bluuebunny/crossref_metadata_2025 huggingface dataset:
A recombinant trispecific single‐chain Fv derivative directed against CD123 and CD33 mediates effective elimination of acute myeloid leukaemia cells by dual targeting
Authors: Kügler Markus , Stein Christoph , Kellner Christian , Mentz Kristin , Saul Domenica , Schwenkert Michael , Schubert Ingo , Singer Heiko , Oduncu Fuat , Stockmeyer Bernhard , Mackensen Andreas , Fey Georg H.
Published: July 2010

Two trivalent constructs consisting of single‐chain Fv antibody fragments (scFvs) specific for the interleukin‐3 receptor α chain (CD123), CD33 and the Fcγ‐receptor III (CD16) were designed and characterized for the elimination of acute myeloid leukaemia (AML) cells. The dual targeting single‐chain Fv triplebody (sctb) [123 × ds16 × 33] and the mono targeting sctb [123 × ds16 × 123] both specifically bound their respective target antigens and were stable in human serum at 37°C for at least 5 d. Both constructs induced potent antibody‐dependent cellular cytotoxicity (ADCC) of two different AML‐derived CD33‐ and CD123 double‐positive cell lines in the low picomolar range using isolated mononuclear cells (MNCs) as effector cells. In these experiments the dual targeting molecule produced significantly stronger lysis than the mono targeting agent. In addition, the sctbs showed a high potency in mediating ADCC of primary leukaemia cells isolated from peripheral blood or bone marrow of seven AML patients. Hence, these novel molecules displayed potent anti‐leukaemic effects against AML cells in vitro and represent attractive candidates for further preclinical development.
research paper: Differential expression of HDAC3, HDAC7 and HDAC9 is associated with prognosis and survival in childhood acute lymphoblastic leukaemia
Authors: Moreno Daniel Antunes , Scrideli Carlos Alberto , Cortez Maria Angélica Abdala , De Paula Queiroz Rosane , Valera Elvis Terci , Da Silva Silveira Vanessa , Yunes José Andres , Brandalise Silvia Regina , Tone Luiz Gonzaga
Published: July 2010

Altered expression of histone deacetylases (HDACs) is a common feature in several human malignancies and may represent an interesting target for cancer treatment, including haematological malignancies. We evaluated the mRNA gene expression profile of 12 HDAC genes by quantitative real‐time polymerase chain reaction in 94 consecutive childhood acute lymphoblastic leukaemia (ALL) samples and its association with clinical/biological features and survival. ALL samples showed higher expression levels of HDAC2, HDAC3, HDAC8, HDAC6 and HDAC7 when compared to normal bone marrow samples. HDAC1 and HDAC4 showed high expression in T‐ALL and HDAC5 was highly expressed in B‐lineage ALL. Higher than median expression levels of HDAC3 were associated with a significantly lower 5‐year event‐free survival (EFS) in the overall group of patients (P = 0·03) and in T‐ALL patients (P = 0·01). HDAC7 and HADC9 expression levels higher than median were associated with a lower 5‐year EFS in the overall group (P = 0·04 and P = 0·003, respectively) and in B‐lineage CD10‐positive patients (P = 0·009 and P = 0·005, respectively). Our data suggest that higher expression of HDAC7 and HDAC9 is associated with poor prognosis in childhood ALL and could be promising therapeutic targets for the treatment of refractory childhood ALL.
Mathematical model for chemotherapeutic drug efficacy in arresting tumour growth based on the cancer stem cell hypothesis
Authors: Ganguly R. , Puri I. K.
Published: July 2010

Abstract.  Objective: Cancer stem cells have been identified as the growth root for various malignant tumours and are thought to be responsible for cancer recurrence following treatment. Materials and methods: Here, a predictive mathematical model for the cancer stem cell hypothesis is used to understand tumour responses to chemotherapeutic drugs and judge the efficacy of treatments in arresting tumour growth. The impact of varying drug efficacies on different abnormal cell populations is investigated through the kinetics associated with their decline in response to therapy. Results and conclusions: The model predicts the clinically established ‘dandelion phenomenon’ and suggests that the best response to chemotherapy occurs when a drug targets abnormal stem cells. We compare continuous and periodic drug infusion. For the latter, we examine the relative importance of the drug cell‐kill rate and the mean time between successive therapies, to identify the key attributes for successful treatment.
Kinetics and cellular sources of cathelicidin during the course of experimental latent tuberculous infection and progressive pulmonary tuberculosis
Authors: Castañeda-Delgado J , Hernández-Pando R , Serrano C J , Aguilar-León D , León-Contreras J , Rivas-Santiago C , Méndez R , González-Curiel I , Enciso-Moreno A , Rivas-Santiago B
Published: July 2010

In spite of advances in immunology on mycobacterial infection, there are few studies on the role of anti-microbial peptides in tuberculosis. The cathelin-related anti-microbial peptide (CRAMP) is the only cathelicidin isolated from mice. In this work we investigated the cellular sources and the production kinetics of this molecule during experimental tuberculosis, using two well-characterized models of latent or chronic infection and progressive disease. The lung of non-infected control mice expressed CRAMP at very low levels. In both models of experimental tuberculosis the main cells immunolabelled for CRAMP were bronchial epithelial cells, macrophages and pneumocytes types II and I. After intratracheal infection with a high bacilli dose (H37Rv strain) in Balb/c mice to produce progressive disease, a high CRAMP gene expression was induced showing three peaks: very early after 1 day of infection, at day 21 when the peak of protective immunity in this model is raised, and at day 28 when the progressive phase starts and the immunoelectronmicroscopy study showed intense immunolabelling in the cell wall and cytoplasm of intracellular bacilli, as well as in cytoplasmic vacuoles. Interestingly, at day 60 post-infection, when advanced progressive disease is well established, characterized by high bacillary loads and extensive tissue damage, CRAMP gene expression decreased but strong CRAMP immunostaining was detected in vacuolated macrophages filled with bacilli. Thus, cathelecidin is highly produced during experimental pulmonary tuberculosis from diverse cellular sources and could have significant participation in its pathogenesis.
Women’s perceptions and experiences of a traumatic birth: a meta‐ethnography
Authors: Elmir Rakime , Schmied Virginia , Wilkes Lesley , Jackson Debra
Published: July 2010

elmir r., schmied v., wilkes l. & jackson d. (2010) Women’s perceptions and experiences of a traumatic birth: a meta‐ethnography. Journal of Advanced Nursing 66(10), 2142–2153.

