Pediatrics | Open Access Articles | Digital Commons Network™

Optimizing Antibiotic Durations Of Therapy In Pediatric Community-Acquired Pneumonia, Gregory Cook, Shubho Sarkar

School of Medicine Faculty Publications

Infectious Diseases Week, IDWeek 2023, October 11-15, 2023, Boston, MA

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Effects Of Covid-19 Vaccination On Pro-Coagulant State Of A Protein S-Deficient Pediatric Patient: New Hope For Treatment Of The Deficiency, Diana Polania-Villanueva, M. Kumar, Lekha Thangada, Jone Garai, Jovanny Zabaleta, Rinku Majumder

School of Medicine Faculty Publications

ISTH 2023 Congress, June 24 - 28, 2023, Montreal, Canada

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Antiphosphatidylserine/Prothrombin Antibodies In Childhood Sle Are Associated With Lupus Anticoagulant Positivity, S. Chugh, R. Pilania, A. Kler, C. Hans, Narender Kumar, S. Singh, J. Ahluwalia

School of Medicine Faculty Publications

ISTH 2023 Congress, June 24 - 28, 2023, Montreal, Canada

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Left Juxtaposition Of The Right Atrial Appendage: Pitfalls In Diagnosis, Kanya Singhapakdi, Wesam Sourour, Thomas Kimball

School of Medicine Faculty Publications

Several congenital anomalies of the right atrial appendage (RAA) have been described including aneurysm, herniation (in association with a pericardial defect), and left juxtaposition. The left juxtaposition of the RAA (LJRAA), first described by Birmingham in 1893 and subsequently introduced by Dixon in 1954, is usually associated with complex cardiac malformations such as obstruction of the left ventricular outflow tract. In this case report, we will describe an unusual variant of LJRAA in the absence of any other cardiac defects, which was initially misinterpreted as an aortic dissection. The correct diagnosis was made after careful reinterpretation and the use of …

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Cystic Fibrosis Year In Review 2022, Jeeyeon Kim, Benjamin Lyman, Adrienne P. Savant

School of Medicine Faculty Publications

Remarkable medical advancements have been made for people with cystic fibrosis (CF) in recent years, with an abundance of research continuing to be conducted worldwide. With concern for limitations in access to highly effective CFTR modulators, as well as the recent Coronavirus Disease-19 pandemic, there has been a consistent effort to understand and improve CF screening, disease burden, diagnosis, and management. Our aim in this review is to present articles from 2022 with an emphasis on clinically relevant studies. We hope this will serve as a broad overview of the research published in the past year.

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Left Pulmonary Artery Occlusion Following Device Closure Of Patent Ductus Arteriosus In Premature Infants, Jesus C. Jaile, Ernest Siwik, Sergio Bartakian

School of Medicine Faculty Publications

Background: Device closure of a patent ductus arteriosus (PDA) is rapidly evolving, with the Amplatzer Piccolo Occluder (Abbott) receiving US Food and Drug Administration approval and becoming the first device approved for PDA closure in patients ≥700 g. We report on the first known cases of complete left pulmonary artery (LPA) occlusion following Piccolo closure of a PDA in premature infants. Methods: Retrospective chart analysis of PDA closures. Results: We have performed over 50 cases of Piccolo device closure of the PDA in preterm neonates in the past 2 years, with these 2 cases representing our only complications (4%). This …

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Protein S Antibody As An Adjunct Therapy For Hemophilia B, Hope P. Wilson, Aliyah Pierre, Ashley L. Paysse, Narender Kumar, Brian C. Cooley, Pratyadipta Rudra, Adrianne W. Dorsey, Diana Polania-Villanueva, Sabyasachi Chatterjee, Maissaa Janbain, Maria C. Velez, Rinku Majumder

School of Medicine Faculty Publications

ABSTRACT: Hemophilia B (HB) is caused by an inherited deficiency of plasma coagulation factor IX (FIX). Approximately 60% of pediatric patients with HB possess a severe form of FIX deficiency (< 1% FIX activity). Treatment typically requires replacement therapy through the administration of FIX. However, exogenous FIX has a limited functional half-life, and the natural anticoagulant protein S (PS) inhibits activated FIX (FIXa). PS ultimately limits thrombin formation, which limits plasma coagulation. This regulation of FIXa activity by PS led us to test whether inhibiting PS would extend the functional half-life of FIX and thereby prolong FIX-based HB therapy. We assayed clotting times and thrombin generation to measure the efficacy of a PS antibody for increasing FIX activity in commercially obtained plasma and plasma from pediatric patients with HB. We included 11 pediatric patients who lacked additional comorbidities and coagulopathies. In vivo, we assessed thrombus formation in HB mice in the presence of the FIXa ± PS antibody. We found an accelerated rate of clotting in the presence of PS antibody. Similarly, the peak thrombin formed was significantly greater in the presence of the PS antibody, even in plasma from patients with severe HB. Furthermore, HB mice injected with PS antibody and FIX had a 4.5-fold higher accumulation of fibrin at the thrombus induction site compared with mice injected with FIX alone. Our findings imply that a PS antibody would be a valuable adjunct to increase the effectiveness of FIX replacement therapy in pediatric patients who have mild, moderate, and severe HB.

