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1. Follow-up at two years of age and early predictors of non-compliance in a cohort of very preterm infants

Author

Patrick Gérardin, Emmanuel Chirpaz, Magali Carbonnier, Duksha Ramful, Corinne Mussard, Sylvain Samperiz, and Claire Mas

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Multivariate analysis, Developmental Disabilities, Population, 03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, Humans, Medicine, 030212 general & internal medicine, education, Not evaluated, education.field_of_study, business.industry, Infant, Newborn, Infant, Obstetrics and Gynecology, Gestational age, Pediatrics, Perinatology and Child Health, Cohort, Premature Birth, Gestation, Female, Observational study, Parity (mathematics), business, Infant, Premature
Abstract

Aim To examine the rates of follow-up at two years of age and perinatal factors associated with non-compliance in an observational population-based cohort of very preterm children enrolled in a routine follow-up program. Method Data review of infants born between 2008 and 2012 in the Observatoire de La Grande Prematurite, Reunion Island cohort. All singletons born alive before 33 weeks of gestational age and resident on the island at two years of age were included. Patients were considered compliant if they were timely evaluated between 20–28 months of age, or non-compliant if they were not evaluated or evaluated after 28 months of age. Results Of the 802 survivors (mean gestational age of 30.3 ± 2.0 months, mean birthweight of 1364 ± 396 g), 468 (58.4%) were examined between 20–28 months, 119 (14.8%) after 28 months of age, and 215 (26.8%) were never evaluated, respectively. In multivariate analysis, factors associated with non-compliance were higher parity (> 2), past history of preterm delivery, maternal diabetes (preexisting or gestational), appropriate for gestational status, and centre of birth. Conclusion Sustainable follow-up of vulnerable neonates remains a challenge in clinical practice. Early predictors of non-compliance can be used to define individualized and local follow-up strategies in these infants at high risk for developmental disabilities.

Published
2017

2. Mortality and severe morbidity of very preterm infants: comparison of two French cohort studies

Author

Anais Godeluck, Victorine Lenclume, Valérie Benhammou, Corinne Mussard, Patrick Truffert, Patrick Gérardin, Pierre-Yves Robillard, Sylvain Samperiz, Pierre-Yves Ancel, Duksha Ramful, Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Epidémiologie Clinique [Saint-Pierre, La Réunion], Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Sud Saint Pierre [Ile de la Réunion], CHU Sud Saint Pierre [Ile de la Réunion], Centre d'Études Périnatales de l'Océan Indien (CEPOI), Université de La Réunion (UR)-Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Centre de Recherche Épidémiologie et Statistique Sorbonne Paris Cité (CRESS (U1153 / UMR_A_1125 / UMR_S_1153)), Institut National de la Recherche Agronomique (INRA)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Université Sorbonne Paris Cité (USPC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Evaluation des technologies de santé et des pratiques médicales - ULR 2694 (METRICS), Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Université Paris Descartes - Paris 5 (UPD5), CIC - Mère Enfant Necker Cochin Paris Centre (CIC 1419), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Neonatal and pediatric intensive care unit [Saint-Denis, La Réunion], The EPIPAGE-2 project has been funded with support from the following organisations: The French Institute of Public Health Research/Institute of Public Health and its partners: the French Health Ministry, the National Institute of Health and Medical Research (INSERM), the National Institute of Cancer, and the National Solidarity Fund for Autonomy (CNSA), The National Research Agency through the French EQUIPEX program of investments in the future (reference ANR-11-EQPX-0038), the PREMUP Foundation, Fondation de France (reference 00050329), and Fondation pour la Recherche Médicale (reference SPF20160936356). The OGP project has been funded by the Agence Régionale de Santé-Indian Ocean regional health agency., ANR-11-EQPX-0038,RE-CO-NAI,Plateforme de REcherche sur les COhortes d'enfants suivis depuis la NAIssance(2011), Santé publique : épidémiologie et qualité des soins-EA 2694 (CERIM), CHU Cochin [AP-HP]-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), ANR-11-EQPX-0038/11-EQPX-0038,RE-CO-NAI,Plateforme de REcherche sur les COhortes d'enfants suivis depuis la NAIssance(2011), Bodescot, Myriam, Equipements d'excellence - Plateforme de REcherche sur les COhortes d'enfants suivis depuis la NAIssance - - RE-CO-NAI2011 - ANR-11-EQPX-0038 - EQPX - VALID, CHU Lille, Université de Lille, METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694, and Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS]

Subjects
Male, Pediatrics, medicine.medical_specialty, Gestational Age, Very low birth weight, Infant, Premature, Diseases, Logistic regression, Severity of Illness Index, Retinopathy of prematurity, Necrotising enterocolitis, 03 medical and health sciences, 0302 clinical medicine, [SDV.MHEP.PED] Life Sciences [q-bio]/Human health and pathology/Pediatrics, Preterm, 030225 pediatrics, Medicine, Humans, 030212 general & internal medicine, Hospital Mortality, Mortality, Bronchopulmonary dysplasia, Cohort studies, Intraventricular haemorrhage, Morbidity, Periventricular leukomalacia, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, business.industry, lcsh:RJ1-570, Infant, Newborn, Gestational age, lcsh:Pediatrics, medicine.disease, 3. Good health, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Pediatrics, Perinatology and Child Health, Cohort, Gestation, Female, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, France, business, Reunion, Infant, Premature, Cohort study, Research Article
Abstract

Background In Reunion Island, a French overseas department, the burden of preterm birth and perinatal mortality exceed those observed in mainland France, despite similar access to standard perinatal care. The purpose of the study was to compare the outcome of two cohorts of NICU-admitted very preterm infants born between 24 and 31 weeks of gestation (WG): the registry-based OGP (Observatoire de la Grande Prématurité, Reunion Island, 2008–2013) cohort, and the nationwide EPIPAGE-2 (mainland France, 2011) observational cohort. Methods The primary outcome was adverse neonatal outcomes defined as a composite indicator of in-hospital mortality or any of three following severe morbidities: bronchopulmonary dysplasia (BPD), necrotising enterocolitis, or severe neurological injury (periventricular leukomalacia or grade III-IV intraventricular haemorrhages). Logistic regression modelling adjusting for confounders was performed. Results A total of 1272 very preterm infants from the Reunionese OGP cohort and 3669 peers from the mainland EPIPAGE-2 cohort were compared. Adverse neonatal outcomes were more likely observed in the OGP cohort (32.6% versus 26.6%, p Conclusions Despite similar guidelines for standard perinatal care, very preterm infants born in Reunion Island have a higher risk for death or severe morbidity compared with those born in mainland France.

Published
2019

3. Characteristics of prescription in 29 Level 3 Neonatal Wards over a 2-year period (2017-2018). An inventory for future research

Author

Elsa Kermorvant-Duchemin, Guillaume Binson, Cécile Desbruyeres, Roselyne Brat, Evelyne Jacqz-Aigrain, Anne-Sophie Pages, Guillaume Escourrou, Duksha Ramful, Séverine Martin-Mons, Gaël Mazeiras, Amélie Moussy-Durandy, Julien Mourdie, Ceneric Alexandre, Francesco Bonsante, Marine Dorsi, Florence Le Bail Dantec, Laurence Caeymaex, Yaovi Kugbe, Olivier Flechelles, Soumeth Abasse, Béatrice Gouyon, Anaelle Pignolet, Massimo Di Maio, Abdellah ElGellab, Yvan Couringa, Mohamed-Amine Yangui, Elodie Marie Garnier, Alexandre Lapillonne, Karine Norbert, Catherine Lafon, Jean-Bernard Gouyon, Hasinirina Razafimahefa, Simon Lorrain, Silvia Iacobelli, Florence Flamein, Delphine Mitanchez, Léila Karaoui, Olivier Girard, Jean-Marc Rosenthal, Centre d'Études Périnatales de l'Océan Indien (CEPOI), Université de La Réunion (UR)-Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Centre Hospitalier Sud Francilien, CH Evry-Corbeil, CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Régional d'Orléans (CHRO), Centre Hospitalier Intercommunal de Créteil (CHIC), Centre Hospitalier Andrée Rosemon [Cayenne, Guyane Française], CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN), Groupe Hospitalier Artois-Ternois Centre Hospitalier d’Arras, Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Centre hospitalier universitaire de Poitiers (CHU Poitiers), CHU Lille, centre hospitalier intercommunal de Poissy/Saint-Germain-en-Laye - CHIPS [Poissy], Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Centre Hospitalier de Saint-Denis [Ile-de-France], Centre Hospitalier Métropole Savoie [Chambéry], Groupe Hospitalier du Havre Hôpital Jacques Monod (MONTIVILLIERS) (GHH), Centre Hospitalier Intercommunal André Grégoire [Montreuil] (CHI André Gregoire), Centre Hospitalier Universitaire de Martinique [Fort-de-France, Martinique], Centre Hospitalier de Mayotte, CHU Pointe-à-Pitre/Abymes [Guadeloupe], Centre Hospitalier Public du Cotentin (CHPC), Centre hospitalier territorial Gaston-Bourret [Nouméa], Grand Hôpital de l'Est Francilien (GHEF), Centre Hospitalier de Lens, Centre hospitalier Saint-Brieuc, Centre Hospitalier René Dubos [Pontoise], Centre hospitalier de Pau, Centre Hospitalier de l'Ouest Guyanais Franck Joly [Saint-Laurent-du-Maroni, Guyane Française], CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital Robert Debré, and Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims (CHU Reims)

Subjects
Male, Pediatrics, Databases, Factual, Organic chemistry, Medication prescription, Neonatal Care, 0302 clinical medicine, Antibiotics, Medicine and Health Sciences, Medicine, 030212 general & internal medicine, Summary of Product Characteristics, Practice Patterns, Physicians', Vitamin D, Routes of Administration, Multidisciplinary, Antimicrobials, Gestational age, Drugs, Vitamins, 3. Good health, Chemistry, Physical Sciences, Female, Infant, Premature, Cohort study, Research Article, medicine.medical_specialty, Prescription Drugs, Science, Cardiology, Gestational Age, Drug Prescriptions, Microbiology, 03 medical and health sciences, Alkaloids, 030225 pediatrics, Caffeine, Microbial Control, Patients' Rooms, Organic compounds, Humans, Medical prescription, Retrospective Studies, Polypharmacy, Pharmacology, business.industry, Infant, Newborn, Chemical Compounds, Biology and Life Sciences, Neonates, Retrospective cohort study, Environmental Exposure, Health Care, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Observational study, Neonatology, business, Developmental Biology
Abstract

