Surgical resection of diffuse pulmonary arteriovenous malformations (PAVMs).

Objective: Patients with pulmonary arteriovenous malformations (PAVM) can have significant morbidity and mortality. Surgical resection in isolation or with embolization is reported to treat diffuse-type PAVMs. Herein, we describe outcomes for children and adults for whom PAVMs were managed with elective surgical resection.

Use of bupivacaine liposomal injectable suspension in children aged 2 to 6 years undergoing cardiac surgery does not accelerate recovery.

Objective: Bupivacaine liposomal injectable suspension is proven safe and effective for selective postsurgical analgesia in children older than 6 years. We evaluated if intraoperative bupivacaine liposomal injectable suspension administration decreases postoperative opioid use, peak pain scores, and length of stay in children aged 2 to 6 years undergoing cardiac surgery via median sternotomy.

Methods: Serial patients aged 2 to 6 years undergoing cardiac surgery received 4 mg/kg bupivacaine liposomal injectable suspension mixed with 0.

Aligning prediction models with clinical information needs: infant sepsis case study.

Objective: Sepsis recognition among infants in the Neonatal Intensive Care Unit (NICU) is challenging and delays in recognition can result in devastating consequences. Although predictive models may improve sepsis outcomes, clinical adoption has been limited. Our focus was to align model behavior with clinician information needs by developing a machine learning (ML) pipeline with two components: (1) a model to predict baseline sepsis risk and (2) a model to detect evolving (dynamic) sepsis risk due to physiologic changes.

Neuroinflammation: An Oligodendrocentric View.

Chronic neuroinflammation, driven by central nervous system (CNS)-resident astrocytes and microglia, as well as infiltration of the peripheral immune system, is an important pathologic mechanism across a range of neurologic diseases. For decades, research focused almost exclusively on how neuroinflammation impacted neuronal function; however, there is accumulating evidence that injury to the oligodendrocyte lineage is an important component for both pathologic and clinical outcomes. While oligodendrocytes are able to undergo an endogenous repair process known as remyelination, this process becomes inefficient and usually fails in the presence of sustained inflammation.

A Predictive Model for Identification of Pediatric Individuals With Common Variable Immunodeficiency through Electronic Medical Records.

Introduction: Common variable immunodeficiency (CVID) is characterized by recurrent sinopulmonary infections. However, in the pediatric population, recurrent sinopulmonary infections early in life are common, which can render key clinical features of CVID less distinctive. Accordingly, the diagnosis of CVID is often delayed due to the heterogeneous nature of the presentation and the broad age of onset.

Use of CD19-targeted Immune Modulation to Eradicate AAV Neutralizing Antibodies.

Neutralizing antibodies (NAb) against adeno-associated virus (AAV) represent a significant obstacle to the efficacy of systemic recombinant AAV vector administration or re-administration. While there are some promising preclinical immunomodulation strategies in development, insights into which B cell subsets and compartments maintain persistent AAV NAb may define the optimal eradication strategy. Given the limited success of CD20-directed monotherapy in previous studies, we hypothesized that CD19-directed approaches that extend targeting into the plasma cell compartments may improve AAV NAb eradication.

Neurobehavioral Profiles in Opioid-Exposed and Unexposed Neonates.

Objective: To describe distinctive profiles of neurobehavior in opioid-exposed and unexposed neonates.

Study Design: The Outcomes of Babies with Opioid Exposure (OBOE) study is a multisite, prospective, observational study in neonates born at term with opioid exposure and unexposed controls. As part of the OBOE study protocol, certified examiners administered the NeoNatal Neurobehavioral Scales, 2 edition (NNNS-II) before 6 weeks postnatal age.

Outpatient Diuretic Use and Respiratory Outcomes in Children with Bronchopulmonary Dysplasia During the First Three Years of Life.