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Small Molecules Targeting Insm1 For The Treatment Of High-Risk Neuroblastoma, Michael S. Lan, Chiachen Chen

School of Medicine Faculty Publications

Human neuroblastoma (NB) is the most common childhood extracranial tumor arising from the sympathetic nervous system. It is also a clinically heterogeneous disease that ranges from spontaneous regression to high-risk stage 4 disease. The cause of this disease remains elusive. However, the amplification of NMYC oncogene occurred in roughly 30% of NB patients, which strongly correlated with the advanced stage of disease subtype and the worse prognosis status. We discovered that N-Myc oncoprotein binds and activates INSM1, a zinc-finger transcription factor of neuroendocrine tumors. We also found that INSM1 modulates N-Myc stability mediated through PI3K/AKT/GSK3β signaling pathway. Therefore, INSM1 emerges …

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A Cryptic Microdeletion Del(12)(P11.21p11.23) Within An Unbalanced Translocation T(7;12)(Q21.13;Q23.1) Implicates New Candidate Loci For Intellectual Disability And Kallmann Syndrome, Afif Ben-Mahmoud, Shotaro Kishikawa, Vijay Gupta, Natalia T. Leach, Yiping Shen, Oana Moldovan, Himanshu Goel, Bruce Hopper, Kara Ranguin, Nicolas Gruchy, Saskia M. Maas, Yves Lacassie, Soo Hyun Kim, Woo Yang Kim, Bradley J. Quade, Cynthia C. Morton, Cheol Hee Kim, Lawrence C. Layman, Hyung Goo Kim

School of Medicine Faculty Publications

In a patient diagnosed with both Kallmann syndrome (KS) and intellectual disability (ID), who carried an apparently balanced translocation t(7;12)(q22;q24)dn, array comparative genomic hybridization (aCGH) disclosed a cryptic heterozygous 4.7 Mb deletion del(12)(p11.21p11.23), unrelated to the translocation breakpoint. This novel discovery prompted us to consider the possibility that the combination of KS and neurological disorder in this patient could be attributed to gene(s) within this specific deletion at 12p11.21-12p11.23, rather than disrupted or dysregulated genes at the translocation breakpoints. To further support this hypothesis, we expanded our study by screening five candidate genes at both breakpoints of the chromosomal translocation …

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Two Pediatric Cases Within A Familial Cluster And Hereditary Diffuse Gastric Carcinoma: A Tale Of 2 Sisters, Lacey Falgout, Lawrence L. Gensler

School of Medicine Faculty Publications

Hereditary diffuse gastric cancer is a familial form of poorly differentiated signet ring cell carcinoma (SRCC) caused by a mutation in the CDH1/E-cadherin gene-mediating cell adhesion. CDH1 mutations are inherited in an autosomal dominant fashion and exhibit a high level of penetrance. SRCC diagnosis is exceedingly rare in the pediatric population. We report a case of SRCC in 2 sisters (10 and 15 years) and their father (41 years).

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Persistent Increase In Serum Ferritin Levels Despite Converting To Permanent Vascular Access In Pediatric Hemodialysis Patients: Pediatric Nephrology Research Consortium Study, Ali Mirza Onder, Md Abu Yusuf Ansari, Fang Deng, Matthew M. Grinsell, Larry Patterson, Jennifer Jetton, Sahar Fathallah-Shaykh, Daniel Ranch, Diego Aviles, Lawrence Copelovitch, Eileen Ellis, Vimal Chadha, Ayah Elmaghrabi, Jen-Jar Lin, Lavjay Butani, Maha Haddad, Olivera Marsenic, Paul Brakeman, Raymond Quigley, H Stella Shin, Rouba Garro, Rupesh Raina, Craig B. Langman

