When is a child with status epilepticus likely to have Dravet syndrome?

PURPOSE: To identify clinical risk factors for Dravet syndrome (DS) in a population of children with status epilepticus (SE). MATERIAL AND METHODS: Children aged between 1 month and 16 years with at least one episode of SE were referred from 6 pediatric neurology centers in Switzerland. SE was defined as a clinical seizure lasting for more than 30min without recovery of normal consciousness. The diagnosis of DS was considered likely in previously healthy patients with seizures of multiple types starting before 1 year and developmental delay on follow-up. The presence of a SCN1A mutation was considered confirmatory for the diagnosis. Data such as gender, age at SE, SE clinical presentation and recurrence, additional seizure types and epilepsy diagnosis were collected. SCN1A analyses were performed in all patients, initially with High Resolution Melting Curve Analysis (HRMCA) and then by direct sequencing on selected samples with an abnormal HRMCA. Clinical and genetic findings were compared between children with DS and those with another diagnosis, and statistical methods were applied for significance analysis. RESULTS: 71 children with SE were included. Ten children had DS, and 61 had another diagnosis. SCN1A mutations were found in 12 of the 71 patients (16.9%; ten with DS, and two with seizures in a Generalized Epilepsy with Febrile Seizures+(GEFS+) context). The median age at first SE was 8 months in patients with DS, and 41 months in those with another epilepsy syndrome (p<0.001). Nine of the 10 DS patients had their initial SE before 18 months. Among the 26 patients aged 18 months or less at initial SE, the risk of DS was significantly increased for patients with two or more episodes (56.3%), as compared with those who had only one episode (0.0%) (p=0.005). CONCLUSION: In a population of children with SE, patients most likely to have DS are those who present their initial SE episode before 18 months, and who present with recurrent SE episodes.

Rousseau's Child: Preschoolers Expect Strangers to Favor Prosocial Actions

Modern thinking about human nature is notoriously divided between two contradictory notions: The Hobbesian tradition portrays men as driven by selfish desires, while the Rousseauian tradition recognizes altruistic proclivities as truemotivations to cooperate. We tested preschoolers' predictions about the prosocial or antisocial manner in which people would behave toward each other. Four stories were presented to 3- and 4-year-old children. In each story, the protagonists could either cooperate, act in terms of their own interests, or adopt a behavior unrelated to the ongoing scenario. Children as young as 3 years of age expected the protagonists to behave prosocially - and even more so if the protagonists were female. The results suggest that, even at an early age, children are inclined to adopt a "Rousseau-like" stance rather than a "suspicious" or "pessimistic" Hobbesian stance.

Family Alliance as a Moderator of the Link Between Maternal Postpartum Depression and Child Symptoms Assessed by Both Parents

We investigated the moderating effect of family relationships on the links between maternal postpartum depression and child symptoms in a low-risk community sample of families with 3-month-old infants (n = 57). The level of maternal depression was assessed by the Montgomery-Asberg Depression Rating Scale from a clinical interview, child symptoms by the Symptom Check List completed by both parents, and family relationships by direct observation of father-mother-baby interactions (Lausanne Trilogue Play). Families were categorized as high coordination or low coordination from their overall coordination level throughout the play. Results showed no significant links between maternal depression level and child symptoms reported by both parents. Mothers with a high depressive level in high coordination families tended to report more symptoms in their child than did mothers with lower depressive scores, whereas this link was not found in low coordination families. Prevention perspectives and clinical implications of these results are discussed.

Associations of baroreflex sensitivity, heart rate variability, and initial orthostatic hypotension with prenatal and recent postnatal methylmercury exposure in the Seychelles Child Development Study at age 19 years.

BACKGROUND: A few studies have suggested an association between prenatal exposure to methylmercury and decreased heart rate variability (HRV) related to autonomic heart function, but no study has examined this association using baroreflex sensitivity (BRS). In this study we assessed the distribution of BRS and immediate orthostatic hypotension (IOH) in young Seychellois adults and their associations with exposure to prenatal and recent postnatal methylmercury. METHODS: Subjects in the Seychelles Child Development Study (SCDS) main cohort were evaluated at age 19 years. Non-invasive beat-to-beat blood pressure (BP) monitoring (Finapres, Ohmeda) was performed at rest and during active standing in 95 consecutive subjects. Recent postnatal mercury exposure was measured in subjects' hair at the age of 19 years and prenatal exposure in maternal hair grown during pregnancy. BRS was estimated by sequence analysis to identify spontaneous ascending and descending BP ramps. HRV was estimated by the following markers: PNN50 (relative numbers of normal-to-normal intervals which are shorter by more than 50 ms than the immediately following normal-to-normal intervals); rMSSD (root mean of the squared sum of successive interval differences); LF/HF (low frequency/high frequency component ratio); ratio of the mean expiratory/inspiratory RR intervals (EI ratio); and the ratio between the longest RR interval 30 s after active standing and the shortest RR interval at 15 s (Max30/Min15). IOH was estimated by the deepest BP fall within the first 15 s after active standing up. RESULTS: Prenatal MeHg exposures were similar in boys and girls (6.7±4.3, 6.7±3.8 ng/g) but recent postnatal mercury levels were higher in males than females (11.2±5.8 vs 7.9±4.3 ng/g, p=0.003). Markers of autonomic heart rate control were within the normal range (BRS: 24.8±7 ms/mm Hg, PNN50: 24.9±6.8%, rMSSD: 68±22, LF/HF: 0.61±0.28) in both sexes. After standing, 51.4% of subjects had a transient systolic BP drop>40 mm Hg, but only 5.3% reported dizziness or had syncope. Prenatal and recent postnatal MeHg levels, overall, were not associated with BRS, E/I ratio, PNN50, rMSSD, LF/HF ratio, Max30/Min15 ratio, and IOH. CONCLUSIONS: This study provides no support for the hypothesis that prenatal or recent postnatal MeHg exposure from fish consumption is associated with impaired autonomic heart rate control.

