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To synthesize and appraise economic evaluations of vision screening to detect vision impairment in children.Literature searches were conducted on seven electronic databases, grey literature, and websites of agencies conducting health technology assessments. Studies were included if they (1) were full, comparative economic evaluations that used cost-utility, cost-benefit, cost-effectiveness, cost-consequence, or cost-analysis methods; (2) described screening services designed to detect amblyopia, strabismus, or uncorrected refractive errors in children under 6 years of age; and (3) published after 1994. High-quality studies were synthesized descriptively. Currencies were reported in 2019 Canadian dollars. Quality was assessed with the Pediatric Quality Appraisal Questionnaire (PQAQ).Vision screening services were conducted by paid staff, volunteers, or health care professionals in schools or clinics. Thirteen studies were published from five countries: China (n = 1), United States (n = 4), United Kingdom (n = 1), Canada (n = 1), and Germany (n = 6). Analytical techniques included cost-utility/cost-effectiveness combination (n = 2), cost-effectiveness analysis (n = 7), cost-utility analysis (n = 1), cost-benefit analysis (n = 1), cost-consequence analysis (n = 1), and cost analysis (n = 1). Incremental cost-effectiveness ratios ranged from C$1,056 to C$151,274 per additional case detected/prevented and from C$9,429 to C$30,254,703 per additional QALY gained, depending on the type of screening service and comparator. Six studies were determined to be of high quality.Vision screening to detect amblyopia for young children may be cost-effective compared with no screening if amblyopia reduced quality of life. Studies varied significantly in the type of screening services and comparators used. Methodological limitations were common. Future studies would be aided immensely by prospective studies on the impact of amblyopia on the health-related quality of life of young children and guidelines on the effective conduct of vision screening.RéSUMé: OBJECTIF: Synthétiser et évaluer des évaluations économiques de dépistages visuels visant à détecter la déficience visuelle chez les enfants. MéTHODE: Nous avons interrogé sept bases de données électroniques, la littérature grise et les sites Web d’organismes effectuant des évaluations des technologies de la santé. Nous avons inclus les études correspondant aux critères suivants : (1) évaluations économiques comparatives exhaustives utilisant l’analyse coûts-utilité, coûts-bénéfices, coûts-efficacité ou coûts-conséquences ou l’analyse des coûts; (2) décrivant des services de dépistage visant à détecter l’amblyopie, le strabisme ou les anomalies de la réfraction non corrigées chez les enfants de moins de six ans; et (3) publiées après 1994. Nous avons fait la synthèse descriptive des études de haute qualité. Les devises ont été converties en dollars canadiens de 2019. Nous avons évalué la qualité des études à l’aide de l’outil PQAQ (Pediatric Quality Appraisal Questionnaire). RéSULTATS: Les services de dépistage visuel étaient offerts par du personnel rémunéré, des bénévoles ou des professionnels de santé dans des écoles ou des cliniques. Treize études ont été publiées dans cinq pays : Chine (n = 1), États-Unis (n = 4), Royaume-Uni (n = 1), Canada (n = 1) et Allemagne (n = 6). Les techniques d’analyse employées étaient la combinaison analyse coûts-utilité/analyse coûts-efficacité (n = 2), l’analyse coûts-efficacité (n = 7), l’analyse coûts-utilité (n = 1), l’analyse coûts-avantages (n = 1), l’analyse coûts-conséquences (n = 1) et l’analyse des coûts (n = 1). Les rapports coût-efficacité différentiels s’échelonnaient entre 1 056 $ CA et 151 274 $ CA par cas supplémentaire détecté/prévenu et entre 9 429 $ CA et 30 254 703 $ CA par année de vie pondérée par la qualité (AVPQ) supplémentaire gagnée, selon le type de service de dépistage et le comparateur. Six études ont été jugées être de haute qualité. CONCLUSION: Comparativement à l’absence de dépistage, les dépistages visuels pour détecter l’amblyopie chez les jeunes enfants peuvent être efficaces par rapport à leur coût lorsque l’amblyopie réduit la qualité de vie. Le type de services de dépistage et les comparateurs utilisés variaient considérablement d’une étude à l’autre. Les contraintes méthodologiques étaient courantes. Les études futures seraient grandement favorisées par des études prospectives des incidences de l’amblyopie sur la qualité de vie liée à la santé chez les jeunes enfants et par des lignes directrices sur l’exécution efficace des dépistages visuels.

Two brothers, completely asymptomatic until their first year of life, started to complain from gingival hypertrophy, progressive development of painful soft tissue masses on the fingers and toes, on the face and on the scalp. There were no neurological symptoms or mental delay for both brothers.

There remains a general misconception that the immune status of the fetus and neonate is immature or insufficient. However, emerging research in immune ontogeny prompts reconsideration of this orthodoxy, reframing this period instead as one of unique opportunity. Vaccine responses (qualitative and quantitative) vary between individuals, and across demographic cohorts. Elements of baseline immune status and function predict vaccine response – some of these factors are well described, others remain a subject of ongoing research, especially with the rapidly expanding field of ‘omics’ research, enabled by development of highly granular immune profiling techniques and increasing computational capacity. Age is one of the strongest predictive factors associated with variability in the response to vaccination; and predictable variation in response to vaccination is a key to identify the crucial underlying mechanisms. Specifically, circulating maternal antibody in the young infant can modulate immune response to vaccination, acting as an ‘undercover adjuvant’ that, counter to current dogma, may offer a pathway to longer lasting, higher quality immune response to vaccination. Exciting avenues for novel research in this area have the potential to dramatically alter how we protect the world's most vulnerable population – the very young., SCOPUS: ar.j, info:eu-repo/semantics/published

Introduction. — The recently identified role of a BRAF somatic mutation in the pathophysiologyof Langerhans cell histiocytosis (LCH) offers new therapeutic options. Herein we describe thecase of a 10-month-old infant with refractory high-risk LCH successfully treated with vemu-rafenib.Observation. — The patient first presented with cutaneous LCH at the age of 2 months. Thedisease remained undiagnosed until she was 6 months old, when it rapidly evolved to a multisys-temic high-risk and life-threatening disease, refractory to 2 lines of chemotherapy. BRAFV600Emutation was found at skin biopsy, and targeted therapy with vemurafenib was started whenshe was 10 months old. The treatment induced a fast and sustained response, but rapid relapseoccurred after treatment discontinuation, leading to resumption of treatment, once moreresulting in a sustained response.Conclusion. — Our case highlights the first-line role of dermatologists in establishing the diag-nosis of LCH, especially in children, in whom the eruption may be difficult to identify, leading todelayed diagnosis. Targeted therapy with vemurafenib has recently been described in children in this indication and our results support its efficacy, highlighting the need for prolonged treat-ment and raising the question of maintenance therapy, as well as the necessity for large-scaleand long-term studies., info:eu-repo/semantics/published

OBJECTIVES: The aim of the study was to assess clinical presentation, endoscopic findings, antibiotic susceptibility and treatment success of Helicobacter pylori (H. pylori) infected pediatric patients. METHODS: Between 2013 and 2016, 23 pediatric hospitals from 17 countries prospectively submitted data on consecutive H. pylori-infected (culture positive) patients to the EuroPedHP-Registry. RESULTS: Of 1333 patients recruited (55.1% girls, median age 12.6 years), 1168 (87.6%) were therapy naïve (group A) and 165 (12.4%) had failed treatment (group B). Patients resided in North/Western (29.6%), Southern (34.1%) and Eastern Europe (23.0%), or Israel/Turkey (13.4%). Main indications for endoscopy were abdominal pain or dyspepsia (81.2%, 1078/1328). Antral nodularity was reported in 77.8% (1031/1326) of patients, gastric or duodenal ulcers and erosions in 5.1% and 12.8%, respectively. Primary resistance to clarithromycin (CLA) and metronidazole (MET) occurred in 25% and 21%, respectively, and increased after failed therapy. Bacterial strains were fully susceptible in 60.5% of group A, but in only 27.4% of group B. Primary CLA resistance was higher in Southern and Eastern Europe (adjusted odds ratio [ORadj] = 3.44, 95% confidence interval [CI] 2.22-5.32, P, SCOPUS: ar.j, info:eu-repo/semantics/published