School of Medicine Faculty Publications

Our objective was to examine serum ferritin trends after conversion to permanent vascular access (PVA) among children who started hemodialysis (HD) using tunneled cuffed catheters (TCC). Retrospective chart reviews were completed on 98 subjects from 20 pediatric HD centers. Serum ferritin levels were collected at the creation of PVA and for two years thereafter. There were 11 (11%) arteriovenous grafts (AVG) and 87 (89%) arteriovenous fistulae (AVF). Their mean TCC use was 10.4 ± 17.3 months. Serum ferritin at PVA creation was elevated at 562.64 ± 492.34 ng/mL, increased to 753.84 ± 561.54 ng/mL (p = < 0.001) in the first year and remained at 759.60 ± 528.11 ng/mL in the second year (p = 0.004). The serum ferritin levels did not show a statistically significant linear association with respective serum hematocrit values. In a multiple linear regression model, there were three predictors of serum ferritin during the first year of follow-up: steroid-resistant nephrotic syndrome as primary etiology (p = 0.035), being from a center that enrolled >10 cases (p = …

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Parents’ Perspectives On The Utility Of Genomic Sequencing In The Neonatal Intensive Care Unit, Amy A. Lemke, Michelle L. Thompson, Emily C. Gimpel, Katelyn C. Mcnamara, Carla A. Rich, Candice R. Finnila, Meagan E. Cochran, James M.J. Lawlor, Kelly M. East, Kevin M. Bowling, Donald R. Latner, Susan M. Hiatt, Michelle D. Amaral, Whitley V. Kelley, Veronica Greve, David E. Gray, Stephanie A. Felker, Hannah Meddaugh, Ashley Cannon, Amanda Luedecke, Kelly E. Jackson, Laura G. Hendon, Hillary M. Janani, Marla Johnston, Lee Ann Merin, Sarah L. Deans, Carly Tuura, Trent Hughes

School of Medicine Faculty Publications

Background: It is critical to understand the wide-ranging clinical and non-clinical effects of genome sequencing (GS) for parents in the NICU context. We assessed parents’ experiences with GS as a first-line diagnostic tool for infants with suspected genetic conditions in the NICU. Methods: Parents of newborns (N = 62) suspected of having a genetic condition were recruited across five hospitals in the southeast United States as part of the SouthSeq study. Semi-structured interviews (N = 78) were conducted after parents received their child’s sequencing result (positive, negative, or variants of unknown significance). Thematic analysis was performed on all interviews. Results: …

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Symptom Management Care Pathway Adaptation Process And Specific Adaptation Decisions, Emily Vettese, Farha Sherani, Allison A. King, Lolie Yu, Catherine Aftandilian, Christina Baggott, Vibhuti Agarwal, Ramamoorthy Nagasubramanian, Kara M. Kelly, David R. Freyer, Etan Orgel, Scott M. Bradfield, Wade Kyono, Michael Roth, Lisa M. Klesges, Melissa Beauchemin, Allison Grimes, George Tomlinson, L. Lee Dupuis, Lillian Sung

School of Medicine Faculty Publications

BACKGROUND: There is substantial heterogeneity in symptom management provided to pediatric patients with cancer. The primary objective was to describe the adaptation process and specific adaptation decisions related to symptom management care pathways based on clinical practice guidelines. The secondary objective evaluated if institutional factors were associated with adaptation decisions. METHODS: Fourteen previously developed symptom management care pathway templates were reviewed by an institutional adaptation team composed of two clinicians at each of 10 institutions. They worked through each statement for all care pathway templates sequentially. The institutional adaptation team made the decision to adopt, adapt or reject each statement, …

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What To Look For In Relationships: Development, Inter-Rater Reliability, And Initial Validity Estimates For A Young Child-Caregiver Relationship Assessment, Joy D. Osofsky, Margaret A. Fields-Olivieri, Andrew L. Frazer, Rebecca A. Graham, Bethany H. Mccurdy, Carl F. Weems

School of Medicine Faculty Publications

Introduction: Observational assessments are important for understanding a range of behaviors and emotions in the young child-caregiver relationship. This paper provides initial data on a multidimensional assessment for professionals who work with young children and their caregivers, the What to Look for in Relationships (WLR). The WLR was designed to assist providers in evaluating strengths and areas for improvement in five areas of young child-caregiver relationship dimensions. This paper reports on the development, interrater reliability, initial convergent and discriminant validity, and incremental utility of the scales. Methods: Data were collected from caregiver-child dyads, who participated in a semi-structured observational caregiver-child …

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Minimalistic Approach To Enhanced Recovery After Pediatric Scoliosis Surgery, Scott A. Barnett, Bryant M. Song, Matthew Bauer, Matthew E. Nungesser, Claudia Leonardi, Michael J. Heffernan

