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Place: United States; This study's objective was to correlate the abnormalities in brain MRIs performed at corrected-term age for minor or moderate neurocognitive disorders in children school-age born extremely premature (EPT) and without serious sequelae such as autism, cerebral palsy, mental impairment. Data were issued from a cross-sectional multicenter study (GP-Qol study, number NCT01675726). Clinical examination and psychometric assessments were performed when the children were between 7 and 10 years old during a day-long evaluation. Term-equivalent age brain MRIs on EPT were analyzed with a standardized scoring system. There were 114 children included in the study. The mean age at the time of evaluation, was 8.47 years old (± 0.70). 59% of children with at least one cognitive impairment and 53% who had a dysexecutive disorder. Only ten EPT (8.7%) presented moderate to severe white and grey matter abnormalities. These moderate to severe grey matter abnormalities were associated with at least two abnormal executive functions [OR 3.08 (95% CI 1.04-8.79), p = 0.04] and language delay [OR 3.25 (95% CI 1.03-9.80), p = 0.04]. These results remained significant in the multivariate analysis. Moderate to severe ventricular dilatation abnormalities (15%, n = 17) were associated with ideomotor dyspraxia [OR 7.49 (95% CI 1.48-35.95), p = 0.02] and remained significant in multivariate analysis [OR 11.2 (95% CI 1.45-131.4), p = 0.02]. Biparietal corrected diameters were moderate abnormal in 20% of cases (n = 23) and were associated to visuo spatial integration delay [OR 4.13 (95% CI 1.23-13.63), p = 0.02]. Cerebral MRI at term-equivalent age with scoring system analysis can provide information on long-term neuropsychological outcomes at school-age in EPTs children having no severe disability.

La mise en place des liens d’attachement concerne tous les acteurs de la périnatalité. Les comportements d’attachement chez l’enfant ainsi que les soins maternels sont observés en maternité puis en consultations par les pédiatres, sages-femmes et infirmières puéricultrices. Néanmoins, dès le suivi de grossesse, tous les professionnels peuvent repérer des éléments témoignant d’une certaine insécurité ou pouvant affecter les représentations maternelles. Détailler la clinique des comportements d’attachement permet d’évaluer cette dimension au cours des soins quotidiens aux nouveaunés et à sa famille. Dans certaines situations particulières telles que l’hospitalisation du nouveau-né, la vulnérabilité psychique maternelle ou au décours d’un accouchement prématuré, les équipes soignantes doivent notamment être attentives aux éléments cliniques de ces premiers liens.

Background: To investigate the relative contributions of prenatal complications, perinatal characteristics, neonatal morbidities and socio-economic conditions on the occurrence of motor, sensory, cognitive, language and psychological disorders in a large longitudinal preterm infant population during the first 7 years after birth.Methods: The study population comprised 4122 infants born at 24 hours, intrauterine growth restriction, preterm labour and maternal hypertension), perinatal characteristics (gender, multiple pregnancies, gestational age, birth weight, APGAR score and intubation or ventilation in the delivery room), neonatal complications (low weight gain during hospitalization, respiratory assistance, severe neurological anomalies, nosocomial infections) and socio-economic characteristics (socio-economic level, parental separation, urbanicity). Based on hazard ratios determined using a propensity score matching approach, population-attributable fractions (PAF) were calculated for each of the four types of determinants and for each developmental disorder.Results: The percentages of motor, sensory, cognitive, language and psychological disorders were 17.0, 13.4, 29.1, 25.9 and 26.1%, respectively. The PAF for the perinatal characteristics were the highest and they were similar for the different developmental disorders considered (around 60%). For the neonatal and socio-economic determinants, the PAF varied according to the disorder, with contributions of up to 17% for motor and 27% for language disorders, respectively. Finally, prenatal complications had the lowest contributions (between 6 and 13%).Conclusions: This study illustrates the heterogeneity of risk factors on the risk of developmental disorder in preterm infants. These results suggest the importance of considering both medical and psycho-social follow-ups of preterm infants and their families.

