Spleen stiffness in a healthy pediatric population undergoing liver magnetic resonance elastography.

Background: Splenic stiffness is a potential imaging marker of portal hypertension. Normative spleen stiffness values are needed to define diagnostic thresholds.

Objective: To report stiffness measurements of the spleen in healthy children undergoing liver magnetic resonance (MR) elastography across MRI vendors and field strengths.

The impact of pediatric post-cardiac arrest care on survival: A multicenter review from the AHA get with the Guidelines®-resuscitation post-cardiac arrest care registry.

Aim: Adherence to post-cardiac arrest care (PCAC) recommendations is associated with improved outcomes for adults. We aimed to describe the survival impact of meeting American Heart Association (AHA) PCAC guidelines in children after cardiac arrest.

Methods: We conducted a retrospective study using Get With The Guidelines® Resuscitation's (GWTG®-R) registry to describe the PCAC of patients ≤ 18 years old who suffered an in-hospital or out-of-hospital cardiac arrest (IHCA or OHCA).

Risk of Motor Vehicle Accidents in Obstructive Sleep Apnea: Comparative Analysis of CPAP Versus Surgery.

Objective: To compare the incidence of motor vehicle accidents (MVAs) among patients with obstructive sleep apnea (OSA) undergoing continuous positive airway pressure (CPAP) therapy and sleep surgery.

Study Design: Retrospective cohort study using the TriNetX national clinical database.

Setting: Analysis of a nationwide patient cohort.

Comprehensive reanalysis of light fluence distribution in pleural photodynamic therapy using standardized anatomical coordinates.

Photodynamic therapy (PDT) has shown promise as an adjuvant treatment for malignant pleural mesothelioma when combined with surgical resection. Accurate light dosimetry is critical for treatment efficacy. This study presents an improved method for analyzing light fluence distribution in pleural PDT using a standardized anatomical coordinate system and advanced computational modeling.

Palovarotene in fibrodysplasia ossificans progressiva: review and perspective.

Introduction: Palovarotene is a retinoic acid receptor gamma agonist that was studied in phase-2 and phase-3 clinical trials for the inhibition of new heterotopic ossification (HO) in fibrodysplasia ossificans progressiva (FOP). Despite numerous setbacks and regulatory delays, palovarotene is now the first approved FOP treatment in the U.S.

Transitioning from the safety of the womb to the outside world for neonates with life-threatening cardiovascular conditions: The IMmediate Postpartum Access to Cardiac Therapy (IMPACT) Procedure.

The IMmediate Postpartum Access to Cardiac Therapy (IMPACT) procedure is a multidisciplinary, collaborative, highly coordinated clinical service in which a planned delivery and intensive neonatal care are offered for conditions where there is a high likelihood of postnatal instability. This process includes prenatal consultation with the parent(s), involving each service engaged with the delivery, postnatal resuscitation, and procedural care. A Cesarean section delivery is planned in an operating room with immediate access to a multifunctional procedural suite where the neonate can undergo rapid cardiac evaluation and initiation of interventional treatments which can have a positive, life-saving impact.

Femur osteoid osteoma in children: are there location-dependent differences in MRI findings?

Background: Osteoid osteomas are most commonly found in the femur and preferentially affect the pediatric population. Magnetic resonance imaging (MRI) findings of femoral osteoid osteomas are not well described.

Objective: To systematically characterize pretreatment MRI findings of clinically confirmed femur osteoid osteomas in children and determine location-dependent differences.

Transferrin Saturation, Serum Iron, and Ferritin in Heart Failure: Prognostic Significance and Proteomic Associations.

Background: Iron deficiency (ID) is currently defined as a serum ferritin level <100 or 100 to 299 ng/mL with transferrin saturation (TSAT) <20%. Serum ferritin and TSAT are currently used to define absolute and functional ID. However, individual markers of iron metabolism may be more informative than current arbitrary definitions of ID.

Predicting right ventricular failure after left ventricular assist device implant: A novel approach.

Aims: Right ventricular (RV) failure (RVF) after left ventricular assist device (LVAD) implant is an important cause of morbidity and mortality. Modern, data-driven approaches for defining and predicting RVF have been under-utilized.

Methods: Two hundred thirty-two patients were identified with a mean age of 55 years; 40 (17%) were women, 132 were (59%) Caucasian and 74 (32%) were Black.

Prenatally Diagnosed Absent Septum Pellucidum and Septo-Optic Dysplasia: A Narrative Review and Practical Recommendations for Pediatric Neurologists.

Evaluation of the cavum septum pellucidum is required in standard second-trimester screening fetal anatomy ultrasound scans. The absence of septum pellucidum triggers further evaluation and referral for subspecialty counseling. Absence of septum pellucidum is linked to other midline anomalies including septo-optic dysplasia.

