Metabolic control of type 1 diabetic patients followed at the University Children's Hospital in Berne: Have we reached the goal?

In type 1 diabetes (T1DM), a good metabolic control is important to reduce and/or postpone complications. Guidelines regarding how to achieve this goal are published by the American Diabetes Association (ADA) and the International Society of Paediatric and Adolescence Diabetes (ISPAD). The aims of this study were to determine the current level of metabolic control in T1DM patients on different treatment regimens, followed at the diabetes outpatient unit of the University Children's Hospital Bern, Switzerland, and to compare it with both the reported data from ten years ago (1998) and with the current guidelines of the ADA and ISPAD.

From nature versus nurture, via nature and nurture, to gene x environment interaction in mental disorders

It is now generally accepted that complex mental disorders are the results of interplay between genetic and environmental factors. This holds out the prospect that by studying G x E interplay we can explain individual variation in vulnerability and resilience to environmental hazards in the development of mental disorders. Furthermore studying G x E findings may give insights in neurobiological mechanisms of psychiatric disorder and so improve individualized treatment and potentially prevention. In this paper, we provide an overview of the state of field with regard to G x E in mental disorders. Strategies for G x E research are introduced. G x E findings from selected mental disorders with onset in childhood or adolescence are reviewed [such as depressive disorders, attention-deficit/hyperactivity disorder (ADHD), obesity, schizophrenia and substance use disorders]. Early seminal studies provided evidence for G x E in the pathogenesis of depression implicating 5-HTTLPR, and conduct problems implicating MAOA. Since then G x E effects have been seen across a wide range of mental disorders (e.g., ADHD, anxiety, schizophrenia, substance abuse disorder) implicating a wide range of measured genes and measured environments (e.g., pre-, peri- and postnatal influences of both a physical and a social nature). To date few of these G x E effects have been sufficiently replicated. Indeed meta-analyses have raised doubts about the robustness of even the most well studied findings. In future we need larger, sufficiently powered studies that include a detailed and sophisticated characterization of both phenotype and the environmental risk.

Alveolarization continues during childhood and adolescence: new evidence from helium-3 magnetic resonance

The current hypothesis that human pulmonary alveolarization is complete by 3 years is contradicted by new evidence of alveolarization throughout adolescence in mammals.

Hereditary angioedema (HAE) in children and adolescents--a consensus on therapeutic strategies

Hereditary angioedema due to C1 inhibitor (C1 esterase inhibitor) deficiency (types I and II HAE-C1-INH) is a rare disease that usually presents during childhood or adolescence with intermittent episodes of potentially life-threatening angioedema. Diagnosis as early as possible is important to avoid ineffective therapies and to properly treat swelling attacks. At a consensus meeting in June 2011, pediatricians and dermatologists from Germany, Austria, and Switzerland reviewed the currently available literature, including published international consensus recommendations for HAE therapy across all age groups. Published recommendations cannot be unconditionally adopted for pediatric patients in German-speaking countries given the current approval status of HAE drugs. This article provides an overview and discusses drugs available for HAE therapy, their approval status, and study results obtained in adult and pediatric patients. Recommendations for developing appropriate treatment strategies in the management of HAE in pediatric patients in German-speaking countries are provided.Conclusion Currently, plasma-derived C1 inhibitor concentrate is considered the best available option for the treatment of acute HAE-C1-INH attacks in pediatric patients in German-speaking countries, as well as for short-term and long-term prophylaxis.

Parental Divorce in Young Adulthood: Some Preliminary Findings

Developmental differences in reactions to parental divorce have not been examined beyond adolescence, and the impact of divorce on older offspring has thus been minimized. Central themes that emerged from an exploratory study of 18-to-23-year-olds suggest that the break-up of their parents' marriage represents a critical event deserving of further attention from researchers and clinicians.

