Autonomic markers of extubation readiness in premature infants.

Background: In premature infants, extubation failure is common and difficult to predict. Heart rate variability (HRV) is a marker of autonomic tone. Our aim is to test the hypothesis that autonomic impairment is associated with extubation readiness.

North American Blastic Plasmacytoid Dendritic Cell Neoplasm Consortium: position on standards of care and areas of need.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with historically poor outcomes and no worldwide consensus treatment approach. Unique among most hematologic malignancies for its frequent cutaneous involvement, BPDCN can also invade other extramedullary compartments, including the central nervous system. Generally affecting older adults, many patients are unfit to receive intensive chemotherapy, and although hematopoietic stem cell transplantation is preferred for younger, fit individuals, not all are eligible.

SARS-CoV-2 placentitis, stillbirth, and maternal COVID-19 vaccination: clinical-pathologic correlations.

Stillbirth is a recognized complication of COVID-19 in pregnant women that has recently been demonstrated to be caused by SARS-CoV-2 infection of the placenta. Multiple global studies have found that the placental pathology present in cases of stillbirth consists of a combination of concurrent destructive findings that include increased fibrin deposition that typically reaches the level of massive perivillous fibrin deposition, chronic histiocytic intervillositis, and trophoblast necrosis. These 3 pathologic lesions, collectively termed SARS-CoV-2 placentitis, can cause severe and diffuse placental parenchymal destruction that can affect >75% of the placenta, effectively rendering it incapable of performing its function of oxygenating the fetus and leading to stillbirth and neonatal death via malperfusion and placental insufficiency.

Risk Factors for Death and Severe Neurological Sequelae in Childhood Bacterial Meningitis.

Introduction: Acute bacterial meningitis (ABM) continues to be a challenge from a diagnostic and therapeutic point of view. Identifying severity risk factors and predictive scores may guide interventions to reduce poor outcome.

Methods: Data from a retrospective study for ABM in children admitted to the Institute of Tropical Medicine in Paraguay was analyzed.

Targeted Metabolomics Revealed a Sex-Dependent Signature for Metabolic Syndrome in the Mexican Population.

Metabolic syndrome (MetS) is a group of several metabolic conditions predisposing to chronic diseases. Individuals diagnosed with MetS are physiologically heterogeneous, with significant sex-specific differences. Therefore, we aimed to investigate the potential sex-specific serum modifications of amino acids and acylcarnitines (ACs) and their relationship with MetS in the Mexican population.

Refining the Neuroimaging Definition of the Dandy-Walker Phenotype.

Background And Purpose: The traditionally described Dandy-Walker malformation comprises a range of cerebellar and posterior fossa abnormalities with variable clinical severity. We aimed to establish updated imaging criteria for Dandy-Walker malformation on the basis of cerebellar development.

Materials And Methods: In this multicenter study, retrospective MR imaging examinations from fetuses and children previously diagnosed with Dandy-Walker malformation or vermian hypoplasia were re-evaluated, using the choroid plexus/tela choroidea location and the fastigial recess shape to differentiate Dandy-Walker malformation from vermian hypoplasia.

ATP6V0C variants impair V-ATPase function causing a neurodevelopmental disorder often associated with epilepsy.

The vacuolar H+-ATPase is an enzymatic complex that functions in an ATP-dependent manner to pump protons across membranes and acidify organelles, thereby creating the proton/pH gradient required for membrane trafficking by several different types of transporters. We describe heterozygous point variants in ATP6V0C, encoding the c-subunit in the membrane bound integral domain of the vacuolar H+-ATPase, in 27 patients with neurodevelopmental abnormalities with or without epilepsy. Corpus callosum hypoplasia and cardiac abnormalities were also present in some patients.

Isolated Absent Septum Pellucidum: A Retrospective Study of Fetal Diagnosis and Postnatal Outcomes.

Background: Absent septum pellucidum (ASP) is a brain abnormality often associated with neuroanatomic abnormalities including septo-optic dysplasia (SOD). We aimed to determine how frequently prenatally diagnosed isolated ASP is confirmed by postnatal imaging and to examine clinical outcomes for ASP.

Methods: This was a retrospective study of maternal-fetal dyads referred to Children's National Hospital from January 1, 2012, to June 30, 2019.

New Insights into Zika in Infants and Children.

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Outcome of Agenesis of the Corpus Callosum Diagnosed by Fetal MRI.

Background: Fetal magnetic resonance imaging (MRI) is increasingly utilized for prenatal diagnosis of agenesis of the corpus callosum (ACC). This study aimed to (1) describe cases of ACC diagnosed by fetal MRI, (2) determine the frequency of postnatal confirmation by MRI, and (3) understand postnatal outcomes of infants with ACC.