Objective: To investigate the link between outpatient respiratory outcomes in children with bronchopulmonary dysplasia (BPD) who are prescribed outpatient diuretics versus those who are not at the time of their initial pediatric pulmonary clinic visit.

Study Design: Patient data were gathered by chart review and validated questionnaires on 1,002 children with BPD discharged between 2008 and 2023 seen at the Children's Hospital of Philadelphia and Johns Hopkins Children's Center outpatient pediatric pulmonary clinics.

Results: Children prescribed outpatient diuretics (n=634) at time of first pulmonary appointment were more likely to have severe BPD and require home supplemental oxygen.

Psychometric properties of the Federal Unit Scale for general psychopathology screening without iatrogenic effects.

Mental health assessment tools play a crucial role in clinical practice, yet many existing scales risk exacerbating the conditions they aim to measure. This study introduces the Federal Unit Scale (FUS), a novel 5-item scale designed to assess a broad range of affect and traits capturing distress tolerance while minimizing potentially triggering language. The FUS was developed with a focus on combat veterans, frontline workers, and first responders.

Adult Growth Hormone Deficiency, Replacement Therapy, and Outcomes in Long-Term Childhood Cancer Survivors.

Context: The consequences of untreated adult growth hormone deficiency (aGHD) among childhood cancer survivors are not well-defined. The lack of evidence and socioeconomic factors may contribute to underutilization of growth hormone therapy (GHT) among survivors with aGHD.

Objectives: To examine the association of GHT use with socioeconomic factors and to assess the impact of untreated aGHD in survivors using insulin-like growth factor-1 (IGF1) as a marker of GH action.

Dynamics of primary cilia in endothelial and mesenchymal cells throughout mouse lung development.

Cilia are specialized structures found on a variety of mammalian cells, with variable roles in the transduction of mechanical and biological signals (by primary cilia, PC), as well as in the generation of fluid flow (by motile cilia). Their critical role in the establishment of a left-right axis in early development is well described, as well as in the defense immune function of multiciliated upper airway epithelium. By contrast, detailed analysis of the ciliary status of specific cell types during organogenesis and postnatal development has received less attention.

Comprehensive Multiomic Analysis Reveals Metabolic Reprogramming Underlying Human Fontan-Associated Liver Disease.

Background: The Fontan operation is the current standard of care for single-ventricle congenital heart disease. Almost all patients with Fontan operation develop liver fibrosis at a young age, known as Fontan-associated liver disease (FALD). The pathogenesis and mechanisms underlying FALD remain little understood, and there are no effective therapies.

Anti-obesity medication for weight loss in early nonresponders to behavioral treatment: a randomized controlled trial.

Current guidelines recommend behavioral treatment (BT) as the first intervention for patients with obesity. However, a substantial minority (35-50%) do not achieve a clinically meaningful loss of ≥5%. Anti-obesity medications (AOMs) are recommended when target weight loss is not achieved; however, their efficacy among BT nonresponders has not been established.

Primitive audiovisual integration of speech.

An unintelligible video recording of a face uttering a sentence and an unintelligible acoustic sinusoid following the frequency variation of a single vocal resonance of the utterance were intelligible when presented together at their veridical synchrony. The intelligibility resulted from audiovisual sensory integration and phonetic perceptual analysis, which depended neither on the separate resolution of linguistic impressions in each modality nor on closed-set reports about a single pair of minimal phonemic contrast features. Likewise, audiovisual integration could not be attributed to Gestalt-derived similarity principles applied unimodally or bimodally.

Is local air pollution concentration a moderator or mediator of the association between residential greenspace and pediatric asthma exacerbations? A longitudinal study of pediatric patients in Philadelphia, Pennsylvania.

Background: Despite mixed evidence supporting the link between greenspace and asthma exacerbations, several studies suggest a negative association. The mechanisms underlying this relationship are unclear, with air pollution concentrations potentially playing a key role. This study investigated whether air pollution concentrations modify or mediate the relationship between residential greenspace and pediatric asthma exacerbations.