Childhood Adoption: Long-Term Effects in Adulthood

Clinicians and researchers have characterized early life experiences as permanent and stable influences on the personality and subsequent life experiences of an individual. Recent conceptualizations have suggested that personal and environmental factors influencing development are not deterministic. Multiple pathways into adulthood are possible. Adoption is one potential early life stressor that may illustrate the usefulness of such conceptualizations for assessing long-term effects in adulthood. Previous studies of adoption have characterized the effects of adoption into adolescence and young adulthood. The purpose of this study was to provide an initial assessment of the long-term impact of adoption. The participants were taken from the Swedish Adoption/Twin Study of Aging. From the original sample, we identified a subsample of 60 pairs of twins who were separated and reared apart, with one member being raised by a biological parent or parents and the other by an adoptive parent or parents with no biological relationship. A series of univariate and multivariate analyses were undertaken to assess the elements associated with being reared in either an adoptive home or the home of biological parent(s). The results suggest few significant effects of adoption on the adult adjustment of adoptees. In particular, the results reflect the important mediating role of childhood socioeconomic status, suggesting that the stress of adoption itself is mediated by the type of rearing environment provided by the adoption process.

Child Aggression in a Post-Communist Country: Race and Gender Aspects in a Cross-Cultural Comparison

Gypsies represent approximately 2.5% of the Czech population, but are considerably over-represented among the unemployed, prisoners, schooldropouts, neglected children, etc. Together with racist attitudes on the part of the majority, this causes strong inter-ethnic tension and obviouseconomic, moral and political problems. This research studied the way in which this situation is reflected in peer relations between Gypsy andmajority children in schools. Six samples of children (totalling 2974 children aged 7-15, of whom 15% were Gypsies) were studied through peernomination, teacher assessment and self-reporting. Gypsy/non-Gypsy and gender dichotomies were correlated with measures of aggression,victimisation and acceptance/rejection. The results showed that Gypsy children, both boys and girls, were more likely to nominate their Gypsy peers as aggressors than they nominatemajority children, implying that they tend to direct their rejection toward their own kind. The number of Gypsy children in a class was also animportant factor with Gypsies being more likely to be accepted and less likely to appear aggressive when they were only one or two in a class, thanin a class where there was a greater number of Gypsy pupils. When whole classes were taken as the unit of analysis, Gypsy children were seen asmore likely to behave aggressively in class by their Gypsy and non-Gypsy counterparts as well as by their teachers. At the same time they aremuch less likely to become victims of aggression than are non-Gypsy children, both boys and girls. Mr. Rican also found that the acceptance/rejection patterns of Gypsy children betray their unsatisfactory socialisation. Among their peers, Gypsyor non-Gypsy, they tend to prefer aggressors or children who teachers describe as showing little discipline or effort to succeed at school. Partialcorrelation to assess the influence of seniority on aggressiveness provided a warning that the recent lengthening of compulsory school attendance islikely to bring an increase in aggressiveness. He believes that Gypsy ethnic identity has lost many of its important positive aspects, making itsnegative aspects more prominent and more dangerous. He does however find some possible ways for teachers to reinforce the positive aspects ofGypsy children's identities in order to support their socialisation at schools.

Family Anomie and Juvenile Deliquency in Post-Communist Romania

The sensitivity of crime rates to social, economic and political influences has long aroused the interest of sociologists who have attempted to explain what kind of relationships might be associated with variations in crime rates between different social groups at different times. The earliest views were put forward by Emil Durkheim, and while later writers have developed (R.K. Merton, L. Srole, A, K. Cohen, etc.) have developed some aspects of his ideas further, his basic ideas of the divorce of the individual from normative standards and the lack of social integration are still valid. Ms. Voicu-Minea looked at the theoretical background in detail but then limited it to a specific social group, the family, asking first why certain individual within vulnerable families and/or negative social influences commit offences while others do not. In modern times the family has undergone massive structural and functional changes. Its former economic function, which once endowed it with a great capacity for social inclusion, has generally vanished, while its formerly crucial role in children's education has been massively reduced. These changes, which are still not complete, can lead to dysfunction and in certain social contexts such as that in post-communist Romanian society, this risk of dysfunction is still greater as unfavourably social circumstances more easily affect such families. The number of cases of juvenile delinquency in Romania has increased sharply ever since the end of the communist system and in 1996 reached the level of 18,317 cases. The sample examined included 1012 juvenile delinquents aged between 14 and 18, taken from all areas of Bucharest. Over 80% of charges related to theft, with more serious offences being relatively rare. The children underwent a series of psychological tests, accompanied by a questionnaire relating to family situation. The results showed that juvenile delinquency in Romania is overwhelmingly male, with 91.8% of offences being committed by boys. Two thirds of the research group were under the age of 16 and only just over one third attended school, with over half having left school before the legal age. While the majority of subjects had a lower than average level of education, they did not always recognise this, with two thirds seeing their level of education as being as good as or better than average. Nearly half the children (43%) did not live with both natural parents and majority came from families with three or more children. This applied both to their original families and to the families in which they were living at the time of the survey. The overwhelming majority of families were living in or around Bucharest, but under one third originated from there. Almost 25% of parents were under-schooled and around one third were unqualified workers. At least 30% of families lived in inadequate accommodation and family incomes were generally low. Ms. Voicu-Minea does however point out that over half the minors from the sample saw their family income as satisfactory or even more than satisfactory. When factors such as bad relationships between parents, corporal punishment, alcohol consumption and criminal records of family members were taken into account, the picture was bleak, making it understandable why over 36% of subjects had run away from home at least once, and in many cases repeatedly and for longer periods. The overwhelming majority of offences (80.8%) were committed in groups of between 2 and 11 persons, usually "friends" but in about 10% of cases member's of the family. IQ tests put about 75% of the sample at slightly under average, the difference being too slight to account for the behaviour problems of the majority. Personality tests, however, showed a different picture. Over 70% of those tested manifested an acute need of tenderness and a similar number a high level of potential aggressiveness. Almost half of the minors expressed such feelings as intolerance or a desire for revenge, and Ms. Voicu-Minea found a clear weakness of the Self. Around half the sample expressed sentiments of abandonment, renunciation and solitude.