Methods: Maternal records from Children's National Hospital between January 2012 and June 2019 with a prenatal neurological consultation, fetal MRI, and ACC on imaging were included.

Plasma Biomarkers of Evolving Encephalopathy and Brain Injury in Neonates with Hypoxic-Ischemic Encephalopathy.

Objective: The objective of the study was to evaluate the relationship between a panel of candidate plasma biomarkers and (1) death or severe brain injury on magnetic resonance imaging (MRI) and (2) dysfunctional cerebral pressure autoregulation as a measure of evolving encephalopathy.

Study Design: Neonates with moderate-to-severe hypoxic-ischemic encephalopathy (HIE) at 2 level IV neonatal intensive care units were enrolled into this observational study. Patients were treated with therapeutic hypothermia (TH) and monitored with continuous blood pressure monitoring and near-infrared spectroscopy.

Neurosarcoidosis in children: A systematic review and summary of cases, imaging and management.

Neurosarcoidosis is a rare disorder in children. We identified 30 pediatric NS cases through a systematic review. Twenty-one (70%) had systemic sarcoidosis with 30% having primary neurosarcoidosis.

A Neurologist's Practical Guide to Conducting a Fetal Consultation.

Fetal and neonatal neurology is increasingly recognized as a subspecialty within child neurology and fellowship training programs are emerging. Most child neurologists have not received formal training in the interpretation of fetal data and the practice of fetal neurology consultation. However, they can be valuable members of the fetal care team and bring important perspective to the diagnosis of fetal neurologic conditions.

Defining Objective Measures of Physician Stress in Simulated Critical Communication Encounters.

Unlabelled: This study had three aims: 1) quantify the difference in stress levels between low and high stress roles during simulated critical communication encounters using objective physiologic data (heart rate variability [HRV]) and subjective measures (State-Trait Anxiety Inventory [STAI]), 2) define the relationship between subjective and objective measures of stress, and 3) define the impact of trainee preparedness and reported self-efficacy on stress levels.

Design: Mixed methods simulation-based study.

Setting: Single center.

Teaching Children to Respond to Questions About the Past: A Preliminary Analysis.

Correct responding to questions regarding events that have occurred in the past are important for conversations and safety. Limited research has demonstrated techniques for teaching this skill to children who do not successfully tact past events. The current study demonstrates a probe-fading method for teaching children to correctly tact stimuli they were exposed to in the past.

Electroencephalogram in low-risk term newborns predicts neurodevelopmental metrics at age two years.

Objective: To determine whether neurodevelopmental biomarkers at 2 years of age are already present in the newborns' EEG at birth.

Methods: Low-risk term newborns were enrolled and studied utilizing EEG prior to discharge from the birth hospital. A 14-channel EEG montage (scalp-level) and source signals were calculated using the EEG.

Maternal psychological distress during the COVID-19 pandemic and structural changes of the human fetal brain.

Background: Elevated maternal psychological distress during pregnancy is linked to adverse outcomes in offspring. The potential effects of intensified levels of maternal distress during the COVID-19 pandemic on the developing fetal brain are currently unknown.

Methods: We prospectively enrolled 202 pregnant women: 65 without known COVID-19 exposures during the pandemic who underwent 92 fetal MRI scans, and 137 pre-pandemic controls who had 182 MRI scans.

Fetal brain MRI: neurometrics, typical diagnoses, and resolving common dilemmas.

This review presents a practical approach to imaging the fetal brain by MRI. Herein, we demonstrate how to measure brain structures and fluid spaces, and discuss the importance of comparing measurements to normative biometric references at a corresponding gestational age. We present some common imaging dilemmas of the technical aspects of fetal MRI with regard to typical regions of abnormality including the cerebrum, the ventricular system, and the posterior fossa, and discuss how to resolve them.

Association of Elevated Maternal Psychological Distress, Altered Fetal Brain, and Offspring Cognitive and Social-Emotional Outcomes at 18 Months.

Importance: Prenatal maternal psychological distress is associated with disturbances in fetal brain development. However, the association between altered fetal brain development, prenatal maternal psychological distress, and long-term neurodevelopmental outcomes is unknown.

Objective: To determine the association of fetal brain development using 3-dimensional magnetic resonance imaging (MRI) volumes, cortical folding, and metabolites in the setting of maternal psychological distress with infant 18-month neurodevelopment.

Frequency-Based Maternal Electrocardiogram Attenuation for Fetal Electrocardiogram Analysis.

Fetal electrocardiogram (ECG) waveform analysis along with cardiac time intervals (CTIs) measurements are critical for the management of high-risk pregnancies. Currently, there is no system that can consistently and accurately measure fetal ECG. In this work, we present a new automatic approach to attenuate the maternal ECG in the frequency domain and enhance it with measurable CTIs.