Variability in triggers for mechanical left ventricular unloading in VA-ECMO: A literature search.

Background: Venoarterial Extracorporeal Membrane Oxygenation (VA-ECMO) is a means of supporting the lungs or the heart and lungs in patients with hemodynamic compromise that is refractory to conventional measures. VA-ECMO is most commonly deployed in a percutaneous fashion with femoral arterial and venous access. While VA-ECMO, particularly in a femoral-femoral configuration, provides both hemodynamic and ventilatory support, it also causes increased afterload on the left ventricle (LV) which in turn may result in LV distension (LVD).

Pediatric cardiac patients with pulmonary hemorrhage supported on ECMO: An ELSO registry study.

Background: Pulmonary Hemorrhage (PH) is a rare but potentially devastating condition and pediatric cardiac patients are at increased risk for. ECMO may be used to safely support these patients, but data is limited.

Methods: Observational retrospective cohort study from the ELSO registry database in pediatric cardiac patients from birth to 18 years old with PH supported on ECMO from January 2011 through December 2020.

Opportunities and Challenges in Using Electronic Health Record Systems to Study Postacute Sequelae of SARS-CoV-2 Infection: Insights From the NIH RECOVER Initiative.

The benefits and challenges of electronic health records (EHRs) as data sources for clinical and epidemiologic research have been well described. However, several factors are important to consider when using EHR data to study novel, emerging, and multifaceted conditions such as postacute sequelae of SARS-CoV-2 infection or long COVID. In this article, we present opportunities and challenges of using EHR data to improve our understanding of long COVID, based on lessons learned from the National Institutes of Health (NIH)-funded RECOVER (REsearching COVID to Enhance Recovery) Initiative, and suggest steps to maximize the usefulness of EHR data when performing long COVID research.

Virtual Car Seat Installation Program Evaluation: Parent Satisfaction and Installation Accuracy.

Background: Motor vehicle crashes are a leading cause of preventable death in the United States, but properly used child safety seats significantly reduce the risk of injury and mortality. In-person car safety seat installation programs were suspended during the COVID-19 pandemic, prompting the development of virtual alternatives. However, the effectiveness of these virtual installation programs remains unknown.

Outpatient Respiratory Outcomes in Extremely Preterm Children During the First 3 Years of Life.

Rationale: Extremely preterm infants are at highest risk for developing bronchopulmonary dysplasia (BPD). This study aimed to examine the relationship between gestational age and respiratory outcomes in children with BPD in the outpatient setting.

Methods: Data were collected from 1025 preterm children with BPD recruited from outpatient bronchopulmonary (BPD) clinics at Johns Hopkins and Children's Hospital of Philadelphia (CHOP).

Engaging Parents of Children With Sickle Cell Disease in Shared Decision-Making for Hydroxyurea: The ENGAGE-HU Study.

Background: Guidelines recommend that hydroxyurea be offered to children with sickle cell disease (SCD) as early as 9 months of age using shared decision-making. To help clinicians implement shared decision-making with parents, we developed the Hydroxyurea Shared Decision-Making (H-SDM) toolkit. We evaluated its effectiveness on parent decisional uncertainty, perceptions of shared decision-making, hydroxyurea knowledge, and the likelihood of being offered and prescribed hydroxyurea.

Nasal biomarker inflammatory profile in response to intranasal corticosteroids in pediatric obstructive sleep apnea syndrome.

Study Objectives: Nasopharyngeal inflammation contributes to pediatric obstructive sleep apnea syndrome (OSAS). Intranasal corticosteroids (INCS) are used to treat pediatric OSAS; a randomized controlled trial (RCT) showed an improvement in OSAS symptoms but without polysomnography or neurobehavioral outcome differences. There is a lack of data demonstrating an objective decrease in the nasal inflammatory biomarker profile (NIBP) associated with INCS.