ObjectivesThe primary objective of this study is to determine the current level of patient medication exposure in Level 3 Neonatal Wards (L3NW). The secondary objective is to evaluate in the first month of life the rate of medication prescription not cited in the Summary of Product Characteristics (SmPC). A database containing all the medication prescriptions is collected as part of a prescription benchmarking program in the L3NW.Material and methodsThe research is a two-year observational cohort study (2017-2018) with retrospective analysis of medications prescribed in 29 French L3NW. Seventeen L3NW are present since the beginning of the study and 12 have been progressively included. All neonatal units used the same computerized system of prescription, and all prescription data were completely de-identified within each hospital before being stored in a common data warehouse.ResultsThe study population includes 27,382 newborns. Two hundred and sixty-one different medications (International Nonproprietary Names, INN) were prescribed. Twelve INN (including paracetamol) were prescribed for at least 10% of patients, 55 for less than 10% but at least 1% and 194 to less than 1%. The lowest gestational ages (GA) were exposed to the greatest number of medications (18.0 below 28 weeks of gestation (WG) to 4.1 above 36 WG) (pConclusionNeonates remain therapeutic orphans. The consequences of polypharmacy in L3NW should be quickly assessed, especially in the most immature infants.

Published
2019

4. Evaluation of the management of pregnancies and infants at risk for congenital syphilis: La Réunion, 2008 to 2014

Author

M Le Chevalier de Préville, Fabrice Cuillier, Stéphanie Robin, N Traversier, J-L Alessandri, and Duksha Ramful

Subjects
Adult, Male, medicine.medical_specialty, Pediatrics, Adolescent, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Pregnancy, Risk Factors, Humans, Medicine, Maternal fetal, 030212 general & internal medicine, Neonatology, Pregnancy Complications, Infectious, Retrospective Studies, 030219 obstetrics & reproductive medicine, business.industry, Obstetrics, Syphilis, Congenital, Infant, Newborn, Obstetrics and Gynecology, Gestational age, Penicillin G, medicine.disease, Treatment Outcome, Congenital syphilis, Practice Guidelines as Topic, Pediatrics, Perinatology and Child Health, Female, France, business
Abstract

To evaluate the maternal-fetal management and follow-up of infants at risk for congenital syphilis.Monocentric retrospective cohort study at the Félix Guyon Hospital, Saint-Denis, La Réunion between January 2008 and December 2014. Management of 38 pregnancies (35 women, 39 fetuses) with a positive syphilis serology was evaluated according to the Centers for Disease Control and Prevention (CDC) guidelines.A total of 68% (n=26) of pregnancies were screened before 16 weeks of gestation, and 26% (n=10) had appropriate treatment. Adverse outcomes were noted in 45% of pregnancies. Neonatal serology was not performed in 7 live-born neonates (19%) and 33 infants were classifiable according to the four CDC scenarios. In the infants, adequate complementary evaluation and appropriate treatment were noted in 42% (n=13) and 55% (n=17) of cases, respectively. Rate of infant follow-up was 76% (n=16). No treatment failure was observed in infants.Reinforcement of public health policies and a better sensitization, training and collaboration among perinatal caregivers are warranted given the gaps revealed in our study.

Published
2016

5. Low Renal Oxygen Saturation at Near-Infrared Spectroscopy on the First Day of Life Is Associated with Developing Acute Kidney Injury in Very Preterm Infants

Author

Sylvain Samperiz, Silvia Iacobelli, Duksha Ramful, Jean-Bernard Gouyon, Francesco Bonsante, Sandrine Daniel, and Christine Binquet

Subjects
Male, medicine.medical_specialty, Renal function, Gestational Age, Infant, Premature, Diseases, urologic and male genital diseases, Kidney, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, 030225 pediatrics, Internal medicine, Intensive care, medicine.artery, medicine, Humans, Infant, Very Low Birth Weight, 030212 general & internal medicine, Prospective Studies, Renal artery, Oxygen saturation (medicine), Creatinine, Spectroscopy, Near-Infrared, business.industry, Acute kidney injury, Hemodynamics, Infant, Newborn, Acute Kidney Injury, medicine.disease, Echocardiography, Doppler, Oxygen, medicine.anatomical_structure, chemistry, Infant, Extremely Premature, Pediatrics, Perinatology and Child Health, Multivariate Analysis, Cardiology, Linear Models, Gestation, Female, business, Biomarkers, Developmental Biology
Abstract

Background: Acute kidney injury (AKI) is a frequent complication in preterm infants, and the identification of early markers of renal hypoperfusion is a chief challenge in neonatal intensive care units. Objectives: To describe the association between early markers of cardiovascular function and renal perfusion with AKI occurrence in a cohort of preterm infants < 32 weeks’ gestation. Methods: 128 infants were prospectively included from birth to discharge. During the first day of life, we assessed cardiovascular function, systemic and organ blood flow by Doppler ultrasound, and monitored cerebral and renal regional oxygen saturation (rSO2) using near-infrared spectroscopy (NIRS). These measures were analyzed in relation to developing AKI and serum creatinine (SCr) peak from day 2 to 7 of life. Results: 12 of 128 infants presented with AKI (9.4%). SCr peak was 155.3 ± 30.2 µmol/L in infants with AKI versus 82.0 ± 16.5 in non-AKI infants (p < 0.001). Among all measures of cardiovascular function and renal perfusion, low mean cerebral and renal rSO2 during the first day of life and a low resistive index at renal artery Doppler were significantly associated with developing AKI. After adjustment for possible confounding factors, low renal rSO2 on the first day of life remained associated with a high SCr peak from day 2 to 7 of life. Conclusion: Low renal rSO2 values during the first day of life correlate with developing AKI in preterm infants < 32 weeks’ gestation. NIRS monitoring of renal function during adaptation seems promising, and its very early use after birth to detect kidney hemodynamic dysfunction deserves further investigations.

Published
2018

6. Recessive loss of function PIGN alleles, including an intragenic deletion with founder effect in La Réunion Island, in patients with Fryns syndrome

Author

Asma Chebil, Duksha Ramful, Thibaud Jouan, Hanitra Randrianaivo, Norbert F. Ajeawung, Nicolas Gruchy, Yannis Duffourd, Bérénice Doray, Christopher T. Gordon, Francesca Filippini, Christine Bole-Feysot, Philippe M. Campeau, Asma Omarjee, Julien Thevenon, Paul Kuentz, Jean-Luc Alessandri, Patrick Nitschke, Laurence Faivre, Jeanne Amiel, Coralie Dumont, Thi Tuyet Mai Nguyen, Marion Gérard, Myriam Oufadem, Guillaume Benoist, Olivier Alibeu, Marie-Line Jacquemont, Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Imagine - Institut des maladies génétiques (IMAGINE - U1163), Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Sud Saint Pierre [Ile de la Réunion], Service de Génétique [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Centre de recherche du CHU Sainte-Justine, CHU Sainte Justine [Montréal], Centre Hospitalier de Mayotte, Equipe GAD (LNC - U1231), Lipides - Nutrition - Cancer [Dijon - U1231] (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement, Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement, FHU TRANSLAD, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Laboratoire de Génétique Médicale (LGM), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de recherche du CHU Sainte-Justine [Montreal], Université de Montréal (UdeM)-CHU Sainte Justine [Montréal], Service de réanimation néonatologique et pédiatrique [La Réunion], Groupe Hospitalier Sud-CHR La réunion, Embryology and genetics of human malformation (Equipe Inserm U1163), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Génétique et épigénétique des maladies métaboliques, neurosensorielles et du développement (Inserm U781), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Université de Caen Normandie (UNICAEN), Normandie Université (NU), Biologie, génétique et thérapies ostéoarticulaires et respiratoires (BIOTARGEN), Normandie Université (NU)-Normandie Université (NU), Pôle Gynécologie Obstétrique [CHM, Mayotte], Centre de génétique - Centre de référence des maladies rares, anomalies du développement et syndromes malformatifs (CHU de Dijon), Hôpital d'Enfants [CHU Dijon], Hôpital du Bocage, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon)-Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Pôle Femme-Mère-Enfant [CHU La Réunion, Saint-Pierre, La Réunion], Plateforme de génomique [SFR Necker], Structure Fédérative de Recherche Necker (SFR Necker - UMS 3633 / US24), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Plate Forme Paris Descartes de Bioinformatique (BIP-D), Université Paris Descartes - Paris 5 (UPD5), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Biologie moléculaire et cellulaire de la différenciation, Université Joseph Fourier - Grenoble 1 (UJF)-Institut Albert Bonniot-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de La Réunion ( CHU La Réunion ), Imagine - Institut des maladies génétiques ( IMAGINE - U1163 ), Centre National de la Recherche Scientifique ( CNRS ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université Paris Descartes - Paris 5 ( UPD5 ), Service de Génétique Clinique [Caen], Université de Caen Normandie ( UNICAEN ), Normandie Université ( NU ) -Normandie Université ( NU ) -CHU Caen, Lipides - Nutrition - Cancer [Dijon - U1231] ( LNC ), Université de Bourgogne ( UB ) -AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Université de Bourgogne ( UB ) -AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Université de Bourgogne ( UB ) -AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Laboratoire de Génétique Médicale ( LGM ), Université de Strasbourg ( UNISTRA ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de Réanimation Néonatale et Pédiatrique, CHD Félix Guyon, Eidgenössische Technische Hochschule - Swiss Federal Institute of Technology in Zürich [Zürich] (ETH Zürich), Université de Lausanne (UNIL), Laboratoire de Chimie Physique Macromoléculaire (LCPM), Université de Lorraine (UL)-Centre National de la Recherche Scientifique (CNRS), Equipe NuTox (LNC - U1231) (NUTOX), Service de Médecine Néonatale [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Centre National de la Recherche Scientifique (CNRS)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Université de Maurice, Service de médecine foetale et foetopathologie, Service de Génétique, Hôpital de Hautepierre [Strasbourg], Department of Experimental Cardiology, Academic Medical Center - Academisch Medisch Centrum [Amsterdam] (AMC), and University of Amsterdam [Amsterdam] (UvA)-University of Amsterdam [Amsterdam] (UvA)-Heart Failure Research Center (HFRC)

