Pediatrics Commons^™

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.

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.

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 …

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 …

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 …

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. …

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.

Assessing Pattern Of The Pediatric Multisystem Inflammatory Syndrome (Pmis) In Children During The Covid-19 Pandemic: Experience From The Emergency Department Of Tertiary Care Center Of A Low-Middle-Income Country, Saleem Akhtar, Iqra Anis, Kumar Nirdosh, Muhammad Ihsan Tayyab Ihsan, Ahmed Raheem Buksh, Surraiya Bano

Department of Paediatrics and Child Health

Background: Pediatric Multisystem Inflammatory Syndrome (PMIS) is a hyperinflammatory condition affecting multiple organs in children, often resembling incomplete Kawasaki Disease during later phases of COVID-19 infection. Data on PMIS in low-middle-income countries, particularly in emergency department settings, is limited.
Objectives: This prospective observational study at Aga Khan University Hospital, Karachi, aimed to determine the frequency, clinical presentation patterns, and laboratory parameters of children with PMIS visiting the emergency department during the COVID-19 pandemic. Secondary objectives included assessing factors associated with in-hospital mortality.
Methods: From March 2020 to September 2021, patients meeting World Health Organization PMIS criteria were enrolled. COVID-19 testing …

Exploring Referral And Service Utilization Patterns Within An Outpatient Interdisciplinary Pediatric Chronic Pain Program, Aimee Hildenbrand, Christina M Amaro, Benjamin Bear, Catherine Soprano, Katherine S Salamon

Department of Medicine Faculty Papers

PURPOSE: We examine referral sources and clinical characteristics for youth presenting to an outpatient interdisciplinary pediatric chronic pain program.

PATIENTS AND METHODS: Referral data were extracted from the electronic health record. PROMIS Pediatric Anxiety and Pain Interference Scales were administered at an initial evaluation visit.

RESULTS: The program received 1488 referrals between 2016 and 2019, representing 1338 patients, with increasing volume of referrals over time. Referrals were primarily from orthopedics (19.6%), physical medicine and rehabilitation (18.8%), neurology (14.4%), and rheumatology (12.6%). Patients referred were primarily female (75.4%), White (80.1%), English-speaking (98.4%) adolescents (median=15.0 years). Of those referred, 732 (54.7%) attended …

Advancing Virtual At-Home Care For Community Health Center Patients Using Patient Self-Care Tools, Technology, And Education [Letter], Cheryl Modica, Joy H. Lewis, Curtis Bay

SOMA Faculty Publications

Abstract

Introduction

Health centers are community-based, patient directed primary care providers that offer accessible, high-quality primary care within medically underserved communities. Screening for cancer and managing complex chronic conditions such as diabetes, hypertension, obesity, and depression are vital services for the vulnerable populations seen by community health centers. Delivering care for complex chronic conditions and preventive services using virtual models that integrate self-care tools and technology is an important approach to increasing access for hard-to-reach patients served by health centers.

Objective

This study aimed to explore the use of a virtual care model, applied using a systems approach and patient-driven …

Large Single Center Experience With Extubation During Neonatal And Pediatric Extracorporeal Membrane Oxygenation, Cara Holton, Johanna I. Orrick, Debra E. Newton, Jenna Miller

Presentations

Introduction: Extubation of neonatal and pediatric patients supported with extracorporeal membrane oxygenation (ECMO) may avoid ventilator induced lung injury, decrease neurosedative exposure, and improve rehabilitation and mobility. Few studies have evaluated incidence and outcomes of pediatric patients extubated during ECMO. Methods: A single-center retrospective cohort study was performed to describe our experience with extubation on ECMO. All patients extubated on ECMO from 2013-2022 were included. Patients who had a tracheostomy prior to ECMO cannulation were excluded. Patients who had a tracheostomy placed during their ECMO run were included if extubation took place prior to tracheostomy placement. Results: Forty patients were …

Community-Acquired Pneumonia, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Clinical Practice Guidelines)

No abstract provided.

Successful Hepatectomy, Anhepatic State, And Liver Transplant On Ecmo, Jenna Miller, Kari L. Davidson, Bhargava Mullapudi, Richard J. Hendrickson, Ryan T. Fischer, Lisa Conley, Wes Ware, Michelle Mckain, Tara Benton

Presentations

We discuss the case of a 13-month-old previously healthy male who presented with acute liver failure (ALF) of unknown etiology. He progressed to fulminant cardiopulmonary failure requiring mechanical ventilation and high dose inotropes with persistent lactate >20. He was cannulated to VA ECMO and tandem continuous renal replacement therapy (CRRT) with single pass albumin dialysis (SPAD) and plasma exchange (PLEX) therapies. He required high dose inotropes on ECMO and had persistently elevated lactate despite maximal medical therapy. Limited imaging studies suggested cirrhotic liver and possible intestinal pneumatosis. Due to evidence of fulminant ALF and potential for a reversible process, he …

Utility Of The 3di Short Version In The Identification And Diagnosis Of Autism In Children At The Kenyan Coast., Patricia Kipkemoi, Symon Kariuki, Joseph Gona, Felicita Wangeci Mwangi, Martha Kombe, Collins Kipkoech, Paul Mwangi, William Mandy, Amina Abubakar, Charles Newton

Institute for Human Development, East Africa

Introduction: The precise epidemiological burden of autism is unknown because of the limited capacity to identify and diagnose the disorder in resource-constrained settings, related in part to a lack of appropriate standardised assessment tools and health care experts. We assessed the reliability, validity, and diagnostic accuracy of the Developmental Diagnostic Dimensional Interview (3Di) in a rural setting on the Kenyan coast.

Methods: Using a large community survey of neurodevelopmental disorders (NDDs), we administered the 3Di to 2,110 children aged between 6 years and 9 years who screened positive or negative for any NDD and selected 242 who had specific symptoms …

Double Aortic Arch With Atresia Of The Left Aortic Arch Proximal To The Left Common Carotid Artery In A Patient With Phace Syndrome – A Management Conundrum, Mohamed Aashiq Abdul Ghayum, Anmol Goyal, Aliessa P. Barnes, Sanket Shah

Posters

Background Cardiac anomalies in PHACE syndrome (P: posterior fossa anomalies, H: hemangiomas, A: arterial lesions, C: cardiac abnormalities, E: abnormalities of the eye) often consists of complex aortic arch anomalies with double aortic arch (DAA) being an uncommon association. Atresia of the proximal segment of the left arch in DAA is extremely rare and can pose a diagnostic and management conundrum due to formation of a loose vascular ring and asymptomatic nature as seen in our case. Case Description A 5-year-old male, diagnosed with PHACE syndrome, at a different center was noted to have an incidental finding on head imaging …

Transposition Of The Great Arteries With Intramural Left Main Coronary Artery: Salient Imaging Findings And Description Of Two Operative Techniques, Joshua Holbert, Manasa Gadiraju, Sanket Shah, Edo Bedzra

Posters

Background: Transposition of the great arteries (TGA) is a common cyanotic critical congenital heart disease, with a feasible prenatal diagnosis. Arterial switch operation (ASO) with/without VSD closure is the preferred surgical approach with added challenge when an intramural coronary artery (IMC) is present. ASO is technically quite demanding with IMC, and incidence of post-operative complications and mortality are potentially higher. We present our recent TGA-IMC experience focusing on salient features identified on echocardiography, CT and invasive angiograms, as well as variations in ASO surgical techniques for repair. Methods: Our echo imaging acquisition required high frequency transducers and shallow image depth …