School of Medicine Faculty Publications

Purpose: Prior studies of enhanced recovery protocols (ERP) have been conducted at large institutions with abundant resources. These results may not apply at institutions with less resources directed to quality improvement efforts. The purpose of this study was to assess the value of a minimalistic enhanced recovery protocol in reducing length of stay (LOS) following PSF for adolescent idiopathic scoliosis. We hypothesized that accelerated transition to oral pain medications and mobilization alone could shorten hospital length of stay in the absence of a formal multimodal pain regimen. Methods: AIS patients aged 10–18 who underwent PSF at a tertiary pediatric hospital …

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Clinical Practice Guideline Recommendation Summaries For Pediatric Oncology Health Care Professionals: A Qualitative Study, Nancy Santesso, Melissa Beauchemin, Paula D. Robinson, Alexandra M. Walsh, Aaron J. Sugalski, Tammy Lo, Ha Dang, Brian T. Fisher, Allison C. Grimes, Andrea Rothfus Wrightson, Lolie C. Yu, Lillian Sung, L. Lee Dupuis

School of Medicine Faculty Publications

Objective To develop a summary format of clinical practice guideline (CPG) recommendations to improve understandability among health care professionals. Methods We developed a summary format based on current research and used the "Think Aloud"technique in one-on-one cognitive interviews to iteratively improve it. Interviews of health care professionals from Children's Oncology Group-member, National Cancer Institute Community Oncology Research Program sites were conducted. After every five interviews (a round), responses were reviewed, and changes made to the format until it was well understood and no new, substantive suggestions for revision were raised. We took a directed (deductive) approach to content analysis of …

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Yoga And Mindfulness For Social-Emotional Development And Resilience In 3–5 Year-Old Children: Non-Randomized, Controlled Intervention, Alessandra N. Bazzano, Yaoyao Sun, Yuanhao Zu, Julia M. Fleckman, Emma A. Blackson, Tejal Patel, Angie Shorty-Belisle, Keith H. Liederman, Cody Roi

School of Medicine Faculty Publications

Background: Early childhood is the key life course period for development of social-emotional skills, providing the foundation for school readiness and resilience in later life. Age-appropriate yoga and mindfulness programs may contribute to the development of critical skills in children. Young children from minoritized communities that face structural racism and health disparities may benefit from programs that support social-emotional development and contribute to future academic success. Systematic reviews of yoga interventions for young children have indicated the potential for effectiveness in supporting social-emotional development, executive function, and physical activity. However, studies of yoga and mindfulness with non-White children are sparse …

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The Diagnosis Of Severe Combined Immunodeficiency: Implementation Of The Pidtc 2022 Definitions, Christopher C. Dvorak, Elie Haddad, Jennifer Heimall, Elizabeth Dunn, Morton J. Cowan, Sung-Yun Pai, Neena Kapoor, Lisa Forbes Satter, Rebecca H. Buckley, Richard J. O'Reilly, Sharat Chandra, Jeffrey J. Bednarski, Olatundun Williams, Ahmad Rayes, Theodore B. Moore, Christen L. Ebens, Blachy J. Davila Saldana, Aleksandra Petrovic, Deepak Chellapandian, Geoffrey D. E. Cuvelier, Mark T. Vander Lugt, Emi H. Caywood, Shanmuganathan Chandrakasan, Hesham Eissa, Frederick D. Goldman, Evan Shereck, Victor M. Aquino, Kenneth B. Desantes, Lolie Yu, Et Al

School of Medicine Faculty Publications

Background: Shearer et al in 2014 articulated well-defined criteria for the diagnosis and classification of severe combined immunodeficiency (SCID) as part of the Primary Immune Deficiency Treatment Consortium's (PIDTC's) prospective and retrospective studies of SCID. Objective: Because of the advent of newborn screening for SCID and expanded availability of genetic sequencing, revision of the PIDTC 2014 Criteria was needed. Methods: We developed and tested updated PIDTC 2022 SCID Definitions by analyzing 379 patients proposed for prospective enrollment into Protocol 6901, focusing on the ability to distinguish patients with various SCID subtypes. Results: According to PIDTC 2022 Definitions, 18 of 353 …

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Mechanisms Of Recovery: Community Perceptions Of Change And Growth Following Multiple Disasters, Howard Osofsky, Joy Osofsky, Leia Y. Saltzman, Estilla Lightfoot, Jule De King, Tonya C. Hansel