Fabry's disease: otoneurologic findings in twelve members of one family

Fabry's disease corresponds to an inherited disorder transmitted by an X-linked recessive gene. It generates a dysfunction of glycosphingolipid metabolism due to an enzymatic deficiency of alpha-galactosidase activity, resulting in glycosphingolipid deposits in all areas of the body. The clinical (heart, kidney, and central nervous system) manifestations are more severe in hemizygous boys than in heterozygous girls. They appear during childhood or adolescence: acroparesthesia, joint pain, angiokeratoma, corneal dystrophy, hypohydrosis or anhydrosis, and renal failure. The otoneurologic symptoms consist of hearing fluctuation, progressive unilateral or bilateral hearing loss, and episodes of vertigo or dizziness. Otoneurologic findings in 12 of 26 members of the same family are presented: the mother and 9 of her 12 children, as well as 2 of her 14 grandchildren: 4 healthy persons, 4 heterozygous female carriers, and 4 hemizygous male patients. Three of the male patients had fluctuation of hearing, sudden hearing loss, and episodes of vertigo and dizziness. The otoneurologic examinations showed a bilateral cochleovestibular deficit (n = 1), a right cochleovestibular deficit (n = 1), and a bilateral hearing loss combined with a right vestibular deficit (n = 1). Histopathologic evidence of glycosphingolipid accumulation in vascular endothelial and ganglion cells, as well as atrophy of the stria and spiral ligament, might explain the otoneurologic symptoms and findings.

On the postnatal development of the striate cortex (V1) in the tree shrew (Tupaia belangeri)

Histological serial sections, three-dimensional reconstructions and morphometry served to study the postnatal development of V1 in tree shrews. The main objectives were to evaluate the expansion of V1, the implications of its growth on the occipital cortex and, vice versa, the effects of the expanding neocortex on the topography of V1. The future V1 was identified on postnatal day 1 by its granular layer IV, covering the superior surface of the occipital cortices including the poles. A subdivision of layer IV, distinctive for the binocular part, was evident in the central region. V1 expanded continuously with age into all directions succeeded by the maturation of layering. The monocular part was recognized from day 15 onward, after the binocular part had reached its medial border. In reference to the retinotopic map of V1, regions emerged in a coherent temporo-spatial sequence delineating the retinal topography in a central to peripheral gradient beginning with the visual streak representation. The growth of V1 was greatest until tree shrews open their eyes, culminated during adolescence, and completed after a subsequent decrease in the young adult. Simultaneous expansion of the neocortex induced a shifting of V1. Translation and elongation of V1 entailed that the occipital cortex covered the superior colliculi along with a downward rotation of the poles. The enlargement of the occipital part of the hemispheres was in addition associated with the formation of a small occipital horn in the lateral ventricles, indicating an incipient 'true' occipital lobe harbouring mainly cortices involved in visual functions.

