INTRODUCTION:Echocardiography is an important tool for diagnosis of cardiac abnormalities that can impact the management and outcome of the sick newborn in the intensive care unit. A preliminary echocardiogram performed by the neonatologist under the supervision of a paediatric cardiologist for interpretation and review is an alternate when there is not a cardiologist on site. The aim of this study was to evaluate frequency of use, neonatal characteristics, and indications of neonatologist-performed echocardiography in a Tertiary Neonatal Care Centre in Tunisia.METHODS:Prospective observational study in a tertiary Neonatal Intensive Care Unit (NICU) in Monastir (Tunisia) from April 2015 to February 2017.An echocardiography was indicated in these situations: cyanosis, signs of circulatory shock, clinical signs of heart failure, presence of a murmur, arrhythmia, and abnormal pulses in upper and/or lower extremities, suspected persistent pulmonary hypertension in neonates, clinically suspected patent ductus arteriosus, maternal diabetes mellitus and polymalformative syndrome. The findings of echocardiography were confirmed by pediatric cardiologist in case of structural or functional cardiac abnormalities.RESULTS:675 echocardiography were performed among them 535 were normal and 25 revealed a persistent arterial duct treated with E2 postaglandins (Prostine®) or paracetamol according to a pre-established protocol. 80 Congenital heart diseases were retained, which represented an incidence of 7 ‰ live births. The second time of our work consisted to study the 55 cases of cardiac diseases confirmed after exclusion of atrial communication. The antenatal diagnosis was made in 11% of cases. The main signs indicating the echocardiogram were the heart murmur (22 cases) followed by cyanosis (6 cases). A malformation association and / or a chromosomal aberration have been noted in 36% of cases. For half of the patients, the cardiac ultrasound was performed before the first 24 hours of life. This examination was completed by a thoracic angioscan in 9 patients. 31% of newborns had an infusion of Prostaglandins for an average duration of 11 days [2-60 days]. One-third of newborns (35 cases) required respiratory assistance. A palliative surgery was made in 7 cases and curative one in 4 cases. The average age at the time of the intervention was 20 days. The neonatal mortality rate was 40%.CONCLUSION:Echocardiography is being utilized progressively on the neonatal unit, and has been indicated to have a high return for both structural and functional cardiac abnormalities. It is important to encourage collaboration with pediatric cardiologists to establish standards for training and to develop guidelines for clinical practice in order to improve neonatal care.

L’érythème annulaire centrifuge récidivant post-angine est une entité récemment individualisée chez deux patientes adultes. Nous rapportons un nouveau cas chez un enfant. Un enfant âgé de 3 ans, sans antécédents pathologiques notables, était adressé à notre consultation pour des lésions érythémateuses d’évolution centrifuge évoluant par poussées depuis 2 mois dont la dernière remontait à 5 jours. À l’examen dermatologique, on notait de multiples plaques maculopapuleuses érythémateuses annulaires à bordure polycyclique et à centre légèrement pigmenté. Ces lésions, qui sont de tailles différentes, siégeaient au niveau des cuisses, de l’avant-bras droit, du poignet droit et du visage. L’examen oto-rhino-laryngologique objectivait une angine érythémateuse. Le reste de l’examen, en particulier cardiaque, ostéoarticulaire et neurologique, était sans anomalies. La reprise de l’interrogatoire révélait la notion d’une angine qui précédait toujours ces poussées. Les explorations paracliniques biologiques étaient normales hormis un taux élevé d’ASLO. En traitant uniquement l’angine, les lésions cutanées disparaissaient. L’érythème annulaire centrifuge récidivant post-angine est une entité récemment décrite par Elfatoiki et al. en 2014. Ils rapportaient deux cas de femmes adultes qui présentaient des épisodes récidivants de lésions annulaires centrifuges succédant à une angine streptococcique, comme notre patient. La récidive de lésions cutanées annulaires centrifuges après des épisodes d’angine, le taux élevé d’ASLO étaient plus en faveur d’un érythème marginé dans le cadre d’un rhumatisme articulaire aigu post-streptococcique. Toutefois, selon Elfatoiki et al., l’absence d’arthralgies, d’atteinte cardiaque et de syndrome inflammatoire biologique font suggérer le diagnostique de cette entité récente. Quoique la place nosographique exacte de cette éruption annulaire récidivante post-streptococcique reste incertaine, le tableau présenté par notre patient cadrait bien avec ce diagnostic.