School of Medicine Faculty Publications

Introduction and purpose: The geographic location of the Gulf South leaves communities in continuous threat, response, and recovery disaster cycles. Hurricane Katrina in 2005 provided an opportunity to study disaster mental health. Less than 5 years after the storm, many Hurricane Katrina survivors were impacted again by the Deepwater Horizon Gulf oil spill. Despite adversities impacting Gulf communities, over 90% of participants reported they were resilient. The purpose of this study was to improve the understanding of the mechanisms that contribute to strengths following adversity in communities affected by repeated disasters. Specifically, we focused on survivor perceptions of personal, spiritual, …

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The Gain Of Function Scn1a Disorder Spectrum: Novel Epilepsy Phenotypes And Therapeutic Implications, Andreas Brunklaus, Tobias Brünger, Tony Feng, Carmen Fons, Anni Lehikoinen, Eleni Panagiotakaki, Mihaela Adela Vintan, Joseph Symonds, James Andrew, Alexis Arzimanoglou, Sarah Delima, Julie Gallois, Donncha Hanrahan, Gaetan Lesca, Stewart Macleod, Dragan Marjanovic, Amy Mctague, Noemi Nuñez-Enamorado, Eduardo Perez-Palma, M. Scott Perry, Karen Pysden, Sophie J. Russ-Hall, Ingrid E. Scheffer, Krystal Sully, Steffen Syrbe, Ulvi Vaher, Murugan Velayutham

School of Medicine Faculty Publications

Brain voltage-gated sodium channel NaV1.1 (SCN1A) loss-of-function variants cause the severe epilepsy Dravet syndrome, as well as milder phenotypes associated with genetic epilepsy with febrile seizures plus. Gain of function SCN1A variants are associated with familial hemiplegic migraine type 3. Novel SCN1A-related phenotypes have been described including early infantile developmental and epileptic encephalopathy with movement disorder, and more recently neonatal presentations with arthrogryposis. Here we describe the clinical, genetic and functional evaluation of affected individuals. Thirty-five patients were ascertained via an international collaborative network using a structured clinical questionnaire and from the literature. We performed whole-cell voltage-clamp electrophysiological recordings comparing …

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Metabolic Pathways Enriched According To Erg Status Are Associated With Biochemical Recurrence In Hispanic/Latino Patients With Prostate Cancer, Natalia L. Acosta-Vega, Rodolfo Varela, Jorge Andrés Mesa, Jone Garai, Melody C. Baddoo, Alberto Gómez-Gutiérrez, Silvia J. Serrano-Gómez, Marcela Nuñez Lemus, Martha Lucía Serrano, Jovanny Zabaleta, Alba L. Combita, María Carolina Sanabria-Salas

School of Medicine Faculty Publications

Background: The role of ERG-status molecular subtyping in prognosis of prostate cancer (PCa) is still under debate. In this study, we identified differentially expressed genes (DEGs) according to ERG-status to explore their enriched pathways and implications in prognosis in Hispanic/Latino PCa patients. Methods: RNA from 78 Hispanic PCa tissues from radical prostatectomies (RP) were used for RNA-sequencing. ERGhigh/ERGlow tumor groups were determined based on the 1.5-fold change median expression in non-tumor samples. DEGs with a False Discovery Rate (FDR) < 0.01 and a fold change >2 were identified between ERGhigh and ERGlow tumors and submitted to enrichment analysis in MetaCore. Survival and association analyses were performed …

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Tachyarrhythmias During Hospitalization For Covid-19 Or Multisystem Inflammatory Syndrome In Children And Adolescents, Audrey Dionne, Kevin G. Friedman, Cameron C. Young, Margaret M. Newhams, Suden Kucukak, Ashley M. Jackson, Julie C. Fitzgerald, Laura S. Smallcomb, Sabrina Heidemann, Gwenn E. Mclaughlin, Katherine Irby, Tamara T. Bradford, Steven M. Horwitz, Laura L. Loftis, Vijaya L. Soma, Courtney M. Rowan, Michele Kong, Natasha B. Halasa, Keiko M. Tarquinio, Adam J. Schwarz, Janet R. Hume, Shira J. Gertz, Katharine N. Clouser, Christopher L. Carroll, Kari Wellnitz, Melissa L. Cullimore, Sule Doymaz, Emily R. Levy