Strengthening of laterality of verbal and visuospatial functions during childhood and adolescence

Cognitive functions in the child's brain develop in the context of complex adaptive processes, determined by genetic and environmental factors. Little is known about the cerebral representation of cognitive functions during development. In particular, knowledge about the development of right hemispheric (RH) functions is scarce. Considering the dynamics of brain development, localization and lateralization of cognitive functions must be expected to change with age. Twenty healthy subjects (8.6-20.5 years) were examined with fMRI and neuropsychological tests. All participants completed two fMRI tasks known to activate left hemispheric (LH) regions (language tasks) and two tasks known to involve predominantly RH areas (visual search tasks). A laterality index (LI) was computed to determine the asymmetry of activation. Group analysis revealed unilateral activation of the LH language circuitry during language tasks while visual search tasks induced a more widespread RH activation pattern in frontal, superior temporal, and occipital areas. Laterality of language increased between the ages of 8-20 in frontal (r = 0.392, P = 0.049) and temporal (r = 0.387, P = 0.051) areas. The asymmetry of visual search functions increased in frontal (r = -0.525, P = 0.009) and parietal (r = -0.439, P = 0.027) regions. A positive correlation was found between Verbal-IQ and the LI during a language task (r = 0.585, P = 0.028), while visuospatial skills correlated with LIs of visual search (r = -0.621, P = 0.018). To summarize, cognitive development is accompanied by changes in the functional representation of neuronal circuitries, with a strengthening of lateralization not only for LH but also for RH functions. Our data show that age and performance, independently, account for the increases of laterality with age.

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.

Using PSA to guide timing of androgen deprivation in patients with T0-4 N0-2 M0 prostate cancer not suitable for local curative treatment (EORTC 30891)

OBJECTIVE: EORTC trial 30891 compared immediate versus deferred androgen-deprivation therapy (ADT) in T0-4 N0-2 M0 prostate cancer (PCa). Many patients randomly assigned to deferred ADT did not require ADT because they died before becoming symptomatic. The question arises whether serum prostate-specific antigen (PSA) levels may be used to decide when to initiate ADT in PCa not suitable for local curative treatment. METHODS: PSA data at baseline, PSA doubling time (PSADT) in patients receiving no ADT, and time to PSA relapse (>2 ng/ml) in patients whose PSA declined to <2 ng/ml within the first year after immediate ADT were analyzed in 939 eligible patients randomly assigned to immediate (n=468) or deferred ADT (n=471). RESULTS: In both arms, patients with a baseline PSA>50 ng/ml were at a>3.5-fold higher risk to die of PCa than patients with a baseline PSAine PSA was between 8 and 50 ng/ml, the risk of PCa death was approximately 7.5-fold higher in patients with PSADT<12 mo than in patients with PSADT>12 mo. Time to PSA relapse after response to immediate ADT correlated significantly with baseline PSA, suggesting that baseline PSA may also reflect disease aggressiveness. CONCLUSIONS: Patients with a baseline PSA>50 ng/ml and/or a PSADT<12 mo were at increased risk to die from PCa and might have benefited from immediate ADT, whereas patients with a baseline PSA<50 ng/ml and a slow PSADT (>12 mo) were likely to die of causes unrelated to PCa, and thus could be spared the burden of immediate ADT.

Patterns of exposure to infectious diseases and social contacts in early life and risk of brain tumours in children and adolescents: an International Case-Control Study (CEFALO)

BACKGROUND Infectious diseases and social contacts in early life have been proposed to modulate brain tumour risk during late childhood and adolescence. METHODS CEFALO is an interview-based case-control study in Denmark, Norway, Sweden and Switzerland, including children and adolescents aged 7-19 years with primary intracranial brain tumours diagnosed between 2004 and 2008 and matched population controls. RESULTS The study included 352 cases (participation rate: 83%) and 646 controls (71%). There was no association with various measures of social contacts: daycare attendance, number of childhours at daycare, attending baby groups, birth order or living with other children. Cases of glioma and embryonal tumours had more frequent sick days with infections in the first 6 years of life compared with controls. In 7-19 year olds with 4+ monthly sick day, the respective odds ratios were 2.93 (95% confidence interval: 1.57-5.50) and 4.21 (95% confidence interval: 1.24-14.30). INTERPRETATION There was little support for the hypothesis that social contacts influence childhood and adolescent brain tumour risk. The association between reported sick days due to infections and risk of glioma and embryonal tumour may reflect involvement of immune functions, recall bias or inverse causality and deserve further attention.

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.