Pediatrics Commons^™

Degree Of Uncertainty In Reporting Imaging Findings For Necrotizing Enterocolitis: A Secondary Analysis From A Pilot Randomized Diagnostic Trial., Alain Cuna, Disa Rathore, Kira Bourret, Erin K. Opfer, Sherwin S. Chan

Manuscripts, Articles, Book Chapters and Other Papers

Diagnosis of necrotizing enterocolitis (NEC) relies heavily on imaging, but uncertainty in the language used in imaging reports can result in ambiguity, miscommunication, and potential diagnostic errors. To determine the degree of uncertainty in reporting imaging findings for NEC, we conducted a secondary analysis of the data from a previously completed pilot diagnostic randomized controlled trial (2019-2020). The study population comprised sixteen preterm infants with suspected NEC randomized to abdominal radiographs (AXRs) or AXR + bowel ultrasound (BUS). The level of uncertainty was determined using a four-point Likert scale. Overall, we reviewed radiology reports of 113 AXR and 24 BUS …

Immunogenicity Phase Ii Study Evaluating Booster Capacity Of Nonadjuvanted Aks-452 Sars-Cov-2 Rbd Fc Vaccine, David G Alleva, Eline A Feitsma, Yester F Janssen, Hendrikus H Boersma, Thomas M Lancaster, Thillainaygam Sathiyaseelan, Sylaja Murikipudi, Andrea R Delpero, Melanie M Scully, Ramya Ragupathy, Sravya Kotha, Jeffrey R Haworth, Nishit J Shah, Vidhya Rao, Shashikant Nagre, Shannon E Ronca, Freedom M Green, Stephen A Shaw, Ari Aminetzah, Schelto Kruijff, Maarten Brom, Gooitzen M Van Dam, Todd C Zion

Faculty, Staff and Students Publications

AKS-452, a subunit vaccine comprising an Fc fusion of the ancestral wild-type (WT) SARS-CoV-2 virus spike protein receptor binding domain (SP/RBD), was evaluated without adjuvant in a single cohort, non-randomized, open-labelled phase II study (NCT05124483) at a single site in The Netherlands for safety and immunogenicity. A single 90 µg subcutaneous booster dose of AKS-452 was administered to 71 adults previously primed with a registered mRNA- or adenovirus-based vaccine and evaluated for 273 days. All AEs were mild and no SAEs were attributable to AKS-452. While all subjects showed pre-existing SP/RBD binding and ACE2-inhibitory IgG titers, 60-68% responded to AKS-452 …

A Case Of Streptococcus Constellatus Bacteremia Secondary To Respiratory Syncytial Virus Bronchiolitis In A Neonate, Ronald G. Lott Iii, Samantha M. Lavertue, Xavier Zonna, Samuel Wlasowicz, Roland Zhang, Ravi Kavuda

Advances in Clinical Medical Research and Healthcare Delivery

Common causes of bacteremia in the neonatal period include group B streptococcus, listeria, and E. coli. We report a rare case of neonatal bacteremia with culture-positive Streptococcus constellatus. Streptococcus constellatus is part of the normal flora of the orogenital regions of the body; when found elsewhere, it causes pyogenic abscesses in the brain and digestive tract. In this case, the Streptococcus constellatus bacteremia was secondary to prolonged hospitalization in a patient with respiratory syncytial virus (RSV) bronchiolitis. This report serves to make physicians aware of and to define treatment options for Streptococcus constellatus as a rarely isolated organism that …

Current Strategies For Increasing Knock-In Efficiency In Crispr/Cas9-Based Approaches, Andrés Felipe Leal, Angelica María Herreno-Pachón, Eliana Benincore-Flórez, Amali Karunathilaka, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Since its discovery in 2012, the clustered regularly interspaced short palindromic repeats (CRISPR) and CRISPR-associated protein 9 (Cas9) system has supposed a promising panorama for developing novel and highly precise genome editing-based gene therapy (GT) alternatives, leading to overcoming the challenges associated with classical GT. Classical GT aims to deliver transgenes to the cells via their random integration in the genome or episomal persistence into the nucleus through lentivirus (LV) or adeno-associated virus (AAV), respectively. Although high transgene expression efficiency is achieved by using either LV or AAV, their nature can result in severe side effects in humans. For instance, …