Subjects
0301 basic medicine, Male, MESH: Limb Deformities, Congenital, Limb Deformities, Congenital, MESH: Phosphotransferases, [SDV.CAN]Life Sciences [q-bio]/Cancer, 030105 genetics & heredity, Biology, MESH: Founder Effect, Article, 03 medical and health sciences, Pulmonary hypoplasia, 0302 clinical medicine, Fryns syndrome, Loss of Function Mutation, Genetics, medicine, Missense mutation, Humans, Allele, [ SDV.GEN.GH ] Life Sciences [q-bio]/Genetics/Human genetics, Genetics (clinical), Loss function, Hernia, Diaphragmatic, [SDV.GEN]Life Sciences [q-bio]/Genetics, MESH: Humans, Phosphotransferases, MESH: Infant, Newborn, Infant, Newborn, Congenital diaphragmatic hernia, Facies, Infant, MESH: Loss of Function Mutation, medicine.disease, MESH: Infant, Hypoplasia, Founder Effect, MESH: Male, MESH: Hernia, Diaphragmatic, MESH: Facies, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, MESH: Gene Deletion, Female, MESH: Female, 030217 neurology & neurosurgery, Gene Deletion, Founder effect
Abstract

International audience; Fryns syndrome (FS) is a multiple malformations syndrome with major features of congenital diaphragmatic hernia, pulmonary hypoplasia, craniofacial dysmorphic features, distal digit hypoplasia, and a range of other lower frequency malformations. FS is typically lethal in the fetal or neonatal period. Inheritance is presumed autosomal recessive. Although no major genetic cause has been identified for FS, biallelic truncating variants in PIGN, encoding a component of the glycosylphosphatidylinositol (GPI)-anchor biosynthesis pathway, have been identified in a limited number of cases with a phenotype compatible with FS. Biallelic variants in PIGN, typically missense or compound missense with truncating, also cause multiple congenital anomalies-hypotonia-seizures syndrome 1 (MCAHS1). Here we report six further patients with FS with or without congenital diaphragmatic hernia and recessive loss of function PIGN alleles, including an intragenic deletion with a likely founder effect in La Réunion and other Indian Ocean islands. Our results support the hypothesis that a spectrum of phenotypic severity is associated with recessive PIGN variants, ranging from FS at the extreme end, caused by complete loss of function, to MCAHS1, in which some residual PIGN function may remain. Our data add FS resulting from PIGN variants to the catalog of inherited GPI deficiencies caused by the disruption of the GPI-anchor biosynthesis pathway.

Published
2018

7. ZORRO: Z Omphaloplasty Repair for Omphalocele

Author

Laurent Gavage, Pauline Clermidi, Fernanda Frade, Jean-Luc Michel, Rani Kassir, Luke Harper, Frédérique Sauvat, and Duksha Ramful

Subjects
0301 basic medicine, Novel technique, Male, Umbilicus (mollusc), Umbilical cord, Surgical Flaps, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Wound Healing, Omphalocele, Umbilicus, business.industry, Umbilicoplasty, Infant, Newborn, Gestational age, General Medicine, Anatomy, Plastic Surgery Procedures, medicine.disease, 030104 developmental biology, medicine.anatomical_structure, Treatment Outcome, Normal position, 030220 oncology & carcinogenesis, Pediatrics, Perinatology and Child Health, Surgery, Female, business, Hernia, Umbilical
Abstract

Purpose To suggest a novel technique for omphalocele closure which uses the circular base of the umbilical cord, thus allowing for a more physiological healing process and natural-looking scar. Methods Among 16 neonates operated for omphalocele between 2011 and 2016, 12 were closed with a one-stage procedure using a Z omphaloplasty (ZORRO). Median gestational age was 36.5 weeks; median birth weight was 3210 g. The umbilical arteries were divided and ligated outside the peritoneal cavity above the parietal musculocutaneous plane. The upper part of the defect was closed vertically in the midline, while the lower part was closed in a circular fashion by imbricating 2 lateral cutaneous Z flaps thus forming a new cordonal base. Results The postoperative course was uneventful in all cases. The reconstructed cordonal bases healed as would a normal umbilical cord, with central umbilication surrounded by healthy skin. With a median follow-up period of 11 months, the umbilicus was in the normal position, with a 0.6 xyphoumbilical/xyphopubic ratio. Conclusions This technique mimics the natural necrosis mechanism and physiological healing of the umbilicus thus allowing for an esthetic and “natural looking” umbilicus.

Published
2017

8. Low Plasma Protein Levels at Birth Are Associated with Poor Cardiovascular Adaptation and Serious Adverse Outcome in Infants with Gestational Age <32 Weeks: The ProHémie Study

Author

Sylvain Samperiz, Sandrine Daniel, Francesco Bonsante, Pierre-Yves Robillard, Christine Binquet, Karim Jamal-Bey, Silvia Iacobelli, Jean-Bernard Gouyon, Duksha Ramful, Anais Godeluck, Centre d'Études Périnatales de l'Océan Indien ( CEPOI ), Université de la Réunion ( UR ) -Centre Hospitalier Universitaire de La Réunion ( CHU La Réunion ), Service de réanimation néonatologique et pédiatrique [La Réunion], Groupe Hospitalier Sud-CHR La réunion, Centre Hospitalier Universitaire de La Réunion ( CHU La Réunion ), Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques ( CIC-EC ), Université de Bourgogne ( UB ) -Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), CHR La réunion, Centre d'Études Périnatales de l'Océan Indien (CEPOI), Université de La Réunion (UR)-Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques (CIC-EC), and Université de Bourgogne (UB)-Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Male, Pediatrics, Time Factors, Cardiovascular System, Ventricular Function, Left, 0302 clinical medicine, Risk Factors, Odds Ratio, 030212 general & internal medicine, Prospective Studies, Prospective cohort study, [ SDV.MHEP.GEO ] Life Sciences [q-bio]/Human health and pathology/Gynecology and obstetrics, Hypoproteinemia, Spectroscopy, Near-Infrared, Gestational age, Blood Proteins, Prognosis, Adaptation, Physiological, humanities, Pathophysiology, Echocardiography, Doppler, 3. Good health, Neurological outcome, Cardiovascular Diseases, Infant, Extremely Premature, Female, Neonatal intensive care, Infant, Premature, medicine.medical_specialty, Gestational Age, [SDV.MHEP.GEO]Life Sciences [q-bio]/Human health and pathology/Gynecology and obstetrics, 03 medical and health sciences, Very-low-birth-weight infants, 030225 pediatrics, medicine, Humans, Postnatal adaptation, Chi-Square Distribution, business.industry, Albumin, Hemodynamics, Infant, Newborn, Retrospective cohort study, Odds ratio, medicine.disease, body regions, Blood pressure, Logistic Models, Regional Blood Flow, Peripheral circulation, Pediatrics, Perinatology and Child Health, Linear Models, Arterial blood pressure, business, Chi-squared distribution, Biomarkers, Developmental Biology, Renal function
Abstract

Background: Retrospective studies suggest that early hypoproteinemia has prognostic value for adverse outcome in preemies, but the underlying pathophysiology is unknown. We hypothesized that the prognostic relevance of hypoproteinemia could be related to its association with impaired cardiovascular function and organ perfusion during transition. Objectives: To describe the plasma protein status and the measures of cardiovascular function according to the outcome in infants Methods: One hundred and twenty-eight infants were prospectively included from birth to discharge. During the first 24 h of life, we assessed the cardiovascular function and systemic and organ blood flow by Doppler ultrasound, and monitored cerebral and renal regional oxygen saturation (cRSO2, rRSO2) using near-infrared spectroscopy. These measures were analyzed in relationship to hypoproteinemia (total plasma protein level Results: Hypoproteinemia was associated with a higher risk of a severe adverse outcome after adjustment of confounding variables (adjusted OR = 6.8; 95% CI 1.3-34). Compared to normoproteinemic infants and after adjustment for gestational age, hypoproteinemic ones had more significantly: hypotension (7 vs. 13%, p = 0.03), abnormal capillary refilling time (20 vs. 36%, p < 0.001), abnormal renal blood flow (resistive index 0.78 ± 0.11 vs. 0.85 ± 0.09, p = 0.04), lower rRSO2 (82.9 ± 9.2 vs. 73.6 ± 10.5%, p = 0.04), and lower systemic vascular resistance (0.155 ± 0.058 vs. 0.108 ± 0.037 mm Hg/L/kg; p = 0.04). The cRSO2 patterns were significantly decreased in infants with severe adverse outcome and independent from protein status. Conclusion: Hypoproteinemia is associated with impaired cardiovascular function. Further studies are required to elucidate the interplay between changes in protein levels, postnatal hemodynamics and clinical outcome.

Published
2017

9. Management of neonatal spontaneous intestinal perforation by peritoneal needle aspiration

Author

Duksha Ramful, Gébus M, Harper L, Sylvain Samperiz, Alessandri Jl, and Michel Jl

Subjects
Male, medicine.medical_specialty, medicine.medical_treatment, Birth weight, Gestational Age, Infant, Premature, Diseases, Enteral administration, 03 medical and health sciences, 0302 clinical medicine, Enteral Nutrition, Cholestasis, 030225 pediatrics, Intestinal Stricture, Laparotomy, Intensive Care Units, Neonatal, medicine, Spontaneous Intestinal Perforation, Birth Weight, Humans, 030212 general & internal medicine, Retrospective Studies, business.industry, Infant, Newborn, Obstetrics and Gynecology, Gestational age, Retrospective cohort study, medicine.disease, Surgery, Infant, Extremely Low Birth Weight, Intestinal Perforation, Needles, Infant, Extremely Premature, Pediatrics, Perinatology and Child Health, Drainage, Female, France, business
Abstract

To describe conservative management of spontaneous intestinal perforation (SIP) in preterm infants using peritoneal needle aspiration (PNA). Monocentric retrospective review of SIP cases treated primarily by PNA between 1999 and 2015 (n=31). Mean gestational age was 29.2±2.4 weeks and birthweight 1149±428 g. SIP occurred at 3.7±2.2 days of life. PNA achieved definitive treatment in 18 patients (60%) with a mean of 1.8 (±0.8) procedures. All patients requiring more than three PNAs had secondary laparotomy. Two patients died and five presented severe cerebral lesions. Full enteral feeding was achieved 42±18 days after SIP. Intestinal morbidity included cholestasis (n=6), intestinal stricture (n=1) and growth restriction (n=22). On follow-up (n=25, median=4 years), no severe impairment was noted. Seventeen children (68%) had a normal development. PNA as primary therapy for SIP is a viable option, resulting in definitive treatment in 60% of cases, with limited mortality and morbidity.