Background We examined how specific cognitive behavioral impairments impacted quality of life (QoL) within a large multicenter cohort of 7-10 year olds surviving extremely preterm (EPT) without major neurodevelopmental disability. Methods Between 7 and 10 years of age, two generic, self-proxy, and parental evaluations were obtained. QoL measurement questionnaires (Kidscreen-10/VSPA (Vecu et Sante Percue de l'Enfant et de l'Adolescent)) were used and compared to a reference population. The general and specific cognitive functions, such as executive functions, behavior and anxiety, and clinical neurologic examination, were also assessed. Results We analyzed 211 school-aged EPT children. The mean gestational age was 26.2 (±0.8) weeks, birth weight was 879 g (±181) and the mean age was 8.4 years (±0.87). Children with a Full-Scale Index Quotient ≥89, who were considered as normal, had a lower QoL. Specific cognitive impairments: comprehensive language delay, visuo-spatial integration defect, and dysexecutive disorders) were the QoL correlates in the domains of school performance and body image. Conclusions School and health care professionals need to increase their focus on EPT children's lower so as to recognize the preterm behavioral/cognitive phenotype and their potential need for supportive measures. Research on preventive interventions is warranted to investigate if these long-term effects of an EPT birth can be attenuated in neonatal period and after.

ObjectivePrevious studies have assessed breastfeeding-support programmes. Among these, osteopathic manipulative treatment (OMT) is a frequently used approach, although without strong evidence of efficacy.MethodsA double-blind randomised controlled trial was conducted between July 2013 and March 2016. Breastfed term infants were eligible if one of the following criteria was met: suboptimal breastfeeding behaviour, maternal cracked nipples or maternal pain. The infants were randomly assigned to the intervention or the control group. The intervention consisted of two sessions of early OMT, while in the control group, the manipulations were performed on a doll behind a screen. The primary outcome was the exclusive breastfeeding rate at 1 month, which was assessed in an intention-to-treat analysis. Randomisation was computer generated and only accessible to the osteopath practitioner. The parents, research assistants and paediatricians were masked to group assignment.ResultsOne hundred twenty-eight mother–infant dyads were randomised, with 64 assigned to each group. In each group, five infants were lost to follow-up. In the intervention group, 31 of 59 (53%) of infants were still exclusively breast fed at 1 month vs 39 of 59 (66%) in the control group, (OR 0.55, 95% CI 0.26 to 1.17; p=0.12). After adjustment for suboptimal breastfeeding behaviour, caesarean section, use of supplements and breast shields, the adjusted OR was 0.44 (95% CI 0.17 to 1.11; p=0.08). No adverse effects were reported in either group.ConclusionOMT did not improve exclusive breast feeding at 1 month.Trial registration numberNCT01890668.

To assess the Global School Adaptation (GSA) questionnaire of children's abilities and classroom behavior administered to teachers of very preterm children at 5 years of age as a predictor of the need for educational support (grade retention, special class, learning support) at age 7.We assessed 858 very preterm children (33 weeks of gestation) at 5 years of age using the GSA and again at 7 years to determine the use of educational support. We examined the association between the GSA score and educational support at 7 years and performed a receiver operating characteristic curve analysis.At 7 years of age, 130 children had educational support (15.2%). Children with a nonoptimal GSA score (45) at 5 years required educational support more often (57.7%) than children with a GSA score of 45 or greater (15.4%) (OR, 7.5; 95% CI, 5.02-11.21). The need for educational support was associated with male sex; a low parent socioeconomic level; lower birth weight, birth head circumference, or gestational age (28-30 weeks of gestation); severe neurologic complications; patent ductus arteriosus ligation; and the use of therapy services at 5 years of age. After adjustment, only the GSA score was associated with educational support at 7 years of age (OR, 0.86; 95% CI, 0.84-0.88). A receiver operating characteristic curve analysis of the GSA performance revealed an optimal cut-off at 48, with a sensitivity of 70.8%, a specificity of 73.5%, and an area under the curve of 0.79.Using a cut-off score of 48, the GSA at 5 years of age may be a useful tool to identify children born preterm at risk of school-based learning difficulties.

