bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Validity of Psychometric Studies on Children with Spina Bifida
Authors: Tew Brian , Laurence K. M. 
Published: July 2010

SUMMARYThe iq scores of 57 cases of spina bifida cystica on tests administered after an interval of five years were compared with earlier scores. The comparison showed highly significant correlations for nine cases with encephalocele, 16 with meningocele and 32 with myelocele, indicating that stability rather than variability is characteristic of the iq scores of children with malformations of the central nervous system.RÉSUMÉLes résultats de 57 enfants atteints de spina bifida aux tests d'intelligence relevés sur une période de cinq ans ont été compares. Les résultats montrent une correlation hautement significative pour 9 cas d'encéphalocèles, 16 cas de méningocèles et 32 cas de myélocèles, indiquant que la stabilité plus que la variation est caractéristique des qi d'enfants présentant des malformations du système nerveux central.ZUSAMMENFASSUNGDie Ergebnisse von Intelligenztesten, die an 57 Kindern mit Spina bifida cystica über eine Zeitspanne von fünf Jahren durchgeführt worden waren, wurden verglichen. Die Ergebnisse zeigten hochsignifikante Korrelationen für neun Fälle mit Encephalocele, 16 Fälle mit Meningocele und 32 Fälle mit Myelozele, was darauf hinweist, daß für die iq‐Scores der Kinder mit Mißbildungen im Bereich des zns eher Stabilität als Variation charakteristisch ist.RESUMENSe compararon los puntajes de 57 niños con espina bifida cistica en tests de inteligencia aplicados durante un intervalo de 5 años. Los resultados mostraron correlaciones altamente significativas en 9 casos de encefalocele, 16 casos de meningocele y 32 casos de mielocele indicando una estabilidad más bien que una variación de las caracteristicas de los puntajes de ci en niños con malformaciones de snc.
Tay‐Sachs Disease: Neurophysiological Studies in 20 Children
Authors: Pampiglione G. , Privett G. , Harden Ann 
Published: July 2010

SUMMARYTwenty children with proven Tach‐Sachs disease underwent neurophysiological investigations during the course of their illness. Although the enzyme defect is demonstrable before birth, the electroencephalograms showed relatively mild abnormalities during the first year of life, but there was a rapid progressive deterioration thereafter until death, which usually occurred before the age of four years. The electroretinogram remained unaffected through the course of the disease, in keeping with the preservation of function of retinal receptor elements, although the ganglion cell layer was grossly affected. Visual evoked responses were recordable in the early stages of the disease, but after 16 months no cortical response to flashes of light could be detected. This combination of neurophysiological features and their evolution are of interest as they differ from those of other neurometabolic diseases of early childhood.ZUSAMMENFASSUNGBei 20 Kindern mit diagnostiziertem Morbus Tay‐Sachs wurden Untersuchung vorgenommen während ihres Krankheitsverlaufs neurophysiologische. Obgleich dieser Enzymdefekt schon vor der Geburt feststellbar ist, finden sich innerhalb des ersten Lebensjahres nur relativ milde Abnormitäten im eeg mit einer raschen Verschlechterung danach bis zum Tode, der gewöhnlich vor Erreichen des Alters von vier Jahren eintritt. Das Elektroretinogramm (erg) blieb während des gesamten Krankheitsverlaufs unbeeinträchtigt mit der Erhaltung der Funktion der retinalen Receptorelemente, obgleich die Ganglienzellschicht stark betroffen ist. Die visuell evozierten Reaktionen (ver) waren in den frühen Abschnitten noch feststellbar, aber über den 16. Lebensmonat hinaus konnten keine corticalen Reaktionen auf Lichtblitze mehr beobachtet werden. Diese Kombination neurophysiologischer Befunde und ihrer Entwicklung sind von Interesse, da sie sich von denen anderer neurometabolischer Erkrankungen der frühen Kindheit unterscheiden.RESUMENSe realizaron investigaciones neurofisiológicas en 20 niños con enfermedad de Tay‐Sachs probada, durante el curso de su enfermedad. Aunque el defecto enzimático es demonstrable antes del nacimiento, el eeg mostró anomalías relativamente moderadas, con una rápida y progresiva deterioración posterior hasta la muerte, que habitualmente ocurrió antes de los cuatro años. El electroretinograma no se afectó durante todo el curso de la enfermedad, paralelamente con la preservación de la función de los elementos retinianos receptores, a pesar de estar muy afectada la capa de células ganglionares. Las respuestas visuales evocadas podían detectarse en los estadíos precoces, pero no se pudo detectar ninguna respuesta cortical a relámpagos luminosos después de los 16 meses. Esta combinación de características neurofisiológicas y su evolución tienen su interés, pues difiere de las de otras enfermedades neurometabólicas de la primera infancia.
Fatal Hepatic Failure in a Child Treated with Phenytoin and Valproic Acid
Authors: Höjer B. , Rane A. 
Published: July 2010