As infants with proven viral infection present lower risk of bacterial infection, we evaluated how molecular methods detecting viruses on respiratory secretions could contribute to etiological diagnostic of these febrile episodes. From November 2010 to May 2011, we enrolled all febrile infants, SCOPUS: ar.j, info:eu-repo/semantics/published

info:eu-repo/semantics/published

La prise en charge de l´ostéomyélite aiguë devient plus délicate depuis l´émergence duStaphylocoque aureusrésistant à la méthicilline d´origine communautaire. Nous avons recueilli les cas d´ostéomyélite aiguë dues à ce germe sur une période de 21 ans (Janvier 1995-Décembre 2016) et nous avons essayé d´analyser les particularités de cette entité pathologique. Notre série comporte 15 enfants, d´âge moyen 9 ans. Le membre inférieur était atteint dans tous les cas. Une notion de traumatisme local a été signalée dans 8 cas et une porte d´entrée cutanée a été trouvée dans 4 cas. Le mode de début était aigu dans 12 cas avec un tableau de pseudo-paralysie du membre atteint. Une staphylococcie pulmonaire avec des signes septico-pyohémiques ont été notés dans un cas. L´hémoculture était positive dans 8 cas. La recherche par PCR de la leucocidine de Panton et Valentine était pratiquée dans 1 cas avec un résultat positif. Tous ces patients ont eu un débridement chirurgical et une antibiothérapie empirique secondairement adaptée. L´évolution était bonne dans 8 cas et mauvaise dans les autres cas avec passage à la chronicité dans 6 cas et un cas de décès. Une fracture pathologique a été signalée dans 3 cas. La survenue d´une ostéomyélite àStaphylocoque aureusrésistant à la méthicilline d´origine communautaire est péjorative. Connaitre ces infections en se basant sur des arguments cliniques et paracliniques est un enjeu important pour une prise en charge thérapeutique spécifique et rapide.

SCOPUS: cp.j, info:eu-repo/semantics/published

International audience; Soft tissue sarcomas in children are rare tumor, representing around 6 to 7% of children cancer. They spread mostly sporadically (90%) and therefore are rarely associated to an underlying constitutional genetic disease (10%). About half of those sarcomas are rhabdomyosarcomas and the others are a very heterogenous histologic group with various bio-pathologies and prognosis. Clinical presentation is mainly a soft tissue lump often difficult to distinguish from more frequent benign causes (malformative, infectious, benign, or pseudotumor). Inappropriate initial diagnosis work-up has a strong impact on soft tissue sarcomas' prognosis. Adapted complementary investigations (first ultrasound and MRI) are important to compile arguments for a malign origin and to indicate a biopsy. However, predictive value of imaging exams still remains imperfect, and histological analysis by percutaneous image-guided biopsy and sometimes by surgical biopsy is often necessary. Authors realize an update on optimal diagnostic pathway including molecular tests in presence of a soft tissue mass in a child.

Introduction:l´objectif de cette étude est d´évaluer l´impact du COVID-19 sur les activités de soins et les recettes au Centre Hospitalier National d´Enfant Albert Royer de Dakar au Sénégal. Méthodes:il s´agissait d´une étude rétrospective, descriptive et analytique comparant les activités de consultations externes, d´hospitalisation et les recettes du premier trimestre des années 2019 et 2020. Résultats:une baisse moyenne de 33% des consultations externes a été notée au premier trimestre de l´année 2020 correspondant à la période de la pandémie comparée au premier trimestre de 2019. Une augmentation des hospitalisations était observée surtout pour les mois de janvier et février. Cependant, au mois de mars, une baisse de 11% était notée. Il en est de même pour les recettes où une baisse n´est observée qu´au mois de mars; elle était de 10%. Conclusion:l´épidémie actuelle au COVID-19 impacte fortement sur les activités de consultations externes, de soins et les recettes du centre hospitalier national d´Enfants Albert Royer. Des mesures efficaces doivent être prises pour éviter les conséquences sur la mortalité et le fonctionnement de la structure.

SCOPUS: re.j, info:eu-repo/semantics/published

The purpose of this commentary is to discuss how legalization of non-medical marijuana (LNMM) in Canada can potentially influence child and adolescent unintentional injuries based on evidence from states (American) and jurisdictions that have already legalized cannabis for recreational purposes. Although the evidence is still not conclusive, LNMM can bring about higher exposure, lower perceived harms, and higher prevalence of cannabis use by minors through role modeling and normalization of behaviour within the household and the community, and higher rates of driving under the influence of cannabis, which can contribute to a higher burden of road traffic injuries. Experience of American states with LNMM shows higher rates of emergency visits for pediatric poisoning due to unintentional ingestion of cannabis-containing foods and severe burns due to explosions during the course of home-based cannabis extraction. While the justification for legalization has created a strict legal framework for improved control of cannabis in Canada, the implications for health and safety of children and adolescents necessitate further study, communication with policy-makers and public health practitioners, and evidence-based education of parents, caregivers, and youth.

Le syndrome de Perthes est défini par l’association d’hémorragies sous conjonctivales, de cyanose cervico-faciale et des pétéchies à des manifestations neurologiques. La compression brutale du thorax ou thoraco-abdominale bloquée en inspiration forcée avec effort à glotte fermée est le mécanisme responsable du syndrome. Le pronostic est en général bon si la durée de la compression est brève et la réanimation cardiorespiratoire commencée précocement. L’objectif de cet article est de décrire les caractéristiques du syndrome de Perthes chez les enfants.

The main objective of this study is to describe the most common childhood diseases occurring in Theresienstadt ghetto during the Second World War as well as applied anti-epidemic measures. A partial objective is to describe medical and nursing care of sick child prisoners. The data was obtained by the method of synthesis of primary and secondary data with the highest importance after adequate external source criticism. Selected monographs, memoirs, survivor diaries, Orders of the day by the Council of the Elders and Reports of the Jewish self-government of Theresienstadt from 1941 to 1945, the Archives of the Jewish Museum in Prague, the Ghetto Museum, the Museum of the History of Polish Jews in Warsaw, Post Bellum online electronic collection of oral historical interviews, and witness accounts. The validity of the presented conclusions is ensured by comparing data from several sources. The most common infections in Theresienstadt children were enteritis, scarlet fever, infectious jaundice, measles, mumps, rubella, varicella, black cough, pneumonia, otitis media, and typhoid fever. Most of these infections had an entirely atypical symptomatology or complications. Children were hospitalized in children's hospitals, in children's rooms of hospitals for adults and infirmaries in children's homes. Albeit diagnostic methods had a high standard, options of treatment were very limited. The most common treatments included bed rest, diet and cold compress. Occasionally, chemotherapeutic agents (e.g. a sulfonamide drug Prontosil) and Aspirin were available. The anti-epidemic measures in the ghetto focused on hygiene, enhancing children's immunity, vaccination and pest control. Hlavním cílem této studie je popsat nejčastější dětské nemoci vyskytující se v terezínském ghettu během druhé světové války, stejně jako aplikovaná protiepidemická opatření. Částečným cílem je popsat lékařskou a ošetřovatelskou péči o nemocné dětské vězně. Údaje byly získány metodou syntézy primárních a sekundárních údajů s nejvyšším významem po odpovídající kritice externích zdrojů. Vybrané monografie, paměti, deníky přeživších, Řády dne Rady starších a Zprávy židovské samosprávy Terezína z let 1941 až 1945, Archiv Židovského muzea v Praze, Muzeum ghetta, Muzeum dějin polských Židů ve Varšavě, Online elektronická sbírka ústních historických rozhovorů a svědectví. Platnost předložených závěrů je zajištěna porovnáním údajů z několika zdrojů. Mezi nejčastější infekce v Terezínských dětech patřila enteritida, spála, infekční žloutenka, spalničky, příušnice, zarděnky, plané neštovice, černý kašel, pneumonie, zánět středního ucha a břišní tyfus. Většina těchto infekcí měla zcela atypickou symptomatologii nebo komplikace. Děti byly hospitalizovány v dětských nemocnicích, v dětských pokojích nemocnic pro dospělé a infirmaries v dětských domovech. I když diagnostické metody měly vysoký standard, možnosti léčby byly velmi omezené. Mezi nejčastější léčby patří odpočinek na lůžku, strava a studený obklad. Příležitostně byly k dispozici chemoterapeutické látky (např. sulfonamidový lék Prontosil) a aspirin. Protie epidemická opatření v ghettu se zaměřila na hygienu, posílení imunity dětí, očkování a hubení škůdců.