Aim.  This study presents the findings a meta‐ethnographic study reporting women’s perceptions and experiences of traumatic birth.

Background.  Childbirth is viewed by many as a life transition that can bring a sense of accomplishment. However, for some women, birth is experienced as a traumatic event with a minority experiencing post‐traumatic stress. A traumatic birth experience can have a significant impact on the physical and emotional well‐being of a woman, her infant and family.

Data source.  The CINAHL, MEDLINE, Scopus and PubMed databases were searched for the period January 1994 to October 2009 using the keywords birth trauma, traumatic birth, qualitative research, birth narrative and birth stories.

Review methods.  A meta‐ethnographic approach was used. Quality appraisal was carried out. An index paper served as a guide in identifying particular findings and comparing them with other findings. This ‘reciprocal translation’ process started with a search for common themes, phrases and metaphors.

Results.  Ten qualitative studies were included in the final sample. Six major themes were identified: ‘feeling invisible and out of control’, ‘to be treated humanely’, ‘feeling trapped: the reoccurring nightmare of my childbirth experience’, ‘a rollercoaster of emotions’, ‘disrupted relationships’ and ‘strength of purpose: a way to succeed as a mother’.

Conclusions.  It is evident that a small percentage of women experience a traumatic birth. Although some women who experience a traumatic birth do not necessarily have physical or psychological adverse outcomes, others identify a significant personal impact. Healthcare professionals must recognize women’s need to be involved in decision‐making and to be fully informed about all aspects of their labour and birth to increase their sense of control.
Collaborative recommendation of e‐learning resources: an experimental investigation
Authors: Manouselis N. , Vuorikari R. , Van Assche F.
Published: July 2010

Repositories with educational resources can support the formation of online learning communities by providing a platform for collaboration. Users (e.g. teachers, tutors and learners) access repositories, search for interesting resources to access and use, and in many cases, also exchange experiences and opinions. A particular class of online services that take advantage of the collected knowledge and experience of users are collaborative filtering ones. The successful operation of such services in the context of real‐life applications requires careful testing and parameterization before their actual deployment. In this paper, the case of developing a learning resources' collaborative filtering service for an online community of teachers in Europe was examined. More specifically, a data set of evaluations of learning resources was collected from the teachers that use the European Schoolnet's learning resource portal. These evaluations were then used to support the experimental investigation of design choices for an online collaborative filtering service for the portal's learning resources. A candidate multi‐attribute utility collaborative filtering algorithm was appropriately parameterized and tested for this purpose. Results indicated that the development of such systems should be taking place considering the particularities of the actual communities that are to be served.
Adaptation provisioning with respect to learning styles in a Web‐based educational system: an experimental study
Authors: Popescu E.
Published: July 2010

Personalized instruction is seen as a desideratum of today's e‐learning systems. The focus of this paper is on those platforms that use learning styles as personalization criterion called learning style‐based adaptive educational systems. The paper presents an innovative approach based on an integrative set of learning preferences that alleviates some of the limitations of similar systems. The adaptive methods used as well as their implementation in a dedicated system (WELSA) are presented, together with a thorough evaluation of the approach. The results of the experimental study involving 64 undergraduate students show that accommodating learning styles in WELSA has a beneficial effect on the learning process.
The Hydra polyp: Nothing but an active stem cell community
Authors: Bosch Thomas C. G. , Anton‐Erxleben Friederike , Hemmrich Georg , Khalturin Konstantin
Published: July 2010

Hydra is a powerful stem cell model because its potential immortality and extensive regeneration capacity is due to the presence of three distinct stem cell lineages. All three lineages conform to a well‐defined spatial distribution across the whole body column of the polyp. Stem cell function in Hydra is controlled by extracellular cues and intrinsic genetic programs. This review focuses on the elusive stem cell niche of the epithelial layers. Based on a comparison of the differences between, and commonalities among, stem cells and stem cell niches in Hydra and other invertebrates and vertebrates, we propose that the whole body column of the polyp may be considered a stem cell “niche” in which stem cell populations are established and signals ensuring the proper balance between stem cells and progenitor cells are integrated. We show that, at over 500 million years old, Hydra offers an early glimpse of the regulatory potential of stem cell niches.
Effect of unilateral endobronchial valve insertion on pulmonary ventilation and perfusion: A pilot study
Authors: CHUNG Steven C. S. , PETERS Matthew J. , CHEN Stephanie , EMMETT Louise , ING Alvin J.
Published: July 2010

Background and objective:  The effects of bronchoscopic lung volume reduction (BLVR) on pulmonary ventilation and perfusion are incompletely understood. In this pilot trial, we investigated serial changes in regional ventilation and perfusion following unilateral endobronchial valve placement in COPD patients with heterogeneous emphysema.

Methods:  At baseline and at days 30 and 90 following BLVR, subjects underwent lung function, 6MWD and St George's Respiratory Questionnaire. Ventilation and perfusion scintigraphy were performed to quantitate and serially compare regional differences.

