Breastfeeding Success and Newborn Health before and during the COVID-19 Pandemic:A Single-Centre Comparative Study.

Introduction: Due to the SARS-CoV-2 pandemic, adjustments in patient and visitor traffic were made in hospitals to limit viral exposure. The primary objective of our study was to compare the breastfeeding success of healthy newborns in a maternity ward during the 2020 lockdown period compared with the same period in the previous year.

Material And Methods: Single-center comparative study based on prospectively collected data.

Severe atypical Lemierre syndrome caused by methicillin-sensitive Staphylococcus aureus: Two pediatric case reports.

Background: Lemierre syndrome is typically associated with ear, nose, and throat (ENT) infections caused by Fusobacterium necrophorum. Since 2002, cases of atypical Lemierre-like syndrome secondary to Staphylococcus aureus have been reported.

Cases: We report two pediatric cases of atypical Lemierre syndrome with a similar presentation: exophthalmia, absence of pharyngitis, metastatic lung infection, and intracranial venous sinus thrombosis.

Renal vein thrombosis in neonates: a case series of diagnosis, treatment and childhood kidney function follow-up.

Background: Neonatal renal vein thrombosis (NRVT) is a rare condition with little data available.

Methods: We retrospectively analyzed newborns diagnosed with NRVT admitted to 3 pediatric nephrology units in Paris from 2005 to 2020.

Results: Twenty-seven patients were analyzed (male = 59%).

Assessing the risk of adverse pregnancy outcomes and birth defects reporting in women exposed to ganciclovir or valganciclovir during pregnancy: a pharmacovigilance study.

Objectives: Cytomegalovirus (CMV) is the leading cause of congenital infection worldwide. Reference anti-CMV treatment is valganciclovir/ganciclovir, which is contraindicated in pregnancy given questions about teratogenicity.

Methods: We analysed reports from VigiBase, the world's largest safety database, and performed a disproportionality analysis of adverse pregnancy outcomes associated with (val)ganciclovir compared with any other drugs or with (val)aciclovir as comparators.

DOCK11 deficiency in patients with X-linked actinopathy and autoimmunity.

Dedicator of cytokinesis (DOCK) proteins play a central role in actin cytoskeleton regulation. This is highlighted by the DOCK2 and DOCK8 deficiencies leading to actinopathies and immune deficiencies. DOCK8 and DOCK11 activate CDC42, a Rho-guanosine triphosphate hydrolases involved in actin cytoskeleton dynamics, among many cellular functions.

A human dynein heavy chain mutation impacts cortical progenitor cells causing developmental defects, reduced brain size and altered brain architecture.

Dynein heavy chain (DYNC1H1) mutations can either lead to severe cerebral cortical malformations, or alternatively may be associated with the development of spinal muscular atrophy with lower extremity predominance (SMA-LED). To assess the origin of such differences, we studied a new Dync1h1 knock-in mouse carrying the cortical malformation p.Lys3334Asn mutation.

Severe Bacterial Infection Initially Misdiagnosed as MIS-C: Caution Needed.

Working in the era of the novel coronavirus disease 2019 can predispose to cognitive bias. We present a case of life-threatening bacterial infection misdiagnosed as multisystem inflammatory syndrome in children. While multisystem inflammatory syndrome in children-related myocardial dysfunction is now a well-recognized complication of coronavirus disease 2019, a rigorous differential diagnosis approach, notably for infectious etiologies, is paramount.

Femtosecond assisted keratopigmentation (FAK) with a 5-millimeter diameter aperture does not affect ocular examination.

Aim: to assess the feasibility of performing a screening of ocular pathologies after Femtosecond laser Assisted Keratopigmentation (FAK) procedure in normal eyes with the aid of multimodal imaging technologies.

Design: A Retrospective cohort study.

Participants: Thirty consecutive international patients (60 eyes) who underwent FAK for purely aesthetic reasons were chosen for this study.

Thyroid-stimulating hormone receptor signaling restores skeletal muscle stem cell regeneration in rats with muscular dystrophy.