School of Medicine Faculty Publications

BACKGROUND: Cardiac complications related to COVID-19 in children and adolescents include ventricular dysfunction, myocarditis, coronary artery aneurysm, and bradyarrhythmias, but tachyarrhythmias are less understood. The goal of this study was to evaluate the frequency, characteristics, and outcomes of children and adolescents experiencing tachyarrhythmias while hospitalized for acute severe COVID-19 or multisystem inflammatory syndrome in children. METHODS AND RESULTS: This study involved a case series of 63 patients with tachyarrhythmias reported in a public health surveillance registry of patients aged 15, 2020, to December 31, 2021, at 63 US hospitals. Patients with tachyarrhythmias were compared with patients with severe COVID-19–related complications …

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Cryptococcal Hsf3 Controls Intramitochondrial Ros Homeostasis By Regulating The Respiratory Process, Xindi Gao, Yi Fu, Shengyi Sun, Tingyi Gu, Yanjian Li, Tianshu Sun, Hailong Li, Wei Du, Chenhao Suo, Chao Li, Yiru Gao, Yang Meng, Yue Ni, Sheng Yang, Tian Lan, Sixiang Sai, Jiayi Li, Kun Yu, Ping Wang, Chen Ding

School of Medicine Faculty Publications

Mitochondrial quality control prevents accumulation of intramitochondrial-derived reactive oxygen species (mtROS), thereby protecting cells against DNA damage, genome instability, and programmed cell death. However, underlying mechanisms are incompletely understood, particularly in fungal species. Here, we show that Cryptococcus neoformans heat shock factor 3 (CnHsf3) exhibits an atypical function in regulating mtROS independent of the unfolded protein response. CnHsf3 acts in nuclei and mitochondria, and nuclear- and mitochondrial-targeting signals are required for its organelle-specific functions. It represses the expression of genes involved in the tricarboxylic acid cycle while promoting expression of genes involved in electron transfer chain. In addition, CnHsf3 responds …

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Extracorporeal Membrane Oxygenation In The Care Of A Preterm Infant With Covid-19 Infection: Case Report, Jessica Patrick-Esteve, Christy Mumphrey, David Yu, Emily Masoumy, Jeremy Lawson, David Hebert, Brian Barkemeyer

School of Medicine Faculty Publications

Coronavirus disease 2019 (COVID-19) was first reported to the World Health Organization (WHO) in December 2019 and has since unleashed a global pandemic, with over 518 million cases as of May 10, 2022. Neonates represent a very small proportion of those patients. Among reported cases of neonates with symptomatic COVID-19 infection, the rates of hospitalization remain low. Most reported cases in infants and neonates are community acquired with mild symptoms, most commonly fever, rhinorrhea and cough. Very few require intensive care or invasive support for acute infection. We present a case of a 2-month-old former 26-week gestation infant with a …

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Variation In The Hemoglobin Glycation Index, James M. Hempe, Daniel S. Hsia

School of Medicine Faculty Publications

A high hemoglobin glycation index (HGI) has been repeatedly associated with greater risk for hypoglycemia in people with diabetes and greater risk for chronic vascular disease in people with or without diabetes. This review explores how different sources of analytical and biological variation in HbA1c and blood glucose individually and collectively affect the clinical information value of HGI. We conclude that HGI is a complex quantitative trait that is a clinically practical biomarker of risk for both hypoglycemia and chronic vascular disease.

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Facilitators And Barriers To Clinical Practice Guideline-Consistent Supportive Care At Pediatric Oncology Institutions: A Children's Oncology Group Study, Aaron J. Sugalski, Tammy Lo, Melissa Beauchemin, Allison C. Grimes, Paula D. Robinson, Alexandra M. Walsh, Nancy Santesso, Ha Dang, Brian T. Fisher, Andrea Rothfus Wrightson, Lolie C. Yu, Lillian Sung, L Lee Dupuis

School of Medicine Faculty Publications

Background Clinical practice guideline (CPG)-consistent care improves patient outcomes, but CPG implementation is poor. Little is known about CPG implementation in pediatric oncology. This study aimed to understand supportive care CPG implementation facilitators and barriers at pediatric oncology National Cancer Institute (NCI) Community Oncology Research Program (NCORP) institutions. Methods Healthcare professionals at 26 pediatric, Children's Oncology Group-member, NCORP institutions were invited to participate in face-to-face focus groups. Serial focus groups were held until saturation of ideas was reached. Supportive care CPG implementation facilitators and barriers were solicited using nominal group technique (NGT), and implementation of specific supportive care CPG recommendations …

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Nucleated Red Blood Cells As A Biomarker For Mortality In Neonates Following Cardiac Surgery, Kurt D. Piggott, Syeda Maqsood, Cynthia L. Warner, Timothy Pettitt, Shengping Yang, Jason Turner, Amira Soliman, Casey Norlin, Latasha Lewis, Ajay Bhatia