Results:  Six out of eight subjects completed the study; all had endobronchial valves targeting their left upper lobe. At day 90 post‐BLVR, there was a trend towards an increase in FEV1 and a mean reduction in St George's Respiratory Questionnaire score of nine units. In the targeted left upper zone there was reduced ventilation and perfusion. Ventilation and perfusion to the right lung; and specifically the right lower zone, significantly increased.

Conclusions:  There appears to be redistribution of ventilation and perfusion to the contralateral lung following endobronchial valve placement. This may be of importance when assessing patients for unilateral BLVR. Selecting patients with heterogeneous disease is emphasized, taking into consideration not just comparison between upper and lower lobes, but between left and right lungs. A larger trial is currently underway, guided by these findings.
Full‐term interstitial retroperitoneal pregnancy with delivery of a healthy infant
Authors: Milićević Srboljub , Jovanović Dragoljub , Vilendečić Zoran , Ljubić Aleksandar , Božanović Tanja , Niketić Ljiljana
Published: July 2010

Implantation in the interstitial part of the oviduct is frequently associated with severe fetal and maternal morbidity. We present a patient with primary interstitial and secondary retroperitoneal ectopic pregnancy. The suspicion of an ectopic pregnancy was raised after an ultrasound examination during the 18th week of pregnancy. An almost complete lack of symptoms led us to discard the possibility of an ectopic pregnancy. This normal‐course pregnancy advanced to term. Due to anhydramnios and fetal malpresentation, a laparotomy was performed at 39 weeks of pregnancy and a healthy infant was delivered via cesarean section.
Vascular endothelial growth factor, fms‐like tyrosine kinase‐1 (Flt‐1) and soluble Flt‐1 gene expressions in Korean pre‐eclamptic placentas
Authors: Park Jong‐Sik , Baik Haing‐Woon , Lee Seong‐Kyu , Na Woo‐Suk , Song Young‐Rae , Yang Yun‐Suk , Park Mi‐Hae , Hwang In‐Taek , Park Joon‐Sook , Oh Kwan‐Young
Published: July 2010

Aim:  To assess the expressions of vascular endothelial growth factor (VEGF), fms‐like tyrosine kinase‐1 (Flt‐1), and soluble Flt‐1 (sFlt‐1) genes in healthy normotensive and pre‐eclamptic placentas of Korean women.

Methods:  We investigated 12 healthy normotensive pregnant women and 10 pre‐eclamptic pregnant women at Eulji University Hospital. The obtained placental tissues were analyzed using reverse transcription polymerase chain reaction and real‐time quantitative polymerase chain reaction.

Results:  The sFlt‐1 messenger ribonucleic acid (mRNA) level was elevated 2.6 times more in pre‐eclamptic placentas than in normal control placentas. However, the VEGF mRNA level of pre‐eclamptic placentas was decreased. There was no difference in the Flt‐1 mRNA level between control and pre‐eclamptic placentas.

Conclusions:  Our study showed that expressions of genes relating to angiogenesis were altered in Korean pre‐eclamptic placentas. These results suggest that the alteration in expressions of sFlt‐1 and VEGF genes might be associated with the pathogenesis of pre‐eclampsia.
Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system
Authors: Nautiyal Manisha , Sweatt Andrew J. , MacKenzie James A. , Mark Payne R. , Szucs Sylvia , Matalon Reuben , Wallin Reidar , Hutson Susan M.
Published: July 2010

The NIPSNAP (4‐nitrophenylphosphatase domain and non‐neuronal SNAP25‐like protein homolog 1) proteins belong to a highly conserved family of proteins of unknown function. We found that NIPSNAP1 binds to the branched‐chain α‐keto acid (BCKA) dehydrogenase enzyme complex, which is disrupted in maple syrup urine disease, a disease of branched‐chain amino acid catabolism that results in neurological dysfunction. Phenylketonuric (PKU) and epileptic mice show altered expression of NIPSNAP1 in the brain. Therefore, the distribution and localization of NIPSNAP1 in rat brain was determined. Results show that NIPSNAP1 is expressed exclusively in neurons including pyramidal neurons in the cerebral cortex, Purkinje neurons in the cerebellum and motor neurons in the spinal cord. Dopaminergic neurons in midbrain and noradrenergic neurons in the brainstem, which are affected in PKU, also express NIPSNAP1. NIPSNAP1 is found to be localized in the mitochondrial matrix and can bind dihydrolipoyl‐transacylase and ‐transacetylase components of the BCKA and pyruvate dehydrogenase complexes in vitro. Our data provide the first experimental evidence for a strictly neuronal expression of this mitochondrial protein in the rat nervous system.
ALBINISM IN BOTSWANA: Albinism in Botswana junior secondary schools – a double case study
Authors: Dart Gareth , Nkanotsang Tiroyaone , Chizwe Ose , Kowa Lily
Published: July 2010

Pupils with albinism potentially face a number of challenges in accessing quality education in schools in Botswana. Physical issues such as poor eyesight related to the condition and the problems of sensitive skin in such a dry and warm climate are both contributing factors to making learning problematic for some pupils. This study by Gareth Dart of the University of Worcester, Tiroyaone Nkanotsang from a junior secondary school in Botswana, Ose Chizwe from the Government of Botswana and Lily Kowa from a junior secondary school in Botswana, considers the educational experience of two particular pupils with albinism in junior secondary schools in Botswana. Case studies were carried out by student teachers on teaching practice. Issues arising from the case studies of the two pupils were combined with the life story of a third student teacher who himself has albinism. The authors conclude by making some recommendations as to how the experience of pupils with albinism might be improved in the light of local conditions and principles of development.
Immigration and Outsourcing: A General‐Equilibrium Analysis
Authors: Bandyopadhyay Subhayu , Wall Howard J.
Published: July 2010

