Medical Specialties Commons^™

Chick Embryo Chorioallantoic Membrane As A Platform For Assessing The In Vivo Efficacy Of Chimeric Antigen Receptor T-Cell Therapy In Solid Tumors, Allison J Nipper, Emilie A K Warren, Kershena S Liao, Hsuan-Chen Liu, Chieko Michikawa, Caroline E Porter, Gabrielle A Wells, Mariana Villanueva, Fabio Henrique Brasil Da Costa, Ratna Veeramachaneni, Hugo Villanueva, Masataka Suzuki, Andrew G Sikora

Faculty, Staff and Student Publications

The fertilized chicken egg chorioallantoic membrane (CAM), a highly vascularized membrane nourishing the developing embryo, also supports rapid growth of three-dimensional vascularized tumors from engrafted cells and tumor explants. Because murine xenograft models suffer limitations of time, cost, and scalability, we propose CAM tumors as a rapid, efficient screening tool for assessing anti-tumor efficacy of chimeric Ag receptor (CAR) T cells against solid tumors. We tested the efficacy of human epidermal growth factor receptor 2 (HER2)-specific CAR T cells against luminescent, HER2-expressing (FaDu, SCC-47) or HER2-negative (MDA-MB-468) CAM-engrafted tumors. Three days after tumor engraftment, HER2-specific CAR T cells were applied …

Mechanism And Rational Combinations With Gp-2250, A Novel Oxathiazine Derivative, In Ovarian Cancer, Mark S Kim, Deanna Glassman, Katelyn F Handley, Adrian Lankenau Ahumada, Nicholas B Jennings, Emine Bayraktar, Katherine Foster, Robiya Joseph, Sanghoon Lee, Robert L Coleman, Anil K Sood

Faculty, Staff and Student Publications

BACKGROUND: GP-2250, a novel analog of taurultam (TRLT), has emerged as a potent anti-neoplastic drug; however, the mechanisms underlying its effects are not well understood. Here, we investigated the mechanism of action and the biological effects of GP-2250 using in vitro and in vivo models.

METHODS: We carried out a series of in vitro (MTT assay, Annexin V/PI assay, colony formation assay, reverse-phase protein array [RPPA], and HRLC/IC analysis) to determine the biological activity of GP-2250 and investigate the mechanism of action. In vivo experiments were carried out to determine the therapeutic efficacy of GP-2250 alone and in combination with …

Autophagic Signaling Promotes Systems-Wide Remodeling In Skeletal Muscle Upon Oncometabolic Stress By D2-Hg, Yaqi Gao, Kyoungmin Kim, Heidi Vitrac, Rebecca L Salazar, Benjamin D Gould, Daniel Soedkamp, Weston Spivia, Koen Raedschelders, An Q Dinh, Anna G Guzman, Lin Tan, Stavros Azinas, David J R Taylor, Walter Schiffer, Daniel Mcnavish, Helen B Burks, Roberta A Gottlieb, Philip L Lorenzi, Blake M Hanson, Jennifer E Van Eyk, Heinrich Taegtmeyer, Anja Karlstaedt

Faculty, Staff and Student Publications

OBJECTIVES: Cachexia is a metabolic disorder and comorbidity with cancer and heart failure. The syndrome impacts more than thirty million people worldwide, accounting for 20% of all cancer deaths. In acute myeloid leukemia, somatic mutations of the metabolic enzyme isocitrate dehydrogenase 1 and 2 cause the production of the oncometabolite D2-hydroxyglutarate (D2-HG). Increased production of D2-HG is associated with heart and skeletal muscle atrophy, but the mechanistic links between metabolic and proteomic remodeling remain poorly understood. Therefore, we assessed how oncometabolic stress by D2-HG activates autophagy and drives skeletal muscle loss.