Recombinant Rod Domain Of Vimentin Reduces Sars-Cov-2 Viral Replication By Blocking Spike Protein-Ace2 Interactions, Fong Wilson Lam, Cameron August Brown, Shannon Elizabeth Ronca

Faculty, Staff and Students Publications

Although the SARS-CoV-2 vaccination is the primary preventive intervention, there are still few antiviral therapies available, with current drugs decreasing viral replication once the virus is intracellular. Adding novel drugs to target additional points in the viral life cycle is paramount in preventing future pandemics. The purpose of this study was to create and test a novel protein to decrease SARS-CoV-2 replication. We created the recombinant rod domain of vimentin (rhRod) in E. coli and used biolayer interferometry to measure its affinity to the SARS-CoV-2 S1S2 spike protein and the ability to block the SARS-CoV-2–ACE2 interaction. We performed plaque assays …

Virtual Facilitation Best Practices And Research Priorities: A Scoping Review, Asya Agulnik, Derrecka Boykin, Denalee M O'Malley, Julia Price, Mia Yang, Mark Mckone, Geoffrey Curran, Mona J Ritchie

Department of Pediatrics Faculty Papers

BACKGROUND: Facilitation is an implementation strategy that supports the uptake of evidence-based practices. Recently, use of virtual facilitation (VF), or the application of facilitation using primarily video-based conferencing technologies, has become more common, especially since the COVID-19 pandemic. Thorough assessment of the literature on VF, however, is lacking. This scoping review aimed to identify and describe conceptual definitions of VF, evaluate the consistency of terminology, and recommend "best" practices for its use as an implementation strategy.

METHODS: We conducted a scoping review to identify literature on VF following the PRISMA-ScR guidance. A search of PubMed, Embase, Web of Science, and …

Cpsf3 Inhibition Blocks Pancreatic Cancer Cell Proliferation Through Disruption Of Core Histone Mrna Processing, Abdulrahman A Alahmari, Aditi H Chaubey, Venkata S Jonnakuti, Arwen A Tisdale, Carla D Schwarz, Abigail C Cornwell, Kathryn E Maraszek, Emily J Paterson, Minsuh Kim, Swati Venkat, Eduardo Cortes Gomez, Jianmin Wang, Katerina V Gurova, Hari Krishna Yalamanchili, Michael E Feigin

Faculty, Staff and Students Publications

Pancreatic ductal adenocarcinoma (PDAC) is a lethal disease with limited effective treatment options, potentiating the importance of uncovering novel drug targets. Here, we target cleavage and polyadenylation specificity factor 3 (CPSF3), the 3′ endonuclease that catalyzes mRNA cleavage during polyadenylation and histone mRNA processing. We find that CPSF3 is highly expressed in PDAC and is associated with poor prognosis. CPSF3 knockdown blocks PDAC cell proliferation and colony formation in vitro and tumor growth in vivo. Chemical inhibition of CPSF3 by the small molecule JTE-607 also attenuates PDAC cell proliferation and colony formation, while it has no effect on cell proliferation …

Phenolic Acids Prevent Sex-Steroid Deficiency-Induced Bone Loss And Bone Marrow Adipogenesis In Mice, Perry C. Caviness, Oxana P. Lazarenko, Michael L. Blackburn, Jennifer F. Chen, Christopher E. Randolph, Jovanny Zabaleta, Fenghuang Zhan, Jin Ran Chen