This paper analyzes immigration and outsourcing in a general‐equilibrium model of international factor mobility. In our model, legal immigration of skilled labor is controlled through a quota, while outsourcing is determined both by the firms in response to market conditions and through policy‐imposed barriers. A loosening of the immigration quota reduces outsourcing, enriches capitalists, leads to losses for native workers, and raises national income. If the nation targets an exogenously determined immigration level, the second‐best outsourcing tax can be either positive or negative. If in addition to the immigration target there is a wage target arising out of income distribution concerns, an outsourcing subsidy is required. We extend the analysis to consider illegal immigration of unskilled labor. A higher legal immigration quota will lead to more (less) illegal immigration if skilled and unskilled labor are complements (substitutes) in production.
FDI Flows to Latin America, East and Southeast Asia, and China: Substitutes or Complements?
Authors: Chantasasawat Busakorn , Fung K. C. , Iizaka Hitomi , Siu Alan
Published: July 2010

Is China diverting foreign direct investment (FDI) from other developing countries? Theoretically, a growing China augments other countries' FDI by creating more production networking and raising demand for resources. However, low Chinese costs lure multinationals away from other production sites. Here we explore this issue empirically. We focus on East and Southeast Asia and Latin America for 1985–2002. We control for the standard determinants of inward FDI, then add China FDI to represent the “China Effect.” We found that China's FDI is positively related to FDI in Asia, while the China Effect is insignificant for Latin America. Also the China Effect is generally not the most important determinant of other countries' FDI. Market sizes and policies such as corporate tax rates and openness tend to be more important.
Negotiating fair pay and conditions: low paid women's experience and perceptions of labour market deregulation and individual wage bargaining
Authors: Jefferson Therese , Preston Alison
Published: July 2010

The OECD is strongly supportive of the industrial relations changes recently introduced in Australia. This article examines the experiences of low paid women in a deregulated labour market context. The research highlights the gendered effects of the recent reforms and cautions against the use of Australia as a model framework.
Origin and flux of a gas seep in the Northern Alps (Giswil, Switzerland)
Authors: ETIOPE G. , ZWAHLEN C. , ANSELMETTI F. S. , KIPFER R. , SCHUBERT C. J.
Published: July 2010

Natural gas seeps in the Alpine region are poorly investigated. However, they can provide useful information regarding the hydrocarbon potential of sedimentary Alpine units and related geofluid migration, typically controlled by pressurized gas accumulations and tectonics. A gas seep located near Giswil, in the Swiss Northern Alps, was investigated, for the first time, for molecular and isotopic gas composition, methane flux to the atmosphere, and gas flux variations over time. The analyses indicated that the gas was thermogenic (CH4 > 96%; δ13C1: −35.5‰ to −40.2‰) and showed evidence of subsurface petroleum biodegradation (13C‐enriched CO2, and very low C3+ concentrations). The source rock in the region is marine Type II kerogen, which is likely the same as that providing thermogenic gas in the nearby Wilen shallow well, close to Lake Sarnen. However, the lack of δ13CCO2 and δ13C3 data for that well prevented us from determining whether the Wilen and Giswil seeps are fed by the same reservoir and seepage system. Gas fluxes from the Giswil seep, measured using a closed‐chamber system, were significant and mainly from two major vents. However, a substantial gas exhalation from the soil occurs diffusely in an area of at least 115 m2, leading to a total CH4 output conservatively estimated to be at least 16 tonnes per year. Gas flux variations, monitored over a 1‐month period by a special tent and flowmeter, showed not only daily meteorological oscillations, but also an intrinsic ‘pulsation’ with periods of enhanced flux that lasted 2–6 h each, occurring every few days. The pulses are likely related to episodes of gas pressure build‐up and discharge along the seepage system. However, to date, no relationship to seismicity in the active Sarnen strike‐slip fault system has been established.
Cognitive Impairment in New Cases of Epilepsy Randomly Assigned to Carbamazepine, Phenytoin and Sodium Valproate
Authors: Forsythe Ian , Butler Richard , Berg Ian , McGuire Ralph
Published: July 2010

Sixty‐four new cases of childhood epilepsy were randomly assigned to either carbamazepine, phenytoin or sodium valproate, and were assessed with cognitive tests before medication and three subsequent times over a year. Carbamazepine in moderate dosage adversely affected memory, but sodium valproate and phenytoin did not.

Altérations cognitives dans les nouveaux cas d'épilepsie traités au hasard par la carbamazépine, la phénytoine et le valproate de sodium

Soixante‐quatre nouveaux cas d'épilepsie de I'enfance ont été orientés au hasard vers un traitement par carbamazépine, phénytoine ou valproate de sodium et furent, évalués par des tests cognitifs avant le début de la médication et ultérieurement trois fois au cours d'une année. La carbamazépine à doses modérées altérait le mémoire, mais le valproate de sodium et la phénytoine ne le faisaient pas.

Kognifive Störungen bei neuen Epilepsiefällen, die wahllos mit Carbamazepin, Phenytoin oder Valproinsäure behandelt worden waren.

64 Neue Fälle mit kindlicher Epilepsie wurden willkürlich entweder mit Carbamazepin, Phenytoin oder Valproinsäure behandelt und vor der Therapie, sowie dreimal danach irn Verlauf eines Jahres, mit kognitiven Tests untersucht. Carbamazepin hatte in mäßiger Dosierung einen negativen Effekt auf das Gedächtnis, Valproinsäure und Phenytoin nicht.