METHODS: We quantified genomic, metabolomic, and proteomic changes in …

#Hashtagthis – Everything You Need To Know About Launching Your Gynecologic Oncology Social Media Research Career: A Report From Gynecologic Oncology Reports And Society Of Gynecologic Oncology Education Committee, Megan L Hutchcraft, Eric Rios-Doria, Tiffany Y Sia, Eleonora Teplinsky, Shannon N Westin, Gregg Nelson

Faculty, Staff and Student Publications

On February 6th, 2024, Gynecologic Oncology Reports and the Society of Gynecologic Oncology Education Committee co-hosted a webinar about ways to use social media for career enhancement and for dissemination of research. During the discussion, we reviewed:i.how to identify one's goals, target audience, and select a social media platform.ii.how to navigate the negatives of social media.iii.how to develop one's online academic brand.iv.how to use social media for academic promotion and career advancement.v.how to use social media as a research tool.vi.how to use visual tools to bring attention to one's research.The objective of this report is to review the literature on …

A Biallelic Variant Of The Rna Exosome Gene, Exosc4, Associated With Neurodevelopmental Defects Impairs Rna Exosome Function And Translation, Milo B Fasken, Sara W Leung, Lauryn A Cureton, Maha Al-Awadi, Adila Al-Kindy, Ambro Van Hoof, Sohail Khoshnevis, Homa Ghalei, Almundher Al-Maawali, Anita H Corbett

Faculty, Staff and Student Publications

The RNA exosome is an evolutionarily conserved complex required for both precise RNA processing and decay. Pathogenic variants in EXOSC genes, which encode structural subunits of this complex, are linked to several autosomal recessive disorders. Here, we describe a missense allele of the EXOSC4 gene that causes a collection of clinical features in two affected siblings. This missense variant (NM_019037.3: exon3:c.560T>C) changes a leucine residue within a conserved region of EXOSC4 to proline (p.Leu187Pro). The two affected individuals show prenatal growth restriction, failure to thrive, global developmental delay, intracerebral and basal ganglia calcifications, and kidney failure. Homozygosity for the …

Nf1 Mutation Disrupts Activity-Dependent Oligodendroglial Plasticity And Motor Learning In Mice, Yuan Pan, Jared D Hysinger, Belgin Yalçın, James J Lennon, Youkyeong Gloria Byun, Preethi Raghavan, Nicole F Schindler, Corina Anastasaki, Jit Chatterjee, Lijun Ni, Haojun Xu, Karen Malacon, Samin M Jahan, Alexis E Ivec, Benjamin E Aghoghovwia, Christopher W Mount, Surya Nagaraja, Suzanne Scheaffer, Laura D Attardi, David H Gutmann, Michelle Monje

Faculty, Staff and Student Publications

Neurogenetic disorders, such as neurofibromatosis type 1 (NF1), can cause cognitive and motor impairments, traditionally attributed to intrinsic neuronal defects such as disruption of synaptic function. Activity-regulated oligodendroglial plasticity also contributes to cognitive and motor functions by tuning neural circuit dynamics. However, the relevance of oligodendroglial plasticity to neurological dysfunction in NF1 is unclear. Here we explore the contribution of oligodendrocyte progenitor cells (OPCs) to pathological features of the NF1 syndrome in mice. Both male and female littermates (4-24 weeks of age) were used equally in this study. We demonstrate that mice with global or OPC-specific Nf1 heterozygosity exhibit defects …

A Visualization And Radiation Treatment Plan Quality Scoring Method For Triage In A Population-Based Context, Alexandra O Leone, Abdallah S R Mohamed, Clifton D Fuller, Christine B Peterson, Adam S Garden, Anna Lee, Lauren L Mayo, Amy C Moreno, Jay P Reddy, Karen Hoffman, Joshua S Niedzielski, Laurence E Court, Thomas J Whitaker

Faculty, Staff and Student Publications

PURPOSE: Our purpose was to develop a clinically intuitive and easily understandable scoring method using statistical metrics to visually determine the quality of a radiation treatment plan.

METHODS AND MATERIALS: Data from 111 patients with head and neck cancer were used to establish a percentile-based scoring system for treatment plan quality evaluation on both a plan-by-plan and objective-by-objective basis. The percentile scores for each clinical objective and the overall treatment plan score were then visualized using a daisy plot. To validate our scoring method, 6 physicians were recruited to assess 60 plans, each using a scoring table consisting of a …

Secondary Endpoint Utilization And Publication Rate Among Phase Iii Oncology Trials, Esther J Beck, Alexander D Sherry, Marcus A Florez, Ramez Kouzy, Joseph Abi Jaoude, Timothy A Lin, Avital M Miller, Adina H Passy, Gabrielle S Kupferman, Roshal R Patel, Fumiko Chino, Victoria Serpas Higbie, Christine M Parseghian, Michael J Overman, Bruce D Minsky, Charles R Thomas, Chad Tang, Pavlos Msaouel, Ethan B Ludmir

