A5005290441- Neonatal Respiratory Health Research
- Congenital Diaphragmatic Hernia Studies
- Mechanical Circulatory Support Devices
- Respiratory Support and Mechanisms
- Intensive Care Unit Cognitive Disorders
- Cardiac Arrest and Resuscitation
- Infant Development and Preterm Care
- Metabolism and Genetic Disorders
- Anesthesia and Neurotoxicity Research
- Biochemical Acid Research Studies
- Neuroscience of respiration and sleep
- Connective tissue disorders research
- Pulmonary Hypertension Research and Treatments
- Pediatric Pain Management Techniques
- Anesthesia and Sedative Agents
- Fibroblast Growth Factor Research
- Neurological diseases and metabolism
- Cancer, Hypoxia, and Metabolism
- Cardiac Arrhythmias and Treatments
- Hyperglycemia and glycemic control in critically ill and hospitalized patients
- Mitochondrial Function and Pathology
- Restraint-Related Deaths
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Gestational Diabetes Research and Management
- Chronic Obstructive Pulmonary Disease (COPD) Research
Maladies génétiques d’expression pédiatrique
2021-2025
Sorbonne Université
2018-2025
Assistance Publique – Hôpitaux de Paris
2013-2025
Inserm
2020-2025
Hôpital Armand-Trousseau
2018-2025
Expression Génétique Microbienne
2024
Université Paris 1 Panthéon-Sorbonne
2024
Sorbonne Paris Cité
2021
Hôpital d'Enfants
2021
Hôpital Antoine-Béclère
2021
Synthesis and apoenzyme attachment of lipoic acid have emerged as a new complex metabolic pathway. Mutations in several genes involved the de novo pathway recently been described (i.e., LIAS, NFU1, BOLA3, IBA57), but no mutation was found so far specific process to apoenzymes pyruvate dehydrogenase (PDHc), α-ketoglutarate (α-KGDHc) branched chain α-keto (BCKDHc) complexes.Exome capture performed boy who developed Leigh disease following gastroenteritis had combined PDH α-KGDH deficiency with...
Objective: To investigate the impact of fetal growth restriction (FGR) on hormonal regulation post-natal and glucose metabolism [via insulin hormone (GH)/Insulin-like Growth factor 1 (IGF1) axis pathways] in small for gestational age (SGA) neonates. Methods: We conducted a monocentric observational prospective comparative study 73 singleton babies born with weight inferior to 2,000 g. analyzed auxological (weight, height head circumference), (GH, IGF1, plasma concentrations) data comparing...
Abstract Introduction Lung biopsy is considered as the last step investigation for diagnosing lung diseases; however, its indication must be carefully balanced with invasiveness. The present study aims to evaluate diagnostic yield of in critically ill patients hospitalized pediatric intensive care unit (ICU). Material and Methods Children who underwent a ICU between 1995 2022 were included. Biopsies performed operating room post‐mortem biopsies excluded. Results Thirty‐one included, median...
Ventilator-associated pneumonia (VAP) is a severe intensive complication and remains under estimated in neonatal care unit (NICU). Center for Disease Control defined criteria adults pediatrics without criteria. The objective of this article was to evaluate the rate, risks factors outcome neonates suffering from ventilator-associated French NICU.We conducted prospective observational study within one-year period our NICU. Three hundred eighty-one 28 days age were included. Data analyses...
Acinar dysplasia (AcDys) is one of the three main diffuse developmental disorders lung. The transcription factor NK2 homeobox 1 (NKX2.1) partly controls synthesis surfactant proteins by type 2 alveolar epithelial cells (AEC2), and germline mutations are known to be associated with brain-lung thyroid syndrome. We report case a full-term neonate who developed refractory respiratory failure pulmonary hypertension requiring venoarterial extracorporeal membrane oxygenation. Histological...
The objective of the study was to assess efficacy reduced sufentanil doses for postoperative analgesia following surgical ductal closure in extremely premature infants.This a retrospective, single-center, cohort comparing 2 dosing regimens used between 2001 and 2010 included all infants born at <28 weeks gestation with closure. Sufentanil were 2007 as standard care. Time divided into 3 epochs distinguish effects practice changes over time from dose change: epoch 1 (2001 2004), (May 2005...
Objectives: Extracorporeal membrane oxygenation is an established therapy for refractory cardiac and/or pulmonary failure that not available in all centers. When infants and children require extracorporeal oxygenation, they are sometimes placed on support peripheral centers where then transferred to specialized The objective of this study first describe one the largest cohorts transported by a mobile unit while oxygenation. Design: We undertook single-center retrospective included patients...