Alteración cognitiva en casos nuevos de epilepsia tratados al azar con carbatnazepina, fenitoína y valproato sódico

Sesenta y cuatro casos de epilepsia infantil fueron tratados al azar con carbamazepina, fenitoina y valproato sódico y fueron examinados con tests cognitivos antes de la medicación y tres veces más en el curso de un año. La carbamazepina a dosis moderadas afectaba adversamente a la memoria, cosa que no hacían ni el valproato ni la fenitoína.
Left Unilateral Neglect in a Preschool Child
Authors: Thompson Nora M. , Ewing‐Cobbs Linda , Fletcher Jack M. , Miner Michael E. , Levin Harvey S.
Published: July 2010

The authors present a case of transient left hemispatial inattention following traumatic brain injury in a three‐year‐old child. Computed tomographic scan revealed a small right frontal and a right temporo‐parietal contusion. Stimuli were developed to quantify the response difference between hemifields in this pre‐literate child. Hemispatial inattention showed resolution at 19 days post‐trauma and had completely resolved six months later. These findings suggest that right hemisphere systems subserve visually guided attention before six years of age.

Négligence gauche unilatérale chez un enfant d'âge préscolaire

Les auteurs rapportent le cas d'une inattention hémispatiale gauche transitoire à la suite d'une lésion cérébrale chez un enfant de trois ans. Les scanner révéla une petite contusion frontale droite et une contusion temporo‐pariétale droite. Des stimulations furent provoquées pour quantifier les différences de réponses entre les deux hémi‐champs chez cet enfant de capacité pré‐scolaire. L'inattention hémispatiale s'atténua 19 jours après le traumatisme et disparut complètement six mois plus tard. Ces données suggèrent que les systèmes hémisphériques droits participent à l'attention sous contrôle visuel avant l'âge de six ans.

Linksseitige laterale Aufmerksamkeitsstörung bei einem Vorschulkind

Die Autoren stellen ein dreijähriges Kind vor, das nach einem Hirntrauma eine passagere räumliche Aufmerksamkeitsstörung links aufwies. Im Computertomogramm zeigten sich rechts frontal und rechts temporoparietal kleine Contusionsherde. Es wurden Stimuli entwickelt, um die Wahrnehungsunterschiede der zwei Sehfeldabschnitte bei diesem Vorschulkind zu quantifizieren. Die räumliche Aufmerksamkeitsstörung begann sich am 19 posttraumatischen Tag zurückzubilden und war sechs Monate später vollständig verschwunden. Diese Befunde deuten darauf hin, daß die visuell gelenkte Aufmerksamkeit vor dem sechsten Lebensjahr von der rechten Hemisphäre gesteuert wird.

Negligencia unilateral izquierda en el preescolar

Los autores presentan un caso de inatención transitoria hemiespacial izquierda después de una lesión traumática del cerebro en un niño de tres años de edad. La TAC reveló una pequeña contusión frontal y temporoparietal del lado izquierdo. Se aplicaron estímulos para cuantificar la diferencia de respuestas entre ambos hemisferios en este niño prelectivo. La inatención hemiespacial empezó a resolverse a los 19 dias después del trauma habiendo desaparecido totalmente seis meses más tarde. Estos hallazgos sugieren que los sistemas del hemisferio derecho subsirven la atención guiada visualmente antes de los seis años de edad.
Variable Course of Canavan Disease in Two Boys with Early Infantile Aspartoacylase Deficiency
Authors: Moers A. v. , Sperner J. , Michael Th. , Scheffner D. , Schutgens R. H. B.
Published: July 2010

This is a report of two patients with Canavan disease from the Federal Republic of Germany. One is a severely retarded, macrocephalic boy, who had the characteristic laboratory findings of Canavan disease and progressive leucodystrophy on neuro‐imaging. The other is retarded, with signs of a cerebral movement disorder showing no deterioration during the first 15 months. The significance of aspartoacylase deficiency in Canavan disease for differential diagnosis, genetic counselling and prenatal diagnosis of leucodystrophy is discussed.

Déficience en aspartoacylase dans la maladie de Canavan

Cet article relate le premier cas rapporté de deux patients avec maladie de Canavan dans la République Fédérale Allemande. Un garçon gravement retardé macrocéphalique présentait les données de laboratoire caractéristiques de la maladie de Canavan et une leucodystrophie progressive à l'imagerie neurologique. La signification de la déficiene en aspartoacylase dans la maladie de Canavan pour le diagnostic differentiel, le conseil gégétique et le diagnostic prénatal de leucodystrophie sont discutés.

Aspartoacylase Mangel beim Canavan Syndrom

Dies ist der erste Bericht über zwei Patienten mit Canavan Syndrom aus der Bundesprepublik Deutschland. Ein hochgradig retardierter, makrozephaler Junge hatte die charakteristischen Laborbefunde für ein Canavan Syndrom und neuroradiologisch eine Leukodystrophie. Die Autoren diskutieren die Bedeutung des Aspartoacylase‐Mangels beim Canavan Syndrom für die Differentialdiagnose, genetische Beratung und pränatale Diagnose der Leukodystrophie.

Deficiencia en aspartoacilasa en la enfermedad de Canavan

Este es el primer dos casos de enfermedad de Canavan en la Républica Federal Alemana. Un varon gravemente retrasado y macrocefálico presentaba los datos de laboratorio de la enfermedad de Canavan y en la neuroimagen una leucodistrofia progresiva. Se discute el significado de la deficiencia en aspartoacilasa, el consejo genético y el diagnóstico prenatal de leucodistrofia.
Height Measurement Of Patients With Neuromuscular Disease And Contractures
Authors: Miller Freeman , Koreska Jan
Published: July 2010

Patients with neuromuscular diseases frequently have joint contractures, which preclude standard measurements of standing height. Standing height, arm‐span, forearm and ulnar‐segment measurements were taken of 116 normal children without contractures. Measurement of the forearm segment had the highest correlation with standing height, but it requires a special ruler. Arm‐span measurement had good correlation with standing height, although this cannot be used if elbow or shoulder contractures are present; in such patients ulnar segment measurement can be used, but it has the lowest correlation with standing height.

