Deficit in memory consolidation (abnormal forgetting rate) in childhood temporal lobe epilepsy. Pre and postoperative long-term observation.

Deficits in memory consolidation have been reported in adult patients with epilepsy but, not to our knowledge, in children. We report the long-term follow-up (9 y. o. to 18 y. o.) of a boy who suffered from temporal lobe epilepsy and underwent a left temporal lobectomy with amygdalo-hippocampal resection at the age of 10. He showed an abnormal forgetting rate when trying to encode new information and a significant deficit for retrieving remote episodic memories (when compared with his twin brother), both consistent with a consolidation disorder. His memory condition slightly improved after cessation of the epilepsy, nevertheless did not normalize. No standard memory assessment could pinpoint his memory problem, hence an adapted methodology was needed. We discuss the nature of the memory deficit, its possible causes and its clinical implications.

Randomized Trial of prophylactic granulocyte colony-stimulating factor during rapid COJEC induction in pediatric patients with high-risk neuroblastoma: the European HR-NBL1/SIOPEN study.

Purpose To reduce the incidence of febrile neutropenia during rapid COJEC (cisplatin, vincristine, carboplatin, etoposide, and cyclophosphamide given in a rapid delivery schedule) induction. In the High-Risk Neuroblastoma-1 (HR-NBL1) trial, the International Society of Paediatric Oncology European Neuroblastoma Group (SIOPEN) randomly assigned patients to primary prophylactic (PP) versus symptom-triggered granulocyte colony-stimulating factor (GCSF; filgrastim). Patients and Methods From May 2002 to November 2005, 239 patients in 16 countries were randomly assigned to receive or not receive PPGCSF. There were 144 boys with a median age of 3.1 years (range, 1 to 17 years) of whom 217 had International Neuroblastoma Staging System (INSS) stage 4 and 22 had stage 2 or 3 MYCN-amplified disease. The prophylactic arm received a single daily dose of 5 μg/kg GCSF, starting after each of the eight COJEC chemotherapy cycles and stopping 24 hours before the next cycle. Chemotherapy was administered every 10 days regardless of hematologic recovery, provided that infection was controlled. Results The PPGCSF arm had significantly fewer febrile neutropenic episodes (P = .002), days with fever (P = .004), hospital days (P = .017), and antibiotic days (P = .001). Reported Common Toxicity Criteria (CTC) graded toxicity was also significantly reduced: infections per cycle (P = .002), fever (P < .001), severe leucopenia (P < .001), neutropenia (P < .001), mucositis (P = .002), nausea/vomiting (P = .045), and constipation (P = .008). Severe weight loss was reduced significantly by 50% (P = .013). Protocol compliance with the rapid induction schedule was also significantly better in the PPGCSF arm shown by shorter time to completion (P = .005). PPGCSF did not adversely affect response rates or success of peripheral-blood stem-cell harvest. Following these results, PPG-GSF was advised for all patients on rapid COJEC induction.

Une cause de cardiomyopathie dilatée chez l'enfant: le déficit primaire en carnitine [A cause of dilated cardiomyopathy in child: primary carnitine deficiency].

AIM: The aim of this case report was to show the importance to research metabolic etiology, especially a carnitine deficiency in dilated cardiomyopathy of children. CASE REPORT: A three years old Togolese child presented muscular hypotonia, dyspnea. Examination showed left galop murmur and systolic murmur 2/6. Chest X-ray showed cardiomegaly (CTI: 0.66), electrocardiogram, a sinusal rythm, left ventricle hypertrophy and T wave abnormalities. Echocardiogram showed a markedly dilated left ventricle with reduced systolic function (EF: 0.43; reference range 0.55-0.80) and moderate mitral regurgitation. The inflammatory signs where negatives. Magnetic resonance imaging don't show signs of ischemic or myocarditis. The levels of free and total plasmatic carnitine decreased: 3μmol/L (N: 18-48μmol/L) and 5μmol/l (N: 29-70μmol/L) respectively. Mutation analysis of the gene SLC22A5 confirms the diagnosis of primary systemic carnitine deficiency. Treatment with oral carnitine was started at 200mg/kg per day. Within three weeks of treatment, we observed the decrease of all symptoms and the left ventricular size and function normalized (EF: 0.62). He has now been on oral carnitine for live. CONCLUSION: Primary carnitine deficiency is a cause of dilated cardiomyopathy in child. It must systematically be suspected when a child presents a primitive cardiomyopathy. The treatment with oral carnitine for live is simple, with excellent prognosis.