Thyroglossal duct cyst of the tongue is a rare entity. Occurrence in the anterior part of the tongue is exceptional. We report in this paper a rare case of thyroglossal cyst of the anterior part of the tongue, discovered in a five-days-old newborn at delivery. Images have shown a cystic mass with homogenous liquid content. A transoral complete resection of the lesion was performed, with no postoperative complication. The histological analysis confirm the diagnosis of lingual thyroglossal duct cyst. There were no recurrence with a follow-up of eight months.

AIMS:To describe the transport of sick neonates to a tertiary care hospital and evaluate their condition at arrival and outcome.METHODS:A multicenter, prospective cohort study was performed in 7 NICUs in Tunisia from 1st april to 31 July 2015.Demographic parameters, transport details and clinical features at arrival were recorded. All neonates were followed up till discharge or death.RESULTS:A total of 239 consecutive neonates were enrolled in the study representing 5.7% of all admitted infants. Maternal risk factors were present in 26% of neonates admitted. Sex-ratio was 1.46. Preterm infants represented 24% of transported babies. Seventeen percent of neonates had severe respiratory distress and 10% had hemodynamic troubles. Referred hospital was not informed in 24% of cases. Regarding the transport mode, 113 newborns (47.5%) were transported in ambulance accompanied by a nurse. Documentation during transfert was present in 14% of cases. Five babies expired on arrival despite resuscitation.  Rate mortality was 8.4%.CONCLUSION:Transporting neonates in developing countries is a challenge. Organized transport services in Tunisia are not always available. So, in cases of at-risk pregnancy, it is safer to transport the mother prior to delivery than to transfer the sick baby after birth.

BACKGROUND:Extremely preterm infants are newborns born before 28 weeks of gestation. Survival of these immature newborns depends on resuscitation and the quality of care during hospitalization.OBJECTIVE:To determine survival and neurologic outcomes at2 years after extremely preterm birth.METHODS:It is a retrospective multicentric study in 5 neonatal intensive care units (NICU) in 2012-2013.All live births less than 28 weeks gestation were included.RESULTS:A total of 109 births were recorded. Prenatal corticosteroids were given in 47% of cases. Mean weight was 989g and mean age was 26 weeks gestation. Ninety percent of patients had respiratory distress syndrome and 67% of them needed respiratory support. Surfactant was given to 29% of newborns. The mortality rate atdischarge was 76%.The first cause of mortality was nosocomial infections. At thecorrected age of 2 years, 27% of survivors had abnormal neurologic outcome.CONCLUSION:In our study, survival and neurologic outcomes ofextremely preterm infants were poor.In this high-risk population, improving perinatal care remains a challenge to improve long-term outcome in Tunisia.

Background Noonan syndrome (NS) is an autosomal dominant multisystem disorder caused by the dysregulation of several genes belonging to the RAS Mitogen Activated Protein Kinase (MAPK) signaling pathway. Incontinentia Pigmenti (IP) is an X-linked, dominantly inherited multisystem disorder. Case presentation This study is the first report of the coexistence of Noonan (NS) and Incontinentia Pigmenti (IP) syndromes in the same patient. We report on the clinical phenotype and molecular characterization of this patient. The patient was examined by a pluridisciplinary staff of clinicians and geneticist. The clinical diagnosis of NS and IP was confirmed by molecular investigations. The newborn girl came to our clinics due to flagrant dysmorphia and dermatological manifestations. The clinical observations led to characterize the Incontinentia Pigmenti traits and a suspicion of a Noonan syndrome association. Molecular diagnosis was performed by Haloplex resequencing of 29 genes associated with RASopathies and confirmed the NS diagnosis. The common recurrent intragenic deletion mutation in IKBKG gene causing the IP was detected with an improved PCR protocol. Conclusion This is the first report in the literature of comorbidity of NS and IP, two rare multisystem syndromes.