Duchenne muscular dystrophy (DMD) is a severe and progressive myopathy leading to motor and cardiorespiratory impairment. We analyzed samples from patients with DMD and a preclinical rat model of severe DMD and determined that compromised repair capacity of muscle stem cells in DMD is associated with early and progressive muscle stem cell senescence. We also found that extraocular muscles (EOMs), which are spared by the disease in patients, contain muscle stem cells with long-lasting regenerative potential.

Concurrent and Predictive Validity of a Cycloid Loops Copy Task to Assess Handwriting Disorders in Children.

Handwriting disorders (HDs) are mainly assessed using script or cursive handwriting tasks. The most common is the scale for children's handwriting, with a French adaptation (BHK). The present study aims to assess the concurrent validity of a pre-scriptural task (copying a line of cycloid loops) with the BHK for the diagnosis of HDs.

Icex: Advances in the automatic extraction and volume calculation of cranial cavities.

The use of non-destructive approaches for digital acquisition (e.g. computerised tomography-CT) allows detailed qualitative and quantitative study of internal structures of skeletal material.

A high-throughput sequencing approach identifies immunotherapeutic targets for bacterial meningitis in neonates.

Background: Worldwide, Escherichia coli is the leading cause of neonatal Gram-negative bacterial meningitis, but full understanding of the pathogenesis of this disease is not yet achieved. Moreover, to date, no vaccine is available against bacterial neonatal meningitis.

Methods: Here, we used Transposon Sequencing of saturated banks of mutants (TnSeq) to evaluate E.

Plasma G ganglioside potential biomarker for diagnosis, prognosis and disease monitoring of GM2-Gangliosidosis.

GM2-Gangliosidosis are a group of inherited lysosomal storage pathologies characterized by a large accumulation of G ganglioside in the lysosome. They are caused by mutation in HEXA or HEXB causing reduced or absent activity of a lysosomal β-hexosaminidase A, or mutation in GM2A causing defect in GM2 activator protein (GM2AP), an essential protein for the activity of the enzyme. Biochemical diagnosis relies on the measurement of β-hexosaminidases A and B activities, which is able to detect lysosomal enzyme deficiency but fails to identify defects in GM2AP.

Valganciclovir is not associated with decreased EBV infection rate in pediatric kidney transplantation.

Introduction: Primary infection or reactivation of Epstein-Barr Virus (EBV) is a significant cause of morbidity and mortality in pediatric kidney transplantation. Valganciclovir (VGC) treatment is recommended for prophylaxis of cytomegalovirus infection, but its role for the prevention of EBV infection remains controversial.

Patients And Methods: All pediatric kidney transplant recipients aged <18 years old were considered for inclusion in this retrospective study.

Dry synovitis, a rare entity distinct from juvenile idiopathic arthritis.

Background: Dry synovitis (DS) is a rare entity as only a few cases have been reported to date. We describe the clinical features, radiological manifestations and course of DS in comparison with rheumatoid factor negative polyarticular juvenile idiopathic arthritis (RFneg-polyJIA).

Methods: We performed a multicenter retrospective collection of data of DS patients who presented with progressive joint limitations without palpable synovitis, absence of elevated acute phase reactants, negative ANA and RF, and imaging showing joint and/or osteochondral involvement.

Genotype-phenotype relationships of truncating mutations, p.E297G and p.D482G in bile salt export pump deficiency.

Background & Aims: Bile salt export pump (BSEP) deficiency frequently necessitates liver transplantation in childhood. In contrast to two predicted protein truncating mutations (PPTMs), homozygous p.D482G or p.

Systemic corticosteroids for the treatment of acute episodes of rhabdomyolysis in lipin-1-deficient patients.

Mutations in the LPIN1 gene constitute a major cause of severe rhabdomyolysis (RM). The TLR9 activation prompted us to treat patients with corticosteroids in acute conditions. In patients with LPIN1 mutations, RM and at-risk situations that can trigger RM have been treated in a uniform manner.

Consequences of General Anesthesia in Infancy on Behavior and Brain Structure.

Background: One in 7 children will need general anesthesia (GA) before the age of 3. Brain toxicity of anesthetics is controversial. Our objective was to clarify whether exposure of GA to the developing brain could lead to lasting behavioral and structural brain changes.