The role of epilepsy in early language development in a child with a congenital lesion in the right hemisphere

Early epilepsy is known to worsen the developmental prognosis of young children with a congenital focal brain lesion, but its direct role is often very difficult to delineate from the other variables. This requires prolonged periods of follow-up with simultaneous serial electrophysiological and developmental assessments which are rarely obtained. We studied a male infant with a right prenatal infarct in the territory of the right middle cerebral artery resulting in a left spastic hemiparesis, and an epileptic disorder (infantile spasms with transient right hemihypsarrhythmia and focal seizures) from the age of 7 months until the age of 4 years. Pregnancy and delivery were normal. A dissociated delay of early language acquisition affecting mainly comprehension without any autistic features was documented. This delay was much more severe than usually expected in children with early focal lesions, and its evolution, with catch-up to normal, was correlated with the active phase of the epilepsy. We postulate that the epilepsy specifically amplified a pattern of delayed language emergence, mainly affecting lexical comprehension, reported in children with early right hemisphere damage.

Parents living with HIV in a high-income country: do patients need specific support?

The number of HIV-infected persons with children and caregiving duties is likely to increase. From this statement, the present study was designed to establish how HIV infected caregivers organise their parenting routines and to determine their support needs. A further aim was to ascertain caregivers' perception of conspicuous behaviours displayed by their children. Finally, it sought to determine the extent to which the caregivers' assessment of their parenting activity is influenced by the required support and their children's perceived conspicuous behaviours. The study design was observational and cross-sectional. Sampling was based on the 7 HIV Outpatient Clinics associated with the national population-based Swiss HIV Cohort Study. It focused on persons living with HIV who are responsible for raising children below the age of 18. A total of 520 caregivers were approached and 261 participated. An anonymous, standardised, self-administered questionnaire was used for data collection. The data were analysed using descriptive statistical procedures and backward elimination multiple regression analysis. The 261 respondents cared for 406 children and adolescents under 18 years of age; the median age was 10 years. The caregivers' material resources were low. 70% had a net family income in a range below the median of Swiss net family income and 30% were dependent on welfare assistance. 73% were undergoing treatment with 86% reporting no physical impairments. The proportion of single caregivers was 34%. 92% of the children were living with their HIV infected caregivers. 80% of the children attended an institution such as a school or kindergarten during the day. 89% of the caregivers had access to social networks providing support. Nevertheless, caregivers required additional support in performing their parenting duties and indicated a need for assistance on the material level, in connection with legal problems and with participation in the labour market. 46% of the caregivers had observed one or more conspicuous behaviours displayed by their children, which indicates a challenging situation. However, most of these caregivers assessed their parenting activity very favourably. Backward elimination multiple regression analysis indicated that a smaller number of support needs, younger age of the eldest child and fewer physical impairments on the part of the caregiver enhance the caregivers' assessment of their parenting activity. Physicians should speak to caregivers living with HIV about their parenting responsibilities and provide the necessary scope for this subject in their consultation sessions. Physicians are in a position to draw their patients' attention to the services available to them.

Violent adolescents and their educational environment: a multilevel analysis.