Faculty, Staff and Student Publications

UNLABELLED: Secondary endpoints (SEP) provide crucial information in the interpretation of clinical trials, but their features are not yet well understood. Thus, we sought to empirically characterize the scope and publication rate of SEPs among late-phase oncology trials. We assessed SEPs for each randomized, published phase III oncology trial across all publications and ClinicalTrials.gov, performing logistic regressions to evaluate associations between trial characteristics and SEP publication rates. After screening, a total of 280 trials enrolling 244,576 patients and containing 2,562 SEPs met the inclusion criteria. Only 22% of trials (62/280) listed all SEPs consistently between ClinicalTrials.gov and the trial protocol. …

Safety And Activity Of Lenalidomide In Combination With Obinutuzumab In Patients With Relapsed Indolent Non-Hodgkin Lymphoma: A Single Group, Open-Label, Phase 1/2 Trial, Ashwath Gurumurthi, Collin K Chin, Lei Feng, Nathan H Fowler, Paolo Strati, Fredrick B Hagemeister, Luis E Fayad, Jason R Westin, Chizobam Obi, Janine Arafat, Ranjit Nair, Raphael E Steiner, Sattva S Neelapu, Christopher R Flowers, Loretta J Nastoupil

Faculty, Staff and Student Publications

BACKGROUND: Rituximab and lenalidomide is a preferred option for relapsed indolent B cell non-Hodgkin lymphoma. Obinutuzumab may be a superior combination partner with lenalidomide given enhanced antibody dependent cellular cytotoxicity and phagocytosis compared to rituximab. Our aim was to determine the recommended phase 2 dose, safety, and activity of lenalidomide in combination with fixed dose of obinutuzumab in relapsed and refractory indolent B cell non-Hodgkin lymphoma.

METHODS: In this single-arm, open-label, phase 1/2 trial, we enrolled patients with relapsed or refractory WHO Grade 1-3A follicular lymphoma, marginal zone lymphoma and small lymphocytic lymphoma and adequate performance status (ECOG 0-2) at …

Cerebrovascular Reactivity Mri As A Biomarker For Cerebral Small Vessel Disease–Related Cognitive Decline: Multi-Site Validation In The Markvcid Consortium, Peiying Liu, Zixuan Lin, Kaisha Hazel, George Pottanat, Cuimei Xu, Dengrong Jiang, Jay J. Pillai, Emma Lucke, Christopher E. Bauer, Brian T. Gold, Steven M. Greenburg, Karl G. Helmer, Kay Jann, Gregory Jicha, Joel Kramer, Pauline Maillard, Rachel M. Mulavelil, Pavel Rodriguez, Claudia L. Satizabal, Kristin Schwab, Sudha Seshadri, Herpreet Singh, Ángel G. Velarde Dediós, Danny J. J. Wang, Rita R. Kalyani, Abhay Moghekar, Paul B. Rosenberg, Sevil Yasar, Marilyn Albert, Hanzhang Lu

Neurology Faculty Publications

INTRODUCTION: Vascular contributions to cognitive impairment and dementia (VCID) represent a major factor in cognitive decline in older adults. The present study examined the relationship between cerebrovascular reactivity (CVR) measured by magnetic resonance imaging (MRI) and cognitive function in a multi-site study, using a predefined hypothesis.

METHODS: We conducted the study in a total of three analysis sites and 263 subjects. Each site performed an identical CVR MRI procedure using 5% carbon dioxide inhalation. A global cognitive measure of Montreal Cognitive Assessment (MoCA) and an executive function measure of item response theory (IRT) score were used as outcomes.

RESULTS: CVR …

Effect Of Male Partners' Involvement And Support On Reproductive, Maternal And Child Health And Well-Being In East Africa: A Scoping Review, Richard Fletcher, Faye Forbes, Abel Fekadu Dadi, Getachew Mullu Kassa, Casey Regan, Anna Galle, Addisu Beyene, Rebecca Liackman, Marleen Temmerman

Centre of Excellence in Women and Child Health

Background and Aims: East African countries have high rates of maternal and childmortality and morbidity. Studies have shown that the involvement of male partners inreproductive health can benefit maternal and child health (MCH). This scoping reviewaims to provide an overview of the evidence across East Africa that describes malepartner involvement and its effect on maternal, reproductive, and child well‐being.