Most studies of inhaled nitric oxide (iNO) for prevention of bronchopulmonary dysplasia (BPD) in premature infants have focused on short-term mortality and morbidity. Our aim was to determine the long-term effects of iNO. A 7-year follow-up was undertaken of infants entered into a multicenter, double-blind, randomized, placebo-controlled trial of iNO for prevention of BPD in premature infants born between 24 and 28 weeks plus six days of gestation. At 7 years, survival and hospital admissions since the 2-year follow-up, home oxygen therapy in the past year, therapies used in the previous month and growth assessments were determined. Questionnaires were used to compare general health, well-being, and quality of life. A total of 305 children were assessed. No deaths were reported. Rates of hospitalization for respiratory problems (6.6 vs. 10.5%, iNO and placebo group, respectively) and use of respiratory medications (6.6 vs. 9.2%) were similar. Two patients who received iNO and one who received placebo had received home oxygen therapy. There were no significant differences in any questionnaire-documented health outcomes. iNO for prevention of BPD in very premature infants with respiratory distress did not result in long-term benefits or adverse long-term sequelae. In the light of current evidence, routine use of iNO cannot be recommended for prevention of BPD in preterm infants., SCOPUS: ar.j, DecretOANoAutActif, info:eu-repo/semantics/published

L´épilepsie pose un problème de santé publique au Sénégal. L´objectif de l´étude était de décrire les aspects cliniques et paracliniques de l´épilepsie de l´enfant à l´Hôpital de la Paix de Ziguinchor (HPZ). Il s´agissait d´une étude documentaire de dossiers d´enfants épileptiques, du 1erjanvier 2015 au 31 décembre 2018. Ont été inclus, les patients âgés ≤ de 15 ans, suivis pour épilepsie à l´HPZ. Etaient exclus, les dossiers incomplets. Cinquante-cinq (37 garçons et 18 filles) enfants ont été colligés; 70,9% avaient un âge < à 5 ans. L´âge moyen était de 4,3 ans. Les patients étaient originaires du milieu rural (60%) et de famille défavorisée (67,3%). Les crises d´épilepsie étaient généralisées (72,7%) et focales (27,3%). Dix-huit patients présentaient une épilepsie idiopathique, 17 une épilepsie non idiopathique. Les facteurs étiologiques étaient dominés par les anomalies de la grossesse et de l´accouchement (29,1%). L´épilepsie est fréquente à l´HPZ. Elle prédomine en milieu rural chez les garçons de moins de 5 ans, issus de famille défavorisée. Les crises généralisées tonico-cloniques et les crises focales sont les tableaux cliniques les plus fréquentes et les anomalies de la grossesse et de l´accouchement sont les étiologies les plus retrouvées.

Background: Knobloch syndrome (OMIM 267750) is a rare autosomal recessive disorder due to genetic defects in the COL18A1 gene. The triad of high myopia, occipital defect, vitreoretinal degeneration has been described as pathognomonic for this condition. Patients with Knobloch syndrome have also extraocular problems as brain and kidney malformations. High genetic and phenotypic variation has been reported in the affected patients. Materials and Methods: Here we provide detailed clinical description of 3 individuals with Knobloch syndrome. Ocular examination and fundus imaging have been performed. Detailed information about systemic conditions has been provided. Results: Mutations in COL18A1 were identified in all three patients. Patient 1 had congenital hip dislocation and patient 2 had renal atrophy, cardiac insufficiency and difficult skin healing. Conclusions: With this report we add to the clinical and genetic knowledge of this rare condition., SCOPUS: ar.j, info:eu-repo/semantics/published

International audience; Objective Maternal obesity is associated with an increase in maternal, foetal and neonatal morbidity and mortality. The aim of our study was to evaluate the relationships between maternal pre-pregnancy body mass index and (1) neonatal outcome in preterm infants, and (2) neurodevelopmental outcome at 2 years of corrected age. Method We conducted a single-centre cohort study. Infants born between 24+0 and 33+6 weeks of gestation between January 2009 and December 2013, hospitalised in the neonatal intensive care unit of Angers University Hospital, and with available data regarding maternal pre-pregnancy body mass index were eligible. Three groups were defined according to maternal body mass index: normal (n = 418), overweight (n = 136) and obese (n = 89). The primary outcome was neurodevelopment at 2 years of corrected age. Children with a non-optimal neuromotor and/or psychomotor assessment and/or a sensory disability were regarded as having a “non-optimal neurodevelopmental outcome”. Neuromotor function was regarded as non-optimal when cerebral palsy was present or when the clinical examination revealed neurological signs of abnormal muscular tone. Psychomotor assessment was regarded as non-optimal if the revised Brunet-Lézine test was < 85 or when the overall score in the parental Ages and Stages Questionnaire (ASQ) was < 185. Finally, sensory disabilities such as blindness and children who required a hearing aid were taken into account. The secondary outcome was the composite criteria of neonatal complications. Multivariable analysis included the following variables: mother’s age, gestational age, smoking during pregnancy, magnesium sulphate and steroid treatment during pregnancy, twin status, gender, socioeconomic status and social security benefits for those with low incomes. Results The study population was composed of 643 preterm infants. Among them, 520 were assessed at 2 years. There was no difference in the proportion of infants with non-optimal neurodevelopmental outcomes between the three groups (16.6% for obese, 13.5% for overweight, 16.9% for normal body mass index mothers; p = 0.73). According to multivariable analysis, being born from an overweight or obese mother was not associated with an increased risk of non-optimal neuro-development at 2 years (adjusted OR = 0.84 [0.40–1.76] for obese, adjusted OR = 0.83 [0.43–1.59] for overweight mothers). There was no difference in the proportion of preterm infants with a non-optimal composite criterion of neonatal complications between the three groups. In the multivariable analysis, being born from an overweight or obese mother was not associated with an increased risk of non-optimal neonatal outcomes (adjusted OR = 0.95 [0.49–1.83] for obese, adjusted OR = 1.18 [0.69–2.01] for overweight mothers). Conclusion In this large prospective cohort of preterm infants born before 34 weeks of gestation, we found no relationship between maternal body mass index and neurodevelopmental outcomes at 2 years of corrected age and no relationship between maternal body mass index and neonatal outcomes. Other prematurity-related factors may be more relevant for neurodevelopmental outcome than the mother’s pre-pregnancy BMI.