The 2024 American Association for Thoracic Surgery expert consensus document: Current standards in donor lung procurement and preservation.

Background: Donor lung procurement and preservation is critical for lung transplantation success. Unfortunately, the large variability in techniques impacts organ utilization rates and transplantation outcomes. Compounding this variation, recent developments in cold static preservation and new technological advances with machine perfusion have increased the complexity of the procedure.

Comparison of the amyloid plaque proteome in Down syndrome, early-onset Alzheimer's disease, and late-onset Alzheimer's disease.

Down syndrome (DS) is strongly associated with Alzheimer's disease (AD) due to APP overexpression, exhibiting Amyloid-β (Aβ) and Tau pathology similar to early-onset (EOAD) and late-onset AD (LOAD). We evaluated the Aβ plaque proteome of DS, EOAD, and LOAD using unbiased localized proteomics on post-mortem paraffin-embedded tissues from four cohorts (n = 20/group): DS (59.8 ± 4.

Lineage-dependence of the neuroblastoma surfaceome defines tumor cell state-dependent and independent immunotherapeutic targets.

Background: Neuroblastoma is a heterogeneous disease with adrenergic (ADRN)- and therapy resistant mesenchymal (MES)-like cells driven by distinct transcription factor networks. Here, we investigate the expression of immunotherapeutic targets in each neuroblastoma subtype and propose pan-neuroblastoma and cell state specific targetable cell-surface proteins.

Methods: We characterized cell lines, patient-derived xenografts, and patient samples as ADRN-dominant or MES-dominant to define subtype-specific and pan-neuroblastoma gene sets.

Endoscopy and Anesthesia Outcomes Associated with Glucagon-Like Peptide-1 Receptor Agonist use in Patients Undergoing Outpatient Upper Endoscopy.

Background And Aims: Glucagon-like peptide-1 receptor agonists (GLP1RAs) can cause delayed gastric emptying, raising concern for retained gastric contents (RGCs) during endoscopy and adverse anesthesia events. We aimed to determine associations between GLP1RA and endoscopy and anesthesia outcomes.

Methods: This single-center retrospective cohort study examined patients prescribed GLP1RA who underwent outpatient endoscopy stratified by exposure at the time of endoscopy.

Maralixibat Reduces Serum Bile Acids and Improves Cholestatic Pruritus in Adolescents With Alagille Syndrome.

Background: Alagille syndrome (ALGS) is a multisystem cholestatic disorder. Maralixibat is approved for the treatment of cholestatic pruritus in ALGS with limited data in adults.

Methods: Participants were included if they received ≥ 2 doses of maralixibat at age ≥ 16 years in one of the three previously published maralixibat ALGS clinical trials.

The Complete Inpatient Record Using Comprehensive Electronic Data (CIRCE) project: A team-based approach to clinically validated, research-ready electronic health record data.

Introduction: The rapid adoption of electronic health record (EHR) systems has resulted in extensive archives of data relevant to clinical research, hospital operations, and the development of learning health systems. However, EHR data are not frequently available, cleaned, standardized, validated, and ready for use by stakeholders. We describe an in-progress effort to overcome these challenges with cooperative, systematic data extraction and validation.

Phenotypic variability and the gender paradox in the R363C variant of Fabry disease.

Fabry disease is an X-linked lysosomal disease caused by variants in the gene. Although Fabry disease is X-linked, gene variants in females can exhibit a wide range of symptoms, challenging the traditional view of Fabry as an X-linked recessive disease. A family is presented here with a 36-year-old female who is symptomatic with chronic kidney disease and her oligosymptomatic 70-year-old father, both of whom have a heterozygous and hemizygous GLA pathogenic variant, respectively, c.

Frequency and reasons that parents decline genetic testing for critically ill neonates.

Purpose: Current literature reports strong support among parents for genetic testing for ill neonates; yet, some parents decline this testing for unknown reasons. We aimed to document the proportion of parents who decline, describe their clinical and demographic characteristics, and categorize their rationales.

Methods: We reviewed medical records to collect and compare clinical and demographic information for patients whose parents consented to and declined recommended genetic testing.

A Multicenter Study of Pulmonary Critical Care Trainees' Perception of Airway Management Training during Fellowship.

Background: There is significant variability in airway management training among pulmonary and critical care medicine (PCCM) fellows.

Objective: To assess the airway management training of PCCM fellows, specifically evaluating the role of the institutional approach to intubations (anesthesia-predominant primary operators vs. PCCM-predominant) to the overall fellows' educational experience.