Newborn With Refractory Seizures Due To Hemimegalencephaly And Tuberous Sclerosis Complex: Case Report and Literature Review.

Background Hemimegalencephaly (HME) is a rare congenital disorder that is initiated during embryonic development with abnormal growth of one hemisphere. Tuberous sclerosis complex (TSC), a genetic disorder, is rarely associated with HME. Methods We present a case of a newborn with HME with a confirmed mutation in the TSC-1 gene and describe the clinical course, findings on (amplitude integrated) electroencephalography (aEEG), cranial ultrasound (CUS), MRI, and the postmortem evaluation.

Physiological versus time based cord clamping in very preterm infants (ABC3): a parallel-group, multicentre, randomised, controlled superiority trial.

Background: Physiological-based cord clamping (PBCC) in preterm infants is beneficial for cardiovascular transition at birth and may optimize placental transfusion. Whether PBCC can improve clinical outcomes is unknown. The aim of the Aeration, Breathing, Clamping (ABC3) trial was to test whether PBCC results in improved intact survival in very preterm infants.

Longitudinal Analysis of Amplitude-Integrated Electroencephalography for Outcome Prediction in Infants with Hypoxic-Ischemic Encephalopathy: A Validation Study.

Objectives: To validate the prognostic accuracy of a previously published tool (HOPE calculator) using longitudinal analysis of amplitude-integrated electroencephalography (aEEG) background activity and sleep-wake cycling to predict favorable or adverse 2-year neurodevelopmental outcome in infants with hypoxic-ischemic encephalopathy (HIE) undergoing therapeutic hypothermia (TH), and to evaluate the predictive value for outcome at 5-8 years of age.

Study Design: Single-center retrospective cohort study in 117 infants who underwent TH for HIE between 2008 and 2022. We scored 2-channel aEEG BGPs, sleep-wake cycling, and seizure activity at 6-hour intervals for 84 hours.

Fifteen Years of Neonatal Therapeutic Hypothermia: Clinical Trends Show Unchanged Post-Rewarming Outcomes despite Reduction in Hypoxic-Ischemic Encephalopathy Severity.

Introduction: Hypoxic-ischemic encephalopathy (HIE) affects 1-2 per 1,000 births and is associated with mortality and long-term neurodevelopmental challenges. At present, therapeutic hypothermia (TH) is the only neuroprotective intervention for these infants. This study examines whether HIE severity, clinical management during TH, and post-rewarming outcomes have changed since its introduction 15 years ago.

State-of-the-art cranial ultrasound in clinical scenarios for infants born at term and near-term.

Neonates admitted to the intensive care unit are at risk of brain injury. Importantly, infants with signs of neurological impairment need prompt diagnosis to guide intervention. Cranial ultrasound (CUS) is the first-line imaging tool for infants born preterm.

Most major bleeds in preterm infants occur in the absence of severe thrombocytopenia: an observational cohort study.

Objective: To describe the incidence of major bleeds according to different platelet counts in very preterm infants, and to explore whether this association is influenced by other risk factors for bleeding.

Design: Observational cohort study.

Setting: A Dutch tertiary care neonatal intensive care unit.

Developmental anatomy of the thalamus, perinatal lesions, and neurological development.

The thalamic nuclei develop before a viable preterm age. GABAergic neuronal migration is especially active in the third trimester. Thalamic axons meet cortical axons during subplate activation and create the definitive cortical plate in the second and third trimesters.

Brain MRI Injury Patterns across Gestational Age among Preterm Infants with Perinatal Asphyxia.

Introduction: Brain injury patterns of preterm infants with perinatal asphyxia (PA) are underreported. We aimed to explore brain magnetic resonance imaging (MRI) findings and associated neurodevelopmental outcomes in these newborns.

Methods: Retrospective multicenter study included infants with gestational age (GA) 24.

Feasibility of neurosonography in CHD-fetuses and controls in a clinical tertiary setting.

Objective: Ultrasonographic examination is the first-tier test to detect abnormal development of central nervous system (CNS). In optimal conditions, neurosonography can detect all important hallmarks of CNS development. It is, however, not known how the performance of this modality is in a routine setting.

Early red blood cell transfusion and the occurrence of intraventricular hemorrhage in very preterm infants.

Background: Preterm infants are at risk of developing both intraventricular hemorrhage (IVH) and anemia of prematurity. Several studies reported an association between early postnatal red blood cell (RBC) transfusion and IVH, however the timing and causality between these two remains unclear.

Aims: To describe the temporal sequence between administration of early RBC transfusion (within the first week of life) and diagnosis of IVH in very preterm infants.

Fetal and neonatal neuroimaging in twin-twin transfusion syndrome.

Objectives: To describe the types of brain injury and subsequent neurodevelopmental outcome in fetuses and neonates from pregnancies with twin-twin transfusion syndrome (TTTS). Additionally, to determine risk factors for brain injury and to review the use of neuroimaging modalities in these cases.