SUMMARYA fatal case of hepatic failure in a five‐year‐old girl who had been treated with phenytoin and valproic acid is presented. The report emphasizes the need for caution in the use of valproate, especially in children with altered liver function, and in combination with phenytoin or phenobarbitone.RÉSUMÉInsuffisance hépatique mortelle chez un enfant traité par phénytoine et acide valproïqueL'article rapporte le cas d'une insuffisance hépatique mortelle chez une fillette de cinq ans qui avait été traitée par phénytoïne et acide valproïque. Les auteurs insistent sur la nécessité de prudence dans l'usage du valproate, spécialement chez les enfants ayant une fonction hépatique altérée et en cas d'association avec phénytoïne ou phénobarbital.ZUSAMMENFASSUNGTödliches Leberversagen bei einem Kind, das mit Phenytoin und Valproat behandelt wurdeEs wird über ein fünfjähriges Mädchen berichtet, das mit Phenytoin und Valproat behandelt worden war und an einem Leberversagen gestorben ist. In dem Bericht wird die Notwendigkeit unterstrichen, bei der Anwendung von Valproat vorsichtig zu sein, insbesondere bei Kindern mit veränderter Leberfunktion und in Kombination mit Phenytoin oder Phenobarbital.RESUMENFallo hepatico fatal en un niño tratado con fenitoina y acido valproicoSe presenta un caso fatal de fallo hepatico en una niña de cinco años de edad que habia sido tratada con fenitoina y acido valproico. La comunicacion insiste en la necesidad de ser cauteloso en el uso del valproato, especialmente en niños con funcion hepatica alterada y en combinacion con fenitoina o fenobarbital.
75,000 SEVERELY DISABLED CHILDREN
Authors: Bradshaw Jonathan , Lawton Dorothy 
Published: July 2010

SUMMARYThis article explores the epidemiological potential of the computerised record of applications to the Family Fund, which contains details of75,000 children with severe disabilities in the UK. It illustrates the strengths and weaknesses of the record, with an analysis of variation in the characteristics of the children and their families by principal handicapping conditions.RÉSUMÉ75 mille enfanls en incapacité sévèreCet article analyse le potentiel épidémiologique des dossiers analysés sur ordinateur provenant du FamilyFund et qui contiennent des données se rapportant à 75 mille enfants du Royaume Uni, avec incapacité sévère. Cette analyse démontre les forces et les faiblesses du dossier type et permet une analyse des variations des caractéristiques des enfants et de leur famille en fonction des principals conditions d'incapacité.ZUSAMMENFASSUNG75,000 schwer behinderte KinderDieser Artikel untersucht das epidemiologische Potential der komputermäßig erfaßten Anträge an den Familienfond, die Einzelheiten über 75,000 Kinder mit schweren Behinderungen im Vereinigten Königreich enthalten. Es werden die Stärken und Schwächen der Komputererfassung dargestellt und es wird eine Analyse über die Veränderung der Charakteristika dieser Kinder und ihrer Familien durch besondere Behinderungsbedingungen gegeben.RESUMEN75,000 niños con minusvalias gravesEste articulo expìora el potencial epidemiológico de la gravación computarizada de instancias realizada en la Fundación de la Familia, que contiene detalles de75,000 niños con minusvalencias graves en el Reino Unido. Ilustra sobre los puntos fuertes y los débiles de los archivos, con un análisis de la variatión de las características de los niños y de sus familias de acuerdo con sus principals minusvalencias.
Measuring mobility limitations in children with cerebral palsy: content and construct validity of a mobility questionnaire (MobQues)
Authors: VAN RAVESTEYN NICOLIEN T , SCHOLTES VANESSA A , BECHER JULES G , ROORDA LEO D , VERSCHUREN OLAF , DALLMEIJER ANNET J 
Published: July 2010