School of Medicine Faculty Publications

Introduction: Nucleated red blood cells (NRBCs) are immature red cells that under normal conditions are not present in the peripheral circulation. Several studies have suggested an association between elevated NRBC and poor outcome in critically ill adults and neonates. We sought to determine if elevations in NRBC value following cardiac surgery and following clinical events during the hospital stay can be used as a biomarker to monitor for mortality risk in neonates post-cardiac surgery. Materials and methods: We constructed a retrospective study of 264 neonates who underwent cardiac surgery at Children's Hospital, New Orleans between 2011 and 2020. Variables included …

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Efficacy And Safety Of Abobotulinumtoxina For Upper Limb Spasticity In Children With Cerebral Palsy: A Randomized Repeat-Treatment Study, Mauricio R. Delgado, Ann Tilton, Jorge Carranzo-Del Rio, Nigar Dursun, Marcin Bonikowski, Resa Aydin, Iwona Maciag-Tymecka, Joyce Oleszek, Edward Dabrowski, Anne-Sophie Grandoulier, Philippe Picaut, The Dysport In Pul Study Group

School of Medicine Faculty Publications

Aim: To assess the efficacy and safety of repeat abobotulinumtoxinA injections in reducing upper limb spasticity in children with cerebral palsy (CP). Method: This was a double-blind, repeat-cycle study (NCT02106351) in children with CP (2–17y). Children were randomized to receive 2U/kg (control), 8U/kg, or 16U/kg abobotulinumtoxinA injections into the target muscle group (wrist or elbow flexors) and additional muscles alongside occupational therapy via a home-exercise therapy program (HETP; minimum five 15min sessions/wk). Children received 8U/kg or 16U/kg plus HETP in cycles 2 to 4. Results: During cycle 1, 210 children (126 males, 84 females; mean age [SD] 9y [4y 5mo], …

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Ace2 Mouse Models: A Toolbox For Cardiovascular And Pulmonary Research, Hongpeng Jia, Xinping Yue, Eric Lazartigues

School of Medicine Faculty Publications

Angiotensin-converting enzyme 2 (ACE2) has been identified as the host entry receptor for the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) responsible for the COVID-19 pandemic. ACE2 is a regulatory enzyme of the renin-angiotensin system and has protective functions in many cardiovascular, pulmonary and metabolic diseases. This review summarizes available murine models with systemic or organ-specific deletion of ACE2, or with overexpression of murine or human ACE2. The purpose of this review is to provide researchers with the genetic tools available for further understanding of ACE2 biology and for the investigation of ACE2 in the pathogenesis and treatment of COVID-19.

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Intratumoral Translocation Positive Heterogeneity In Pediatric Alveolar Rhabdomyosarcoma Tumors Correlates To Patient Survival Prognosis, Katrina Gleditsch, Jorge Peñas, Danielle Mercer, Ayesha Umrigar, James Briscoe, Matthew Stark, Fern Tsien, Andrew D. Hollenbach

School of Medicine Faculty Publications

Alveolar rhabdomyosarcoma (ARMS) is characterized by one of three translocation states: t(2;13) (q35;q14) producing PAX3-FOXO1, t(1;13) (p36;q14) producing PAX7-FOXO1, or translocation-negative. Tumors with t(2;13) are associated with greater disease severity and mortality than t(1;13) positive or translocation negative patients. Consistent with this fact, previous work concluded that a molecular analysis of RMS translocation status is essential for the accurate determination of prognosis and diagnosis. However, despite this knowledge, most diagnoses rely on histology and in some cases utilize fluorescence in situ hybridization (FISH) probes unable to differentiate between translocation products. Along these same lines, diagnostic RT-PCR analysis, which can differentiate …

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Full-Text Articles in Pediatrics

Optimizing Antibiotic Durations Of Therapy In Pediatric Community-Acquired Pneumonia, Gregory Cook, Shubho Sarkar

School of Medicine Faculty Publications

Effects Of Covid-19 Vaccination On Pro-Coagulant State Of A Protein S-Deficient Pediatric Patient: New Hope For Treatment Of The Deficiency, Diana Polania-Villanueva, M. Kumar, Lekha Thangada, Jone Garai, Jovanny Zabaleta, Rinku Majumder

School of Medicine Faculty Publications

Antiphosphatidylserine/Prothrombin Antibodies In Childhood Sle Are Associated With Lupus Anticoagulant Positivity, S. Chugh, R. Pilania, A. Kler, C. Hans, Narender Kumar, S. Singh, J. Ahluwalia