A retrospective cross-sectional study was carried out in Wallonia (the southern region of Belgium) in which a 20-question breastfeeding (BF) module was included in an immunization survey. The purpose of this paper is to compare exclusive breastfeeding (EBF) prevalences and BF practices for mothers giving birth in Baby-friendly Hospital Initiative (BFHI) and non-BFHI maternity facilities. A total of 557 mothers responded to BF questions when their child was 18–24 months old; 26.7% of them delivered in a BFHI maternity facility. At discharge, a larger proportion of children were exclusively breastfed if they were born in a BFHI maternity facility (76.5% vs. 65.8%, p =.02). The median duration of EBF (15.0 vs. 12.9 weeks, p =.3), and the proportion of children exclusively breastfed at 5 months (16.8% vs 15.8%, p = 1.0) were similar in both groups. Few mothers knew that EBF was recommended for the first 6 months of life (28.6% in BFHI vs 23.1% in non-BFHI, p =.2). For most groups of the population examined, the rates of BF tended to be higher in BFHI facilities, but many differences were not significant. More specifically, BFHI seemed to boost BF practices among mothers more likely to breastfeed, but the Initiative did not seem to trigger enhanced BF practices in mothers traditionally less likely to breastfeed (except for indifferent/negative partner's attitude and mothers of Belgian origin). Influencing the BF practices of mothers less likely to breastfeed requires a special attention with complementary actions in maternity facilities as well as in community services., SCOPUS: ar.j, info:eu-repo/semantics/published

Introduction: Tumors of the cerebellum are the most common brain tumors in children. Modern treatment and aggressive surgery have improved the overall survival. Consequently, growing numbers of survivors are at high risk for developing adverse and long-term neurological deficits including deficits of cognition, behavior, speech, and language. Post-operative cerebellar mutism syndrome (pCMS) is a well-known and frequently occurring complication of cerebellar tumor surgery in children. In the acute stage, children with pCMS may show deterioration of cerebellar motor function as well as pyramidal and cranial neuropathies. Most debilitating is the mutism or the severe reduction of speech and a range of neurobehavioral symptoms that may occur. In the long term, children that recover from pCMS continue to have more motor, behavioral, and cognitive problems than children who did not develop pCMS after cerebellar tumor surgery. The severity of these long-term sequelae seems to be related to the length of the mute phase. Aim of this narrative review: The impact of pCMS on patients and families cannot be overstated. This contribution aims to discuss the present knowledge on the natural course, recovery, and rehabilitation of children with pCMS. We suggest future priorities in developing rehabilitation programs in order to improve the long-term quality of life and participation of children after cerebellar tumor surgery and after pCMS in particular., SCOPUS: re.j, info:eu-repo/semantics/published

Introduction: As only ten cases have been described in current literature, urachal mucinous cystadenoma seems rare. All studied cases concerned adults or adolescents; this paper reports the first infant presenting urachal mucinous cystadenoma. Case report: A seven-month-old boy affected by urachal mucinous cystadenoma, presented as only sign persistent purulent umbilical discharge. Ultrasonography showed nodular structure with liquid content evoking urachal cyst. Radical surgical excision was performed. Histopathology proved diagnosis of urachal mucinous cystadenoma. No complications were observed. Follow-up consultation eight days postoperative was reassuring, evaluating infection and recurrence absence, and scar healing. Discussion: Preservation of urachus lumen may lead to urachal diseases: patent, diverticulum, sinus and cyst. Mucinous cystadenoma is rarely found in urachus. Solely umbilical purulent discharge was observed, in contrast to all reported cases. Same type of investigation, ultrasonography, was conducted to evoke diagnosis. Since urachal mucinous cystadenoma may transform into adenocarcinoma, prophylactic excision is recommended, and prognosis depends on its radicalness. No long-term outcome for infants is available in literature. We propose annual follow-up by ultrasonography. Conclusion: Urachal mucinous cystadenoma in infants is uncommon. Umbilical discharge must indicate ultrasonography. Surgical excision is recommended in case of suspicion. Long term outcomes are yet to be evaluated., SCOPUS: ar.j, info:eu-repo/semantics/published

Supplemental Digital Content is available in the text., Background: The European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) published recommendations regarding protection for the paediatric endoscopist during the coronavirus 2019 (COVID-19) pandemic.The aim of this survey was to investigate whether European paediatric gastroenterology centres applied the recommendations and how this extraordinary situation was handled by the different centres. Results: Twelve paediatric European gastroenterology centres participated. Nine centres (75%) screened their patients for possible COVID-19 infection before the procedure, the same amount of hospitals changed their practice based on the ESPGHAN recommendations. Six-seven percentage of the centres reduced the staff in the endoscopy suite, 83% of the units used FFP2/3 masks and protective goggles during the procedure and 75% wore waterproof gowns. Conclusion: Uniform guidelines could not be applied by all European hospitals at a certain time point of the viral spread, as different regions of Europe were not only affected differently by COVID-19, but also had different access to personal protective equipment.

i?½ 2016 The Authors. Tropical Medicine & International Health Published by John Wiley & Sons Ltd. Background: Evaluating clinician compliance with recommended steps in clinical guidelines provides one measure of quality of process of care but can result in a multiplicity of indicators across illnesses, making it problematic to produce any summative picture of process quality, information that may be most useful to policy-makers and managers. Objective: We set out to develop a clinically logical summative measure of the quality of care provided to children admitted to hospital in Kenya spanning the three diagnoses present in 60% or more of admissions that would provide a patient-level measure of quality of care in the face of comorbidity. Methods: We developed a conceptual model of care based on three domains: assessment, diagnosis and treatment of illnesses. Individual items within domains correspond to recommended processes of care within national clinical practice guidelines. Summative scores were created to reduce redundancy and enable aggregation across illnesses while maintaining a clear link to clinical domains and our conceptual model. The potential application of the score was explored using data from more than 12 000 children from eight hospitals included in a prior intervention study in Kenya. Results: Summative scores obtained from items representing discrete clinical decision points reduced redundancy, aided balance of score contribution across domains and enabled direct comparison of disease-specific scores and the calculation of scores for children with comorbidity. Conclusion: This work describes the development of a summative Paediatric Admission Quality of Care score measured at the patient level that spans three common diseases. The score may be an efficient tool for assessing quality with an ability to adjust for case mix or other patient-level factors if needed. The score principles may have applicability to multiple illnesses and settings. Future analysis will be needed to validate the score.

IntroductionTo evaluate the consequences of bronchopulmonary dysplasia (BPD) on academic outcomes and healthcare use in adolescents born very preterm.MethodsThis cohort study included 15-year-old adolescents born very preterm (< 32 weeks) between 2011 and 2013, with and without BPD, and controls born full term. Data regarding academic performance, current medical follow-up, and family characteristics were collected. Multivariate logistic regression was used to quantify relationships between academic outcomes and BPD.ResultsFrom the 1341 children included in the initial cohort, 985 adolescents were eligible and 351 included (55 preterms with a history of BPD, 249 without, and 47 controls). Among adolescents born very preterm, a history of BPD was associated with a higher risk to attend a school for children with special needs (p < 0.05) and to have repeated a grade (p = 0.01). It was also associated with an increased number of medical and paramedical consultations. A history of BPD was not associated with the parents' employment status, family structure, or the presence of younger siblings.ConclusionThis study highlights that a history of BPD is associated with poorer academic outcomes and high healthcare use in adolescence.

Background Children with juvenile idiopathic arthritis (JIA) have impaired physical abilities. TNF-α plays a crucial role in this pathogenesis, but it is also involved in the use of lipids and muscle health. Objective of this study was to explore substrate oxidation and impact of TNF blockade on energy metabolism in children with JIA as compared to healthy children. Methods Fifteen non-TNF-blockaded and 15 TNF-blockaded children with JIA and 15 healthy controls were matched by sex, age, and Tanner stage. Participants completed a submaximal incremental exercise test on ergocycle to determine fat and carbohydrate oxidation rates by indirect calorimetry. Results The maximal fat oxidation rate during exercise was lower in JIA children untreated by TNF blockade (134.3 ± 45.2 mg.min− 1) when compared to the controls (225.3 ± 92.9 mg.min− 1, p = 0.007); but was higher in JIA children under TNF blockade (163.2 ± 59.0 mg.min− 1, p = 0.31) when compared to JIA children untreated by TNF blockade. At the same relative exercise intensities, there was no difference in carbohydrate oxidation rate between three groups. Conclusions Lipid metabolism during exercise was found to be impaired in children with JIA. However, TNF treatment seems to improve the fat oxidation rate in this population. Trial registration In ClinicalTrials.gov, reference number NCT02977416, registered on 30 November 2016.

