Publications by authors named "Portefaix A"

Article Synopsis
  • Diabetic ketoacidosis (DKA) is a serious condition that can occur in children with type 1 diabetes, and stopping intravenous insulin can lead to high blood sugar again.
  • This study evaluated two treatment protocols in children recovering from DKA: one where insulin was given after stopping IV infusion, and another where long-acting insulin was given before stopping the IV.
  • Results showed that the new protocol with early long-acting insulin significantly reduced the risk of ketosis rebound compared to the old protocol.
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Regarding nirsevimab immunization status, among 1085 infants hospitalized for bronchiolitis, the odds of hospitalization for respiratory syncytial virus bronchiolitis were 4.7 times higher for nonimmunized children. Immunized infants were less likely to require oxygen supplementation (20.

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Unlabelled: Cystinosis metabolic bone disease (CMBD) is an emerging concept in infantile nephropathic cystinosis, patients presenting with bone pains, fractures, and deformations during teenage or early adulthood. The underlying mechanisms remain unclear. Our aim was to explore the pro-inflammatory profile of osteoclastic lineage in cystinotic patients.

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Background And Purpose: Disabling dystonia despite optimal medical treatment is common in Wilson disease (WD). No controlled study has evaluated the effect of deep brain stimulation (DBS) on dystonia related to WD. This study was undertaken to evaluate the efficacy of DBS on dystonia related to WD.

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Article Synopsis
  • * Conducted retrospectively, the research included infants born before 32 weeks of gestation from 2012 to 2021, analyzing various support methods used and outcomes of early or late weaning failure.
  • * Results showed that 43% of infants were weaned before 6 months of age, with factors like the duration of invasive ventilation and postnatal steroid treatment linked to later weaning failures.
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  • The study investigates mineral bone disorder in patients with chronic kidney disease after kidney transplantation, focusing on bone biomarkers and microarchitecture changes before and 6 months after the surgery.
  • A subgroup of patients aged 10 to 18 who underwent their first kidney transplant was compared to healthy controls, revealing initially higher bone densities but notable declines in trabecular microarchitecture at the radius six months post-transplant.
  • Despite some improvements in bone health, many patients persisted with metabolic issues like acidosis and elevated parathyroid hormone levels after the transplant, indicating ongoing bone metabolism concerns.
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Endocrine disrupting chemicals (EDCs) may impact children's health, with medicines as a possible exposure source. Objective: to assess the potential impact of substances in paediatric medications and essential oils on children as EDC. It is a systematic review of five databases including Medline following the PECOT approach.

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Article Synopsis
  • Endocrine disruptors (ED) are widespread pollutants linked to chronic diseases, particularly affecting vulnerable groups like infants and children, highlighting a need for better information for parents.
  • A study conducted at Lyon Mother and Child Hospital surveyed 746 individuals, including 444 pediatric healthcare professionals and 302 parents, revealing that while most had heard of ED, only 10% of parents and 5% of professionals felt well-informed about them.
  • The findings showed that professionals generally had better knowledge than parents (73% vs. 60%), but only a small percentage had engaged with scientific literature or training on EDs, indicating the necessity for specific educational initiatives.
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Background: This study aimed to compare the humoral responses to mRNA COVID-19 vaccination in people living with HIV (PWH) and HIV-negative individuals.

Methods: We included PWH with an undetectable viral load under ART and HIV-negative participants from the French nationwide ANRS COV-POPART cohort who had received two doses of vaccine as a primary vaccination. We compared humoral response between controls and PWH, stratified by CD4 cell count (<200/mm and ≥200/mm CD4 cell counts) at 1, 6, and 12 months after primary vaccination.

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Purpose: MRI is the main imaging modality for pediatric brain tumors, but amino acid PET can provide additional information. Simultaneous PET-MRI acquisition allows to fully assess the tumor and lower the radiation exposure. Although symptomatic posterior fossa tumors are typically resected, the patient management is evolving and will benefit from an improved preoperative tumor characterization.

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  • Toxic shock syndrome (TSS) is a serious but rare illness that can lead to significant health problems, especially in children, and the study aimed to test the effectiveness and safety of intravenous immunoglobulin (IG) therapy compared to a control treatment in pediatric patients.
  • The study involved a randomized clinical trial with 28 participants aged 1 month to 18 years, assessing the feasibility of the trial based on inclusion rates, protocol compliance, and data completeness over a follow-up period of one year.
  • The results indicated that while the trial was feasible with a high inclusion rate and low missing data, there was no significant difference in clinical outcomes between the IG and control groups, although more adverse events were noted in the control group.
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Background: This study aimed to collect obstetric anesthesia practice and patient-reported outcomes as an update to the last French Obstetric Anesthesia survey from 1996.