RŕSUMŕ

Mesure de la taille chez les patients prŕsentant une affection neuro‐musculaire et des contractures

Les patients atteints d'affection neuro‐musculaires ont souvent des contractures articulaires qui empêchent la mesure de la taille debout. La taille debout, I'envergure, les mesures de I'avant‐bras et du cubitus ont ŕtŕ relevŕs chez 116 enfants normaux sans contracture. La mesure de longueur de I'avant‐bras prŕsentait la meilleure corrŕlation avec la taille debout, mais exige des règiles d'application. L'envergure prŕsentait une bonne corrŕlation avec la taille debout mais elle ne peut être utilisŕe en cas de contracture des coudes ou des ŕpaules; chez de tels sujets, la mesure du cubitus peut être utilisŕe mais elle prŕsente la plus faible corrŕlation avec la taille debout.

Bestimmung der Körpergröße bei Patienten mit neuromuskulärer Erkrankung und Kontrakturen

Patienten mir neuromuskulären Erkrankungen haben häufig Gelenkkontrakturen, die eine Größenbestimmung im Stehen ausschließen. Bei 116 gesunden Kindern ohne Kontrakturen wurden Länge, Armspanne, Unterarm und Ulnasegment gemessen. Die beste Korrelation bestand zwischen Unterarmlänge und Körpergröße, aber man braucht dazu ein Speziallineal. Die Armspanne zeigte eine gute Korrelation zur stehend gemessenen Körpergröße, sie kann aber bei Ellenbogen‐ und Schulterkontrakturen nicht verwendet werden. Bei diesen Patienten kann man die Messung des Ulnasegmentes heranziehen, das allerdings die geringstre Korrelation zur Körpergröße aufweist.

Estatura de pacientes con enfermedad neuromuscular y contracturas

Los pacientes con enfermedades neuromusculares tienen con frecuencia contracturas a nivel de las articulaciones que dificultan la medición de la estatura en bipedestación. Se midió la estatura en bipedestación, la envergadura de brazos y del antebrazo y segmento cubital en 116 niños normales sin contracturas. La longitud del antebrazo tenia la más alta correlación con la estatura en bipedestación, pero requiere una regla especial. La envergadura tenía una buena correlación con la estatura de pie, si bien no puede ser usada si hay contracturas en codo o hombro. En estos pacientes puede ser usada si hay contracturas en codo o hombro. En estos pacientes puede usarse la longitud del segmento cubital, aunque tiene la correlación más baja con la estatura de pie.
CEREBRAL PALSY IN TWINS
Authors: Laplaza F. Javier , Root Leon , Tassanawipas Anant , Cervera Paloma
Published: July 2010

Eighty‐six children with cerebral palsy (CP) born as a product of twin pregnancies were studied. Data regarding their co‐twins were also gathered. The authors' findings suggest that (1) monozygotic twins have a higher risk of CP than dizygotic twins, (2) twin order at birth does not seem to be a relevant factor in the etiology of CP, (3) mothers less than 24 or more than 34 years of age had the highest incidence of children with CP as a product of a twin pregnancy, and (4) the data do not support a genetic basis for the disease.

L'IMC et les jumeaux

Quatre‐vingt six enfants IMC nés d'une grossesse gemellaire ont étéétudies. Les données concernant leurs jumeaux ont également été recuillis. Les élements recueillis par les auteurs suggérent: (1) que les jumeaux monozygotes ont un risque d'IMC plus élevé que les jumeaux dizygotes; (2) l'ordre de naissance entre jumeaux ne semble pas être un facteur significatif pour l'apparition d'une IMC; (3) les mères de moins de 24 ans et de plus de 34 ans présentant le risque le plus éléve d'un enfant IMC au cours d'une grossesse gemellaire; et (4) les données ne sont pas en faveur d'une origine génétique de l'affection.

Cerebralparese bei Zwillingen

86 Kinder aus Zwillingsschwangerschaften mit Cerebralparese (CP) wurden untersucht. Außerdem wurden Daten von ihren Zwillingsgeschwistern gesammelt. Die Befunde lassen vermuten, daß: (1) monozygote Zwillinge ein höheres Risiko für CP haben als dizygote, (2) die Geburtenfolge kein relevanter Faktor für die Ätiologie der CP zu sein scheint, (3) Mütter unter 24 oder über 34 Jahre bei Zwillingsschwangerschaften am häufigsten Kinder mit CP hatten, und (4) die Daten keinen Anhalt für eine genetische Ursache der Erkrankung geben.

Parálisis cerebral en mellizos

Se estudiaron ochenta y seis niños con parálisis cerebral (PC) nacidos como producto de embarazos gemelares. También se recogieron datos relativos a sus hermanos gemelos. Los hallazgos de los autores sugieren que: (1) los gemelos monozigótìcos tienen un riesgo mayor de tener PC que los dizigóticos; (2) el orden del gemelo al nacer no parece ser un factor relevante en la etiologia de la PC; (3) las madres de menos de 24 años o de más de 34 años tienen la mayor incidencia de niños con PC como producto de un embarazo gemelar; y (4) los datos no apoyan una base genética para la enfermedad.
NEONATAL TETANUS: INCIDENCE AND IMPROVED OUTCOME WITH DIAZEPAM
Authors: Okuonghae Humphrey O. , Airede Abdulkareem I.
Published: July 2010

During a four‐year prospective study at a referral hospital, 61 patients with neonatal tetanus were encountered and the contribution of continuous intravenous diazepam was noted. The over‐all incidence during the study was 21·8/1000, with a mortality rate of 8·2 per cent. Signs associated with fatal outcome were hypothermia, generalized rigidity and opisthotonus. The mean dose of diazepam used was 25·8mg/kg/day, in combination with sodium phenobarbitone (mean dose 10·7mg/kg/day). The results suggest that high‐dose intravenous (continuous) diazepam is effective, economical and feasible for reducing mortality in neonatal tetanus. Side‐effects were minimal.