Published
2017

10. Antenatal presentation of hereditary lymphedema type I

Author

Pascal Brouillard, J. L. Alessandri, E. Boudon, T. Abossolo, Yael Levy, Duksha Ramful, Miikka Vikkula, M. Kieffer-Traversier, and François Cartault

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Hydrops Fetalis, Mutation, Missense, Prenatal diagnosis, Ultrasonography, Prenatal, Pregnancy, Hydrops fetalis, Chylous ascites, Genetics, medicine, Humans, Primary lymphedema, Lymphedema, Genetics (clinical), business.industry, Milroy's disease, Infant, Newborn, Chylothorax, General Medicine, Vascular Endothelial Growth Factor Receptor-3, medicine.disease, Dysplasia, Etiology, Female, business
Abstract

Fetal edema can present as limited subcutaneous edema, fluid accumulation in body cavities or hydrops fetalis. Hydrops fetalis is the end stage of a variety of fetal/maternal disorders and nonimmune etiology represents more than 3/4 of cases. Lymphatic dysplasia may account for a subset of patients with nonimmune and "idiopathic" hydrops fetalis, fetal chylous ascites or chylothorax. We present two unrelated patients with antenatal features of hereditary lymphedema syndrome, in whom Milroy disease was diagnosed after birth. At least, 20 genes have been identified to cause primary lymphedema, with sometimes antenatal features. Hereditary lymphedema syndrome should be considered in cases of nonimmune hydrops fetalis/fetal edema after ruling out the more common etiologies.

Published
2015

11. Effect of early low-dose hydrocortisone on survival without bronchopulmonary dysplasia in extremely preterm infants (PREMILOC): a double-blind, placebo-controlled, multicentre, randomised trial

Author

Olivier, Baud, Laure, Maury, Florence, Lebail, Duksha, Ramful, Fatima, El Moussawi, Claire, Nicaise, Véronique, Zupan-Simunek, Anne, Coursol, Alain, Beuchée, Pascal, Bolot, Pierre, Andrini, Damir, Mohamed, Corinne, Alberti, Annick, Tibi, Réanimation et Pédiatrie Néonatales, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), PremUp Foundation, Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Sorbonne Universités-Université Paris Descartes - Paris 5 ( UPD5 ) -CHI Créteil-Institut de Recherche pour le Développement ( IRD ) -Université Paris-Sud - Paris 11 ( UP11 ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Université Paris Diderot - Paris 7 ( UPD7 ), Physiopathologie, conséquences fonctionnelles et neuroprotection des atteintes du cerveau en développement, Université Paris Diderot - Paris 7 ( UPD7 ) -IFR2-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Hôpital de Corbeil-ESsonnes, Centre Hospitalier Universitaire de La Réunion ( CHU La Réunion ), Centre Hospitalier de Poissy Saint-Germain (CHIPS), centre hospitalier universitaire, hôpital Nord, Hôpital Antoine Béclère, Université Paris-Sud - Paris 11 ( UP11 ) -Assistance publique - Hôpitaux de Paris (AP-HP), Médecine Néonatale et Réanimation Pédiatrique, CH René Dubos, Centre Hospitalier Universitaire de Rennes, Néonatalogie, Centre Hospitalier de Saint-Denis, CHU Grenoble, Université Joseph Fourier - Grenoble 1 ( UJF ) -CHU Grenoble, Epidémiologie Clinique et Evaluation Economique Appliquées aux Populations Vulnérables ( Inserm U1123 - ECEVE ), Université Paris Diderot - Paris 7 ( UPD7 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Institut de Recherche pour le Développement (IRD)-Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-CHI Créteil-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris Diderot - Paris 7 (UPD7)-IFR2-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre Hospitalier Universitaire [Rennes], Centre Hospitalier de Saint-Denis [Ile-de-France], Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, Epidémiologie Clinique et Evaluation Economique Appliquées aux Populations Vulnérables (ECEVE (U1123 / UMR_S_1123)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Descartes - Paris 5 (UPD5)-CHI Créteil-Institut de Recherche pour le Développement (IRD)-Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7), Institut National de la Santé et de la Recherche Médicale (INSERM)-AP-HP Hôpital universitaire Robert-Debré [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Universités-Université Paris Descartes - Paris 5 (UPD5)-CHI Créteil-Institut de Recherche pour le Développement (IRD)-Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7), Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), and Institut National de la Santé et de la Recherche Médicale (INSERM)-AP-HP Hôpital universitaire Robert-Debré [Paris]-Université Paris Diderot - Paris 7 (UPD7)

Subjects
Male, Pediatrics, medicine.medical_specialty, Hydrocortisone, [SDV]Life Sciences [q-bio], Anti-Inflammatory Agents/administration & dosage, Anti-Inflammatory Agents, Placebo, Hydrocortisone/administration & dosage/analogs & derivatives, law.invention, 03 medical and health sciences, 0302 clinical medicine, Randomized controlled trial, Double-Blind Method, law, 030225 pediatrics, Intensive care, Medicine, Humans, 030212 general & internal medicine, Bronchopulmonary Dysplasia/prevention & control, Bronchopulmonary Dysplasia, Intention-to-treat analysis, ddc:618, [ SDV ] Life Sciences [q-bio], business.industry, Postmenstrual Age, Infant, Newborn, Gestational age, General Medicine, medicine.disease, 3. Good health, Logistic Models, Treatment Outcome, Bronchopulmonary dysplasia, Infant, Extremely Premature, Gestation, Female, France, business
Abstract

International audience; SummaryBackground Bronchopulmonary dysplasia, a major complication of extreme prematurity, has few treatment options. Postnatal steroid use is controversial, but low-dose hydrocortisone might prevent the harmful effects of inflammation on the developing lung. In this study, we aimed to assess whether low-dose hydrocortisone improved survival without bronchopulmonary dysplasia in extremely preterm infants. Methods In this double-blind, placebo-controlled, randomised trial done at 21 French tertiary-care neonatal intensive care units (NICUs), we randomly assigned (1:1), via a secure study website, extremely preterm infants inborn (born in a maternity ward at the same site as the NICU) at less than 28 weeks of gestation to receive either intravenous low-dose hydrocortisone or placebo during the first 10 postnatal days. Infants randomly assigned to the hydrocortisone group received 1 mg/kg of hydrocortisone hemisuccinate per day divided into two doses per day for 7 days, followed by one dose of 0·5 mg/kg per day for 3 days. Randomisation was stratified by gestational age and all infants were enrolled by 24 h after birth. Study investigators, parents, and patients were masked to treatment allocation. The primary outcome was survival without bronchopulmonary dysplasia at 36 weeks of postmenstrual age. We used a sequential analytical design, based on intention to treat, to avoid prolonging the trial after either efficacy or futility had been established. This trial is registered with ClinicalTrial.gov, number NCT00623740. Findings 1072 neonates were screened between May 25, 2008, and Jan 31, 2014, of which 523 were randomly assigned (256 hydrocortisone, 267 placebo). 255 infants on hydrocortisone and 266 on placebo were included in analyses after parents withdrew consent for one child in each group. Of the 255 infants assigned to hydrocortisone, 153 (60%) survived without bronchopulmonary dysplasia, compared with 136 (51%) of 266 infants assigned to placebo (odds ratio [OR] adjusted for gestational age group and interim analyses 1·48, 95% CI 1·02–2·16, p=0·04). The number of patients needed to treat to gain one bronchopulmonary dysplasia-free survival was 12 (95% CI 6–200). Sepsis rate was not significantly different in the study population as a whole, but subgroup analyses showed a higher rate only in infants born at 24–25 weeks gestational age who were treated with hydrocortisone (30 [40%] of 83 vs 21 [23%] of 90 infants; sub-hazard ratio 1·87, 95% CI 1·09–3·21, p=0·02). Other potential adverse events, including notably gastrointestinal perforation, did not differ significantly between groups. Interpretation In extremely preterm infants, the rate of survival without bronchopulmonary dysplasia at 36 weeks of postmenstrual age was significantly increased by prophylactic low-dose hydrocortisone. This strategy, based on a physiological rationale, could lead to substantial improvements in the management of the most premature neonates. Funding Assistance Publique-Hôpitaux de Paris

Published
2016

12. Biological Impact of Recent Guidelines on Parenteral Nutrition in Preterm Infants

Author

Farid Boubred, Alain Beuchée, Duksha Ramful, Géraldine Gascoin, Olivier Baud, Elodie Zana-Taïeb, Pierre Tourneux, Isabelle Guellec, CHU Trousseau [APHP], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Laboratoire Traitement du Signal et de l'Image (LTSI), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire [Rennes], Néonatalogie, Division of Neonatology, La Conception Hospital, Assistance Publique-Hôpitaux de Marseille and Faculté de Médecine, Université de la Méditerranée - Aix-Marseille 2, Neonatal Unit and Intensive Care, Pédiatrie, CHU Amiens, Université de Maurice, CHU Cochin [AP-HP], Réanimation et Pédiatrie Néonatales, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), PremUp Foundation, Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Universités-Université Paris Descartes - Paris 5 (UPD5)-CHI Créteil-Institut de Recherche pour le Développement (IRD)-Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7), Physiopathologie, conséquences fonctionnelles et neuroprotection des atteintes du cerveau en développement, Université Paris Diderot - Paris 7 (UPD7)-IFR2-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Descartes - Paris 5 (UPD5)-CHI Créteil-Institut de Recherche pour le Développement (IRD)-Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7), Université de Rennes (UR)-Institut National de la Santé et de la Recherche Médicale (INSERM), Institut de Recherche pour le Développement (IRD)-Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Diderot - Paris 7 (UPD7)-CHI Créteil-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Angers, Laboratoire Traitement du Signal et de l'Image ( LTSI ), Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Centre Hospitalier Universitaire de Rennes, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 ( UPD7 ), Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Sorbonne Universités-Université Paris Descartes - Paris 5 ( UPD5 ) -CHI Créteil-Institut de Recherche pour le Développement ( IRD ) -Université Paris-Sud - Paris 11 ( UP11 ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Université Paris Diderot - Paris 7 ( UPD7 ), and Université Paris Diderot - Paris 7 ( UPD7 ) -IFR2-Institut National de la Santé et de la Recherche Médicale ( INSERM )