School of Medicine Faculty Publications

Phenolic acids, such as hippuric acid (HA) and 3-(3-hydroxyphenyl) propionic acid (3-3-PPA), can be produced from microbiome digestion of polyphenols. Previously it was found that HA and 3-3-PPA facilitate bone formation and suppress bone resorption. However, the mechanism of action by which HA and 3-3-PPA protect bone from degeneration is currently unknown. In this report, we present that HA and 3-3-PPA suppression of bone resorption is able to ameliorate bone loss in an ovariectomy (OVX) osteopenic mouse model though not to the extent of Zoledronic acid (ZA). HA and 3-3-PPA treatments were shown to significantly decrease bone marrow adipocyte-like cell …

Pulmonary Fibrosis Stakeholder Summit: A Joint Nhlbi, Three Lakes Foundation, And Pulmonary Fibrosis Foundation Workshop Report, Sydney B Montesi, Christian R Gomez, Michael Beers, Robert Brown, Ishanu Chattopadhyay, Kevin R Flaherty, Christine Kim Garcia, Brigitte Gomperts, Lida P Hariri, Cory M Hogaboam, R Gisli Jenkins, Naftali Kaminski, Grace Hyun J Kim, Melanie Königshoff, Martin Kolb, Darrell N Kotton, Jonathan A Kropski, Joseph Lasky, Chelsea M Magin, Toby M Maher, Mark Mccormick, Bethany B Moore, Cheryl Nickerson-Nutter, Justin Oldham, Anna J Podolanczuk, Ganesh Raghu, Ivan Rosas, Steven M Rowe, William T Schmidt, David Schwartz, Jessica E Shore, Cathie Spino, J Matthew Craig, Fernando J Martinez

Faculty, Staff and Students Publications

Despite progress in elucidation of disease mechanisms, identification of risk factors, biomarker discovery, and the approval of two medications to slow lung function decline in idiopathic pulmonary fibrosis and one medication to slow lung function decline in progressive pulmonary fibrosis, pulmonary fibrosis remains a disease with a high morbidity and mortality. In recognition of the need to catalyze ongoing advances and collaboration in the field of pulmonary fibrosis, the NHLBI, the Three Lakes Foundation, and the Pulmonary Fibrosis Foundation hosted the Pulmonary Fibrosis Stakeholder Summit on November 8–9, 2022. This workshop was held virtually and was organized into three topic …

Economic Impacts Of The Covid-19 Pandemic On Families Of Children With Autism And Other Developmental Disabilities., Olivia M. Pokoski, Hayley Crain, Carolyn Diguiseppi, Sarah M. Furnier, Eric J. Moody, Cy Nadler, Karen Pazol, Jessica Sanders, Lisa D. Wiggins, Maureen S. Durkin

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: To control the spread of the coronavirus disease (COVID-19), many jurisdictions throughout the world enacted public health measures that had vast socio-economic implications. In emergency situations, families of children with developmental disabilities (DDs), including autism, may experience increased difficulty accessing therapies, economic hardship, and caregiver stress, with the potential to exacerbate autism symptoms. Yet, limited research exists on the economic impacts of the COVID-19 pandemic on families of children with autism or another DD compared to families of children from the general population.

OBJECTIVES: To assess impact of the COVID-19 pandemic related to parental employment and economic difficulties in …

Pediatric Spinal Ependymoma With Chromothripsis Of Chromosome 6: A Case Report And Review Of The Literature., Keela R. Scott, Melissa Gener, Elena Repnikova

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Ependymomas are the third most common central nervous system tumor in the pediatric population; however, spinal ependymomas in children are rare. Ependymomas affecting the spinal cord most frequently occur in adults of 20-40 years of age. The current World Health Organization classification system for ependymomas is now composed of ten different entities based on histopathology, location, and molecular studies, with evidence that the new classification system more accurately predicts clinical outcomes.