Objective. Evaluate whether saliva could be a useful alternative to serum for routine therapeutic drug monitoring of caffeine in preterm infants using the enzyme multiplied immunoassay technique (EMIT) assay. Methods. We conducted a prospective study including preterm infants (less than 34 weeks' amenorrhea) admitted to the intensive care and neonatal medicine department. All infants received 5, 10, 15, 20 and 25 mg/kg/day of citrate caffeine intravenously from the first to the fifth day of birth, respectively. For each patient, two concomitant blood and saliva samples corresponding to the trough concentrations were collected 24 hours after each caffeine dose. The caffeine concentrations were determined using the EMIT (R) 2000 caffeine assay. Results. Thirteen preterm infants were included. The saliva and the serum caffeine concentration increased proportionally to the administered dose. Saliva and serum kinetics were comparable and the saliva caffeine concentrations were correlated to the serum ones (r(2) = 0.76). Conclusion. Saliva caffeine monitoring by EMIT is a valid, useful and safe alternative to serum in preterm infants. (C) 2017 Societe francaise de pharmacologie et de therapeutique. Published by Elsevier Masson SAS. All rights reserved.

Les bruits en réanimation induisent des effets indésirables chez les nouveau-nés, leurs familles et le personnel. L’étude a évalué les niveaux sonores en réanimation pédiatrique avant et après la mise en place d’un outil de monitorage du bruit de type SoundEar® associée à une formation du personnel.Une cartographie du service a déterminé la zone la plus exposée au bruit et les périodes du nycthémère les plus bruyantes. Une formation a alors été organisée avec sensibilisation du personnel aux niveaux sonores, aux effets néfastes sur les nouveau-nés et un monitorage SoundEar® a été installé. Les niveaux sonores ont été mesurés avant et 1, 2 et 3 mois après le début de la formation.Le niveau sonore moyen était élevé notamment au niveau du poste central de l’unité et durant les transmissions (Leq moyen : 60,6 ± 3,6 dB(A) ; pics sonores : 94,8 ± 6,8 dB(A)). Une diminution (p < 0,001) a été observée à 1 et 2 mois mais pas à 3 mois après la formation, ces niveaux restant trop élevés par rapport aux recommandations.Une formation du personnel et l’utilisation continue d’un outil de monitorage du bruit réduit les niveaux sonores à 1 et 2 mois, mais 3 mois après la mise en place du dispositif cet effet disparaît. La réduction du niveau sonore est significative, mais modeste, sans atteindre les valeurs recommandées. Ainsi, l’utilisation combinée et régulière de ces deux actions en réanimation doit être envisagée et répétée au moins tous les 3 mois.The sound level in the neonatal intensive care unit (NICU) may induce adverse effects for neonates, their family, and the staff. This study evaluated the sound level in a NICU before and after the implementation of an educational program.A baseline audit determined the most exposed area of the NICU and the most exposed periods over 24 h. Then an educational program started, including sound level measurement methods, side effects for neonates, results from the baseline audit, and new visual monitoring equipment (SoundEar®). Sound levels were measured before, 1, 2, and 3 months after starting the educational program and the use of SoundEar®. The NICU staff was blind to the periods of sound level measurements.The base noise level was high, especially near the central part of the NICU and during transmission time (mean Leq: 60.6 ± 3.6 dB(A); sound peaks: 94.8 ± 6.8 dB(A)). A decrease in the sound level (P < 0.001) was found 1 and 2, but not 3 months after starting the educational program. It remained high compared to the guidelines.Human activity was responsible for most of the sound level. An educational program was effective in reducing the sound level, but did not reach the guideline's target. The continuous use of sound-monitoring equipment after starting the project reduced the sound level for 2 months, but no longer. Therefore, a continuous educational program about the sound level in the NICU including feedback monitoring every 2–3 months should be encouraged.

Neurenteric cysts are the association of an endodermal cyst with a vertebral dysplasia. This congenital malformation can be asymptomatic or manifest itself through respiratory signs due to airway c...