Subjects
Parenteral Nutrition, Pediatrics, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Renal function, 03 medical and health sciences, 0302 clinical medicine, 030225 pediatrics, Hyperchloremic acidosis, medicine, Homeostasis, Humans, 030212 general & internal medicine, Amino Acids, Adverse effect, ComputingMilieux_MISCELLANEOUS, Acidosis, [ SDV ] Life Sciences [q-bio], business.industry, Infant, Newborn, Gastroenterology, Phosphorus, Metabolic acidosis, medicine.disease, 3. Good health, Parenteral nutrition, Pediatrics, Perinatology and Child Health, Dietary Proteins, medicine.symptom, business, Infant, Premature, Hypophosphatemia
Abstract

International audience; Objectives - Recent guidelines for preterm neonates recommend early initiation of parenteral nutrition (PN) with high protein and relatively high caloric intake. This review considers whether these changes could influence homeostasis in very preterm infants during the first few postnatal weeks. Methods - This systematic review of relevant literature from searches of PubMed and recent guidelines was reviewed by investigators from several perinatal centers in France. Results - New recommendations for PN could be associated with metabolic acidosis via the increase in the amino acid ion gap, hyperchloremic acidosis, and ammonia acidosis. The introduction of high-intake amino acids soon after birth could induce hypophosphatemia and hypercalcemia, simulating a "repeat feeding-like syndrome" and could be prevented by the early intake of phosphorus, especially in preterm infants born after fetal growth restriction. Early high-dose amino acid infusions are relatively well tolerated in the preterm infant with regard to renal function. Additional studies, however, are warranted to determine markers of protein intolerance and to specify the optimal composition and amount of amino acid solutions. Conclusions - Optimal PN following new guidelines in very preterm infants, despite their demonstrated benefits on growth, may induce adverse effects on ionic homeostasis. Clinicians should implement appropriate monitoring to prevent and/or correct them.

Published
2015

13. Emergence and clinical insights into the pathology of Chikungunya virus infection

Author

Jean Jacques Hoarau, Stéphanie Robin, Anne Ribera, Duksha Ramful, Marie Christine Jaffar-Bandjee, Bernard Alex Gaüzère, Philippe Gasque, Jimmy Selambarom, Pascale Krejbich-Trotot, Groupe de Recherche en Immunopathologies et maladies infectueuses (GRI), Université de La Réunion (UR)-Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Laboratoire de Chimie des Substances Naturelles et des Sciences des Aliments (LCSNSA), Université de La Réunion (UR), and Laboratoire de Biochimie et Génétique Moléculaire (LBGM)

Subjects
Male, Microbiology (medical), Pathology, medicine.medical_specialty, [SDV]Life Sciences [q-bio], Disease, Global Health, medicine.disease_cause, Communicable Diseases, Emerging, Microbiology, Aedes, Pregnancy, Virology, Global health, medicine, Animals, Humans, Chikungunya, Pregnancy Complications, Infectious, biology, Alphavirus Infections, business.industry, Transmission (medicine), Vaccination, virus diseases, Tropical disease, Viral Vaccines, biology.organism_classification, medicine.disease, Rash, Insect Vectors, 3. Good health, Infectious Diseases, Insect Repellents, Encephalitis, Female, medicine.symptom, business, Chikungunya virus
Abstract

International audience; Major epidemics of Chikungunya have re-emerged with millions of cases worldwide. What was once largely a tropical disease in poorer countries is now recognized as a major global health issue. The disease is perpetuated by the alphavirus Chikungunya, and is transmitted by Aedes mosquitoes. The infection is highly symptomatic, with fever, skin rash and incapacitating arthralgia, which can evolve to chronic arthritis and rheumatism in elderly patients. Mother-to-child transmission, encephalitis, Guillain–Barré syndrome and deaths have been noted. In this article, we will highlight the epidemiological, clinical, virological and immunological aspects of the disease and mention the therapies that have been used during recent epidemics. Novel prevention measures to control the mosquito and a new vaccine are highly warranted.

Published
2010

14. Renal cystic dysplasia, paucity of bile ducts, situs inversus, bowing of the femora in two siblings in the Reunion Island: a ciliopathy?

Author

Claire Brayer, Jean-Luc Alessandri, Duksha Ramful, Jean-Pierre Rivière, Marie-Claire Gubler, François Cartault, and Fabrice Cuillier

Subjects
Adult, Male, Fatal outcome, DNA Mutational Analysis, Kinesins, Pathology and Forensic Medicine, Fatal Outcome, Pregnancy, Humans, Medicine, Cilia, Femur, Genetics (clinical), business.industry, Siblings, General Medicine, Anatomy, Kidney Diseases, Cystic, Situs Inversus, medicine.disease, Renal cystic dysplasia, Radiography, Ciliopathy, Situs inversus, Pediatrics, Perinatology and Child Health, Female, Bile Ducts, Bone Diseases, business, Reunion
Published
2009

15. Neurocognitive Outcome of Children Exposed to Perinatal Mother-to-Child Chikungunya Virus Infection: The CHIMERE Cohort Study on Reunion Island

Author

Gilles Beullier, Liliane Cotte, Jean-Luc Alessandri, Brahim Boumahni, Isabelle Tiran-Rajaoefera, Alain Fourmaintraux, Irénée Boya, Olivier Rollot, Patrick Gérardin, Magali Carbonnier, Monique Kaminski, Sylvain Samperiz, Marie-Christine Jaffar-Bandjee, Xavier Fritel, Duksha Ramful, Tahir Noormahomed, Alain Michault, François Favier, Jocelyn Okoï, Marc Bintner, Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), CIC régional épidémiologie clinique/essais cliniques - Ile de la Réunion (CIC-EC), Institut National de la Santé et de la Recherche Médicale (INSERM), Recherche Epidémiologique en Santé Périnatale et Santé des Femmes et des Enfants (UMR_S 953), Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Immunopathology and Infection Research Grouping (IRG), Université de La Réunion (UR)-Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Centre Hospitalier Gabriel Martin, Centre hospitalier de l'Est Réunion, Saint-Benoît, Clinique Sainte Clotilde, CAMSP La Rose, Clinique Durieux, Le Tampon, La Réunion, Centre hospitalier universitaire de Poitiers (CHU Poitiers), INSERM CIC 0802 (INSERM - CHU de Poitiers), Université de Poitiers-Centre hospitalier universitaire de Poitiers (CHU Poitiers)-Institut National de la Santé et de la Recherche Médicale (INSERM), HAL UPMC, Gestionnaire, Recherche Epidémiologique en Santé Périnatale et Santé des Femmes et des Enfants, Institut National de la Santé et de la Recherche Médicale (INSERM) - Assistance publique - Hôpitaux de Paris (AP-HP) - CHU Tenon [APHP] - CHU Cochin [APHP] - Université Paris-Sud - Paris 11 (UP11) - Université Paris Descartes - Paris 5 (UPD5) - Université Pierre et Marie Curie - Paris 6 (UPMC), Service de gynécologie et obstétrique [Poitiers], Université de Poitiers - CHU de Poitiers, CHU de Poitiers, Groupe de Recherche en Immunopathologies et maladies infectueuses (GRI), Université de la Réunion - Centre hospitalier Régional Site Félix Guyon, Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Descartes - Paris 5 (UPD5)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris-Sud - Paris 11 (UP11), Centre Hospitalier Universitaire de La Réunion ( CHU La Réunion ), CIC régional épidémiologie clinique/essais cliniques - Ile de la Réunion ( CIC-EC ), Institut National de la Santé et de la Recherche Médicale ( INSERM ), Recherche Epidémiologique en Santé Périnatale et Santé des Femmes et des Enfants ( UMR_S 953 ), Université Paris-Sud - Paris 11 ( UP11 ) -Université Pierre et Marie Curie - Paris 6 ( UPMC ) -Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Immunopathology and Infection Research Grouping ( IRG ), Université de la Réunion ( UR ) -Centre hospitalier Régional Site Félix Guyon, Centre Hospitalier Gabriel-Martin, CH Gabriel-Martin, Clinique Sainte-Clotilde, Centre hospitalier universitaire de Poitiers ( CHU Poitiers ), and INSERM CIC 0802 ( INSERM CIC 0802, CHU de Poitiers )

Subjects
Pediatrics, Viral Diseases, Epidemiology, Developmental Disabilities, [ SDV.MHEP.PED ] Life Sciences [q-bio]/Human health and pathology/Pediatrics, Developmental and Pediatric Neurology, Rate ratio, Diagnostic Radiology, [ SDV.NEU.SC ] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Cognitive Sciences, Child Development, Infectious Diseases of the Nervous System, Pregnancy, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Emerging Viral Diseases, Medicine and Health Sciences, Public and Occupational Health, Pregnancy Complications, Infectious, Pediatric Epidemiology, Cognitive Neurology, lcsh:Public aspects of medicine, Radiology and Imaging, Child Health, Gestational age, [SDV.NEU.SC]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Cognitive Sciences, Magnetic Resonance Imaging, 3. Good health, [SDV] Life Sciences [q-bio], [ SDV.MHEP.MI ] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Infectious Diseases, Neurology, Arboviral Infections, Child, Preschool, [SDV.MHEP.MI] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Female, Behavioral and Social Aspects of Health, Chikungunya virus, Cohort study, Research Article, Neglected Tropical Diseases, medicine.medical_specialty, Pediatric Critical Care, lcsh:Arctic medicine. Tropical medicine, lcsh:RC955-962, Cognitive Neuroscience, Neuroimaging, Microbiology, Infectious Disease Epidemiology, Cerebral palsy, [SDV.MHEP.PED] Life Sciences [q-bio]/Human health and pathology/Pediatrics, Developmental Neuroscience, Diagnostic Medicine, Virology, medicine, Humans, Retrospective Studies, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, Neonatal encephalopathy, business.industry, Public Health, Environmental and Occupational Health, Infant, Newborn, Biology and Life Sciences, Infant, Retrospective cohort study, lcsh:RA1-1270, medicine.disease, Tropical Diseases, Infectious Disease Transmission, Vertical, Health Care, Small for gestational age, Chikungunya Fever, Neonatology, business, Neurocognitive, Reunion, Viral Transmission and Infection, [SDV.NEU.SC] Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Cognitive Sciences, Neuroscience
Abstract