Objectif Evaluer la qualite de vie des enfants en âge scolaire nes avant 28 SA et qui n’ont aucune incapacite majeure. Population et methode Etude transversale multicentrique (QPQoL study) incluant des nouveau-nes Resultats Quarante (7,5%) enfants gravement handicapes ont ete exclus. Parmi les 471 enfants eligibles, 169 (36%) etaient perdus de vu lors du suivi et donc 302 (64%) etaient inclus dans l’etude. Le terme moyen de naissance etait de 26.2 SA (± 0.8), le poids moyen de naissance etait de 879 g (± 181) et l’âge moyen de suivi des enfants etait de 8.4 ans (± 0.8). 48% des participants presentaient des deficiences cognitives mineures ou moderees d’apres leur Indice FSIQ. La memoire de travail, l’attention et la flexibilite mentale avaient une moyenne faible par rapport au groupe de reference. Hormis les relations familiales, l’evaluation de la qualite de vie par VSPA et Kidscreen 10 a ete significativement plus faible selon le point de vue des enfants et des parents. Les enfants ont declare une qualite de vie significativement diminuee dans les domaines suivants: les relations amicales, l’estime de soi et les loisirs, tandis que parents avaient indique: le bien-etre psychologique, le travail scolaire et la vitalite. Les enfants ayant un FSIQ ≥ 89, consideres comme normaux, avaient une qualite de vie inferieure. Des deficiences cognitives/comportementales specifiques (retard de langage, dyspraxie visuo-spatiale et troubles dysexecutifs) etaient les determinants significatifs de la qualite de vie dans les domaines des performances scolaires et de l’image corporelle. Conclusion La qualite de vie d’un enfant ne tres grand premature en âge scolaire sans deficience grave etait inferieure a celle d’une population de reference d’enfants nes a terme, tant du point de vue des parents que de celui de l’enfant. Cette evaluation devrait permettre de mieux comprendre le devenir a long terme et d’offrir un soutien adapte a l’enfant et a sa famille. Ethique This study was approved by the CPP (Committee for the Protection of Persons) (18/12/2012 ref 12.018) and is registered on ClinicalTrials.gov, number NCT01675726 .

International audience; The objective of this study was to quantify the possible decrease in school performance at five years of age in preterm children associated with parental separation or divorce, and to test whether this effect varies according to the child's age at the time of the separation. This study included 3,308 infants delivered at < 35 weeks of gestation born between 2003 and 2011 who were enrolled in the population-based LIFT cohort and who had an optimal neurodevelopmental outcome at two years of age. These infants were evaluated by their teachers to assess their abilities and behavior when they had reached five years of age, using the Global School Adaptation (GSA) questionnaire. The mean GSA score was 50.8 points. Parental separations (assessed as parents either living together or living separately) were associated with a decrease in school performance at five years of age, although this was only the case for children who exhibited difficulties at school (3.7 points, p < 0.01). A decrease in school performance only occurred when parental separations took place between 3 and 5 years after the child's birth. Parental separation was associated with a decrease in these children's levels of motivation, autonomy, and manual dexterity. This study indicates that preterm infants of parents who had separated are particularly at risk of a lower scholar performance.

International audience; Background: The quality of life (QOL) of children with and without intellectual disability (ID) has become a growing concern in the literature but the QOL instruments are not adapted for use with young children and children with ID. The objective of this study was to validate a French adaptation of the abbreviated form of the Multidimensional Student’s Life Satisfaction Scale (MSLSS).Method: Confirmatory factor analyses were conducted on the data collected from 411 children aged between 5 and 11 years old. Internal consistency, test–retest fidelity, and convergent validity were tested.Results: The preliminary results of this study show that the model fitted the data collected to assess the life satisfaction of children, χ2 (395) = 705.340; p

ObjectiveThe objective of this study was to investigate both the effects of low gestational age and infant’s neurodevelopmental outcome at 2 years of age on the risk of parental separation within 7 years of giving birth.DesignProspective.Setting24 maternity clinics in the Pays-de-la-Loire region.ParticipantsThis study included 5732 infants delivered at Outcome measureRisk of parental separation (parents living together or parents living separately).ResultsTen percent (572/5732) of the parents reported having undergone separation during the follow-up period. A mediation analysis showed that low gestational age had no direct effect on the risk of parental separation. Moreover, a non-optimal neurodevelopment at 2 years was associated with an increased risk of parental separation corresponding to a HR=1.49(1.23 to 1.80). Finally, the increased risk of parental separation was aggravated by low socioeconomic conditions.ConclusionsThe effect of low gestational age on the risk of parental separation was mediated by the infant’s neurodevelopment.

Background: Preterm infants are at greater risk of developmental impairment and require close follow-up for early and optimal care. Objectives: The objective of the present study was to determine from which age the parental Ages and Stages Questionnaire (ASQ) allows detection of school difficulties in preterm children Methods: Preterm children from the regional Loire Infant Follow-up Team network were evaluated with the Global School Adaptation (GSA) assessment tool at 5 years of age and at least one parental-completed ASQ at 18, 24, or 36 months. Children belonging to the first decile of the GSA score (Results: GSA scores were obtained in 1,775 infants at 5 years of age, and at least one ASQ score at 18, 24, or 36 months was completed. Upon ROC analysis, we observed that the 18-, 24-, and 36-month ASQ scores produced respective area under the ROC curve values of 0.66 (0.64-0.69), 0.72 (0.70-0.75), and 0.77 (0.75-0.80) for predicting a GSA score in the first decile. An ASQ cutoff value of 255 at 36 months showed optimal discriminatory power for identifying significant school difficulties at 5 years of age. Conclusions: The 36-month ASQ is a simple and cost-effective tool that can be employed to help predict future severe school difficulties at 5 years of age in preterm-born children.

