Cerebellar disorders in childhood: cognitive problems

Over the last decade, increasing evidence of cognitive functions of the cerebellum during development and learning processes could be ascertained. Posterior fossa malformations such as cerebellar hypoplasia or Joubert syndrome are known to be related to developmental problems in a marked to moderate extent. More detailed analyses reveal special deficits in attention, processing speed, visuospatial functions, and language. A study about Dandy Walker syndrome states a relationship of abnormalities in vermis lobulation with developmental problems. Further lobulation or volume abnormalities of the cerebellum and/or vermis can be detected in disorders as fragile X syndrome, Downs's syndrome, William's syndrome, and autism. Neuropsychological studies reveal a relation of dyslexia and attention deficit disorder with cerebellar functions. These functional studies are supported by structural abnormalities in neuroimaging in these disorders. Acquired cerebellar or vermis atrophy was found in groups of children with developmental problems such as prenatal alcohol exposure or extreme prematurity. Also, focal lesions during childhood or adolescence such as cerebellar tumor or stroke are related with neuropsychological abnormalities, which are most pronounced in visuospatial, language, and memory functions. In addition, cerebellar atrophy was shown to be a bad prognostic factor considering cognitive outcome in children after brain trauma and leukemia. In ataxia teleangiectasia, a neurodegenerative disorder affecting primarily the cerebellar cortex, a reduced verbal intelligence quotient and problems of judgment of duration are a hint of the importance of the cerebellum in cognition. In conclusion, the cerebellum seems to play an important role in many higher cognitive functions, especially in learning. There is a suggestion that the earlier the incorrect influence, the more pronounced the problems.

Effects of instructional changes on student learning of electrochemistry in an IB chemistry course

This study investigated the effectiveness of incorporating several new instructional strategies into an International Baccalaureate (IB) chemistry course in terms of how they supported high school seniors’ understanding of electrochemistry. The three new methods used were (a) providing opportunities for visualization of particle movement by student manipulation of physical models and interactive computer simulations, (b) explicitly addressing common misconceptions identified in the literature, and (c) teaching an algorithmic, step-wise approach for determining the products of an aqueous solution electrolysis. Changes in student understanding were assessed through test scores on both internally and externally administered exams over a two-year period. It was found that visualization practice and explicit misconception instruction improved student understanding, but the effect was more apparent in the short-term. The data suggested that instruction time spent on algorithm practice was insufficient to cause significant test score improvement. There was, however, a substantial increase in the percentage of the experimental group students who chose to answer an optional electrochemistry-related external exam question, indicating an increase in student confidence. Implications for future instruction are discussed.

Uncovering poiesis : the role of production in technical communication, work, and public life

This dissertation is a report on a collaborative project between the Computer Science and the Humanities Departments to develop case studies that focus on issues of communication in the workplace, and the results of their use in the classroom. My argument is that case study teaching simulates real-world experience in a meaningful way, essentially developing a teachable way of developing phronesis, the reasoned capacity to act for the good in public. In addition, it can be read as a "how-to" guide for educators who may wish to construct their own case studies. To that end, I have included a discussion of the ethnographic methodologies employed, and how it was adapted to our more pragmatic ends. Finally, I present my overarching argument for a new appraisal of the concept of techné. This reappraisal emphasizes its productive activity, poiesis, rather than focusing on its knowledge as has been the case in the past. I propose that focusing on the telos, the end outside the production, contributes to the diminishment, if not complete foreclosure, of a rich concept of techné.

[Acute asthma attacks in childhood]

The diagnosis of an acute asthmatic attack in a child is made on a clinical basis. The severity of the exacerbation can be assessed by physical examination and measurement of the transcutaneous oxygenation saturation. A blood gas analysis can be helpful in this assessment. A child with a severe asthma exacerbation should be promptly referred to an emergency department of a hospital. Oxygen should be given to keep the oxygen saturation above 92% and short-acting, selective beta-2 agonists should be administered. Beta-2 agonists can be delivered by intermittent nebulization, continuous nebulization or by metered dose inhaler (MDI) with a spacer They can also be given intravenously in patients who are unresponsive to escalating therapy. The early administration of systemic corticosteroids is essential for the management of acute asthma in children. When tolerated, systemic corticoseroids can be given orally but inhaled corticosteroids are not recommended. Oxygen delivery, beta-2 agonists and steroid therapy are the mainstay of emergency treatment. Hypovolemia should be corrected either intravenously or orally. Administration of multiple doses of ipratropium bromide has been shown to decrease the hospitalization rate in children and adolescents with severe asthma. Clinical response to initial treatment is the main criterion for hospital admission. Patients with failure to respond to treatment should be transferred to an intensive care unit. A critical aspect of management of the acute asthma attack in a child is the prevention of similar attacks in the future.