OBJECTIVE: This study examined the respective roles of personal and environmental factors in youth violence in a nationally representative sample of 7548 postmandatory school students and apprentices ages 16-20 years in Switzerland. METHODS: Youth violence was defined as having committed at least one of the following in the previous 12 months: attacking an adult, snatching something, carrying a weapon, or using a weapon in a fight. Different ecological levels were tested, resulting in a three-level model only in males (individual, classroom, and school) as the low prevalence of female violence did not allow for a multilevel analysis. Dependent variables were attributed to each level. For males, the classroom level (10%) and the school level (24%) accounted for more than one third in interindividual variance. RESULTS: Factors associated with violence perpetration in females were being a victim of physical violence and sensation seeking at the individual level. In males, practicing unsafe sex, sensation seeking, being a victim of physical violence, having a poor relationship with parents, being depressed, and living in a single-parent household at the individual level; violence and antisocial acts at the classroom level; and being in a vocational school at the school level showed a correlation with violence perpetration. CONCLUSION: Interventions at the classroom level as well as an explicit school policy on violence and other risk behaviors should be considered a priority when dealing with the problem of youth violence. Furthermore, prevention should take into account gender differences.

Isolated disturbance of written language-acquisition as an initial symptom of epileptic aphasia in a 7-year-old child - a 3-year follow-up-study

A 7-year-old right-handed girl developed partial complex seizures with a left-sided onset. A brief period of post-ictal aphasia of the conduction type was documented before seizure control and complete normalization of oral language were obtained. We also found that she had a history of previous unexplained difficulty with written language acquisition that had occurred prior to the clinically recognized epilepsy and a subsequent loss of this ability. This rapidly improved with control of the epilepsy. The evolution of written language were been followed for 3 years, and continued improvement has occurred with fluctuations related to her epilepsy. This observation adds support to the growing body of data indicating that specific cognitive disturbances can be due to epilepsy in young children. It shows the vulnerability of skills which are in a period of active development, and the possibility that oral/written language can be differentially involved by cerebral dysfunction in the young child.

Effects of a school-based physical activity program on physical and psychosocial quality of life in elementary school children: a cluster-randomized trial.

The present study tested the effect of a school-based physical activity (PA) program on quality of life (QoL) in 540 elementary school children. First and fifth graders were randomly assigned to a PA program or a no-PA control condition during one academic year. QoL was assessed by the Child Health Questionnaire at baseline and postintervention. Based on mixed linear model analyses, physical QoL in first graders and physical and psychosocial QoL in fifth graders were not affected by the intervention. In first graders, the PA intervention had a positive impact on psychosocial QoL (effect size [d], 0.32; p < .05). Subpopulation analyses revealed that this effect was caused by an effect in urban (effect size [d], 0.38; p < .05) and overweight first graders (effect size [d], 0.45; p < .05). In conclusion, a school-based PA intervention had little effect on QoL in elementary school children.

Effets du stress sur l'évolution des représentations parentales au cours des 12 premiers mois de vie d'un enfant né avec une fente faciale = Stress influence on parental representations' development during the first year of a child born with a facial cleft

La venue au monde d'un enfant avec une fente faciale constitue un événement stressant, voire traumatique, pour les parents. Que ce soit à travers le risque de symptômes associés, la logistique d'alimentation ou par simples raisons d'esthétisme, de nombreuses personnes vivent l'annonce de la présence d'une fente faciale comme une intrusion brutale dans le vécu de la grossesse et une interruption momentanée de l'investissement représentationnel du foetus. À travers notre recherche, nous montrons qu'un haut stress n'influe pas forcément négativement sur le développement de la relation entre le parent et l'enfant et que, au contraire, dans le cas des pères, il peut contribuer à l'implication émotionnelle envers le bébé. Le moment de l'annonce de la malformation a aussi une importance dans les représentations à propos de l'enfant puisque si elle est faite durant la période prénatale, elle permet aux parents un réarrangement des représentations afin de mieux accueillir le bébé le jour de sa naissance. The birth of a child with a facial cleft is often seen as a very stressing event for both parents. Risks of comorbidity, difficulty in nutrition or just aesthetical reasons are frequently invoked while describing this moment. The announcement of the deformation is felt like a brutal intrusion in representations associated to pregnancy. Then, we can observe a kind of disinvestment of the coming baby. Through our study, we find that high stress has not always a bad issue on relationship between parents and infants. In contrary, this kind of chronic stress can preserve the well-being of the relationship, especially for fatherhood. The time of facial deformation announcement also plays its part in representations about the baby. Before birth, it opens room for reordering and modifying representations, then the baby will be accepted as it should be on his birthday.