Maternal 25-hydroxyvitamin D (25-OHD) deficiency during pregnancy may increase the risk of preterm and small-for-gestational age (SGA) birth, but studies report conflicting results. We used a multicenter prospective cohort of 2813 pregnant women assessed for 25-OHD levels in the first trimester of pregnancy to investigate the association between maternal 25-OHD concentrations and risks of preterm birth (&lt, 37 weeks) and SGA (birthweight &lt, 10th percentile). Odds ratios were adjusted (aOR) for potential cofounders overall and among women with light and dark skin separately, based on the Fitzpatrick scale. 25-OHD concentrations were &lt, 20 ng/mL for 45.1% of the cohort. A total of 6.7% of women had a preterm birth. The aOR for preterm birth associated with the 1st quartile of 25-OHD concentrations compared to the 4th quartile was 1.53 (95% confidence interval (CI): 0.97&ndash, 2.43). In stratified analyses, an association was observed for women with darker skin (aOR = 2.89 (95% CI: 1.02&ndash, 8.18)), and no association with lighter skin. A total of 11.9% of births were SGA and there was no association overall or by skin color. Our results do not provide support for an association between maternal first trimester 25-OHD deficiency and risk of preterm or SGA birth overall, the association with preterm birth risk among women with darker skin requires further investigation.

Background Cutaneous microcystic lymphatic malformations (CMLMs) are rare conditions in children and adults. They present as clusters of vesicles full of lymph and blood to various extents, inducing maceration, esthetic impairment, pain, and impaired quality of life. The treatment is challenging. Sirolimus is an inhibitor of mammalian target of rapamycin (mTOR) involved in angio-lymphangiogenesis. Topical sirolimus has recently been reported as effective in a few reports of patients with CMLMs. The objective is to compare the efficacy and safety of a 12-week application of 0.1% topical sirolimus versus topical vehicle in CMLMs in children and adults. Methods This French blinded multicenter within-person randomized controlled phase 2 trial aims to include 55 patients aged ≥ 6 years who have a primary CMLM. The CMLM will be divided into two equal areas that will be randomly allocated to 0.1% topical sirolimus or topical vehicle applied for 12 weeks. At the end of the 12-week period, the patient/parent will treat the whole area of CMLM with 0.1% topical sirolimus on remaining lesions, for eight more weeks. Patients will be seen at week 20 (treatment will be stopped) and at month 12 to evaluate long-term efficacy. The primary outcome will be improvement of the CMLM in the area treated with topical sirolimus compared to the area treated with topical vehicle by the investigator physician (blinded to the treatment) with the Physician Global Assessment score at week 12. Secondary outcomes will include: assessment of efficacy by independent experts on the basis of standardized photographs; impact on quality of life; efficacy for oozing, bleeding, erythema, and thickness evaluated by the investigators; and global efficacy as well as efficacy for functional and aesthetic impairment evaluated by the patient. Systemic passage of sirolimus will be measured at weeks 6, 12, and 20, and at week 16 for CMLMs ≥ 900 cm2. Discussion For patients with CMLMs, topical sirolimus could be a non-invasive and well-tolerated therapeutic option. If the trial demonstrates efficacy and safety of this treatment, this result will lead to a real change in the management of this condition, and 0.1% sirolimus cream would become the first-line treatment. Trial registration ClinicalTrials.gov, NCT03972592. Registered on 3 June 2019. EU Clinical Trials Register EudraCT, 2018–001359-11.

La pycnodysostose est une maladie osseuse génétique très rare associant une ostéocondensation, un syndrome dysmorphique et un retard de croissance. Rappeler les anomalies phénotypiques, les signes radiologiques, la prise en charge thérapeutiques et évolutifs de la Pycnodysostose chez un enfant de 11 ans. Enfant de 11 ans, a été réferé par son dentiste pour évaluation de son état clinique. Il est de parents consanguin de premier degré, qui présentait depuis l'âge de 3 ans des fractures spontanées à répétition. L'examen trouvait un syndrome dysmorphique fait de bosse frontale, fontanelle antérieure persistante, micrognathie, malformations des doigts, malposition dentaire, ongles incurvées, thorax asymétrique, attitude scoliotique du rachis dorsal, avec retard statural important (-4DS). Les radiographies du squelette montraient une densification des os de la base du crâne, une persistance de la fontanelle antérieure, une malposition dentaire, une densification diaphysaire et métaphysaire des os longs prédominants au niveau des membres inférieurs avec présence des cals vicieux et des phalanges grêles des mains. L'ostéodensitométrie était normale. Devant les signes cliniques et les manifestations radiologiques, le diagnostic retenu est une pycnodysostose. Un conseil génétique a été proposé pour la famille ainsi qu'une prise en charge dentaire et orthopédiques. La pycnodysostose est une entité rare de diagnostic parfois difficile et tardif, elle pose un problème du diagnostic avec l'ostéoporose. Le traitement est essentiellement préventif des fractures et des caries dentaires.

Background: Much controversy exists about the optimal management of a patent ductus arteriosus (PDA) in preterm infants, especially in those born at a gestational age (GA) less than 28weeks. No causal relationship has been proven between a (haemodynamically significant) PDA and neonatal complications related to pulmonary hyperperfusion and/or systemic hypoperfusion. Although studies show conflicting results, a common understanding is that medical or surgical treatment of a PDA does not seem to reduce the risk of major neonatal morbidities and mortality. As the PDA might have closed spontaneously, treated children are potentially exposed to iatrogenic adverse effects. A conservative approach is gaining interest worldwide, although convincing evidence to support its use is lacking. Methods: This multicentre, randomised, non-inferiority trial is conducted in neonatal intensive care units. The study population consists of preterm infants (GA1.5mm. Early treatment (between 24 and 72h postnatal age) with the cyclooxygenase inhibitor(COXi) ibuprofen (IBU) is compared with an expectative management (no intervention intended to close a PDA). The primary outcome is the composite of mortality, and/or necrotising enterocolitis (NEC) Bell stage ≥ IIa, and/or bronchopulmonary dysplasia (BPD) defined as the need for supplemental oxygen, all at a postmenstrual age (PMA) of 36weeks. Secondary outcome parameters are short term sequelae of cardiovascular failure, comorbidity and adverse events assessed during hospitalization and long-term neurodevelopmental outcome assessed at a corrected age of 2 years. Consequences regarding health economics are evaluated by cost effectiveness analysis and budget impact analysis. Discussion: As a conservative approach is gaining interest, we investigate whether in preterm infants, born at a GA less than 28weeks, with a PDA an expectative management is non-inferior to early treatment with IBU regarding to the composite outcome of mortality and/or NEC and/or BPD at a PMA of 36weeks., SCOPUS: ar.j, info:eu-repo/semantics/published

Background: Indirect neonatal hyperbilirubinemia (INH) is a common neonatal disorder worldwide which can remain benign if prompt management is available. However there is a higher morbidity and mortality risk in settings with limited access to diagnosis and care. The manuscript describes the characteristics of neonates with INH, the burden of severe INH and identifies factors associated with severity in a resource-constrained setting. Methods: We conducted a retrospective evaluation of anonymized records of neonates hospitalized on the Thai-Myanmar border. INH was defined according to the National Institute for Health and Care Excellence guidelines as 'moderate' if at least one serum bilirubin (SBR) value exceeded the phototherapy threshold and as 'severe' if above the exchange transfusion threshold. Results: Out of 2980 records reviewed, 1580 (53%) had INH within the first 14days of life. INH was moderate in 87% (1368/1580) and severe in 13% (212/1580). From 2009 to 2011, the proportion of severe INH decreased from 37 to 15% and the mortality dropped from 10% (8/82) to 2% (7/449) coinciding with the implementation of standardized guidelines and light-emitting diode (LED) phototherapy. Severe INH was associated with: prematurity (, SCOPUS: ar.j, info:eu-repo/semantics/published