Background Little is known about the neurocognitive outcome in children exposed to perinatal mother-to-child Chikungunya virus (p-CHIKV) infection. Methods The CHIMERE ambispective cohort study compared the neurocognitive function of 33 p-CHIKV-infected children (all but one enrolled retrospectively) at around two years of age with 135 uninfected peers (all enrolled prospectively). Psychomotor development was assessed using the revised Brunet-Lezine scale, examiners blinded to infectious status. Development quotients (DQ) with subscores covering movement/posture, coordination, language, sociability skills were calculated. Predictors of global neurodevelopmental delay (GND, DQ≤85), were investigated using multivariate Poisson regression modeling. Neuroradiologic follow-up using magnetic resonance imaging (MRI) scans was proposed for most of the children with severe forms. Results The mean DQ score was 86.3 (95%CI: 81.0–91.5) in infected children compared to 100.2 (95%CI: 98.0–102.5) in uninfected peers (P, Author Summary Chikungunya virus (CHIKV), an alphaviral infection transmitted by day-biting Aedes mosquitoes, is widespread in Asia and in Africa. Usually, CHIKV causes a self-limiting arthritide, except in debilitated people and in neonates, for whom it can lead to severe disease. Mother-to-child perinatal transmission of CHIKV is a rare event that can occur in the setting of large-scale outbreaks when the risk of maternal viremia at the term of pregnancy becomes non-negligible. In that event, CHIKV can give rise to neonatal infection with a probability of 50%, and prostration and encephalopathy, the two leading clinical pictures in the neonate, represent a continuum in the gradation of an undiscovered central nervous system involvement. We have followed-up 33 children infected at birth between June 2005 and April 2006 on the island of La Réunion, Indian Ocean, and 135 uninfected controls, for assessing their neurodevelopmental performance around the age of two years. Fifty-one percent of infected children had a global neurodevelopmental delay compared to 15% of uninfected peers. Multivariate analysis and neuroradiology suggest without irrefutable evidence a causal relationship between CHIKV infection and neurocognitive outcomes. Our findings suggest that CHIKV infection, acquired in the perinatal period, can cause severe disease with lifelong expected disability.

Published
2014

16. Fryns syndrome without diaphragmatic hernia, DOOR syndrome or Fryns-like syndrome? Report on patients from Indian Ocean islands

Author

Céline Laurain, Fabrice Cuillier, Duksha Ramful, Laure Jacquemot-Dekkak, Sylvain Samperiz, Marie Kieffer-Traversier, Valérie Malan, Claire Brayer, Jean-Luc Alessandri, Maeva Grondard, Florence Pierre, Isabelle Tiran-Rajaofera, Service de Réanimation Néonatale et Pédiatrique, CHD Félix Guyon, Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Génétique et épigénétique des maladies métaboliques, neurosensorielles et du développement (Inserm U781), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de La Réunion ( CHU La Réunion ), Génétique et épigénétique des maladies métaboliques, neurosensorielles et du développement ( Inserm U781 ), and Université Paris Descartes - Paris 5 ( UPD5 ) -Institut National de la Santé et de la Recherche Médicale ( INSERM )

Subjects
Male, Pediatrics, medicine.medical_specialty, Polyhydramnios, Hernia, Hearing Loss, Sensorineural, Rudiger Syndrome, [SDV]Life Sciences [q-bio], Limb Deformities, Congenital, Nails, Malformed, Sensorineural, Craniofacial Abnormalities, Congenital, Fatal Outcome, Fryns syndrome, DOOR syndrome, Indian Ocean Islands, Intellectual Disability, Genetics, medicine, Humans, Diaphragmatic hernia, Hearing Loss, Genetics (clinical), Hernias, Hernia, Diaphragmatic, Comparative Genomic Hybridization, [ SDV ] Life Sciences [q-bio], business.industry, Congenital diaphragmatic hernia, Facies, Infant, Malformed, Microdeletion syndrome, Hand Deformities, medicine.disease, Hypoplasia, Limb Deformities, Phenotype, Nails, Female, Hernias, Diaphragmatic, Congenital, business, Hand Deformities, Congenital, Diaphragmatic
Abstract

International audience; We report on six patients (five unpublished patients) from the Indian Ocean islands, with coarse face, cleft lip or palate, eye anomalies, brachytelephalangy, nail hypoplasia, various malformations (genitourinary or cerebral), abnormal electroencephalograms with impaired neurological examination and lethal outcome. Massive polyhydramnios was noted in the third trimester of pregnancy and neonatal growth was normal or excessive. The combination of the features is consistent with the diagnosis of Fryns syndrome (FS) without congenital diaphragmatic hernia. Besides chromosomal aberrations and microdeletion syndrome, differential diagnoses include conditions overlapping with FS such as Simpson-Golabi-Behmel, and conditions with hypoplasia/absence of the distal phalanges such as DOOR syndrome, Schinzel-Giedion syndrome, and Rudiger syndrome.

Published
2014

17. Antibody kinetics in infants exposed to Chikungunya virus infection during pregnancy reveals absence of congenital infection

Author

Brahim Boumahni, Sylvain Samperiz, Alain Michault, Marie-Christine Jaffar-Bandjee, Patrick Gérardin, Xavier Fritel, Duksha Ramful, Olivier Rollot, Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), CIC régional épidémiologie clinique/essais cliniques - Ile de la Réunion (CIC-EC), Institut National de la Santé et de la Recherche Médicale (INSERM), Groupe de Recherche en Immunopathologies et maladies infectueuses (GRI), Université de La Réunion (UR)-Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Service de gynécologie et obstétrique [Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Inserm U1018, CIC0802, Centre hospitalier universitaire de Poitiers (CHU Poitiers)-Centre hospitalier universitaire de Poitiers (CHU Poitiers)-Centre de recherche en épidémiologie et santé des populations (CESP), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Poitiers - Faculté de Médecine et de Pharmacie, Université de Poitiers-Université de Poitiers-CIC - Poitiers, Université de Poitiers-Centre hospitalier universitaire de Poitiers (CHU Poitiers)-Direction Générale de l'Organisation des Soins (DGOS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Direction Générale de l'Organisation des Soins (DGOS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Laboratoire de Bactériologie-Parasitologie-Virologie-Hygiène, Groupe Hospitalier Sud Réunion (GHSR), Microbiology Laboratory, Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Réanimation médicale néonatale et pédiatrique, CHR la Reunion site Sud, INSERM CIC 0802 (INSERM - CHU de Poitiers), Université de Poitiers-Centre hospitalier universitaire de Poitiers (CHU Poitiers)-Institut National de la Santé et de la Recherche Médicale (INSERM), Recherche Epidémiologique en Santé Périnatale et Santé des Femmes et des Enfants (UMR_S 953), and Université Paris-Sud - Paris 11 (UP11)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Adult, Infectious Disease Transmission, [SDV]Life Sciences [q-bio], 030231 tropical medicine, Antibodies, Viral, medicine.disease_cause, Asymptomatic, Immunoglobulin G, Antibodies, Serology, 03 medical and health sciences, 0302 clinical medicine, Pregnancy, Immunology and Allergy, Medicine, Humans, Vertical, Chikungunya, Viral, Pregnancy Complications, Infectious, Alphavirus infection, 0303 health sciences, biology, 030306 microbiology, business.industry, Alphavirus Infections, Infant, Newborn, Infectious, virus diseases, Infant, medicine.disease, Newborn, Virology, Infectious Disease Transmission, Vertical, 3. Good health, Pregnancy Complications, Infectious Diseases, Immunization, Immunoglobulin M, Immunology, biology.protein, Female, medicine.symptom, business, Chikungunya virus
Abstract

International audience; To search for serological evidence of congenital infection in apparently healthy neonates born to women infected with the Chikungunya virus (CHIKV) during pregnancy, monitoring for CHIKV-specific antibodies was performed within the CHIMERE cohort study (Reunion island, 2006-2008). CHIKV-specific antibody kinetics showed no evidence of asymptomatic congenital infection as neonates were tested negative for CHIKV-specific IgM antibodies at birth and 368 infants with CHIKV-specific IgG antibodies seroreversed completely (mean seroreversion time: 7.7 months). Seroreversion time of transplacental CHIKV IgG antibodies was inversely correlated with the stage of pregnancy at which exposure took place and end-term small for gestational infants seroreversed earlier.

Published
2014

18. Tibial developmental field defect in valproic acid embryopathy: Report on three cases

Author

C Le Caignec, Bertrand Isidor, J.-L. Alessandri, Anthony S. David, J.M. Laville, Dominique Martin-Coignard, Duksha Ramful, and J. Ghazouani

Subjects
musculoskeletal diseases, Adult, Male, medicine.medical_specialty, Adolescent, Limb Deformities, Congenital, Pregnancy, Internal medicine, Genetics, medicine, Humans, Femur, Tibia, Child, Genetics (clinical), Valproic Acid, Comparative Genomic Hybridization, Tibial agenesis, business.industry, Infant, Newborn, Infant, Aplasia, musculoskeletal system, medicine.disease, VACTERL association, Surgery, Radiography, Fetal Diseases, Endocrinology, Agenesis, Child, Preschool, Prenatal Exposure Delayed Effects, Female, Valproic acid embryopathy, business, medicine.drug
Abstract

Prenatal exposure to valproic acid (VA) is associated with an increased risk of congenital malformations, especially limb defects like radial ray defects. Tibial developmental field defect in VA embryopathy remains exceptional. We report on three patients presenting with tibial hypo/aplasia associated with either femoral bifurcation or radial ray defect following prenatal exposure to VA. Femoral bifurcation and tibial agenesis has been described in the Gollop–Wolfgang complex and in the tibial agenesis-ectrodactyly syndrome. Tibial agenesis has also been reported in VACTERL association. The relation between prenatal exposure to VA and tibial agenesis is discussed. © 2010 Wiley-Liss, Inc.