Preterm infants present higher risk of non-optimal neurodevelopmental outcome. Fetal and postnatal growth, in particular head circumference (HC), is associated with neurodevelopmental outcome. Objectives: We aimed to calculate the relationship between HC at birth, HC delta Z-score (between birth and hospital discharge), and non-optimal neurodevelopmental outcome at 2 years of corrected age in preterm infants. Methods: Surviving infants born ≤34 weeks of gestation were included in the analysis. The relationship between the risk of being non-optimal at 2 years and both HC at birth and HC growth was assessed. The 2 Z-scores were considered first independently and then simultaneously to investigate their effect on the risk of non-optimality using a generalized additive model. Results: A total of 4,046 infants with both HC measures at birth and hospital discharge were included. Infants with small HC at birth (Z-score 2) while adjusting for gestational age, twin status, sex, and socioeconomic information. Conclusions: HC at birth and HC dZ-score between birth and hospital discharge are synergistically associated to neurodevelopmental outcome at 2 years of corrected age, in a population-based prospective cohort of preterm infants born ≤34 weeks of gestation.

Objective To assess genetic mutations and associated somatic anomalies in a series of patients with 46,XY gonadal dysgenesis (GD). Design Single center retrospective study. Setting University pediatric hospital. Patient(s) Fourteen patients with 46,XY GD. Intervention(s) None. Main Outcome Measure(s) Genotype-phenotype relationship. Result(s) The presenting symptom was disorders of sex development (6 patients), primary amenorrhea (2 patients), discordance between 46,XY karyotype and female external genitalia (3 patients), discovery of Mullerian structures at surgery (2 patients), or diagnosed in the evaluation of a gonadal tumor (1 patient). Mullerian structures were shown by ultrasound evaluation in 7 of 13 patients, genitography in 3 of 6 patients and/or surgery in 8 of 10 patients (3 not seen at imaging), or only by histologic examination (1 patient). Three patients had gonadoblastoma and/or seminoma. A mutation was found in 7 patients of whom 2 had family history of reproductive problems and 5 had associated somatic anomalies. The mutations were FOG2/ZFPM2 (1 patient), SRY (2 patients), WT1 (1 patient), or deletions of distal chromosome 9p (3 patients). Among the three other patients with associated anomalies and no mutation, two had ectodermal dysplasia and one had leukemia. Conclusion(s) Mutations were observed in half of the patients with 46,XY GD with Mullerian structures. We also describe for the first time the association between GD and ectodermal dysplasia. Mullerian structures can be found in some cases only by histologic examination, which should be coupled to preventive gonadectomy because of the risk of tumor formation.

Background Mutations of the NR5A1 gene encoding steroidogenic factor-1 have been reported in association with a wide spectrum of 46,XY DSD (Disorder of Sex Development) phenotypes including severe forms of hypospadias. Methodology/Principal Findings We evaluated the frequency of NR5A1 gene mutations in a large series of patients presenting with 46,XY DSD and hypospadias. Based on their clinical presentation 77 patients were classified either as complete or partial gonadal dysgenesis (uterus seen at genitography and/or surgery, n = 11), ambiguous external genitalia without uterus (n = 33) or hypospadias (n = 33). We identified heterozygous NR5A1 mutations in 4 cases of ambiguous external genitalia without uterus (12.1%; p.Trp279Arg, pArg39Pro, c.390delG, c140_141insCACG) and a de novo missense mutation in one case with distal hypospadias (3%; p.Arg313Cys). Mutant proteins showed reduced transactivation activity and mutants p.Arg39Pro and p.Arg313Cys did not synergize with the GATA4 cofactor to stimulate reporter gene activity, although they retained their ability to physically interact with the GATA4 protein. Conclusions/Significance Mutations in NR5A1 were observed in 5/77 (6.5%) cases of 46,XY DSD including hypospadias. Excluding the cases of 46,XY gonadal dysgenesis the incidence of NR5A1 mutations was 5/66 (7.6%). An individual with isolated distal hypopadias carried a de novo heterozygous missense mutation, thus extending the range of phenotypes associated with NR5A1 mutations and suggesting that this group of patients should be screened for NR5A1 mutations.