Tétanos néo‐natal: incidence et amélioration du pronostic par diazépam

Durant une étude prospective de quatre ans dans un hôpital ouvert, 61 patients présentèrent un tétanos néo‐natal et l'apport de diazépam intraveineux fut noté. L'incidence globale durant l'étude fut de 21,8 pour mille, avec un taux de mortalité de 8,2 pour cent. Les signes associés à un pronostic fatal furent l'hypothermie, la raideur généralisée et l'opisthotonos. La dose moyenne de diazépam utilisée fut de 25,8 mg/kg/jour, en association avec du phénobarbital sodique (dose moyenne 10,7 mg/kg/jour. Les résultats suggèrent que le diazépam continu à hautes doses est efficace, économique et pratique pour réduire la mortalité au cours du tétanos néo‐natal. Les effets secondaires furent minimes.

Neonataler Tetanus: Häufigkeit un Verbesserung des Endergebnisses durch Diazepam

Bei einer 4‐Jahresstudie an einem Versorgungskrankenhaus fand man 61 Patienten mit neonatalem Tetanus und untersuchte die Wirkung einer kontinuierlichen Diazepamgabe. Die Gesamt‐Inzidenz während der Studie betrug 21.8/1000, mit einer Mortalitätsrate von 8.2 Prozent. Die wichtigsten Symptome bei tödlichem Ausgang waren Hypothermie generalisierte Rigidität und Opisthotonus. Die mittlere verabreichte Diazepamdosis betrug 25.8mg/kg/Tag in Kombination mit Phenobarbital (mittlere Dosis 10.7mg/kg/Tag). Die Eregebnisse zeigen, daß hochdosierte intravenöse (kontinuierliche) Diazepamgaben bei neonatalem Tetanus wirkungsvoll, ökonomisch und geeignet sind, die Mortalität zu senken. Nebenwirkungen waren minimal.

Tétanos neonatal: incidencia y mejoria en el curso con diazepam

En un estudio prospective de cuatro años en un hospital general, se hallaron 61 pacientes con tétanos neonatal y se anotó la contribución de la administración de diazepam intravenosa contínua. La incidencia global durante el estudio fue de 21,8/1000 con un nivel de mortalidad de 8,2 por ciento. Los signos asociados a un curso fatal fueron hipotermia, rigidez generalizada y opistótonos. La dosis promedia de diazepam utilizada fue de 25,8mg/kg/dia en combinación con fenobarbital sódico (dosis media de 10,7mg/kg/dia). Los resultados sugieren que las dosis altas endovenosas continuas son efectivas, económicas y accesibles para reducir la mortalidad en el tétanos neonatal. Los efectos secundarios fueron mínimos.
THE PREVALENCE OF CEREBRAL VISUAL DISTURBANCE IN CHILDREN WITH CEREBRAL PALSY
Authors: Schenk‐Rootlieb A. J. F. , van Nieuwenhuizen O. , van der Graaf Y. , Wittebol‐Post D. , Willemse J.
Published: July 2010

Assessment of visual acuity using the visual acuity card procedure in 164 children with cerebral palsy revealed low visual acuity in 71 per cent. Results of ophthalmological examination were available for 74 of these patients, but could not explain adequately the low visual acuity of 36 of the 43 patients (84 per cent) assessed by both the acuity card procedure and other techniques. There is a high probability that cerebral visual disturbance is present in these patients. Awareness of visual disability when compiling a programme of visual and neurodevelopmental stimulation for children with cerebral palsy is essential.

La prévalence des troubles centraux de la vision dans l'IMC

L'appréciation de l'acuité visuelle à partir de tableux d'acuité visuelle chez 164 sujets IMC a révélé une acuité visuelle basse dans 71 pour cent des cas. Les résultats des examerts ophtalmologiques étaient disponibles chez 74 sujets mais ne pouvaient expliquer l'acuité visuelle basse de 36 des 43 sujets (84 pour cent) évalués à la fois par les tableaux d'acuité visuelle et d'autres techniques. Il y a une haute probabilité qu'il existait un trouble visuel central chez ces sujets. La reconnaissance d'un déficit visuel en établissant un programme de stimulation visuelle et neurodéveloppementale chez les IMC est essentielle.

Die Häufigkeit der cerebralen Sehstörung bei Cerebralparese

Die Beurteilung der Sehschärfe mit dem Acuity Card Verfahren bei 164 Patienten mit Cerebralpares zeigte bei 71 Prozent eine verminderte Sehschärfe. Von 74 dieser Patienten waren die Ergebnisse der augenärztlichen Untersuchung verfügbar, sie konnten aber bei 36 der 43 Patienten (84 Prozent), die sowohl mit dem Acuity Card Verfahren als auch mit anderen Methoden untersucht wurden waren, die verminderte Sehschärfe nicht hinreichend erklären. Wenn man ein Programm zur visuellen und entwicklungsneurologischen Stimulation für Kinder mit Cerebralparese zusammenstellt, ist es sehr wichtig auf das Vorliegen einer Sehstöbrung zu achten.

Prevalencia de la alteración visual cerebral en la parálisis cerebral

La evaluación de la agudeza visual utilizando el procedimiento de las tarjetas de agudeza visual en 164 pacientes con parálisis cerebral reveló una agudeza visual baja en el 71 por ciento de los casos. En 74 des estos pacientes se pudo obtener un examenoftalmológico, pero ello no pudo explicar adecuadamente la disminución visual de 36 de los 43 pacientes (84 por ciento) evaluados al mismo tiempo con las cartas de agudeza y con otros técnicas. Existe una alta probabilidad de que en estos casos la alteración visual sea de origen cerebral. Es esencial el darse cuenta de la presencia de la alteración visual cuando se crea un programa de estimulación visual y neuroevolutiva en niños con parálisis cerebral.
NON‐VERBAL COMMUNICATION SKILLS OF SURGICALLY TREATED CHILDREN WITH INFANTILE SPASMS
Authors: Caplan Rochelle , Guthrie Donald , Mundy Peter , Sigman Marian , Shields Donald , Sherman Tracy , Peacock Warwick J.
Published: July 2010

The authors present preliminary findings on the effects of surgery on the development of early nonverbal social communication skills in eight children with intractable infantile spasms. After a mean follow‐up of 15·2 months, there was no statistically significant change in the post‐surgical nonverbal communication behavior of these children beyond the expected developmental change. Implications of these findings for the developmental impairment associated with infantile spasms are discussed.