Long-term follow-up after stroke in childhood

Over the last few years, the importance of paediatric stroke has become more and more evident; however, there is still little known about long-term neurological and especially neuropsychological outcome of these children. By retrospective chart review, questionnaire and clinical examination with structured interview, we analysed initial presentation, aetiology and long-term outcome of children suffering ischaemic childhood stroke between 1985 and 1999. A total of 20 children (13 boys) suffered acute arterial ischaemic events. Aetiology was detected in 14, and suspected in another five. Follow-up after 1-15 years (mean 7 years) was possible for 16 children; two had died and two were lost to follow-up. Only two were completely healthy, five suffered mild, six moderate, and three severe handicap. Eleven children presented with combined neurological and neuropsychological problems. Neurological problems were mild to moderate hemisyndrome in 11, dysphasia, epilepsy and other in six each. Mild to severe neuropsychological problems were detected in 13 children, school problems in eight, attention deficits in nine and behaviour problems in seven, increased fatigability and headache in six each. Recurrence was observed in three children, all due to progressive underlying disease. Outcome was most affected by the presence of combined cortical/subcortical and least affected by subcortical infarction. Epilepsy affected neuropsychological outcome. CONCLUSION: although prognosis of paediatric stroke is better than for adult stroke, neurological and especially neuropsychological long-term problems significantly influence the lives of these children. Careful long-term follow-up to support these children in their school career and integration into professional life is necessary. Future studies should evaluate whether specific treatments during the acute episode could improve outcome for these children.

Non-infectious causes of uveitis in 70 Swiss children

Many diseases are linked with uveitis, but few studies have specifically looked at the noninfectious triggers of childhood uveitis in Central Europe. The charts of 70 paediatric patients with non-infectious uveitis admitted to the Department of Pediatrics, University of Bern, Switzerland, between 1983 and 1998 were therefore reviewed. In the patients the age at presentation with uveitis ranged between 0.3 and 16 y, median 8.5 y. Based on the localization, uveitis anterior was diagnosed in most cases (n = 40; 57%), followed by panuveitis (n = 20; 29%) and uveitis posterior (n = 10; 14%). Uveitis was chronic in 54 (77%) and acute in 16 (23%), bilateral in 38 (54%) and unilateral in 32 (46%) cases. An associated condition was noted in 32 (46%) cases: juvenile idiopathic arthritis in 24 cases, sarcoidosis and juvenile spondyloarthropathy in 3 cases, and Sjögren's syndrome and Behçet's disease in 1 case each. In the remaining 38 (54%) patients, no associated condition was diagnosed. It is concluded that in Swiss children, uveitis can be due to a wide spectrum of non-infectious diseases, juvenile idiopathic arthritis being the leading cause. In the majority of the children, no associated condition was recognized.

An outbreak of mumps in a population partially vaccinated with the Rubini strain

Since 1991, 6 years after the recommendation of universal childhood triple vaccination against measles, mumps and rubella (M + M + R), Switzerland has been confronted with an increasing number of mumps cases affecting both vaccinated and unvaccinated children. The M + M + R vaccine mainly used in the Swiss population after 1986 contains the highly attenuated Rubini strain of mumps virus. We analysed an outbreak of 102 suspected mumps cases by virus isolation, determination of IgM antibodies to mumps virus in 27 acute phase sera, and verification of vaccination histories. Mumps was confirmed by virus isolation in 88 patients, of whom 72 had previously received the Rubini vaccine strain. IgM antibodies to mumps virus were detected in 24/27 acute phase serum samples. A group of 92 subjects from the same geographic area without signs of mumps virus infection served as controls. IgG antibodies to mumps virus and vaccination status were assessed in these children. The vaccination rate in these controls was 61%, with equal seropositivity for unvaccinated and Rubini-vaccinated subjects. These data support other recent reports which indicate an insufficient protective efficacy of current mumps vaccines.