Open access article; International audience; Rare neurodevelopmental syndromes often present social cognitive deficits that may underlie difficulties in social interactions and increase the risk of psychosis or autism spectrum disorders. However, little is known regarding the specificities of social cognitive impairment across syndromes while it remains a major challenge for the care. Our review provides an overview of social cognitive dysfunctions in rare diseases associated with psychiatric symptoms (with a prevalence estimated between 1 in 1,200 and 1 in 25,000 live births: 22q11.2 deletion syndrome, Angelman syndrome, Fragile X syndrome, Klinefelter syndrome, Prader-Willi syndrome, Rett syndrome, Smith-Magenis syndrome, Turner syndrome, and Williams syndrome) and shed some light on the specific mechanisms that may underlie these skills in each clinical presentation. We first detail the different processes included in the generic expression "social cognition" before summarizing the genotype, psychiatric phenotype, and non-social cognitive profile in each syndrome. Then, we offer a systematic review of the social cognitive abilities and the disturbed mechanisms they are likely associated with. We followed the PRISMA process, including the definition of the relevant search terms, the selection of studies based on clear inclusion, and exclusion criteria and the quality appraisal of papers. We finally provide insights that may have considerable influence on the development of adapted therapeutic interventions such as social cognitive training (SCT) therapies specifically designed to target the psychiatric phenotype. The results of this review suggest that social cognition impairments share some similarities across syndromes. We propose that social cognitive impairments are strongly involved in behavioral symptoms regardless of the overall cognitive level measured by intelligence quotient. Better understanding the mechanisms underlying impaired social cognition may lead to adapt therapeutic interventions. The studies targeting social cognition processes offer new thoughts about the development of specific cognitive training programs, as they highlight the importance of connecting neurocognitive and SCT techniques.

Summary Background The quality of life (QoL) of children receiving vitamin K antagonist (VKA) treatment has been scarcely studied. Aim To assess QoL of children, and its evolution, throughout our non-selective international normalized ratio (INR) self-monitoring education programme. Methods Children and parents completed QoL questionnaires (Qualin, PedsQL) during education sessions. Scores were compared with those from controls. Results A total of 111 children (mean ± standard deviation age 8.7 ± 5.4 years) were included over a 3-year period. Indications for VKA treatment were congenital heart diseases (valve replacement [42.3%], total cavopulmonary connection [29.7%]), myocardiopathy (11.7%), coronary aneurysm (7.2%), venous/intracardiac thrombosis (4.5%), pulmonary artery hypertension (1.8%), arrhythmia (0.9%) and extra-cardiac disease (1.8%). Eighty children, 105 mothers and 74 fathers completed the QoL questionnaires. QoL was good among children aged 1 − 4 years and moderately impaired in those aged between 5 and 18 years. There was no significant relationship between self-reported QoL and patient's sex, type of VKA, number of group sessions attended, disease duration or time of diagnosis (prenatal or postnatal). QoL scores were significantly lower among children with congenital heart diseases compared with other diseases. There were few differences in QoL between children under transient VKA treatment and those treated for life. Parental proxy QoL scoring correlated well with but was significantly lower than child self-assessments. QoL reported by mothers increased throughout the education programme, independently of any improvement of the health condition. Conclusions This QoL study provides original data from a large cohort of children and their parents participating in a formalized INR self-monitoring education programme for VKA treatment.

International audience; Background: McCune-Albright syndrome is a rare disorder characterized by endocrine disorders, café-au-lait spots and fibrous dysplasia of bone that occurs early in life.Methods: A series of 14 pediatric cases were followed between 1994 and 2013 by the competence center for rare endocrine diseases and constitutional bone diseases at CHU de Nancy (France). The diagnosis is based on the presence of at least two symptoms.Results: The mean follow-up was 6 years (1-17 years). The sex ratio was six girls per boy. The incidence was 0.28 cases/million population/year. Mean age at diagnosis was 6 years. A mutation in the GNAS gene was found in 33% of patients tested. Gonadal involvement (13/14 cases), including early peripheral puberty and ovarian cysts in girls (82%) occurred on average at 4 years of age. Bone involvement (10/14 cases) appeared on average at 5 years of age and was most often multiple (80%) with fracture risk, and the skull, with a neurosensory risk.Conclusion: Clinical definition and methods of screening and monitoring can be improved to allow for an earlier intervention. It must be multidisciplinary and take into account the disability and quality of life of the patient.

Aim: To assess the management and outcome of the congenital hypothyroidism (CH) patients followed at our institution since the introduction of systemic neonatal screening for CH. Study Design: The records of 139 CH patients referred to our center between 1978 and 2014 were retrospectively reviewed. Biochemical and imaging data at diagnosis, initial treatment and growth were analyzed. Results: 111 patients had thyroid dysgenesis (64 ectopy, 46 athyreosis and 1 hypoplasia) and 28 patients had a gland in situ (17 dyshormonogenesis/goiter and 11 normal-sized gland). Levothyroxine treatment was initiated at a median age of 11 days with a mean dose of 11.4 μg/kg/day. Compared to those with ectopy, patients with athyreosis had higher thyroid-stimulating hormone (TSH) and lower thyroxine at diagnosis as well as more delayed bone maturation. Between 1978 and 2014, we observed earlier treatment and earlier TSH normalization. Birth auxology was slightly above the mean of the reference population. Growth at 1 and 6 years and school progression at 11 years were similar to those of the reference population. Conclusion: Ectopy is the commonest cause of CH. Children with CH treated early with a mean levothyroxine dose of 11.4 μg/kg/day had a median TSH of 3.07 mU/l at 1 month of age and normal growth., SCOPUS: ar.j, info:eu-repo/semantics/published

Duodenal atresia is a common cause of neonatal obstruction. It is frequently associated with other malformations such as Down syndrome, cardiac anomalies, malrotation or annular pancreas. Double duodenal atresia is an exceptional malformation. There are only few publications on this subject and none are in association with “Cri du Chat” (Cat Cry) syndrome. We present a newborn, prenatally diagnosed with duodenal atresia and with “Cri du Chat” syndrome. The double duodenal atresia was actually of two different types (type I and type II), associated with malrotation. The second atresia was a peroperative finding at reintervention, five days later. We wish to share our experience in order to avoid unnecessary surgery and co-morbidities., SCOPUS: ar.j, info:eu-repo/semantics/published

Background: In Western countries, many children are affected by the separation of their parents. The study's main objective was to analyse the parental behaviours potentially influential for preschool children's health by family structure (parents together or separated). Methods: We conducted a cross-sectional study based on data collected from examinations as part of free preventive medical consultations in the French Community of Belgium. During the assessment of 30,769 infants aged 28 to 32 months, information was collected on the parents' use of tobacco, brushing of the infant's teeth, being monitored by a dentist, and receiving vision screening. The chi2 test was applied and the odds ratios were derived to compare the two groups of children (exposed/not exposed to parental separation). Multivariate logistic regression analyses were used to adjust the effect of exposure. Results: Nearly one in ten (9.8%) did not live with both parents under the same roof. Taking into account the social and cultural environment and other potential confounders at our disposal, we found that in the event of parental separation, behaviours differ in comparison with situations where parents live together; the adjusted odds ratios (ORs) (95% confidence interval) for the infant's exposure to tobacco, absence of teeth brushing, lack of monitoring by a dentist and absence of visual screening, were respectively 1.7 (1.2-2.0), 1.1 (0.9-1.2), 1.3 (1.1-1.6), 1.2 (1.1-1.2), and 1.2 (1.1-1.4). Conclusions: This study confirms the suspicion that parental separation is an independent risk factor for parental behaviours that negatively influence the infant's health. If these results are confirmed, this it could affect the work of the family doctors and paediatricians, especially in terms of family support and information to parents., SCOPUS: ar.j, info:eu-repo/semantics/published

Background: This study aims to determine the prevalence and characteristics of Staphylococcus aureus in Belgian cystic fibrosis (CF) patients. Methods: Non-duplicate respiratory samples from 510 CF-patients (2012−2013) were examined. One isolate per patient was analysed unless different phenotypes were recovered. Isolates were investigated for mecA/mecC, toxins presence, spa-typing, MLST and SCCmec-typing. Potential livestock-associated (LA) isolates were examined for their immune-evasion-cluster (IEC) genes. Results: S. aureus (n = 380), including 41 small-colony variants (SCVs), were isolated from 66.7% patients. The prevalence of methicillin-resistant S. aureus (MRSA) colonization was 4.9%. Two MRSA isolates carried toxic shock syndrome toxin 1 (TSST-1). Most MRSA (65%) belonged to two nosocomial epidemic clones (CC5, CC8) widespread in Belgium. Methicillin susceptible S. aureus (MSSA) showed great genetic diversity. Five of 33 isolates belonging to potential LA-lineages were IEC negative, including three methicillin-resistant isolates, suggesting an animal origin. Conclusions: The MRSA-prevalence in Belgian CF-patients remained constant (2001−2013), but SCV-prevalence increased. Most MRSA belonged to health-care-associated clones. Three patients carrying LA-MRSA were found, requiring further investigation to determine the risk factors for LA-MRSA acquisition., SCOPUS: ar.j, info:eu-repo/semantics/published