Published
2010

19. One-step management of apple-peel atresia

Author

A. Maurel, Duksha Ramful, S. De Napoli-Cocci, M.B. Aulagne, Luke Harper, J.L. Michel, and S. Mazouzi

Subjects
Male, medicine.medical_specialty, business.industry, Primary anastomosis, Infant, Newborn, Intestinal Atresia, Retrospective cohort study, General Medicine, Short bowel syndrome, medicine.disease, Surgery, Jejunal atresia, Parenteral nutrition, Apple peel atresia, Atresia, Medicine, Humans, Female, Parenteral Nutrition, Total, business, Survival rate, Digestive System Surgical Procedures, Retrospective Studies
Abstract

Purpose: Apple-peel atresia is a rare form of bowel atresia associating proximal jejunal atresia with a typical coil-shaped distal small bowel. Many of the children suffering from this condition are still managed with initial enterostomy followed by delayed anastomosis.Patients and method: Between 2000 and 2007, we managed four patients with apple-peel atresia. Three by primary anastomosis and one by initial enterostomy with delayed anastomosis.Results: Total duration of total parenteral nutrition (TPN) was an average 109 days. Survival rate was 100% with an average follow-up of 4.3 years.Conclusion: Nowadays, the prognosis of apple-peel atresia depends mainly on prolonged TPN-related morbidity rather than immediate postoperative complications. We therefore recommend that those infants affected be managed by primary anastomosis in order to reduce the duration of parenteral nutrition, followed by careful nutrition by teams experienced in neonatal TPN and short bowel syndrome.

Published
2010

20. Binder phenotype and brachytelephalangic chondrodysplasia punctata secondary to maternal vitamin K deficiency

Author

Duksha Ramful, Fabrice Cuillier, Jean-Luc Alessandri, Service de Réanimation Néonatale et Pédiatrique, CHD Félix Guyon, Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Groupe de Recherche en Immunopathologies et maladies infectueuses (GRI), and Université de La Réunion (UR)-Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion]

Subjects
medicine.medical_specialty, Chondrodysplasia Punctata, [SDV]Life Sciences [q-bio], Pathology and Forensic Medicine, 03 medical and health sciences, Congenital, Young Adult, Pregnancy, Internal medicine, Vitamin K deficiency, Medicine, Humans, Chondrodysplasia punctata, Brachytelephalangic Chondrodysplasia Punctata, Child, Genetics (clinical), ComputingMilieux_MISCELLANEOUS, 030304 developmental biology, 0303 health sciences, business.industry, 030305 genetics & heredity, Infant, Newborn, Infant, General Medicine, Hand Deformities, medicine.disease, Newborn, Phenotype, 3. Good health, Radiography, Pregnancy Complications, Endocrinology, K deficiency, Pediatrics, Perinatology and Child Health, Female, Vitamin K Deficiency, Anatomy, business, Hand Deformities, Congenital, Maternal vitamin
Abstract

International audience; no abstract

Published
2010

21. Chikungunya Virus Infection during Pregnancy, Réunion, France, 2006

Author

Patrick Gérardin, Xavier Fritel, Marie-Christine Jaffar-Bandjee, Monique Kaminski, Jacques Bideault, Louis Lagarde, Olivier Rollot, Eric Orvain, Fabrice Cuillier, Sylvain Samperiz, Liliane Cotte, Barbara Dhuime, Alain Michault, Bernard-Alex Gaüzère, Duksha Ramful, Alain Fourmaintraux, Service de gynécologie et obstétrique [Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), CIC régional épidémiologie clinique/essais cliniques - Ile de la Réunion (CIC-EC), Institut National de la Santé et de la Recherche Médicale (INSERM), Réanimation médicale néonatale et pédiatrique, CHR la Reunion site Sud, Groupe de Recherche en Immunopathologies et maladies infectueuses (GRI), Université de La Réunion (UR)-Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Centre Hospitalier Intercommunal Saint Benoît- Saint André, CHI de Saint-Benoit-Saint-André, Centre Hospitalier Gabriel Martin, Clinique Sainte Clotilde, Laboratoire de Bactériologie-Parasitologie-Virologie-Hygiène, Groupe Hospitalier Sud Réunion (GHSR), Recherches épidémiologiques en santé périnatale et santé des femmes, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Gabriel-Martin, CH Gabriel-Martin, and Clinique Sainte-Clotilde

Subjects
newborns, Epidemiology, viruses, Infectious Disease Transmission, [SDV]Life Sciences [q-bio], lcsh:Medicine, Antibodies, Viral, medicine.disease_cause, Disease Outbreaks, 0302 clinical medicine, Pregnancy, Vertical, 030212 general & internal medicine, Chikungunya, Viral, Prospective Studies, Pregnancy Complications, Infectious, Réunion, Obstetrics, Transmission (medicine), Reverse Transcriptase Polymerase Chain Reaction, Infectious, Pregnancy Outcome, 3. Good health, Infectious Diseases, Population study, Female, France, Pregnancy Trimesters, Chikungunya virus, Microbiology (medical), medicine.medical_specialty, 030231 tropical medicine, Antibodies, lcsh:Infectious and parasitic diseases, 03 medical and health sciences, medicine, Humans, lcsh:RC109-216, Alphavirus infection, business.industry, Alphavirus Infections, Research, lcsh:R, Infant, Newborn, Outbreak, Infant, medicine.disease, Newborn, Infectious Disease Transmission, Vertical, Pregnancy Complications, mothers, 030221 ophthalmology & optometry, business
Abstract

Except for prenatal hospital admissions for those infected, this virus had no effect on outcomes., Mother-to-child transmission of chikungunya virus was reported during the 2005–2006 outbreak on Réunion Island, France. To determine the effects of this virus on pregnancy outcomes, we conducted a study of pregnant women in Réunion in 2006. The study population was composed of 1,400 pregnant women (628 uninfected, 658 infected during pregnancy, 27 infected before pregnancy, and 87 infected on unknown dates). We compared pregnancy outcomes for 655 (628 + 27) women not infected during pregnancy with 658 who were infected during pregnancy. Infection occurred during the first trimester for 15% of the infected women, the second for 59%, and the third for 26%. Only hospital admission during pregnancy differed between infected and uninfected women (40% vs. 29%). Other outcomes (cesarean deliveries, obstetric hemorrhaging, preterm births, stillbirths after 22 weeks, birthweight, congenital malformations, and newborn admissions) were similar. This virus had no observable effect on pregnancy outcomes.

Published
2010

22. Severe bullous skin lesions associated with Chikungunya virus infection in small infants

Author

Stéphanie Robin, Léon Benhamou, Jacques Marichy, Marie-Christine Jaffar-Bandjee, Khaled Ezzedine, Julie Zettor, Duksha Ramful, Jean-Luc Alessandri, and Jean-Pierre Rivière

Subjects
Male, medicine.medical_specialty, Pathology, Biopsy, Enzyme-Linked Immunosorbent Assay, Antibodies, Viral, medicine.disease_cause, Polymerase Chain Reaction, Diagnosis, Differential, Epidemiology, Humans, Immunologic Factors, Medicine, Chikungunya, Alphavirus infection, skin and connective tissue diseases, Retrospective Studies, Skin, Skin Diseases, Vesiculobullous, integumentary system, Alphavirus Infections, business.industry, Infant, Newborn, Immunoglobulins, Intravenous, Infant, Retrospective cohort study, Viral Load, medicine.disease, Dermatology, Treatment Outcome, DNA, Viral, Pediatrics, Perinatology and Child Health, Female, Viral disease, Differential diagnosis, business, Chikungunya virus, Total body surface area, Viral load, Follow-Up Studies
Abstract

The aim of this study was to describe a pediatric case series of Chikungunya infection associated with extensive bullous skin lesions, a severe and unknown form of the disease, during the 2005-2006 outbreak in La Réunion Island.Retrospective descriptive hospital-based study in children presenting blistersor = 10% of total body surface area with laboratory-confirmed Chikungunya infection.Eight boys and five girls with a mean age of 3.4 months were included. Blistering began after an average of 2 days after onset of fever and affected 21.5% (10% to 35%) of the total body surface area. Reverse transcription-polymerase chain reaction of blister fluid (n = 5) was positive with a mean viral load sometimes higher than in concurrent serum. Histopathologic examination (n = 10) showed intraepidermal blisters. Hospitalization and repeated dressing changes under general anesthesia were required. No death occurred. On follow-up, long term repigmentation was excellent with sometimes cosmetic sequelae.Chikungunya should be included in the differential diagnosis of febrile blistering dermatoses in small infants in epidemic areas.