CASE PRESENTATION: We present the case of a 16-year-old Caucasian female patient with a history of type 2 neurofibromatosis with multiple schwannomas, meningioma, and spinal ependymoma. Chromosome analysis …

A Case Report And Review On The Anesthetic Considerations Of Anterior Mediastinal Masses, Carter A. Schulz, Joshua D. Mohs, Andrew M. Reuter

Aesculapius Journal (Health Sciences & Medicine)

Case summary: A 15-year-old female presented with chest pain, dyspnea, and weight loss. A chest X-ray and subsequent CT scan revealed a large anterior mediastinal mass with cervical lymphadenopathy and pericardial effusion. Two biopsies under general anesthesia were performed, with the latter confirming classic Hodgkin lymphoma, nodular sclerosis subtype.

Discussion: This case underscores the significant clinical challenges posed by anterior mediastinal masses due to their proximity to vital cardiovascular and respiratory structures. Physiologic changes during general anesthesia can exacerbate these challenges. Preoperative assessment, including imaging and symptom evaluation, is crucial to guide operative planning. It is key to individualize the …

Pain Screening In Youth With Sickle Cell Disease: A Quality Improvement Study, Dennis C. Vroom, Samuel N. Rodgers-Melnick, Amma Owusu-Ansah, Jeffery A. Dusek

Student Scholarship

Background: Youth with sickle cell disease (SCD) face several challenges as they age, including increased pain frequency, duration, and interference. The purpose of this study was to (i) determine the feasibility of routine pain screening; (ii) identify and describe various clinical pain presentations; and (iii) understand preferences/resources related to engaging in integrative health and medicine (IHM) modalities within an outpatient pediatric SCD clinic. Methods: During routine outpatient visits, patients aged 8–18 completed measures of pain frequency, duration, and chronic pain risk (Pediatric Pain Screening Tool [PPST]). Participants screening positive for (i) persistent or chronic pain or (ii) medium or high …

Measures Of Function In Physical Therapy Assessment Of Children With Cerebral Palsy: A Clinical Audit, Marvin D. Zotomayor

Philippine Journal of Physical Therapy

Introduction: The assessment of outcomes in children with cerebral palsy (CP) is important for monitoring their progress, evaluating interventions, and guiding health policies. This study aimed to (1) identify the most common outcome measures used in the clinics to assess function in children with CP, and (2) determine whether the outcome measures used in the clinics meet the current standards of assessment for the purpose of improving patient care decisions, research, and quality assurance.

Methodology: A retrospective record audit study design was used to determine if the current practice in the clinics on the assessment of function in children with …

Probiotic Limosilactobacillus Reuteri Dsm 17938 Changes Foxp3 Deficiency-Induced Dyslipidemia And Chronic Hepatitis In Mice, Erini Nessim Kostandy, Ji Ho Suh, Xiangjun Tian, Beanna Okeugo, Erin Rubin, Sara Shirai, Meng Luo, Christopher M. Taylor, Kang Ho Kim, J. Marc Rhoads, Yuying Liu

School of Medicine Faculty Publications

The probiotic Limosilactobacillus reuteri DSM 17938 produces anti-inflammatory effects in scurfy (SF) mice, a model characterized by immune dysregulation, polyendocrinopathy, enteropathy, and X-linked inheritance (called IPEX syndrome in humans), caused by regulatory T cell (Treg) deficiency and is due to a Foxp3 gene mutation. Considering the pivotal role of lipids in autoimmune inflammatory processes, we investigated alterations in the relative abundance of lipid profiles in SF mice (± treatment with DSM 17938) compared to normal WT mice. We also examined the correlation between plasma lipids and gut microbiota and circulating inflammatory markers. We noted a significant upregulation of plasma lipids …