Methods: This was a retrospective cohort study of consecutive TTTS pregnancies treated with laser surgery in a single fetal therapy center between January 2010 and January 2020.

Efficacy of Levetiracetam as Add-On Therapy in the Treatment of Seizures in Neonates.

Introduction: There is no consensus regarding the efficacy of add-on therapy with levetiracetam (LEV) in the treatment of seizures in neonates. The aim of this study was to evaluate the efficacy of add-on therapy with LEV for achieving >80% seizure reduction after phenobarbital (PB) treatment.

Methods: Retrospective cohort study of near term neonates admitted to the neonatal intensive care unit with EEG-confirmed seizures despite treatment with PB as first-line therapy and using LEV as 2nd-, 3rd- or 4th-line treatment.

The Current Knowledge of Cerebral Magnetic Resonance Imaging in Monochorionic Twins: A Systematic Review of the Last 20 Years.

The distinct placental angioarchitecture in monochorionic (MC) pregnancies increases the risk of complications such as twin-twin transfusion syndrome (TTTS), twin anemia polycythemia sequence (TAPS), and selective fetal growth restriction (sFGR). The aim of this systematic review was to evaluate the incidence, type, and severity of cerebral injury and structural brain development on fetal and/or neonatal cerebral magnetic resonance imaging (MRI) in MC twins with or without complications. Twenty-three studies were included, covering a wide range of complications observed during MC pregnancies, with studies involving sIUFD ( = 12), TTTS ( = 7), mixed complications ( = 2), TAPS ( = 1), and uncomplicated MC pregnancy ( = 1).

Neuroimaging in infants with congenital cytomegalovirus infection and its correlation with outcome: emphasis on white matter abnormalities.

Objective: To evaluate the association between neuroimaging and outcome in infants with congenital cytomegalovirus (cCMV), focusing on qualitative MRI and quantitative diffusion-weighted imaging of white matter abnormalities (WMAs).

Methods: Multicentre retrospective cohort study of 160 infants with cCMV (103 symptomatic). A four-grade neuroimaging scoring system was applied to cranial ultrasonography and MRI acquired at ≤3 months.

Early recognition of characteristic conventional and amplitude-integrated EEG patterns of seizures in SCN2A and KCNQ3-related epilepsy in neonates.

Purpose: Early recognition of seizures in neonates secondary to pathogenic variants in potassium or sodium channel coding genes is crucial, as these seizures are often resistant to commonly used anti-seizure medications but respond well to sodium channel blockers. Recently, a characteristic ictal amplitude-integrated electroencephalogram (aEEG) pattern was described in neonates with KCNQ2-related epilepsy. We report a similar aEEG pattern in seizures caused by SCN2A- and KCNQ3-pathogenic variants, as well as conventional EEG (cEEG) descriptions.

The Association of Dexamethasone and Hydrocortisone with Cerebellar Growth in Premature Infants.

Objectives: Corticosteroids are used to prevent or treat lung disease of prematurity. While neurological side effects have been reported, detailed effects on cerebellar growth are unknown. This study aimed to compare cerebellar growth in premature infants who received dexamethasone or hydrocortisone to premature infants who did not receive postnatal corticosteroids.

Ultrasonographic Estimation of Ventricular Volume in Infants Born Preterm with Posthemorrhagic Ventricular Dilatation: A Nested Substudy of the Randomized Controlled Early Versus Late Ventricular Intervention Study (ELVIS) Trial.

Objective: To study the potential role of ventricular volume (VV) estimation in the management of posthemorrhagic ventricular dilatation related to the need for ventriculoperitoneal (VP)-shunt insertion and 2-year neurodevelopmental outcome in infants born preterm.

Study Design: We included 59 patients from the Early vs Late Ventricular Intervention Study from 4 participating centers. VV was manually segmented in 209 3-dimensional ultrasound scans and estimated from 2-dimensional ultrasound linear measurements in a total of 1226 ultrasounds.

Children Newly Diagnosed with Fetal and Neonatal Alloimmune Thrombocytopenia: Neurodevelopmental Outcome at School Age.

Objective: To evaluate the neurodevelopmental outcome at school age in children newly diagnosed with fetal and neonatal alloimmune thrombocytopenia (FNAIT).

Study Design: This observational cohort study included children diagnosed with FNAIT between 2002 and 2014. Children were invited for cognitive and neurological testing.

Discovery of a Series of Potent, Selective, and Orally Bioavailable Nucleoside Inhibitors of CD73 That Demonstrates Antitumor Activity.

CD73 (ecto-5'-nucleotidase) has emerged as an attractive target for cancer immunotherapy of many cancers. CD73 catalyzes the hydrolysis of adenosine monophosphate (AMP) into highly immunosuppressive adenosine that plays a critical role in tumor progression. Herein, we report our efforts in developing orally bioavailable and highly potent small-molecule CD73 inhibitors from the reported hit molecule to lead molecule and then finally to compound .