Diagnostic et traitement des tumeurs intra-oculaires chez l'enfant [Diagnosis and treatment of intraocular tumors in the child]

PURPOSE: To remind of the absolute necessity for early diagnosis in the presence of ocular signs in children giving rise to possible intraocular tumours. METHOD: Based on our own experience of intraocular tumours in children, together with findings from the literature, diagnostic criteria and methods of treatment are presented. RESULTS: Retinoblastoma is the predominant cause of intraocular tumours in children, representing over 80% of cases under the age of 15 years. Other diseases may give rise to the same initial signs, usually leukocoria, sometimes strabismus, more rarely other atypical signs. Elements taken into account for diagnosis include age, sex, laterality, heredity, size of the globe, clinical aspect of the tumours, presence of calcifications and vitreous seeding. Full fundus examination under general anaesthetic is usually necessary. Biological examination, ultrasonography, computerized tomography and MRI enable an accurate diagnosis to be made in the majority of doubtful cases. The management of retinoblastoma is adapted for each individual case from the wide range of treatments available. Enucleation, radioactive applicators (...), brachytherapy (...), cryo- and photocoagulation represent classical measures. Primary chemotherapy, combined with other treatments such as thermotherapy, has become the treatment of choice in those cases where external beam radiotherapy has been used up to now, or in some instances before enucleation. Enucleation is usually carried out for medullo-epitheliomas, but brachytherapy may offer an alternative. CONCLUSION: Any unexplained ocular sign in children should be considered as a possible retinoblastoma, making an accurate and certain diagnosis imperative. Early treatment may save not only the life but also the vision of patients carrying this highly malignant lesion.

Psychological distress in adult survivors of childhood cancer: the Swiss Childhood Cancer Survivor study.

PURPOSE: To evaluate the degree of psychological distress in adult childhood cancer survivors in Switzerland and to characterize survivors with significant distress. METHODS: Childhood cancer survivors who were age younger than 16 years when diagnosed between 1976 and 2003, had survived more than 5 years, and were currently age 20 years or older received a postal questionnaire. Psychological distress was assessed using the Brief Symptom Inventory (BSI). Raw scores were transformed into T scores according to the German norm sample, and the proportion of participants being at increased risk for psychological distress was calculated (case rule: T > or = 63). t tests and univariable and multivariable logistic regressions were used for statistical analyses. RESULTS: One thousand seventy-six survivors (63.% of eligible survivors, 71.9% of contacted survivors) returned the questionnaire, 987 with complete data on BSI. Comparison with the norm populations showed lower T scores (T < 50) in the Global Severity Index (GSI; T = 46.2), somatization (T = 47.6), obsessive-compulsive tendencies (T = 46.9), and anxiety (T = 48.4). However, more childhood cancer survivors (especially women) had increased distress for GSI (14.4%), interpersonal sensitivity (16.5%), depression (13.4%), aggression (16.9%), and psychotic tendencies (15.6%) than the expected 10% from the norm population. Caseness was associated with female sex, being a single child, older age at study, and self-reported late effects, especially psychological problems. CONCLUSION: Results show that childhood cancer survivors, on average, have less psychological distress than a norm population but that the proportion of survivors at risk for high psychological distress is disproportionally large. Monitoring psychological distress in childhood cancer survivors may be desirable during routine follow-up, and psychological support should be offered as needed.