Molecular-Guided Therapy For The Treatment Of Patients With Relapsed And Refractory Childhood Cancers: A Beat Childhood Cancer Research Consortium Trial., Giselle L Saulnier Sholler, Genevieve Bergendahl, Elizabeth C. Lewis, Jacqueline Kraveka, William Ferguson, Abhinav B. Nagulapally, Karl Dykema, Valerie I Brown, Michael S. Isakoff, Joseph Junewick, Deanna Mitchell, Jawhar Rawwas, William Roberts, Don Eslin, Javier Oesterheld, Randal K. Wada, Devang Pastakia, Virginia Harrod, Kevin Ginn, Raya Saab, Kevin Bielamowicz, Jason Glover, Eugenia Chang, Gina K. Hanna, Daniel Enriquez, Tyler Izatt, Rebecca F Halperin, Abigail Moore, Sara A. Byron, William P D Hendricks, Jeffrey M. Trent

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Children with relapsed central nervous system (CNS tumors), neuroblastoma, sarcomas, and other rare solid tumors face poor outcomes. This prospective clinical trial examined the feasibility of combining genomic and transcriptomic profiling of tumor samples with a molecular tumor board (MTB) approach to make real‑time treatment decisions for children with relapsed/refractory solid tumors.

METHODS: Subjects were divided into three strata: stratum 1-relapsed/refractory neuroblastoma; stratum 2-relapsed/refractory CNS tumors; and stratum 3-relapsed/refractory rare solid tumors. Tumor samples were sent for tumor/normal whole-exome (WES) and tumor whole-transcriptome (WTS) sequencing, and the genomic data were used in a multi-institutional MTB to make real‑time treatment …

Assessment Of Social Vulnerability Impact In Care And Prognosis Of Sinonasal Cancers In The United States, Achilles A. Kanaris, David J. Fei-Zhang, Lily B. Fletcher, Stephanie S. Smith, Urjeet A. Patel, Jill N. D'Souza, Daniel C. Chelius, Anthony M. Sheyn, Jeffrey C. Rastatter

School of Medicine Faculty Publications

Key Points: Social determinants of health interactively influence sinonasal cancer care and prognosis. Housing-transportation and socioeconomic status showed the largest associations with disparities. The social vulnerability index can reveal the social determinants of sinonasal cancers.

Design, Synthesis, X-Ray Crystallography, And Biological Activities Of Covalent, Non-Peptidic Inhibitors Of Sars-Cov-2 Main Protease, Md Ashraf-Uz-Zaman, Teck Khiang Chua, Xin Li, Yuan Yao, Bala Krishna Moku, Chandra Bhushan Mishra, Vasanthi Avadhanula, Pedro A Piedra, Yongcheng Song

Faculty, Staff and Students Publications

Highly contagious SARS-CoV-2 coronavirus has infected billions of people worldwide with flu-like symptoms since its emergence in 2019. It has caused deaths of several million people. The viral main protease (Mpro) is essential for SARS-CoV-2 replication and therefore a drug target. Several series of covalent inhibitors of Mpro were designed and synthesized. Structure-activity relationship studies show that 1) several chloroacetamide- and epoxide-based compounds targeting Cys145 are potent inhibitors with IC50 values as low as 0.49 μM, and 2) Cys44 of Mpro is not nucleophilic for covalent inhibitor design. High resolution X-ray studies revealed the protein-inhibitor interactions and mechanisms of inhibition. …

Development And Validation Of Parent-Reported Gastrointestinal Health Scale In Mecp2 Duplication Syndrome, Davut Pehlivan, Sukru Aras, Daniel G Glaze, Muharrem Ak, Bernhard Suter, Kathleen J Motil

Faculty, Staff and Students Publications

BACKGROUND/AIMS: We aimed to develop a validated patient-reported Gastrointestinal Health Scale (GHS) specific to MECP2 Duplication Syndrome (MDS) to be used in clinical trials.

METHODS: MDS parents completed a Gastrointestinal Health Questionnaire (GHQ) to investigate the most relevant and important items associated with gastrointestinal problems in MECP2-related disorders. Item reduction was executed according to EORTC guidelines. We performed reliability and validity studies for the finalized scale.