Hypoxic-ischemic encephalopathy (HIE) is a rare neonatal condition affecting about 1‰ births. Despite a significant improvement in the management of this condition in the last ten years, HIE remains associated with high rates of death and severe neurological disability. From September 2015 to March 2017, a French national cohort of HIE cases was conducted to estimate the extent of long-term moderate and severe neurodevelopmental disability at 3 years and its determinants. This prospective population-based cohort includes all moderate or severe cases of HIE, occurring in newborns delivered between 34 and 42 completed weeks of gestation and admitted to a neonatal intensive care unit. Detailed data on the pregnancy, delivery, and newborn until hospital discharge was collected from the medical records in maternity and neonatology units. All clinical examinations including biomarkers, EEG, and imaging were recorded. To ensure the completeness of HIE registration, a registry of non-included eligible neonates was organized, and the exhaustiveness of the cohort is currently checked using the national hospital discharge database. Follow-up is organized by the regional perinatal network, and 3 medical visits are planned at 18, 24 and 36 months. One additional project focused on early predictors, in particular early biomarkers, involves a quarter of the cohort. This cohort study aims to improve and update our knowledge about the incidence, the prognosis and the etiology of HIE, and to assess medical care. Its final objective is to improve the definition of this condition and develop prevention and management strategies for high-risk infants. NCT02676063 . Date of registration (Retrospectively Registered): February 8, 2016.

International audience; Background: Fortification of human milk (HM) increases its osmolality, which is associated with an increased risk of necrotizing enterocolitis. The impact of new fortifiers on osmolality is not well-known, nor are the kinetics regarding the increase in osmolality. Aim: To determine the optimum fortifier composition for HM fortification by measuring the osmolality of fortified HM made with three powder multicomponent fortifiers (MCFs) and a protein fortifier (PF). Methods: The osmolality of HM was assessed at 2 (H2) and 24 (H24) h after fortification to compare the effects of MCF (MCF1-3) and PF used in quantities that ensured that infants' nutrient needs would be met (MCF: 4 9/100 ml HM; PF: 0.5 g or 1 g/100 ml HM). To evaluate the early kinetics associated with the osmolality increase, the osmolality of HM fortified with MCF1 or MCF2 was also measured at 0, 1, 5, 10, 15, 20, 30, 40, 50, 60, 90, and 120 min after fortification. Results: The osmolality increased significantly immediately after fortification, depending on the type of fortification used and the quantity of MCF and PF used, rather than the time elapsed after fortification. The maximum value at H24 was 484 mOsm/kg. The mean increase in osmolality between H2 and H24 was 3.1% (p < 0.01) (range: 0.2-10.8%). Most of the increase (>70%) occurred immediately after fortification. Conclusion: When choosing a fortifier, its effect on HM osmolality should be considered. As most of the increase in osmolality occurred immediately, bedside fortification is not useful to prevent the increase in osmolality, and further research should focus on improving fortifier composition.

International audience; OBJECTIVE: The objective was to determine whether maternal nutritional factors are associated with transient neonatal hyperinsulinism (HI).DESIGN AND SETTING: Case control study in 4 French tertiary Obstetrics and Neonatology Departments between 2008 and 2015.METHODS: Sixty-seven mothers of neonates diagnosed with transient hyperinsulinism and 113 mothers of controls were included. The screening for hyperinsulinemic hypoglycemia in neonates was performed because of clinical symptoms suggestive of hypoglycemia or in the presence of conventional risk factors (small-for-gestational-age, prematurity, anoxo-ischemia, hypothermia, macrosomia, gestational diabetes). Hyperinsulinemic hypoglycemia was confirmed in the HI neonates and ruled out in the controls. This allowed for comparing maternal nutrition in cases and controls in a context of similar risk factors. One to 2 mothers of control neonates were included per case, and a food frequency questionnaire was addressed to the mothers between day 5 and day 10 after the birth of their newborn.RESULTS: Crude odds ratio showed that maternal weight gain, abnormal fetal rate, C-section, gender, consumption of fresh cooked vegetables, fresh fruits and fruit juices, low fat diary products, light fat products, and daily bread were significantly associated with hyperinsulinism. Maternal body mass index, hypertension, gestational diabetes, birth weight percentile, gestational age and 5-minute Apgar score were not related to HI. In a multiple backward logistic regression model, consumption of fresh cooked vegetable ≥1/day (OR = 0.33 [0.14-0.77]) and light-fat products ≥1/week (OR = 0.24 [0.08-0.71]) was protective against hyperinsulinism, whereas gestational weight gain >20 kg (OR = 9.5 [2.0-45.5]) and between 15-20 kg (OR = 4.0 [1.2-14.0]), abnormal fetal heart rate (OR = 4.4 [1.6-12.0]), and C-section (OR = 3.4 [1.3-8.9]) were risk factors.CONCLUSIONS: A diet rich in fresh cooked vegetable and reduced in fat, together with the avoidance of a high gestational weight gain may be protective against transient neonatal hyperinsulinism.

Background: Very preterm birth (24 to < 32 week's gestation) is a major public health issue due to its prevalence, the clinical and ethical questions it raises and the associated costs. It raises two major clinical and ethical dilemma: (i) during the perinatal period, whether or not to actively manage a baby born very prematurely and (ii) during the postnatal period, whether or not to continue a curative treatment plan initiated at birth. The Wallonia-Brussels Federation in Belgium counts 11 neonatal intensive care units. Methods: An inventory of key practices was compiled on the basis of an online questionnaire that was sent to the 65 neonatologists working in these units. The questionnaire investigated care-related decisions and practices during the antenatal, perinatal and postnatal periods, as well as personal opinions on the possibility of standardising and/or legislating for end-of-life decisions and practices. The participation rate was 89% (n = 58). Results: The results show a high level of homogeneity pointing to overall agreement on the main principles governing curative practice and the gestational age that can be actively managed given the current state of knowledge. There was, however, greater diversity regarding principles governing the transition to end-of-life care, as well as opinions about the need for a common protocol or law to govern such practices. Conclusion: Our results reflect the uncertainty inherent in the complex and diverse situations that are encountered in this extreme area of clinical practice, and call for qualitative research and expert debates to further document and make recommendations for best practices regarding several "gray zones" of end-of-life care in neonatology, so that high quality palliative care may be granted to all neonates concerned with end-of-life decisions., SCOPUS: ar.j, info:eu-repo/semantics/published

ObjectiveWe aimed to determine paediatric hospital preparedness for a mass casualty disaster involving children in both prehospital and hospital settings. The study findings will serve to generate recommendations, guidelines and training objectives.Design and settingThe AMAVI-PED study is a cross-sectional survey. An electronic questionnaire was sent to French physicians with key roles in specialised paediatric acute care.ResultsIn total, 81% (26 of 32) of French University Hospitals were represented in the study. A disaster plan AMAVI with a specific paediatric emphasis was established in all the paediatric centres. In case of a mass casualty event, paediatric victims would be initially admitted to the paediatric emergency department for most centres (n=21; 75%). Paediatric anaesthesiologists, paediatric surgeons and paediatric radiologists were in-house in 20 (71%), 5 (18%) and 12 (43%) centres, respectively. Twenty-three (82%) hospitals had a paediatric specialised mobile intensive care unit and seven (25%) of these could provide a prehospital emergency response. Didactic teaching and simulation exercises were implemented in 20 (71%) and 22 (79%) centres, respectively. Overall, physician participants rated the level of readiness of their hospital as 6 (IQR: 5–7) on a 10-point readiness scale.ConclusionPaediatric preparedness is very heterogeneous between the centres. Based on the study findings, we suggest that a national programme must be defined and guidelines generated.