Managing the Sick Child in the Era of Declining Malaria Transmission: Development of ALMANACH, an Electronic Algorithm for Appropriate Use of Antimicrobials.

OBJECTIVE: To review the available knowledge on epidemiology and diagnoses of acute infections in children aged 2 to 59 months in primary care setting and develop an electronic algorithm for the Integrated Management of Childhood Illness to reach optimal clinical outcome and rational use of medicines. METHODS: A structured literature review in Medline, Embase and the Cochrane Database of Systematic Review (CDRS) looked for available estimations of diseases prevalence in outpatients aged 2-59 months, and for available evidence on i) accuracy of clinical predictors, and ii) performance of point-of-care tests for targeted diseases. A new algorithm for the management of childhood illness (ALMANACH) was designed based on evidence retrieved and results of a study on etiologies of fever in Tanzanian children outpatients. FINDINGS: The major changes in ALMANACH compared to IMCI (2008 version) are the following: i) assessment of 10 danger signs, ii) classification of non-severe children into febrile and non-febrile illness, the latter receiving no antibiotics, iii) classification of pneumonia based on a respiratory rate threshold of 50 assessed twice for febrile children 12-59 months; iv) malaria rapid diagnostic test performed for all febrile children. In the absence of identified source of fever at the end of the assessment, v) urine dipstick performed for febrile children <2 years to consider urinary tract infection, vi) classification of 'possible typhoid' for febrile children >2 years with abdominal tenderness; and lastly vii) classification of 'likely viral infection' in case of negative results. CONCLUSION: This smartphone-run algorithm based on new evidence and two point-of-care tests should improve the quality of care of <5 year children and lead to more rational use of antimicrobials.

Successful embolization of a delayed splenic rupture following trauma in a child.

Delayed rupture of the spleen following trauma is an exceedingly rare phenomenon in children. In the case we have experienced, arterial embolization was successfully performed, surgery was avoided, and functional splenic tissue was preserved. Embolization is of value in the management of blunt splenic injuries in hemodynamically stable children, even after delayed rupture. The exact criteria for its use remain to be established.

The Making of Experimental Economics: Witness Seminar on the Emergence of a Field

This book is the transcript of a witness seminar on the history of experimental economics, in which eleven high-profile experimental economists participated, including Nobel Laureates Vernon Smith, Reinhard Selten and Alvin Roth. The witness seminar was constructed along four different topics: skills, community, laboratory, and funding. The transcript is preceded by an introduction explaining the method of the witness seminar and its specific set-up and resuming its results. The participants' contribution and their lively discussion provide a wealth of insights into the emergence of experimental economics as a field of research.

Development and initial validation of the Parental PELICAN Questionnaire (PaPEQu) - an instrument to assess parental experiences and needs during their child's end-of-life care.

AIM: To develop and test the Parental PELICAN Questionnaire, an instrument to retrospectively assess parental experiences and needs during their child's end-of-life care. BACKGROUND: To offer appropriate care for dying children, healthcare professionals need to understand the illness experience from the family perspective. A questionnaire specific to the end-of-life experiences and needs of parents losing a child is needed to evaluate the perceived quality of paediatric end-of-life care. DESIGN: This is an instrument development study applying mixed methods based on recommendations for questionnaire design and validation. METHOD: The Parental PELICAN Questionnaire was developed in four phases between August 2012-March 2014: phase 1: item generation; phase 2: validity testing; phase 3: translation; phase 4: pilot testing. Psychometric properties were assessed after applying the Parental PELICAN Questionnaire in a sample of 224 bereaved parents in April 2014. Validity testing covered the evidence based on tests of content, internal structure and relations to other variables. RESULTS: The Parental PELICAN Questionnaire consists of approximately 90 items in four slightly different versions accounting for particularities of the four diagnostic groups. The questionnaire's items were structured according to six quality domains described in the literature. Evidence of initial validity and reliability could be demonstrated with the involvement of healthcare professionals and bereaved parents. CONCLUSION: The Parental PELICAN Questionnaire holds promise as a measure to assess parental experiences and needs and is applicable to a broad range of paediatric specialties and settings. Future validation is needed to evaluate its suitability in different cultures.