RESULTS: A total of 106 surveys were eligible for item reduction and validation processes. The initial 55 items were reduced to 38 items based on parent responses, expert opinion, and initial confirmatory factor analysis …

Impact Of An Integrated Health, Nutrition, And Early Child Stimulation And Responsive Care Intervention Package Delivered To Preterm Or Term Small For Gestational Age Babies During Infancy On Growth And Neurodevelopment: Study Protocol Of An Individually Randomized Controlled Trial In India (Small Babies Trial), Ranadip Chowdhury, Rukman Manapurath, Ingvild Fossgard Sandøy, Ravi Prakash Upadhyay, Neeta Dhabhai, Saijuddin Shaikh, Harish Chellani, Tarun Shankar Choudhary, Abhinav Jain, Jose Martines, Nita Bhandari, Tor A Strand, Sunita Taneja

Faculty, Staff and Student Publications

BACKGROUND: Preterm and term small for gestational age (SGA) babies are at high risk of experiencing malnutrition and impaired neurodevelopment. Standalone interventions have modest and sometimes inconsistent effects on growth and neurodevelopment in these babies. For greater impact, intervention may be needed in multiple domains-health, nutrition, and psychosocial care and support. Therefore, the combined effects of an integrated intervention package for preterm and term SGA on growth and neurodevelopment are worth investigating.

METHODS: An individually randomized controlled trial is being conducted in urban and peri-urban low to middle-socioeconomic neighborhoods in South Delhi, India. Infants are randomized (1:1) into two strata …

Revolutionizing Esophageal Atresia Treatment: Newly Approved Technique To Bypass Surgery, Gabriella Llorente

Mako: NSU Undergraduate Student Journal

Esophageal atresia (EA) is a rare congenital defect where the esophagus, during the early stages of development, does not fully form. This causes there to be no connection between the oral cavity and the stomach. Complications with EA treatment come into play when there is a long gap between the two segments of the esophagus, meaning they cannot simply be sewed together using anastomosis. A new device called Flourish has recently been approved by the FDA and may be a new and more humanitarian approach to solving long gap esophageal atresia in infants.

An Excitatory Projection From The Basal Forebrain To The Ventral Tegmental Area That Underlies Anorexia-Like Phenotypes, Jing Cai, Yanyan Jiang, Yuanzhong Xu, Zhiying Jiang, Claire Young, Hongli Li, Joshua Ortiz-Guzman, Yizhou Zhuo, Yulong Li, Yong Xu, Benjamin R Arenkiel, Qingchun Tong

Faculty, Staff and Students Publications

Maladaptation in balancing internal energy needs and external threat cues may result in eating disorders. However, brain mechanisms underlying such maladaptations remain elusive. Here, we identified that the basal forebrain (BF) sends glutamatergic projections to glutamatergic neurons in the ventral tegmental area (VTA) in mice. Glutamatergic neurons in both regions displayed correlated responses to various stressors. Notably, in vivo manipulation of BF terminals in the VTA revealed that the glutamatergic BF → VTA circuit reduces appetite, increases locomotion, and elicits avoidance. Consistently, activation of VTA glutamatergic neurons reduced body weight, blunted food motivation, and caused hyperactivity with behavioral signs of …

Implementation Of Rapid Genomic Sequencing In Safety-Net Neonatal Intensive Care Units: Protocol For The Virtual Genome Center (Vigor) Proof-Of-Concept Study., Alissa M. D'Gama, Sonia Hills, Jessica Douglas, Vanessa Young, Casie A. Genetti, Monica H. Wojcik, Henry A. Feldman, Timothy W. Yu, Margaret G. Parker, Pankaj B. Agrawal, T. Allcroft, Vineet Bhandari, L. Cantu, D. Honrubia, A. Kritzer, Q. Li, L. Rhein, R. Rothstein, O. Salinas, A. Santana, K. Schmitz-Abe, A. Serna, F. Shapiro, A. B. Shenoy, L. Simoncini, B. Sinha, A. S. Verran, A. Sousa, M. T. Newsam