Aim  The objective of this study was to assess the validity of a mobility questionnaire (MobQues) that was developed to measure parent‐reported mobility limitations in children with cerebral palsy (CP).Method  The parents of 439 children with CP (256 males and 183 females; age range 2–18y; Gross Motor Function Classification System [GMFCS] levels I–IV) completed the mobility questionnaire (MobQues). To assess content validity, we linked all meaningful concepts of the MobQues items to the International Classification of Functioning, Disability and Health (ICF). To assess construct validity, we compared the total scores of the two versions of the MobQues (MobQues47 and MobQues28) according to GMFCS level, and determined Pearson’s correlation coefficient (r) with the Gross Motor Function Measure‐66 (GMFM‐66).Results  Content validity was demonstrated by the fact that 46 of the 47 MobQues items were linked to categories in the ‘Mobility’ chapter of the ICF. Construct validity was demonstrated by the finding that MobQues scores decreased with increasing GMFCS levels (p<0.001). In a subgroup of 162 children, positive correlations were found between total scores and the GMFM‐66 (MobQues47, r=0.75; MobQues28, r=0.67, p<0.001).Interpretation  The results of this study provide evidence supporting the content and construct validity of the MobQues as a measure of mobility limitation in children with CP.
Level of motivation in mastering challenging tasks in children with cerebral palsy
Authors: MAJNEMER ANNETTE , SHEVELL MICHAEL , LAW MARY , POULIN CHANTAL , ROSENBAUM PETER 
Published: July 2010

Aim  The aim of this study was to describe and identify factors associated with motivation in children with cerebral palsy (CP).Method  Children with CP were recruited for this cross‐sectional study. Children were assessed using the Leiter Intelligence Test, the Gross Motor Function Measure, and the Vineland Adaptive Behavior Scale. Parents completed the Dimensions of Mastery Questionnaire (DMQ) and questionnaires on demographics, child behaviour, and family functioning.Results  The parents of 74 children (46 males, 28 females; mean age 9y 2mo, SD 2y 1mo, range 5y 10mo–12y 11mo) completed the DMQ. Just over half of the children (39/74) were classified at Gross Motor Function Classification System (GMFCS) level I, with 13 classified at GMFCS level II, one at level III, six at level IV, and 14 at level V; one child was not classified. The most common diagnoses were spastic hemiplegia and quadriplegia (23 each), followed by diplegia (14). The highest motivation scores were obtained for the dimensions of mastery pleasure and social persistence and the lowest for persistence with motor or cognitive tasks. Age and sex were not predictive of scores on the DMQ. Higher IQ (r=0.41), better motor ability (r=0.43), and fewer limitations in self‐care, communication, and socialization (r=0.44–0.53) were positively associated with motivation total score. A negative impact of the child’s disability on the family was associated with lower motivation (r=−0.44). Positive social behaviours were positively correlated with motivation (r=0.38–0.66), whereas hyperactivity and peer problems were negatively associated.Interpretation  High motivation was associated with fewer activity limitations and behavioural problems and reduced family burden. Low motivation may adversely influence a child’s functional potential and the effectiveness of interventions. Strategies focusing on the child, peers, adults, or activities are proposed to enhance the children’s motivation to engage in more challenging activities.
Physical activity measurement instruments for children with cerebral palsy: a systematic review
Authors: CAPIO CATHERINE M , SIT CINDY H P , ABERNETHY BRUCE , ROTOR ESMERITA R 
Published: July 2010

Aim  This paper is a systematic review of physical activity measurement instruments for field‐based studies involving children with cerebral palsy (CP).Method  Database searches using PubMed Central, MEDLINE, CINAHL Plus, PsycINFO, EMBASE, Cochrane Library, and PEDro located 12 research papers, identifying seven instruments that met the inclusion criteria of (1) having been developed for children aged 0 to 18 years, (2) having been used to evaluate a physical activity dimension, and (3) having been used in a field‐based study involving children with CP. The instruments reviewed were the Activities Scale for Kids – Performance version (ASKp), the Canada Fitness Survey, the Children’s Assessment of Participation and Enjoyment/Preferences for Activities of Children (CAPE/PAC), the Compendium of Physical Activities, the Physical Activity Questionnaire – Adolescents (PAQ‐A), StepWatch, and the Uptimer. Second‐round searches yielded 11 more papers, providing reliability and validity evidence for the instruments.Results  The instruments measure physical activity frequency, mode, domain, and duration. Although most instruments demonstrated adequate reliability and validity, only the ASKp and CAPE/PAC have established reliability and validity for children with physical disabilities; the Uptimer has established concurrent validity. No instrument measuring intensity in free‐living has been validated or found reliable for children with CP.Interpretation  The findings suggest that further studies are needed to examine the methodological properties of physical activity measurement in children with CP. Combining subjective and objective instruments is recommended to achieve better understanding of physical activity participation.
BH3‐only protein Bid is dispensable for seizure‐induced neuronal death and the associated nuclear accumulation of apoptosis‐inducing factor
Authors: Engel Tobias , Caballero‐Caballero Aurelien , Schindler Clara K. , Plesnila Nikolaus , Strasser Andreas , Prehn Jochen H.M. , Henshall David C. 
Published: July 2010