Capacité de communication non verbale chez les enfants présentant des spasmes infantiles et traités par chirurgie

Les auteurs rapportent des résultats préliminaires sur les effets de la chirurgie concernant la capacité de communication sociale précoce non verbale chez huit enfants présentant des spasmes infantiles irréductibles. Aprés un suivi moyen de 15,2 mois, il n'y avait pas de modification statistiquement significative sur le comportement de communication non verbale après chirurgie chez ces enfants, au delà des transformations développementales attendues. Les implications de ces données dans les altérations de développement associées aux spasmes infantiles sont discutées.

Non‐verbale Kommunikation bei Kindern mit Säuglingskrämpfen nach chirurgischer Intervention

Die Autoren stellen vorläufige Befunde der frühen non‐verbalen sozialen Kommunikations‐entwicklung bei acht Kindern mit unbeherrschbaren Säuglingskrämpfen nach chirurgischer Intervention vor. Nach einem mittleren Kontrollzeitraum von 15·2 Monaten fand sich nach der Operation keine statistisch signifikante Veränderung des Kommunikationsverhaltens bei diesen Kindern über den erwarteten Entwicklungsfortschritt hinaus. Es wird diskutiert, inwieweit diese Befunde für die Entwicklungsstörung bei Säuglingskrämpfen von Bedeutung sind.

Habilidad comunicativa no verbal en niños tratados quirúrgicamente por espasmos infantiles

Los autores presentan los hallazgos preliminares de los efectos de la cirugía sobre el desarrollo de la comunicación precoz social no verbal en ocho niños con espasmos infantiles intratables. Después de un seguimiento promedio de 15,2 meses, no había ningún cambio estadísticamente significativo en el comportamiento comunicativo no verbal postquirúrgico en estos niños, más allá del cambio esperado en el desarrollo. Se discuten la implicaciones de estos hallazgos sobre la alteración del desarrollo asociado a los espasmos infantiles.
Cor Pulmonale as a Complication of Ventriculo‐atrial Shunts Reviewed
Authors: Sleigh G. , Dawson A. , Penny W. J.
Published: July 2010

The authors present the case of a nine‐year‐old patient with spina bifida and hydrocephalus treated with a ventriculo‐atrial shunt who developed fatal pulmonary hypertension 5 1/2 years after his last valve revision; he had been well and active up to one month before his death. Details of the dramatic cardiovascular findings are given. The problems of treatment, once symptoms occur, and the difficulty of pre‐symptomatic detection are discussed.

Coeur pulmonaire compliquant une dérivation ventriculoatriale

Les auteurs rapportent le cas d'un patient de neuf ans présentant un spina bifida et une hydrocéphalie traitée par une dérivation ventriculoatriale qui développa une hypertension pulmonaire mortelle cinq ans et demi après la dermière révision de valve; il fut extrěment bien et actif jusqu'au mois précédent le décès. Les détails des données cardio‐vasculaires dramatiques sont précisés. Les problèmes de traitement, une fois les symptomes apparus et la difficultée de la détection pré‐symptomatique sont discutés.

'Cor pulmonale' als Komplikotion bei einem ventrikulo‐avialen shunt

Die Autoren stellen den Fall eines neun‐jährigen Patienten mit Spina bifida und Hydrozephalus und ventrikulo‐atrialem Shunt vor, der 5 ½ Jahre nach der letzten Ventilrevision eine zum Tode fiihrende pulmonale Hypertension entwickelte; er war bis zu einem Monat vor seinem Tod sehr gesund und aktiv. Es werden Einzelheiten der dramatischen kardio‐vaskulären Befunde aufgeführt. Die Probleme der Behandlung—wenn Symptome auftreten—und die Schwierigkeiten einer prä‐symptomatischen Diagnose werden diskutiert.

'Cor pulmonale ' como complicación de derivaciones ventriculo‐otriales. Revisión

Los autores presentant el caso de una paciente de nueve años con espína bifida e hidrocefalia, tratada con una derivación ventriculo‐atrial que padeció una hipertension pulmonar fatal 5 anAos y medio después de la última revision de su válvula. Estaba extremadamente bien y activa hasta un mes antes de su muerte. Se dan detalles de los marcados hallazgos cardiovasculares. Se discuten los problemas de tratamiento una vez aparecidos los sintomas y la dificultad de una detección.
Protracted Clinical Course for Patientsd with Canavan Disease
Authors: Zelnik N. , Luder A. S. , Elpeleg O. N. , Gross‐Tsur V. , Amir N. , Hemli J. A. , Fattal A. , Harel S.
Published: July 2010

Before the establishment of h'‐acetylaspartic aciduria due to aspartoacylase deficiency as the cause of Canavan disease, diagnosis was based on the characteristic clinical features and spongiform encephalopathy, a pathological response shared by a number of other unrelated conditions. Thus confusion exists in the literature about the phenotype of spongiform encephalopathy (Canavan disease), with reports of 'juvenile' and 'congenital' forms, as well as the classical infantile type. In this report. six of 22 patients with infantile‐onset Canavan disease survived beyond six years of age. This phenotypical pattern might be the result of better medical management and care, rather than evidence of genetic heterogeneity.