Cooper Medical School of Rowan University Departmental Research

INTRODUCTION: Rapid genomic sequencing (rGS) in critically ill infants with suspected genetic disorders has high diagnostic and clinical utility. However, rGS has primarily been available at large referral centres with the resources and expertise to offer state-of-the-art genomic care. Critically ill infants from racial and ethnic minority and/or low-income populations disproportionately receive care in safety-net and/or community settings lacking access to state-of-the-art genomic care, contributing to unacceptable health equity gaps. VIrtual GenOme CenteR is a 'proof-of-concept' implementation science study of an innovative delivery model for genomic care in safety-net neonatal intensive care units (NICUs).

METHODS AND ANALYSIS: We developed a …

Parenting Influences On Frontal Lobe Gray Matter And Preterm Toddlers' Problem-Solving Skills, Josselyn S Muñoz, Megan E Giles, Kelly A Vaughn, Ying Wang, Susan H Landry, Johanna R Bick, Dana M Demaster

Faculty, Staff and Student Publications

Children born preterm often face challenges with self-regulation during toddlerhood. This study examined the relationship between prematurity, supportive parent behaviors, frontal lobe gray matter volume (GMV), and emotion regulation (ER) among toddlers during a parent-assisted, increasingly complex problem-solving task, validated for this age range. Data were collected from preterm toddlers (n = 57) ages 15–30 months corrected for prematurity and their primary caregivers. MRI data were collected during toddlers’ natural sleep. The sample contained three gestational groups: 22–27 weeks (extremely preterm; EPT), 28–33 weeks (very preterm; VPT), and 34–36 weeks (late preterm; LPT). Older toddlers became more compliant as …

Clinical Presentation, Classification, And Outcomes Of Cardiogenic Shock In Children, Kriti Puri, Jacob C Jentzer, Joseph A Spinner, Kyle D Hope, Iki Adachi, Sebastian C Tume, Hari P Tunuguntla, Swati Choudhry, Antonio G Cabrera, Jack F Price

Faculty, Staff and Students Publications

Background: Despite growing cardiogenic shock (CS) research in adults, the epidemiology, clinical features, and outcomes of children with CS are lacking.

Objectives: This study sought to describe the epidemiology, clinical presentation, hospital course, risk factors, and outcomes of CS among children hospitalized for acute decompensated heart failure (ADHF).

Methods: We examined consecutive ADHF hospitalizations (< 21 years of age) from a large single-center retrospective cohort. Patients with CS at presentation were analyzed and risk factors for CS and for the primary outcome of in-hospital mortality were identified. A modified Society for Cardiovascular Angiography and Interventions shock classification was created and patients were staged accordingly.

Results: A total of 803 hospitalizations for ADHF were identified in 591 unique patients (median age 7.6 years). CS occurred in 207 (26%) hospitalizations. ADHF hospitalizations with CS were characterized by worse systolic function (P = 0.040), higher B-type natriuretic peptide concentration (P = 0.032), …

Relationship Between Obesity And Global Longitudinal Strain In The Pediatric Single Ventricle Fontan Population Across Ventricular Morphologies., Nitin Madan, Doaa Aly, Melanie Kathol, Amulya Buddhavarapu, Thomas Rieth, Ashley K. Sherman, Daniel Forsha

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Obesity is associated with diminished myocardial function as measured by strain echocardiography in children and young adults with normal cardiac anatomy. Data are lacking about the effect of obesity on myocardial strain in patients with a single ventricle. In this study, the relationship between body mass index (BMI) and single ventricle myocardial strain in the Fontan population was assessed.