L’œdème aigu hémorragique du nourrisson (OAHN) est une vascularite rare et bénigne survenant chez des nourrissons de 4 à 24 mois. Les lésions cutanées, prenant parfois l’aspect d’un purpura ecchymotique ou en nappe, peuvent faire évoquer le diagnostic de purpura fulminans lorsqu’une fièvre y est associée et entraîner alors la mise en route d’une antibiothérapie. Nous rapportons le cas d’un nourrisson de 7 mois dont la présentation clinique initiale d’OAHN a fait suspecter un purpura fulminans. L’évolution rapide des lésions a fait instaurer une antibiothérapie par céfotaxime et amikacine. Le diagnostic d’OAHN a été porté le lendemain devant l’aspect des lésions cutanées, la stabilité hémodynamique de l’enfant et la négativité du bilan bactériologique. Un entérovirus (Coxsackie virus B5) a été mis en évidence secondairement par réaction de polymérisation en chaîne spécifique (PCR) dans le liquide céphalorachidien. Après arrêt de l’antibiothérapie, l’évolution a été spontanément favorable. L’imputabilité de l’entérovirus dans le déclenchement de cet OAHN est discutée.Acute hemorrhagic edema of infancy is a rare but benign vasculitis occurring in infants aged from 4 to 24 months. Skin lesions can take various forms, including extensive hemorrhagic purpura, and can therefore be mistaken for purpura fulminans if associated with fever, which leads to initiating broad-spectrum antibiotic treatment. In the present case, we describe a 7-month-old boy with acute hemorrhagic edema of infancy and rapidly extensive purpura lesions that led to intravenous cefotaxime and amikacin treatment. Diagnosis was made on the next day by a dermatologist, based on the typical aspect of skin lesions, hemodynamic stability, and negative bacteriological samples. Coxsackie virus B5, a pathogenic enterovirus, was found by specific PCR in cerebrospinal fluid. The outcome was spontaneously favorable after discontinuation of antibiotics on day 2. We discuss the imputability of the enterovirus in triggering this case of acute hemorrhagic edema of infancy.

Poster: ECR 2017 / C-1399 / MRI of fetal urinary tract by: A. BenSalem 1, A. Khalfalli2, M. ABDELAALI2, R. Laamiri2, K. Ben Ameur2, F.-Z. Chioukh2, C. Hafsa2; 1Monastir , Selectionnez un departement/TN, 2Monastir/TN

La prise en charge diététique de l’allergie aux protéines du lait de vache requiert l’élimination du régime de toute trace de protéines de lait. Pour les nourrissons non allaités au sein, la principale mesure diététique consiste à remplacer le lait infantile standard par une formule dont l’allergénicité de la fraction protéique a été supprimée. Celle-ci peut être à base d’hydrolysats de protéines de lait de vache ou de riz ou encore à base d’acides aminés. La surveillance de la croissance staturopondérale des enfants atteints est indispensable. La réintroduction du lait, cru ou cuit, se fait toujours prudemment et progressivement. Lorsque l’enfant grandit et reste allergique au lait de vache, l’utilisation de formes cuites du lait facilite considérablement son alimentation.Dietary management of cow's milk allergy is based on the elimination of all cow's milk proteins from the diet. For non-breastfed infants, the main dietetic move is the replacement of the standard infant formula with a formula for which the protein fraction has been modified. This formula can be based on hydrolyzed cow's milk proteins, rice proteins or amino acids. Monitoring the growth of these children is essential. The reintroduction of cow's milk, raw or heated, should always be made carefully and gradually. As the child grows and remains allergic, the use of heated (baked) milk substantially eases his/her feeding.

Le syndrome ARC (arthrogrypose-atteinte rénale [tubulopathie proximale]-cholestase) est une maladie génétique rare, autosomique récessive, responsable d’une atteinte multisystémique rapidement létale. Un nouveau-né, né de parents apparentés, a développé à partir de 10 jours de vie un ictère cholestatique associé à une déshydratation et un syndrome de Fanconi. Le frottis sanguin a montré un aspect très caractéristique de plaquettes « grises » et une mutation à l’état homozygote a été mise en évidence dans le gène VPS33B (l’un des deux gènes responsables du syndrome, codant pour une protéine impliquée dans le trafic des vésicules intracellulaires). Cette mutation (c.604-2A > G), présente à l’état hétérozygote chez les parents, affecte une base invariante du site accepteur d’épissage et n’a pas été encore rapportée à notre connaissance. L’enfant est décédé à l’âge de 3 mois. Un diagnostic prénatal ayant pu être proposé à la famille une nouvelle grossesse a été menée à son terme et une petite fille hétérozygote est née, indemne de la maladie. Cette affection rare s’ajoute à la liste des cholestases néonatales. Le diagnostic évoqué sur la complexité du phénotype est grandement facilité par l’examen du frottis sanguin.ARC syndrome (arthrogryposis – renal dysfunction – cholestasis) is a rare lethal multisystemic autosomal recessive disease. A newborn of consanguineous parents of Algerian descent presented cholestatic jaundice, dehydration, and Fanconi syndrome at 10 days of life. The blood smear showed a very characteristic gray appearance of platelets. A homozygous mutation was evidenced in the VPS33B gene. This gene codes for a protein involved in trafficking of intracellular vesicles. The mutation (c.604-2A > G) present in the heterozygous state in the parents affects an invariant base of the splice acceptor site and to our knowledge has not been reported yet. This child died at the age of 3 months. Prenatal diagnosis was offered to the family; another pregnancy was carried to completion and a girl was born without the disease. The combination of cholestasis and proximal tubulopathy should suggest the diagnosis in a newborn with orthopedic problems. A blood smear greatly facilitates diagnosis.