Methods: Maternity units were randomly selected across France and surveyed for 7 consecutive days from February, 2016, to January, 2017. Data was gathered prospectively by questionnaires filled out by patients and anesthesia providers.

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Despite the high prevalence of late-onset sepsis (LOS) in neonatal intensive care units, a reliable diagnosis remains difficult. This prospective, multicenter cohort study aimed to identify biomarkers early to rule out the diagnosis of LOS in 230 neonates ≥7 days of life with signs of suspected LOS. Blood levels of eleven protein biomarkers (PCT, IL-10, IL-6, NGAL, IP-10, PTX3, CD14, LBP, IL-27, gelsolin, and calprotectin) were measured.

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Autotaxin (ATX) is a secreted enzyme with a lysophospholipase D activity, mainly secreted by adipocytes and widely expressed. Its major function is to convert lysophosphatidylcholine (LPC) into lysophosphatidic acid (LPA), an essential bioactive lipid involved in multiple cell processes. The ATX-LPA axis is increasingly studied because of its involvement in numerous pathological conditions, more specifically in inflammatory or neoplastic diseases, and in obesity.

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Background: The recommended dose of ephedrine in adults (0.1 mg kg) frequently fails to treat hypotension after induction of general anaesthesia in neonates and infants less than 6 months of age. The aim of this study was to determine the optimal dose of ephedrine in this population for the treatment of hypotension after induction of general anaesthesia with sevoflurane.

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Despite various international regulatory initiatives over the last 20 years, many challenges remain in the field of paediatric drug development and evaluation. Indeed, drug research and development is still focused essentially on adult indications, thereby excluding many paediatric patients, limiting the feasibility of trials and favouring competing developments. Off-label prescribing persists and the development of age-appropriate dosage forms for children remains limited.

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The diagnosis of serious bacterial infection (SBI) in young febrile children remains challenging. This prospective, multicentre, observational study aimed to identify new protein marker combinations that can differentiate a bacterial infection from a viral infection in 983 children, aged 7 days-36 months, presenting with a suspected SBI at three French paediatric emergency departments. The blood levels of seven protein markers (CRP, PCT, IL-6, NGAL, MxA, TRAIL, IP-10) were measured at enrolment.

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Background: The underlying mechanisms of obesity in X-linked hypophosphatemia (XLH) are not known. We aimed to evaluate whether FGF21, an endocrine FGF involved in the regulation of carbohydrate-lipid metabolism, could be involved.

Methods: We performed a prospective multicenter cross-sectional study comparing FGF23, Klotho, and FGF21 levels in teenagers with XLH compared to healthy controls (VITADOS cohort) after matching for age, gender, and puberty.

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Background And Aims: Intracranial Hypertension (ICH) is a life-threatening complication of brain injury. The invasive measurement of intracranial pressure (ICP) remains the gold standard to diagnose ICH. Measurement of Optic Nerve Sheath Diameter (ONSD) using ultrasonography is a non-invasive method for detecting ICH.

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Background: Hypercalciuria is one of the most frequent metabolic disorders associated with nephrolithiasis and/or nephrocalcinosis possibly leading to chronic kidney disease (CKD) and bone complications in adults. Orphan diseases with different underlying primary pathophysiology share inappropriately increased 1,25(OH)D levels and hypercalciuria, e.g.

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Aims: To identify all available trigger tools applicable to the pediatric population in hospital settings to detect adverse drug events (ADEs) and to describe their performances by positive predictive value (PPV).

Methods: PubMed® was searched until December 2021. The reference sections were also consulted for new articles.

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Background And Purpose: The EREMI project was set up to collect data on adverse drug reactions (ADRs) occurring due to off-label and/or unlicensed drugs prescribed to hospitalised children in France. These events were evaluated by a regional pharmacovigilance centre (RPC) and an adjudication committee (AC). The aim of this study was to assess the agreement between these two different entities on their evaluation of ADRs.

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Multiple Inflammatory Syndrome in Children (MIS-C) is a delayed and severe complication of SARS-CoV-2 infection that strikes previously healthy children. As MIS-C combines clinical features of Kawasaki disease and Toxic Shock Syndrome (TSS), we aimed to compare the immunological profile of pediatric patients with these different conditions. We analyzed blood cytokine expression, and the T cell repertoire and phenotype in 36 MIS-C cases, which were compared to 16 KD, 58 TSS, and 42 COVID-19 cases.

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