment methods. The literature on each of these methods is reviewed citing the strengths, weaknesses, prevalence results, and other practical considerations for each method. Previous conclusions about the prevalence of FAS and total FASD in the United States (US) population are summarized. Active approaches which provide clinical outreach, recruitment, and diagnostic services in specific populations have been demonstrated to produce the highest prevalence estimates. We then describe and review studies utilizing in-school screening and diagnosis, a special type of active case ascertainment. Selected results from a number of in-school studies in South Africa, Italy, and the US are highlighted. The particular focus of the review is on the nature of the data produced from in-school methods and the specific prevalence rates of FAS and total FASD which have emanated from them. We conclude that FAS and other FASD are more prevalent in school populations, and therefore the general population, than previously estimated. We believe that the prevalence of FAS in typical, mixed-racial, and mixed-socioeconomic populations of the US is at least 2 to 7 per 1,000. Regarding all levels of FASD, we estimate that the current prevalence of FASD in populations of younger school children may be as high as 2,5% in the US and some Western European countries. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009; 15:176,192. [source] Prevention of fetal alcohol spectrum disorders,DEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 3 2009R. Louise Floyd Abstract Alcohol use among women of childbearing age is a leading, preventable cause of birth defects and developmental disabilities in the United States. Although most women reduce their alcohol use upon pregnancy recognition, some women report drinking during pregnancy and others may continue to drink prior to realizing they are pregnant. These findings emphasize the need for effective prevention strategies for both pregnant and nonpregnant women who might be at risk for an alcohol-exposed pregnancy (AEP). This report reviews evidence supporting alcohol screening and brief intervention as an effective approach to reducing problem drinking and AEPs that can lead to fetal alcohol spectrum disorders. In addition, this article highlights a recent report of the National Task Force on Fetal Alcohol Syndrome and Fetal Alcohol Effect that describes effective interventions to reduce alcohol use and AEPs, and outlines recommendations on promoting and improving these strategies. Utilizing evidence-based alcohol screening tools and brief counseling for women at risk for an AEP and other effective population-based strategies can help achieve future alcohol-free pregnancies. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:193,199. [source] Constraint-induced movement therapy (CIMT): Pediatric applicationsDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 2 2009Kathleen Brady Abstract The purpose of this article is to describe theoretical and research bases for constraint-induced movement therapy (CIMT), to discuss key features and variations in protocols currently in use with children, and to review the results of studies of efficacy. CIMT has been found to be an effective intervention for increasing functional use of the hemiparetic upper extremity in adults with chronic disability from stroke. CIMT developed out of behavioral research on the phenomenon of "learned nonuse" of an upper extremity, commonly observed following sensory and/or motor CNS injury, in which failure to regain use persists even after a period of partial recovery. CIMT includes three key elements: (1) constraining the use of the less-impaired upper extremity (UE); (2) intensive, repetitive daily therapist-directed practice of motor movements with the impaired UE for an extended period (2,3 weeks); and (3) shaping of more complex action patterns through a process of rewarding successive approximations to the target action. Mechanisms responsible for success are thought to be separate but complementary, that is, operant conditioning (reversal of learned nonuse) and experience-driven cortical reorganization. CIMT has recently been extended to children with hemiparesis secondary to perinatal stroke or other CNS pathology. Numerous case studies, as well as a small number of randomized controlled or controlled clinical trials have reported substantial gains in functional use of the hemiplegic UE following CIMT with children. Protocols vary widely in terms of type of constraint used, intensity and duration of training, and outcome measures. In general, all report gains in functional use, with minimal or no adverse effects. Continued research is needed, to clarify optimal protocol parameters and to further understand mechanisms of efficacy. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:102,111. [source] Activity-based restorative therapies: Concepts and applications in spinal cord injury-related neurorehabilitationDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 2 2009Cristina L. Sadowsky Abstract Physical rehabilitation following spinal cord injury-related paralysis has traditionally focused on teaching compensatory techniques, thus enabling the individual to achieve day-to-day function despite significant neurological deficits. But the concept of an irreparable central nervous system (CNS) is slowly being replaced with evidence related to CNS plasticity, repair, and regeneration, all related to persistently maintaining appropriate levels of neurological activity both below and above the area where the damage occurred. It is now possible to envision functional repair of the nervous system by implementing rehabilitative interventions. Making the transition from "bench to bedside" requires careful analysis of existing basic science evidence, strategic focus of clinical research, and pragmatic implementation of new therapeutic tools. Activity, defined as both function specific motor task and exercise appears to be a necessity for optimization of functional, metabolic, and neurological status in chronic paralysis. Crafting a comprehensive rehabilitative intervention focused on functional improvement through neurological gains seems logical. The terms activity-based restorative therapies, activity-based therapies, and activity-based rehabilitation have been coined in the last 10 years to describe a new fundamental approach to deficits induced by neurological paralysis. The goal of this approach is to achieve activation of the neurological levels located both above and below the injury level using rehabilitation therapies. This article reviews basic and clinical science evidence pertaining to implementation of physical activity and exercise as a therapeutic tool in the management of chronic spinal cord-related neurological paralysis. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:112,116. [source] Mathematical skills in Williams syndrome: Insight into the importance of underlying representationsDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 1 2009Kirsten O'Hearn Abstract Williams syndrome (WS) is a developmental disorder characterized by relatively spared verbal skills and severe visuospatial deficits. Serious impairments in mathematics have also been reported. This article reviews the evidence on mathematical ability in WS, focusing on the integrity and developmental path of two fundamental representations, namely those that support judgments of "how much" (i.e., magnitude) and "how many" (i.e., number of objects). Studies on magnitude or "number line" representation in WS suggest that this core aspect of mathematical ability, is atypical in WS throughout development, causing differences on some but not all aspects of math. Studies on the representation of small numbers of objects in WS are also reviewed, given the proposed links between this type of representation and early number skills such as counting. In WS, representation appears to be relatively typical in infancy but limitations become evident by maturity, suggesting a truncated developmental trajectory. The math deficits in WS are consistent with neurological data indicating decreased gray matter and hypoactivation in parietal areas in WS, as these areas are implicated in mathematical processing as well as visuospatial abilities and visual attention. In spite of their deficits in core mathematical representations, people with WS can learn many mathematical skills and show some strengths, such as reading numbers. Thus individuals with WS may be able to take advantage of their relatively strong verbal skills when learning some mathematical tasks. The uneven mathematical abilities found in persons with WS provide insight into not only appropriate remediation for this developmental disorder but also into the precursors of mathematical ability, their neural substrates, and their developmental importance. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:11,20. [source] A review of mathematical learning disabilities in children with fragile X syndromeDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 1 2009Melissa M. Murphy Abstract The prevalence rate of mathematical learning disabilities (MLD) among children with fragile X syndrome who do not meet criteria for intellectual and developmental disabilities (,50% of female children) exceeds the rate reported in the general population. The purpose of this article is two-fold: (1) to review the findings on MLD in persons with fragile X syndrome; and (2) to discuss fragile X syndrome as a possible model for understanding pathways to MLD. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:21,27. [source] Mathematical development in spina bifidaDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 1 2009Lianne H. English Abstract Spina bifida (SB) is a neural tube defect diagnosed before or at birth that is associated with a high incidence of math disability often without co-occurring difficulties in reading. SB provides an interesting population within which to examine the development of mathematical abilities and disability across the lifespan and in relation to the deficits in visual-spatial processing that are also associated with the disorder. An overview of math and its cognitive correlates in preschoolers, school-age children and adults with SB is presented including the findings from a longitudinal study linking early executive functions in infancy to the development of later preschool and school age math skills. These findings are discussed in relation to socio-historical perspectives on math education and implications for intervention and directions for further research are presented. © 2009 Wiley-Liss, Inc. Dev Disabil Res Rev 2009;15:28,34. [source] Inhibitory functioning across ADHD subtypes: Recent findings, clinical implications, and future directionsDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 4 2008Zachary W. Adams Abstract Although growing consensus supports the role of deficient behavioral inhibition as a central feature of the combined subtype of ADHD (ADHD/C; Barkley 1997 Psychol Bull 121:65,94; Nigg 2001 Psychol Bull 127:571,598), little research has focused on how this finding generalizes to the primarily inattentive subtype (ADHD/I). This question holds particular relevance in light of recent work suggesting that ADHD/I might be better characterized as a disorder separate from ADHD/C (Diamond 2005 Dev Psychopathol 17:807,825; Milich et al. 2001 Clin Psychol Sci Pract 8:463,488). This article describes major findings in the area of inhibitory performance in ADHD and highlights recent research suggesting important areas of divergence between the subtypes. In particular, preliminary findings point to potential differences between the subtypes with respect to how children process important contextual information from the environment, such as preparatory cues that precede responses and rewarding or punishing feedback following behavior. These suggestive findings are discussed in the context of treatment implications, which could involve differential intervention approaches for each subtype targeted to the specific deficit profiles that characterize each group of children. Future research avenues aimed toward building a sound theoretical model of ADHD/I and a better understanding of its relation to ADHD/C are also presented. Specifically, investigators are encouraged to continue studying the complex interplay between inhibitory and attentional processes, as this area seems particularly promising in its ability to improve our understanding of the potentially distinct pathologies underlying the ADHD subtypes. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:268,275. [source] The neurobiological profile of girls with ADHDDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 4 2008E. Mark Mahone Abstract Since boys are more commonly diagnosed with Attention Deficit Hyperactivity Disorder (ADHD) than girls, the majority of theories and published research studies of ADHD have been based on samples comprised primarily (or exclusively) of boys. While psychosocial impairment in girls with ADHD is well established, the neuropsychological and neurobiological basis of these deficits is less consistently observed. There is growing evidence that boys' and girls' brains develop and mature at different rates, suggesting that the trajectory of early anomalous brain development in ADHD may also be sex-specific. It remains unclear, however, whether earlier brain maturation observed in girls with ADHD is protective. In this review, we outline the current theory and research findings that seek to establish a unique neurobiological profile of girls with ADHD, highlighting sex differences in typical brain development and among children with ADHD. The review highlights findings from neurological, neurocognitive, and behavioral studies. Future research directions are suggested, including the need for longitudinal neuroimaging and neurobehavioral investigation beginning as early as the preschool years, and continuing through adolescence and adulthood, with consideration of identified sex differences in the development of ADHD. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:276,284. [source] Attention deficits, Attention-Deficit Hyperactivity Disorder, and intellectual disabilitiesDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 4 2008Curtis K. Deutsch Abstract Attention-Deficit Hyperactivity Disorder (ADHD) and its earlier nosologic classifications have been extensively investigated since the 1960s, with PubMed listings alone exceeding 13,000 entries. Strides have been made in the diagnosis and treatment of ADHD in individuals with intellectual function in the normal range, as described in companion reviews in this special issue. In contrast, comparatively little is known about ADHD in intellectual developmental disabilities (IDD) despite the possibility that ADHD is statistically overrepresented among individuals with IDD (Pearson et al. 1997 Attention-deficit/hyperactivity disorder in mental retardation: nature of attention deficits. In: Burack J, Enns J, editors. Attention, development, and psychopathology. New York: Guilford Press. p 205,229; Pearson et al. 2000 Am. J. Ment. Retard. 105:236,251). Here, we provide a review of diagnostic controversies in ADHD with IDD, and discuss several topics that are currently attracting research efforts in the field. These include behavioral phenotyping and attempts to come to grips with problems of behavioral and etiological heterogeneity. Additionally, we consider issues relating to methodologically sound assessment of attention disorders and evidence-based intervention procedures that may clarify and/or ameliorate attention deficits in individuals with IDD. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:285,292. [source] Neurodevelopmental outcomes of children with low-grade gliomasDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 3 2008M. Douglas Ris Abstract As a group, children with low-grade gliomas (LGGs) enjoy a high rate of long-term survival and do not require the intensity of neurotoxic treatments used with higher risk pediatric brain tumors. Because they are generally considered to have favorable neurobehavioral outcomes, they have not been studied as thoroughly as higher-grade brain tumors by late-effects researchers. In this article, we review the literature on the neurobehavioral effects associated with low-grade gliomas and conclude that, (1) this is a large, understudied group of survivors of pediatric brain tumors; (2) recent small- and large-scale studies document increased risk in multiple cognitive-behavioral domains after treatment for LGGs compared with healthy peers; (3) such risk is not uniform but varies with tumor location and treatments; and (4) a life span development perspective is essential to a complete understanding of the risks faced by these children. More research on the most efficacious biopsychosocial treatment models for improving the outcomes of survivors of low-grade glioma is recommended, informed by a better understanding of theireducational needs. Investigations of genetic influences on outcome as well as prospective studies of these patients as they age are also recommended. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:196,202. [source] Neurodevelopmental impact on children treated for medulloblastoma: A review and proposed conceptual modelDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 3 2008Shawna L. Palmer Abstract The population of survivors following diagnosis and treatment for medulloblastoma is thankfully on the rise. An increased focus on the quality of that survivorship has expanded the concept of cure to include efforts aimed at improving long-term cognitive outcome. It is well established in the literature that decline in overall intellect and academic performance is experienced by a majority of those undergoing treatment for pediatric medulloblastoma. This decline is believed to be secondary to decline in core cognitive abilities, which in turn are related to underlying damage to neuroanatomical substrates. A review of research on neurodevelopmental impacts following diagnosis and treatment for pediatric medulloblastoma is presented. Particular consideration is given to studies recently published that also reflect critical collaboration among those within the fields of neuropsychology and neuro-imaging. Results from the review are combined within a conceptual model upon which to guide future research and clinical efforts. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:203,210. [source] Pharmacogenetics of the neurodevelopmental impact of anticancer chemotherapyDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 3 2008Philippe Robaey Abstract Pharmacogenetics holds the promise of minimizing adverse neurodevelopmental outcomes of cancer patients by identifying patients at risk, enabling the individualization of treatment and the planning of close follow-up and early remediation. This review focuses first on methotrexate, a drug often implicated in neurotoxicity, especially when used in combination with brain irradiation. The second focus is on glucocorticoids that have been found to be linked to adverse developmental effects in relation with the psychosocial environment. For both examples, we review how polymorphisms of genes encoding enzymes involved in specific mechanisms of action could moderate adverse neurodevelopmental consequences, eventually through common final pathways such as oxidative stress. We discuss a multiple hit model and possible strategies required to rise to the challenge of this integrative research. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:211,220. [source] The nervous system and gastrointestinal functionDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 2 2008Muhammad A. Altaf Abstract The enteric nervous system is an integrative brain with collection of neurons in the gastrointestinal tract which is capable of functioning independently of the central nervous system (CNS). The enteric nervous system modulates motility, secretions, microcirculation, immune and inflammatory responses of the gastrointestinal tract. Dysphagia, feeding intolerance, gastroesophageal reflux, abdominal pain, and constipation are few of the medical problems frequently encountered in children with developmental disabilities. Alteration in bowel motility have been described in most of these disorders and can results from a primary defect in the enteric neurons or central modulation. The development and physiology of the enteric nervous system is discussed along with the basic mechanisms involved in controlling various functions of the gastrointestinal tract. The intestinal motility, neurogastric reflexes, and brain perception of visceral hyperalgesia are also discussed. This will help better understand the pathophysiology of these disorders in children with developmental disabilities. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:87,95. [source] Neurophysiology of hunger and satietyDEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 2 2008Pauline M. Smith Abstract Hunger is defined as a strong desire or need for food while satiety is the condition of being full or gratified. The maintenance of energy homeostasis requires a balance between energy intake and energy expenditure. The regulation of food intake is a complex behavior. It requires discrete nuclei within the central nervous system (CNS) to detect signals from the periphery regarding metabolic status, process and integrate this information in a coordinated manner and to provide appropriate responses to ensure that the individual does not enter a state of positive or negative energy balance. This review of hunger and satiety will examine the CNS circuitries involved in the control of energy homeostasis as well as signals from the periphery, both hormonal and neural, that convey pertinent information regarding short-term and long-term energy status of the individual. © 2008 Wiley-Liss, Inc. Dev Disabil Res Rev 2008;14:96,104. [source] | |||||||||||||||||||