International audience; The Francophone Society of Bone Marrow Transplantation and Cellular Therapy (SFGM-TC) organized the 7th allogeneic hematopoietic stem cell transplantation clinical practices harmonization workshop series in September 2016 in Lille, France. The objective of our workshop is to provide a discussion on the conservation and congelation of hematopoietic stem cells in a pediatric setting as well as our recommendations for this technique.

BackgroundA congenital diaphragmatic hernia (DH) can result in severe lung hypoplasia that increases the risk of morbidity and mortality after birth; however, little is known about the cardiorespiratory transition at birth.MethodsUsing phase-contrast X-ray imaging and angiography, we examined the cardiorespiratory transition at birth in rabbit kittens with DHs. Surgery was performed on pregnant New Zealand white rabbits (n=18) at 25 days' gestation to induce a left-sided DH. Kittens were delivered at 30 days' gestation, intubated, and ventilated to achieve a tidal volume (V t) of 8 ml/kg in control and 4 ml/kg in DH kittens while they were imaged.ResultsFunctional residual capacity (FRC) recruitment and V t in the hypoplastic left lung were markedly reduced, resulting in a disproportionate distribution of FRC into the right lung. Following lung aeration, relative pulmonary blood flow (PBF) increased equally in both lungs, and the increase in pulmonary venous return was similar in both control and DH kittens.ConclusionThese findings indicate that nonuniform lung hypoplasia caused by DH alters the distribution of ventilation away from hypoplastic and into normally grown lung regions. During transition, the increase in PBF and pulmonary venous return, which is vital for maintaining cardiac output, is not affected by lung hypoplasia., SCOPUS: ar.j, info:eu-repo/semantics/published

International audience; Objectives To describe neurodevelopmental outcomes at 2 years corrected age for children born alive at 22-26, 27-31, and 32-34 weeks' gestation in 2011, and to evaluate changes since 1997.Design Population based cohort studies, EPIPAGE and EPIPAGE-2.Setting France.Participants 5567 neonates born alive in 2011 at 22-34 completed weeks' gestation, with 4199 survivors at 2 years corrected age included in follow-up. Comparison of outcomes reported for 3334 (1997) and 2418 (2011) neonates born alive in the nine regions participating in both studies.Main outcome measures Survival; cerebral palsy (2000 European consensus definition); scores below threshold on the neurodevelopmental Ages and Stages Questionnaire (ASQ; at least one of five domains below threshold) if completed between 22 and 26 months corrected age, in children without cerebral palsy, blindness, or deafness; and survival without severe or moderate neuromotor or sensory disabilities (cerebral palsy with Gross Motor Function Classification System levels 2-5, unilateral or bilateral blindness or deafness). Results are given as percentage of outcome measures with 95% confidence intervals.Results Among 5170 liveborn neonates with parental consent, survival at 2 years corrected age was 51.7% (95% confidence interval 48.6% to 54.7%) at 22-26 weeks' gestation, 93.1% (92.1% to 94.0%) at 27-31 weeks' gestation, and 98.6% (97.8% to 99.2%) at 32-34 weeks' gestation. Only one infant born at 22-23 weeks survived. Data on cerebral palsy were available for 3599 infants (81.0% of the eligible population). The overall rate of cerebral palsy at 24-26, 27-31, and 32-34 weeks' gestation was 6.9% (4.7% to 9.6%), 4.3% (3.5% to 5.2%), and 1.0% (0.5% to 1.9%), respectively. Responses to the ASQ were analysed for 2506 children (56.4% of the eligible population). The proportion of children with an ASQ result below threshold at 24-26, 27-31, and 32-34 weeks' gestation were 50.2% (44.5% to 55.8%), 40.7% (38.3% to 43.2%), and 36.2% (32.4% to 40.1%), respectively. Survival without severe or moderate neuromotor or sensory disabilities among live births increased between 1997 and 2011, from 45.5% (39.2% to 51.8%) to 62.3% (57.1% to 67.5%) at 25-26 weeks' gestation, but no change was observed at 22-24 weeks' gestation. At 32-34 weeks' gestation, there was a non-statistically significant increase in survival without severe or moderate neuromotor or sensory disabilities (P=0.61), but the proportion of survivors with cerebral palsy declined (P=0.01).Conclusions In this large cohort of preterm infants, rates of survival and survival without severe or moderate neuromotor or sensory disabilities have increased during the past two decades, but these children remain at high risk of developmental delay.

Objective: To determine the performance of a targeted microarray-based cell-free DNA (cfDNA) test (Harmony Prenatal Test®) for the identification of pregnancies at increased risk for 22q11.2 deletion. Methods: Test performance was determined in 2 steps including a total of 1,953 plasma samples. Analytical validation was performed in 1,736 plasma samples. Clinical verification of performance was performed in an additional 217 prospectively ascertained samples from pregnancies with fetal deletion status determined by diagnostic testing. Results: Analytical sensitivity was 75.4% (95% CI: 67.1-82.2%) based on 122 samples with deletions ranging from 1.96 to 3.25 Mb. In 1,614 presumed unaffected samples, specificity was determined to be at least 99.5% (95% CI: 99.0-99.7%). In the clinical cohort, 5 of 7 samples from pregnancies affected with 22q11.2 deletion were determined to have a high probability of deletion. There were no false positive results in the 210 unaffected samples in this cohort. These clinical data are consistent with the performance demonstrated in the analytical validation. Conclusions: cfDNA testing using a targeted microarray-based technology is able to identify pregnancies at increased risk for 22q11.2 deletions of 3.0 Mb and smaller while maintaining a low false positive rate., SCOPUS: ar.j, info:eu-repo/semantics/published

Background Accurate evaluation of aortic root geometry is necessary in congenital aortic valve lesions in children, to guide surgical or angiographical intervention. Aim To compare aortic annulus diameters measured by two- and three-dimensional transthoracic echocardiography (2D- and 3D-TTE), to determine the feasibility and reproducibility of 3D imaging and assess the dynamic changes during the cardiac cycle. Methods Thirty children without heart disease were prospectively included. Two orthogonal aortic annulus diameters were measured offline using multiplanar reconstruction in diastole and in systole and were compared with the measurement of the aortic annulus diameter by 2D-TTE. Results Mean age was 11 ± 3.6 years. Feasibility of 3D imaging was 100%. The coefficients of intra- and interobserver variability were 3.5% and 6%, respectively. The 2D mean diameter was significantly smaller than the 3D maximum diameter in systole (1.94 vs. 2.01 mm; p = 0.005). 2D and 3D measurements were well correlated (p

Osteomyelitis is an inflammatory process caused by microorganisms and usually accompanied by bone destruction. The process may be limited to one portion of the bone or spread to several areas such as the bone marrow, periosteum or cortex.1 It is an infection that can occur at all ages. In children, the average age of onset is 6 years. Today, many of these risks factors are poorly understood or inadequately addressed in healthcare. If improperly treated, the infection can progress to chronicity, with possible recurrence several years after the acute episode. Staphylococcus aureus is the most frequently isolated pathogen. The treatment of acute osteomyelitis should be started at the earliest stage and initiated in hospital with intravenous antibiotics. The antibiotic molecules used must have good penetration in the bone and be bactericidal. The choice of the molecule for empirical treatment must take into account the local epidemiological features and results of bacteriological cultures. According to epidemiological data, the prevalence of Methicillin-resistant Staphylococcus aureus (MRSA) varies greatly from one country to another and from one continent to another. Overcrowding and low social-economic background are factors favouring the spread of MRSA in the community. Apart from ensuring early referral, the medical community also needs to do research on the main challenges facing us in the control of acute osteomyelitis, a disease that is especially serious in children, such as improved diagnosis, detection of drug resistance, shortened treatment regimens and clinical trials of new drugs.