BACKGROUND:The reliability of blood glucose monitoring in neonatology is not always confirmed. The aim of this study was to evaluate the reliability of blood glucose measurements made with three different devices in newborns.PATIENTS AND METHODS:The study was prospective, conducted in a medical and neonatal intensive care department over a period of 4 months. Capillary glucose level was measured with three different glucometers and compared with venous glucose level determined using the hexokinase method. An ANOVA and Scheffe test were used for the correlation analysis.RESULTS:Three hundred and nine infants were included, with a mean age of 55h and a mean term of 39 weeks of gestation. Mean blood glucose in the laboratory was 0.62±0.15g/L, 0.71±0.17g/L for Accu-Chek(®) Active, 0.80±0.17g/L for Accu-Chek(®) Performa, and 0.83±0.12g/L for Bionime. An ANOVA showed statistically significant differences between the measurements made by glucometers compared to the reference blood glucose levels, and the Scheffé method showed that glucometers overestimated the real plasma glucose levels.CONCLUSION:None of the devices used in this study was satisfactory. However, an estimation of blood glucose taking into consideration this numerical overestimation would allow early detection of hypoglycemia.

La surveillance de la glycémie est importante en néonatalogie. Elle repose sur la mesure de la glycémie capillaire à la bandelette dont la fiabilité n’est pas toujours vérifiée chez le nouveau-né. L’objectif de notre travail était d’évaluer la fiabilité des résultats de la glycémie capillaire mesurée par trois appareils différents par rapport à la glycémie plasmatique chez le nouveau-né. Il s’est agi d’une étude prospective menée sur une période de 4 mois. La glycémie capillaires était mesurée par trois lecteurs (Accu-Chek® Active, Accu-Chek® Performa et Bionime) et comparée à une glycémie concomitante dosée sur un prélèvement veineux. Trois cent neuf nouveau-nés ont été inclus. Leur âge moyen était de 55 h lors du prélèvement. Le terme moyen était de 39 semaines d’aménorrhée (SA). La glycémie veineuse moyenne était de 0,62 ± 0,15 g/L, celle mesurée par les différents appareils était à 0,71 ± 0,17 g/L pour Accu-Chek® Active, 0,80 ± 0,17 g/L pour Accu-Chek® Performa et 0,83 ± 0,12 g/L pour Bionime. La différence entre les mesures capillaires et la glycémie veineuse était significative. Le test de Schiffé a montré que les 3 lecteurs de bandelettes surestimaient la glycémie réelle chez le nouveau-né. Il est nécessaire de revoir les seuils d’intervention médicale en fonction du type d’appareil utilisé, surtout en cas d’hypoglycémie. The reliability of blood glucose monitoring in neonatology is not always confirmed. The aim of this study was to evaluate the reliability of blood glucose measurements made with three different devices in newborns. The study was prospective, conducted in a medical and neonatal intensive care department over a period of 4 months. Capillary glucose level was measured with three different glucometers and compared with venous glucose level determined using the hexokinase method. An ANOVA and Scheffe test were used for the correlation analysis. Three hundred and nine infants were included, with a mean age of 55 h and a mean term of 39 weeks of gestation. Mean blood glucose in the laboratory was 0.62 ± 0.15 g/L, 0.71 ± 0.17 g/L for Accu-Chek® Active, 0.80 ± 0.17 g/L for Accu-Chek® Performa, and 0.83 ± 0.12 g/L for Bionime. An ANOVA showed statistically significant differences between the measurements made by glucometers compared to the reference blood glucose levels, and the Scheffé method showed that glucometers overestimated the real plasma glucose levels. None of the devices used in this study was satisfactory. However, an estimation of blood glucose taking into consideration this numerical overestimation would allow early detection of hypoglycemia.