J. Neurochem. (2010) 115, 92–101.AbstractProlonged seizures activate members of the Bcl‐2 homology domain 3‐only sub‐group of the Bcl‐2 protein family, which are essential for initiation of apoptosis signaling. Bid is a potent pro‐apoptotic Bcl‐2 homology domain 3‐only protein, which upon proteolytic activation translocates to mitochondria to promote activation of the Bax/Bak sub‐group of the pro‐apoptotic Bcl‐2 family and thereby contributes to release of apoptogenic molecules, such as cytochrome c and possibly apoptosis‐inducing factor (AIF). Bid‐deficient mice have been reported to show reduced lesion volumes after ischemia and trauma in vivo but a causal role for Bid in the setting of seizure‐induced neuronal death has not been investigated. In this study, we studied Bid activation following status epilepticus in mice and compared hippocampal damage between wild‐type and Bid‐deficient animals. Full‐length Bid was detected in normal mouse hippocampus and the cleaved (activated) p15 fragment of Bid was detected shortly after status epilepticus. Bid‐deficient mice underwent equivalent electrographic seizure responses during status epilepticus as wild‐type animals. Hippocampal counts of degenerating neurons and surviving neuron‐specific nuclear protein‐positive cells were not significantly different between wild‐type and Bid‐deficient mice. Additionally, nuclear translocation of AIF was not reduced in Bid‐deficient compared with wild‐type animals subjected to status epilepticus. The present study demonstrates that AIF is not dependent on Bid for mitochondrial release and nuclear import in this model and that while Bid is cleaved during seizure‐induced neuronal death, it may be functionally redundant or even not essential.
Sense of coherence and other predictors of pain and health following laparoscopic cholecystectomy
Authors: Barthelsson Cajsa , Nordström Gun , Norberg Åke 
Published: July 2010

Scand J Caring Sci; 2011; 25; 143–150

Sense of coherence and other predictors of pain and health following laparoscopic cholecystectomy Introduction:  Pain is the most common symptom following laparoscopic cholecystectomy (LC) and might delay discharge from hospital after day surgery. A patient’s ability to manage stressful situations can be assessed by the sense of coherence (SOC) and has been proposed to predict health. The aim of this study was to investigate predictors of average pain the first postoperative week after LC, and predictors of changes in perceived health, with special reference to individual coping resources measured by the Sense of Coherence Scale. Furthermore, a test–re‐test was performed on SOC to evaluate the stability in the context of LC surgery.Method:  Seventy‐three patients completed questionnaires about SOC, health status, pain, anxiety, symptom occurrence and symptom distress preoperatively, postoperative day 1–7 and after 1 and 6 months following LC.Results:  By multiple regression, 23% of the variability in pain intensity could be explained by the variables age, SOC and education. Age was the strongest predictor. Further, 19% of the change in health between day 7 and 1 month could be explained by the two variables symptom distress the first postoperative day and SOC. The test–re‐test of SOC had a correlation coefficient (r) of 0.55. Forty‐six patients (63%) remained within ± 10% of their preoperative SOC score at 6 months, 11 patients (15%) decreased and 16 patients (22%) increased their SOC values.Conclusion:  SOC was found to be a significant but weak predictor of pain intensity the first week after LC. Furthermore, patients scoring low SOC values experienced a delay in their health improvement. SOC was more unstable over time than previously suggested. Further, interventional studies are needed to clarify if SOC might be a clinically useful measure to identify vulnerable patients undergoing LC surgery.
BIODIVERSITY RESEARCH: Geographical linkages between threats and imperilment in freshwater fish in the Mediterranean Basin
Authors: Clavero Miguel , Hermoso Virgilio , Levin Noam , Kark Salit 
Published: July 2010