Immunocompromised children are likely to develop a refractory acute respiratory distress syndrome (ARDS). The usefulness of providing extracorporeal life support (ECLS) these patients is subject debate. aim our study was report the outcomes and compare factors associated with mortality between immunocompromised non-immunocompromised supported veno-venous ECMO.We performed retrospective monocentric in French pediatric ECMO center Armand Trousseau Hospital, including all aged from 1 month 18...
Extracorporeal membrane oxygenation (ECMO) is widely used for children treated refractory respiratory failures or cardiogenic shock. Its duration depends on organ functions recovery. Weaning decided using macro-circulatory tools, but microcirculation not well evaluated. Sidestream dark-field video imaging to assess the perfusion of sublingual microvascular vessels. The aim this study was predictive value microcirculatory indices in ECMO weaning. This prospective monocentric examined...
BACKGROUND: Immunocompromised children are likely to develop a refractory acute respiratory distress syndrome (ARDS). The usefulness of providing extracorporeal life support (ECLS) these patients is subject debate. aim our study was report the outcomes and compare factors associated with mortality between immunocompromised non-immunocompromised supported veno-venous ECMO. METHODS: We performed retrospective monocentric in French pediatric ECMO center Armand Trousseau Hospital, including all...
There are presently only scant data published about neonatal diaphragmatic paralysis and the options for its clinical management. We observed a case of unilateral secondary to thoracic surgery in preterm neonate, we successfully managed it using neurally adjusted ventilator assist (NAVA) whose have been electronically recorded real time. Parents gave written consent publication. A female baby, born at 25 weeks’ gestation, was diagnosed with haemodynamically patent ductus arteriosus, which...
Deep sedation in critically ill children undergoing extracorporeal membrane oxygenation (ECMO) can be challenging. Volatile anesthetics like sevoflurane a good alternative for patients hospitalized pediatric intensive care units, whom adequate is difficult to obtain.We report here the first case of patient under receiving by using AnaConDa-S device. This 2-year-old girl, suffering from congenital diaphragmatic hernia, was put on due persistent pulmonary hypertension following metapneumovirus...
OBJECTIVE: Defining the best ventilatory settings under ECMO remains a challenging question. Despite well-defined ARDS treatment before initiation, there is no recommendation on how to ventilate patient for P-ARDS. Only few descriptive studies are available during respiratory ECMO. We aim at evaluating usefulness of protective ventilation bundle and its capacity reduce pressure in our center.METHODS: performed monocentric retrospective study from January 2007 December 2018. All children aged...
Abstract Objective Sedation is necessary for patients to achieve comfort and safety, but prolonged sedation can lead the need increased doses, resulting in withdrawal syndrome delayed extubation. Inhaled anaesthetics (IAs) may cause less while providing similar sedative effects intravenous agents. This study aims describe efficacy of halogenated IAs during identify any adverse on PICU. Design a retrospective, bicentric cohort study. Setting The was conducted at two PICUs university hospitals...
Abstract Purpose: To evaluate the frequency of metabolic secondary systemic insults (mSSI) and its association with mortality in newborns encephalopathy. Methods This single-center observational study neonatal encephalopathy (NE) patients 2018–2020 defined mSSI as at least one abnormal finding for any glucose, sodium, and/or carbon dioxide from routine blood samples during first 3 days life. Thresholds were hypoglycemia, <2 mmol/L; hyperglycemia, >10 hyponatremia, <130...
<b>Introduction:</b> Lung biopsy is the gold standard for diagnosis of diffuse lung diseases. Its indication has to be balanced with its invasiveness. The present study aims at identifying best indications biopsies in pediatric patients hospitalized intensive care unit (ICU). <b>Methods:</b> Children ICU who underwent a were included between 1995 and 2022. Biopsies performed operating room, post-mortem autopsies excluded. <b>Results:</b> Thirty-one median age 18 days (2 10.8 years); 21...
<b>Background:</b> A premature arrest of fetal lung development can be responsible for different diffuse developmental diseases including the most early and severe form, acinar dysplasia (AcDys). Apart from rare mutations in TBX4, molecular causes are poorly known (deMello et Reid 2000). We herein report case AcDys related to a NK2 homeobox 1 (NKX2.1) likely pathogenic variation. <b>Methods:</b> Molecular analyses were performed full-term neonate who developed refractory respiratory failure...
<b>Introduction:</b> Mature surfactant protein (SP)-A, secreted in alveolar space, is a combination of SP-A1 and SP-A2 oligomers, mainly reported as octadecamers. Heterozygous mutations the genes encoding SP-A (<i>SFTPA1</i> <i>SFTPA2</i>) are associated with interstitial lung disease (ILD) adenocarcinoma. To date, no specific treatment exists. The present study investigates effect mutant or on oligomerization. <b>Methods:</b> cDNA expression vector carrying non synonymous <i>SFTPA1</i>...