RNA interference-mediated c-MYC inhibition prevents cell growth and decreases sensitivity to radio- and chemotherapy in childhood medulloblastoma cells

BACKGROUND: With current treatment strategies, nearly half of all medulloblastoma (MB) patients die from progressive tumors. Accordingly, the identification of novel therapeutic strategies remains a major goal. Deregulation of c-MYC is evident in numerous human cancers. In MB, over-expression of c-MYC has been shown to cause anaplasia and correlate with unfavorable prognosis. METHODS: To study the role of c-MYC in MB biology, we down-regulated c-MYC expression by using small interfering RNA (siRNA) and investigated changes in cellular proliferation, cell cycle analysis, apoptosis, telomere maintenance, and response to ionizing radiation (IR) and chemotherapeutics in a representative panel of human MB cell lines expressing different levels of c-MYC (DAOY wild-type, DAOY transfected with the empty vector, DAOY transfected with c-MYC, D341, and D425). RESULTS: siRNA-mediated c-MYC down-regulation resulted in an inhibition of cellular proliferation and clonogenic growth, inhibition of G1-S phase cell cycle progression, and a decrease in human telomerase reverse transcriptase (hTERT) expression and telomerase activity. On the other hand, down-regulation of c-MYC reduced apoptosis and decreased the sensitivity of human MB cells to IR, cisplatin, and etoposide. This effect was more pronounced in DAOY cells expressing high levels of c-MYC when compared with DAOY wild-type or DAOY cells transfected with the empty vector. CONCLUSION: In human MB cells, in addition to its roles in growth and proliferation, c-MYC is also a potent inducer of apoptosis. Therefore, targeting c-MYC might be of therapeutic benefit when used sequentially with chemo- and radiotherapy rather than concomitantly.

Diminished prefrontal brain function in adults with psychopathology in childhood related to attention deficit hyperactivity disorder

The aim of the present study was to investigate prefrontal brain function and cognitive response control in patients with personality disorders who either suffered or did not suffer from psychopathology related to attention deficit hyperactivity disorder (ADHD) during childhood. For this purpose, 36 psychiatric out-patients with personality disorders--24 of whom showed ADHD-related psychopathology during childhood assessed by the German short form of the Wender Utah Rating Scale--and 24 healthy controls were investigated electrophysiologically by means of a cued Go-NoGo task (Continuous Performance Test). Topographical analyses were conducted to individually quantify the NoGo anteriorisation (NGA), a neurophysiological correlate of prefrontal response control that has been suggested to reflect activation of the anterior cingulate cortex. ADHD patients exhibited a significantly reduced mean NGA and diminished amplitudes of the Global Field Power, as well as a reduced increase of fronto-central P300 amplitudes, in NoGo-trials compared with the healthy controls, whereas patients with personality disorders alone did not differ from the control group in any of the electrophysiological parameters. The results indicate that ADHD-related psychopathology is associated with prefrontal brain dysfunction, probably related to processes of response inhibition and/or cognitive response control.

Suprasellar germinomas in childhood and adolescence: diagnostic pitfalls

The clinical and neuro-endocrine data of seven young male patients with suprasellar germinomas seen between 1984 and 1992 are reported. The most common initial symptom was 'idiopathic' central diabetes insipidus (DI), which occurred in all seven patients. The time interval between the appearance of this first clinical sign and the definitive diagnosis of a suprasellar germinoma ranged from 3 to 66 months. Raised prolactin levels and growth hormone deficiency were indicators of a process located in the hypothalamic-pituitary region. An increased beta-HCG level in the serum or the CSF confirmed the diagnostic suspicion of a germinoma and was helpful as a tumor marker in follow-up. Neuro-radiologic studies (CT or MRI) were also disappointing in the early stage when patients presented only with DI. Later on, as patients developed additional symptoms or signs related to the tumor, imaging studies were positive. Given the variable rate of tumor progression, the nonspecific early signs of hypothalamic-pituitary dysfunction (DI) as well as the often negative early imaging studies, the diagnosis of suprasellar germinoma is difficult but should always be considered in the presence of so-called 'idiopathic' central DI. Repeated brain MRIs are mandatory in young patients with idiopathic DI in order not to miss an underlying suprasellar germinoma.

Using Moodle as a support tool for teaching in Higher Education in Portugal: an exploratory study

The use of virtual learning environments in Higher Education (HE) has been growing in Portugal, driven by the Bologna Process. An example is the use of Learning Management Systems (LMS) that translates an opportunity to leverage the use of technological advances in the educational process. The progress of information and communication technologies (ICT) coupled with the great development of Internet has brought significant challenges to educators that require a thorough knowledge of their implementation process. These field notes present the results of a survey among teachers of a private HE institution in its use of Moodle as a tool to support face-to-face teaching. A research methodology essentially of exploratory nature based on a questionnaire survey, supported by statistical treatment allowed to detect motivations, type of use and perceptions of teachers in relation to this kind of tool. The results showed that most teachers, by a narrow margin (58%), had not changed their pedagogical practice as a consequence of using Moodle. Among those that did 67% attended institutional internal training. Some of the results obtained suggest further investigation and provide guidelines to plan future internal training.