School of Medicine Faculty Publications

Left Juxtaposition Of The Right Atrial Appendage: Pitfalls In Diagnosis, Kanya Singhapakdi, Wesam Sourour, Thomas Kimball

School of Medicine Faculty Publications

Cystic Fibrosis Year In Review 2022, Jeeyeon Kim, Benjamin Lyman, Adrienne P. Savant

School of Medicine Faculty Publications

Left Pulmonary Artery Occlusion Following Device Closure Of Patent Ductus Arteriosus In Premature Infants, Jesus C. Jaile, Ernest Siwik, Sergio Bartakian

School of Medicine Faculty Publications

Protein S Antibody As An Adjunct Therapy For Hemophilia B, Hope P. Wilson, Aliyah Pierre, Ashley L. Paysse, Narender Kumar, Brian C. Cooley, Pratyadipta Rudra, Adrianne W. Dorsey, Diana Polania-Villanueva, Sabyasachi Chatterjee, Maissaa Janbain, Maria C. Velez, Rinku Majumder

School of Medicine Faculty Publications

Small Molecules Targeting Insm1 For The Treatment Of High-Risk Neuroblastoma, Michael S. Lan, Chiachen Chen

School of Medicine Faculty Publications

School of Medicine Faculty Publications

Two Pediatric Cases Within A Familial Cluster And Hereditary Diffuse Gastric Carcinoma: A Tale Of 2 Sisters, Lacey Falgout, Lawrence L. Gensler

School of Medicine Faculty Publications

School of Medicine Faculty Publications

School of Medicine Faculty Publications

School of Medicine Faculty Publications

What To Look For In Relationships: Development, Inter-Rater Reliability, And Initial Validity Estimates For A Young Child-Caregiver Relationship Assessment, Joy D. Osofsky, Margaret A. Fields-Olivieri, Andrew L. Frazer, Rebecca A. Graham, Bethany H. Mccurdy, Carl F. Weems

School of Medicine Faculty Publications

Minimalistic Approach To Enhanced Recovery After Pediatric Scoliosis Surgery, Scott A. Barnett, Bryant M. Song, Matthew Bauer, Matthew E. Nungesser, Claudia Leonardi, Michael J. Heffernan

School of Medicine Faculty Publications

School of Medicine Faculty Publications

Yoga And Mindfulness For Social-Emotional Development And Resilience In 3–5 Year-Old Children: Non-Randomized, Controlled Intervention, Alessandra N. Bazzano, Yaoyao Sun, Yuanhao Zu, Julia M. Fleckman, Emma A. Blackson, Tejal Patel, Angie Shorty-Belisle, Keith H. Liederman, Cody Roi

School of Medicine Faculty Publications

School of Medicine Faculty Publications

Mechanisms Of Recovery: Community Perceptions Of Change And Growth Following Multiple Disasters, Howard Osofsky, Joy Osofsky, Leia Y. Saltzman, Estilla Lightfoot, Jule De King, Tonya C. Hansel

School of Medicine Faculty Publications

School of Medicine Faculty Publications

School of Medicine Faculty Publications

School of Medicine Faculty Publications

School of Medicine Faculty Publications

Extracorporeal Membrane Oxygenation In The Care Of A Preterm Infant With Covid-19 Infection: Case Report, Jessica Patrick-Esteve, Christy Mumphrey, David Yu, Emily Masoumy, Jeremy Lawson, David Hebert, Brian Barkemeyer

School of Medicine Faculty Publications

Variation In The Hemoglobin Glycation Index, James M. Hempe, Daniel S. Hsia

School of Medicine Faculty Publications

School of Medicine Faculty Publications

Nucleated Red Blood Cells As A Biomarker For Mortality In Neonates Following Cardiac Surgery, Kurt D. Piggott, Syeda Maqsood, Cynthia L. Warner, Timothy Pettitt, Shengping Yang, Jason Turner, Amira Soliman, Casey Norlin, Latasha Lewis, Ajay Bhatia

School of Medicine Faculty Publications

School of Medicine Faculty Publications

Ace2 Mouse Models: A Toolbox For Cardiovascular And Pulmonary Research, Hongpeng Jia, Xinping Yue, Eric Lazartigues

School of Medicine Faculty Publications

Intratumoral Translocation Positive Heterogeneity In Pediatric Alveolar Rhabdomyosarcoma Tumors Correlates To Patient Survival Prognosis, Katrina Gleditsch, Jorge Peñas, Danielle Mercer, Ayesha Umrigar, James Briscoe, Matthew Stark, Fern Tsien, Andrew D. Hollenbach

School of Medicine Faculty Publications