METHODS AND RESULTS: Thirty-eight abnormal BMI Fontan cases (21 overweight and 17 obese) and 30 normal BMI Fontan controls matched based on single ventricular morphology, age, and sex were included in the study. Ventricular morphology was categorized as single right …

Neonatal Intensive Care Unit Resource Use For Infants At 22 Weeks' Gestation In The Us, 2008-2021, Matthew A Rysavy, Monica M Bennett, Kaashif A Ahmad, Ravi M Patel, Zubin S Shah, Dan L Ellsbury, Reese H Clark, Veeral N Tolia

Faculty, Staff and Student Publications

IMPORTANCE: During the past decade, clinical guidance about the provision of intensive care for infants born at 22 weeks' gestation has changed. The impact of these changes on neonatal intensive care unit (NICU) resource utilization is unknown.

OBJECTIVE: To characterize recent trends in NICU resource utilization for infants born at 22 weeks' gestation compared with other extremely preterm infants (≤28 weeks' gestation) and other NICU-admitted infants.

DESIGN, SETTING, AND PARTICIPANTS: This is a serial cross-sectional study of 137 continuously participating NICUs in 29 US states from January 1, 2008, through December 31, 2021. Participants included infants admitted to the NICU. …

Opportunities For Pharmacogenetic Testing To Guide Dosing Of Medications In Youths With Medicaid., Sonya Tang Girdwood, Matthew Hall, James W. Antoon, Kathryn Kyler, Derek J. Williams, Samir S. Shah, Lucas E. Orth, Jennifer Goldman, James A. Feinstein, Laura B. Ramsey

Manuscripts, Articles, Book Chapters and Other Papers

IMPORTANCE: There are an increasing number of medications with a high level of evidence for pharmacogenetic-guided dosing (PGx drugs). Knowledge of the prevalence of dispensings of PGx drugs and their associated genes may allow hospitals and clinical laboratories to determine which pharmacogenetic tests to implement.

OBJECTIVES: To investigate the prevalence of outpatient dispensings of PGx drugs among Medicaid-insured youths, determine genes most frequently associated with PGx drug dispenses, and describe characteristics of youths who were dispensed at least 1 PGx drug.

DESIGN, SETTING, AND PARTICIPANTS: This serial cross-sectional study includes data from 2011 to 2019 among youths aged 0 to …

Hospital-Level Nicu Capacity, Utilization, And 30-Day Outcomes In Texas, David C Goodman, Patrick Stuchlik, Cecilia Ganduglia-Cazaban, Jon E Tyson, Joanna Leyenaar, Elenir B C Avritscher, Mathew Rysavy, Kanekal S Gautham, David Lynch, Therese A Stukel

Faculty, Staff and Student Publications

IMPORTANCE: Risk-adjusted neonatal intensive care unit (NICU) utilization and outcomes vary markedly across regions and hospitals. The causes of this variation are poorly understood.

OBJECTIVE: To assess the association of hospital-level NICU bed capacity with utilization and outcomes in newborn cohorts with differing levels of health risk.

DESIGN, SETTING, AND PARTICIPANTS: This population-based retrospective cohort study included all Medicaid-insured live births in Texas from 2010 to 2014 using linked vital records and maternal and newborn claims data. Participants were Medicaid-insured singleton live births (LBs) with birth weights of at least 400 g and gestational ages between 22 and 44 weeks. …

Prenatal Diagnosis Of A Ductal-Dependent Branch Pulmonary Artery: Extra Vessels In The 3-Vessel And Trachea View., Anmol Goyal, Maria Kiaffas, Tara Swanson, Melanie Kathol, Sanket Shah, Nitin Madan

Manuscripts, Articles, Book Chapters and Other Papers

Obtaining a three-vessel (3V) and three-vessel and trachea (3VT) view from the fetal upper mediastinum is now considered to be part of standard imaging protocol for routine obstetric cardiac screening examinations. We report two fetal cases of an anomalous pulmonary artery origin, utilizing the standard 3V and 3VT views. Further imaging led to a rare diagnosis of bilateral ductus arteriosus with discontinuous branch pulmonary arteries in the absence of any other congenital heart defect. We briefly discuss the imaging features, differential diagnoses, and management of this rare entity.