The Position of xMOOCs in Educational Systems

The idea of xMOOCs initially aimed at fundamentally changing the US tertiary education system by providing open mass education. This attempt failed for a number of reasons. They include: the ignorance of the importance and benefits of face-to-face instruction, the high workload imposed on students by xMOOCs, the consequences of current xMOOC didactics, the financing of the high costs, and the difficulties of integration into the teaching organization. As a consequence, xMOOCs are turning into methods for professional continuing education including a business model that covers the institution’s cost.

The role of specialisation in the promotion of young football talents: A person-oriented study

This paper investigates the controversial question whether it is more effective to promote specialisation in a specific sport at the beginning of a career or whether to encourage a broad range of sports when promoting competitive sports talents in order for them to achieve a high level of performance in adulthood. The issue of promoting talents depends on human developmental processes and therefore raises developmental scientific questions. Based on recent, dynamic-interactionist concepts of development, we assume a person-oriented approach focussing on the person as a whole rather than individual features. Theoretical considerations lead to four interacting factors being summarised to form a subsystem: childhood training. The relative weights of these factors lead to patterns. By relating these to a performance criterion at the age of peak performance, particularly promising developmental patterns may be identified. One hundred fifty-nine former Swiss football talents were retrospectively interviewed about their career and the data analysed using the LICUR method. Two early career patterns were identified as having a favourable influence on adult performance. Both are characterised by an above-average amount of in-club training. One pattern also exhibits an above-average amount of informal football played outside the club, the other above-average scores for activity in other sports. Hence, comprehensive training and practice inside and outside the club form the basis for subsequent football expertise.

Life partnerships in childhood cancer survivors, their siblings, and the general population

Background: Life partnerships other than marriage are rarely studied in childhood cancer survivors (CCS). We aimed (1) to describe life partnership and marriage in CCS and compare them to life partnerships in siblings and the general population; and (2) to identify socio-demographic and cancer-related factors associated with life partnership and marriage. Methods: As part of the Swiss Childhood Cancer Survivor Study (SCCSS), a questionnaire was sent to all CCS (aged 20–40 years) registered in the Swiss Childhood Cancer Registry (SCCR), aged <16 years at diagnosis, who had survived ≥5 years. The proportion with life partner or married was compared between CSS and siblings and participants in the Swiss Health Survey (SHS). Multivariable logistic regression was used to identify factors associated with life partnership or marriage. Results: We included 1,096 CCS of the SCCSS, 500 siblings and 5,593 participants of the SHS. Fewer CCS (47%) than siblings (61%, P < 0.001) had life partners, and fewer CCS were married (16%) than among the SHS population (26%, P > 0.001). Older (OR = 1.14, P < 0.001) and female CCS (OR = 1.85, <0.001) were more likely to have life partners. CCS who had undergone radiotherapy, bone marrow transplants (global PTreatment = 0.018) or who had a CNS diagnosis (global PDiagnosis < 0.001) were less likely to have life partners. Conclusion: CCS are less likely to have life partners than their peers. Most CCS with a life partner were not married. Future research should focus on the effect of these disparities on the quality of life of CCS.

Veridicality of self-concept of strengths in male adolescents

Changes in physical self-concept and veridicality (i.e., the appropriateness of one’s self-perceptions to reality) during childhood and adolescence are related to performance in physical fi tness, exercise, and global self-esteem, particularly in male adolescents. This cross-sectional study examined the relation between the self-concept of strength and actual strength in groups of male adolescents ages 12 to 15 years. For this purpose, 889 male adolescents (M age = 13.2 yr., SD = 1.0) were questioned about their self-concept of strength, after which a test of strength was carried out. The results showed that the correlation between self- concept of strength and actual strength was higher in older (14- and 15-year-old) than younger (12- and 13-year-old) adolescents. There was a statistically signifi cant association between age group and veridicality, indicating that realistic self-concept is underrepresented among younger, but overrepresented among older adolescents. No association was found between the adolescents’ physical activity and veridicality. The consequences for further research on the development of veridicality in the physical domain are discussed.