AbstractAim  The level of imperilment of mediterranean freshwater fish is among the highest recorded for any group of organisms evaluated to date. Here, we describe the geographical patterns in the incidence of threats affecting mediterranean freshwater fish and test whether the effects of specific threats are spatially related to the degree of imperilment of fish faunas.Location  The Mediterranean Basin Biome.Methods  From the IUCN Red List, we recorded the six main threats to 232 endemic freshwater fish species. We used data on fish distributions from IUCN to characterize the spatial patterns in the incidence of threats (as percentage of species affected) through multivariate statistics. We studied the relationships between threat incidence and two estimators of imperilment (proportion of species threatened and an index of extinction risk) at two spatial scales (10 × 10 km and basins) using partial least squares regressions (PLSR) that incorporated the effects of species richness and mean range size.Results  The main axis of variation in the incidence of threats to freshwater fish split areas mainly affected by invasive species from those areas where species are threatened by pollution and agriculture. Wherever invasive species and water extraction were predominant threats, fish assemblages consistently tended to be more imperilled.Main conclusions  As far as we know, this is the first large‐scale analysis on the spatial relationships between the incidence of threats and level of imperilment of any taxonomic group. Our results highlight the primary role of invasive species and water extraction as drivers of native fish declines in the Mediterranean Basin. Large‐scale patterns described here should be generated by local‐scale impacts of both threats on fish biodiversity, widely reported in Mediterranean areas. Because all the species under concern are endemic, control of invasive species and reducing overexploitation of freshwater resources should be conservation priorities for mediterranean freshwater systems.
English in the real world vs. English at school: Finnish English teachers' and students' views
Authors: Ranta Elina 
Published: July 2010

In this study, 108 students and 34 non‐native teachers of English in Finnish upper secondary schools were surveyed regarding their views on the teaching targets and practical goals underpinning their use of English inside and outside school. Two questionnaires were distributed, covering both quantitative and qualitative questions. The results suggest that Finnish students and teachers are well aware of the lingua franca role of English in the ‘real world’ and generally welcome diversity. At the same time, standard models and goals appear to surround ‘school English’, as reflected in the examinations which terminate upper secondary education.Tutkimuksessa tarkasteltiin suomalaisten lukion englanninopettajien ja opiskelijoiden näkemyksiä englanninopetuksen tavoitteista ja päämääristä lingua franca –englannin näkökulmasta, ts. miten englannin rooli kansainvälisenä kielenä näkyy opettajien ja opiskelijoiden ajatuksissa suhteessa kouluopetukseen. Laadullisia ja määrällisiä kysymyksiä sisältäneeseen kyselytutkimukseen vastasi 108 opiskelijaa ja 34 lukion englanninopettajaa. Tulokset osoittavat, että sekä opettajat että oppilaat ovat hyvin tietoisia englannin roolista kansainvälisenä lingua francana ‘tosielämässä’, mutta tämä tietoisuus ei juurikaan näy ‘kouluenglannissa’, joka nähdään edelleen paljolti standardikielen ja äidinkielisen puhujan näkökulmasta. Ristiriita johtuu todennäköisesti ainakin osittain lukion päättävästä ylioppilaskokeesta ja sen asettamista vaatimuksista.
Influence of glutathione S‐transferase M1 and T1 homozygous null mutations on the risk of antituberculosis drug‐induced hepatotoxicity in a Caucasian population
Authors: Leiro Virginia , Fernández‐Villar Alberto , Valverde Diana , Constenla Lucía , Vázquez Rafael , Piñeiro Luis , González‐Quintela Arturo 
Published: July 2010

AbstractObjectives: Genetic variations in enzymes of isoniazid metabolism confer an increased risk for antituberculosis drug‐induced hepatotoxicity in Asian populations. The present study was aimed at investigating the possible association of antituberculosis drug‐induced hepatotoxicity with polymorphisms at the glutathione S‐transferase (GST) gene in a Caucasian population.Methods: A prospective case–control study was nested in a cohort of patients with active tuberculosis who were treated with a combination of isoniazid, rifampicin and pyrazinamide. Cases constituted patients with antituberculosis drug‐induced hepatotoxicity (n=35), and controls constituted patients without any evidence of this complication (n=60). Homozygous null polymorphisms at GST loci M1 and T1 were analysed from genomic DNA from all participants.Results: The GSTT1 homozygous null polymorphism was significantly associated with antituberculosis drug‐induced hepatotoxicity [odds ratio (OR) 2.60, 95% confidence interval (CI) 1.08–6.24, P=0.03]. No significant association was observed between the GSTM1 homozygous null polymorphism and antituberculosis drug‐induced hepatotoxicity (OR 0.73, 95% CI 0.31–1.73, P=0.48).Conclusion: The GSTT1 homozygous null polymorphism may be a risk factor of antituberculosis drug‐induced hepatotoxicity in Caucasians.