Published
2009

23. Chikungunya fever: CNS infection and pathologies of a re-emerging arbovirus

Author

Jean Luc Alessandri, Pascale Krejbich Trotot, Marie Christine Jaffar-Bandjee, Duksha Ramful, Pascale Guiraud, Renubala Sahoo, Melanie Denizot, Bernard Alex Gaüzère, Philippe Gasque, Ghislaine Lee-Pat-Yuen, Trina Das, Jean Jacques Hoarau, Stéphanie Robin, Groupe de Recherche en Immunopathologies et maladies infectueuses (GRI), Université de La Réunion (UR)-Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Service de Réanimation Néonatale et Pédiatrique, and CHD Félix Guyon

Subjects
[SDV]Life Sciences [q-bio], 030231 tropical medicine, Neuroinfection, Biology, medicine.disease_cause, Communicable Diseases, Arbovirus, Communicable Diseases, Emerging, 03 medical and health sciences, 0302 clinical medicine, medicine, alphavirus, Animals, Humans, Chikungunya, Neurodegeneration, Alphavirus infection, innate immunity, 030304 developmental biology, Emerging, Aedes, 0303 health sciences, Alphavirus Infections, General Neuroscience, virus diseases, Tropical disease, Outbreak, Meningoencephalitis, medicine.disease, biology.organism_classification, Virology, 3. Good health, Immunology, Central Nervous System Viral Diseases, Chikungunya virus, Encephalitis
Abstract

International audience; Chikungunya virus (CHIKV) is transmitted by Aedes mosquitoes and causes an acute symptomatic illness with fever, skin rash, and incapacitating arthralgia, which can evolve into chronic rheumatoid arthritis in elderly patients. This is a tropical disease originally described in central/east Africa in the 1960s, but its 2004 re-emergence in Africa and rapid spread in lands in and around the Indian Ocean (Reunion island, India, Malaysia) as well as Europe (Italy) led to almost 6 million cases worldwide. The risk of importation and spreading diseases with long-term sequelae is even greater today given the global distribution of the vectors (including in the Americas), increased tourism and the apparent capacity of CHIKV to produce high levels of viremia (10(9)-10(12) virus/ml of blood) and new mutants. CHIKV-associated neuropathology was described early in the 1960s, but it is the unprecedented incidence rate in Indian Ocean areas with efficient clinical facilities that allowed a better description of cases with severe encephalitis, meningoencephalitis, peripheral neuropathies and deaths among newborns (mother-to-child infection), infants and elderly patients. Death rates following CHIKV infection were estimated at 1:1000 cases in la Reunion's outbreak. These clinical observations have been corroborated by experimental infection in several mouse models, leading to CNS pathologies. We further describe in this review the capacity of CHIKV to infect neurons and glial cells, delineate the fundamental innate (intrinsic) immune defence mechanisms to protect from infection and argue about the possible mechanisms involved in the encephalopathy.

Published
2009

24. Perlman syndrome: report, prenatal findings and review

Author

Duksha Ramful, Stéphanie Robin, Jean-Pierre Rivière, Sandrine Ernould, Fabrice Cuillier, Jean-Luc Alessandri, Stefan de Napoli-Cocci, and Sylvie Rossignol

Subjects
Polyhydramnios, medicine.medical_specialty, Pediatrics, Prenatal diagnosis, Consanguinity, Kidney, Wilms Tumor, Ultrasonography, Prenatal, Fetal Macrosomia, Fatal Outcome, Pregnancy, Internal medicine, Prenatal Diagnosis, Genetics, medicine, Fetal macrosomia, Humans, Genetic Predisposition to Disease, Perlman syndrome, Genetics (clinical), business.industry, Infant, Newborn, Wilms' tumor, Syndrome, medicine.disease, Amniotic Fluid, Endocrinology, Overgrowth syndrome, Nephromegaly, Female, medicine.symptom, business
Abstract

Perlman syndrome is a rare overgrowth syndrome characterized by polyhydramnios with neonatal macrosomia, nephromegaly, distinctive facial appearance, renal dysplasia, nephro-blastomatosis, and predisposition to Wilms tumor (WT). We report on a newborn with prenatal sonographic signs of Perlman syndrome, large fetal ascites, nephromegaly and macrosomia. The clinical course was marked by neonatal distress, renal failure and refractory hypoxemia leading to death at 2 days of life. Renal histologic examination showed bilateral nephroblastomatosis. Genetic or epigenetic alterations of the 11p15 region (involved in the BWS), including mutation of the CDKN1C gene were excluded. No mutation of the GPC3 gene was identified. We review the 28 patients who have been reported with Perlman syndrome. The prognosis of Perlman syndrome is poor with a high neonatal mortality rate. Among the infants who survived beyond the neonatal period, 64% developed a WT and all had a developmental delay. Fetal macrosomia, ascites and polyhydramnios are frequent manifestations. Clinical overlaps with other overgrowth syndromes particularly Beckwith-Wiedemann syndrome and Simpson-Golabi-Behmel syndrome have been emphasized. Perlman syndrome is considered as an autosomal recessive condition. We review 19 patients from seven sibships with parental consanguinity in two families only. The other cases were sporadic. The 28 reported patients had only 10 unaffected sibs. The low percentage of consanguinity among parents is also puzzling for a rare recessive condition. The molecular basis of Perlman syndrome is unknown. (Epi)genetic anomalies of 11p15 and mutations in GPC3 were not studied in most of the previous reports.

Published
2008

25. Neurologic manifestations of pediatric chikungunya infection

Author

Gérald Rigou, Duksha Ramful, Jean-Luc Alessandri, Florence Le Seach, Stéphanie Robin, and Marie-Christine Jaffar-Bandjee

Subjects
Male, Pathology, medicine.medical_specialty, Pediatrics, Adolescent, Disease, Electroencephalography, medicine.disease_cause, Cerebrospinal fluid, Indian Ocean Islands, medicine, Humans, Neurologic sequelae, Chikungunya, Age of Onset, Child, Pathological, Retrospective Studies, medicine.diagnostic_test, business.industry, Reverse Transcriptase Polymerase Chain Reaction, Age Factors, Encephalitis, Arbovirus, Headache, Infant, Newborn, Brain, Infant, medicine.disease, Echoencephalography, Magnetic Resonance Imaging, Meningitis, Viral, Child, Preschool, Pediatrics, Perinatology and Child Health, Acute disseminated encephalomyelitis, Consciousness Disorders, RNA, Viral, Female, Neurology (clinical), business, Chikungunya virus, Encephalitis
Abstract

Chikungunya virus, a mosquito-borne arbovirus, was responsible for a massive epidemic in La Réunion Island during 2005 to 2006. The disease is usually benign, but neurologic involvement, with sometimes fatal outcome, has been described. We report a retrospective hospital-based pediatric series of 30 children (23 boys and 7 girls) who presented neurologic manifestations of chikungunya such as encephalitis (n = 12), febrile seizures (n = 10), meningeal syndrome (n = 4), and acute encephalopathy (n = 4). Cerebrospinal fluid biological and cytological analyses (n = 23) were unremarkable except for 1 case of acute disseminated encephalomyelitis. The presence of viral genome in cerebrospinal fluid was inconstantly positive. Brain magnetic resonance imaging (MRI) scans (n = 14) were abnormal in 5 cases. Electroencephalography was nonspecific. Two patients died. At discharge and 6 months later, 5 children had neurologic sequelae. Patients with initial severe neurologic presentation and having pathological brain MRI had more sequelae or fatal disease.

Published
2008

26. Intensive Care Unit Admission for Pandemic (H1N1) 2009, Reunion Island, 2009

Author

Duksha Ramful, Laurent Filleul, Mounir El Bock, Khaled Ezzedine, David Vandroux, Bernard Alex Gaüzère, Marie-Christine Jaffar-Bandjee, Denis Malvy, Service de microbiologie, Groupe Hospitalier Sud-CHR La réunion, Centre Hospitalier Universitaire de La Réunion (CHU La Réunion), Service de médecine interne et maladies tropicales, CHU Bordeaux [Bordeaux]-Groupe hospitalier Saint-André, Cellule Interrégionale d'Epidémiologie (CIRE), Cellule Interregionale d'Epidemiologie (CIRE), Groupe de Recherche en Immunopathologies et maladies infectueuses (GRI), Université de La Réunion (UR)-Centre hospitalier Félix-Guyon [Saint-Denis, La Réunion], Laboratoire d'Immunologie et Parasitologie, Université Bordeaux Segalen - Bordeaux 2, Centre Hospitalier Universitaire de La Réunion ( CHU La Réunion ), Groupe de Recherche en Immunopathologies et maladies infectueuses ( GRI ), and Université de la Réunion ( UR ) -Centre hospitalier Régional Site Félix Guyon

Subjects
Male, Epidemiology, intensive care units, H1N1 virus, lcsh:Medicine, Total population, 030204 cardiovascular system & hematology, law.invention, La Réunion, 0302 clinical medicine, Influenza A Virus, H1N1 Subtype, law, Pregnancy, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Pandemic, Health care, 030212 general & internal medicine, Pregnancy Complications, Infectious, Indian Ocean, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, [ SDV.MHEP.ME ] Life Sciences [q-bio]/Human health and pathology/Emerging diseases, [ SDV.SPEE ] Life Sciences [q-bio]/Santé publique et épidémiologie, Middle Aged, Intensive care unit, 3. Good health, [ SDV.MHEP.MI ] Life Sciences [q-bio]/Human health and pathology/Infectious diseases, Infectious Diseases, Mainland, Female, Medical emergency, influenza, Microbiology (medical), Adult, Adolescent, letter, lcsh:Infectious and parasitic diseases, 03 medical and health sciences, Young Adult, Influenza, Human, medicine, Humans, viruses, lcsh:RC109-216, Letters to the Editor, Pandemics, Aged, business.industry, pandemic, lcsh:R, medicine.disease, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, business, Reunion, Demography
Abstract

International audience; We report results of the prospective surveillance system established in the largest intensive care unit (ICU) of Reunion Island (25 beds). This system covers 500,000 residents (62% of the total population) and monitors the daily status of patients >17 years of age who had a positive reverse transcription–PCR (RT-PCR) for pandemic (H1N1) 2009 virus. Reunion Island is a French overseas territory in the Southern Hemisphere, with health care facilities similar to those of mainland France. Patients were followed up until discharge from the ICU or death. Data were collected during July 15–September 30, 2009.

Published
2011

27. Myocarditis associated with 2009 influenza A (H1N1) virus in children

Author

Nicolas Boimond, Duksha Ramful, and Alban-Elouen Baruteau

Subjects
Myocarditis, Reverse Transcriptase Polymerase Chain Reaction, business.industry, MEDLINE, Retrospective cohort study, General Medicine, medicine.disease, medicine.disease_cause, Virology, Disease Outbreaks, Influenza A Virus, H1N1 Subtype, DNA, Viral, Influenza, Human, Pediatrics, Perinatology and Child Health, Influenza A virus, Influenza A (H1N1) virus, Humans, Medicine, Child, Cardiology and Cardiovascular Medicine, business, Reunion, Retrospective Studies
Published
2010

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