Methods: Ten databases were searched to identify quantitative data on male'sinvolvement in East Africa. Studies reporting qualitative data, “intention to use” dataor only reporting on male partner's education or economic status were excluded.Studies were organized into five a priori categories: antenatal …

Contributors To Faculty Wellness In An Academic Emergency Department Before And During The Pandemic: A Qualitative Study, Nicholas D. Hartman, Casey Glass, Manoj Pariyadath, Kathleen Hosmer, Meagan Hunt, Nicklaus Ashburn, Cedric Lefebvre

Journal of Wellness

Introduction: Burnout is well known as a prevalent hazard in emergency medicine (EM) careers, but the literature is less clear about what specific work-related factors actually contribute to wellness and burnout among academic EM faculty.

Objectives: 1) To explore themes and specific concerns that contribute to faculty wellness in an academic emergency department, in a qualitative fashion. 2) To determine whether these themes and concerns changed appreciably in the time of the COVID-19 pandemic.

Methods: EM faculty members at an academic institution were invited to participate in the study, including an on-line survey and a structured interview. Survey results were …

Evaluating Hmgb1 As A Biomarker For Metabolic Dysregulation And Progression Of Diabetes In A Mexican American Cohort, Peter Elvin, Walid Fakhouri, Craig Hanis, Cláudia Cristina Biguetti

School of Podiatric Medicine - Student Research

Aim/Hypothesis: This study focuses on HMGB1 to evaluate its association with the development and progression of diabetes within a Mexican American cohort of non-diabetic (ND), pre-diabetic (Pre-D), and diabetic (D) individuals.

Methods: Data from a cohort of 40 individuals, standardized by age (61.76±11.77), BMI (32.52±7.02), and sex (58% male, 42% female) was analyzed. We assessed several immunological markers utilizing ELISA assays from buffy coats. After excluding out of range samples, the remaining 20 individuals displayed statistical significance in HMGB1. Pearson's correlation coefficient examined the relationship between HMGB1 levels and glycemic changes (HOMA-IR, HOMA-B, HOMA-IS², and HbA1c). Statistical significance …

Variation In Systemic Corticosteroid Prescribing During Asthma-Related Hospitalizations Across Children’S Hospitals, Sian Best, Matt Hall, Jessica L. Bettenhausen, Shelby Chesbro, Nicholas Clark, Megan Collins, Adrienne Deporre, Jonathan Ermer, Bridgette Jones, Leah Jones, Jessica Markham, Elisha Mccoy, Maria Newmaster, Laura Plencner, Henry T. Puls, Smit Shah, Kathryn Kyler

Presentations

Background: Asthma exacerbations are a leading cause of pediatric hospitalizations, and systemic corticosteroids remain a key component of inpatient treatment. Emergency department-focused studies have shown dexamethasone to be equivalent to prednisone/prednisolone in terms of outcomes such as readmission rates, but less is known about the trends in dexamethasone use versus other systemic corticosteroids, and associated outcomes in the inpatient setting. Objectives/Goal: To describe variability and trends in inpatient systemic corticosteroid prescribing practices for acute asthma exacerbations, and to determine associations between the prescribed steroid and hospitalization outcomes. Methods/Design: This retrospective cross-sectional study utilized the PHIS database to examine patients aged …

Transcriptomics Analysis Reveals Potential Regulatory Role Of Nsmase2 (Smpd3) In Nervous System Development And Function Of Middle-Aged Mouse Brains, Zhihui Zhu, Timothy S. Mcclintock, Erhard Bieberich

Markey Cancer Center Faculty Publications

Neutral sphingomyelinase-2 (nSMase2), gene name sphingomyelin phosphodiesterase-3 (Smpd3), is a key regulatory enzyme responsible for generating the sphingolipid cer- amide. The function of nSMase2 in the brain is still controversial. To better under- stand the functional roles of nSMase2 in the aging mouse brain, we applied RNA-seq analysis, which identified a total of 1462 differentially abundant mRNAs between +/fro and fro/fro, of which 891 were increased and 571 were decreased in nSMase2-deficient mouse brains. The most strongly enriched GO and KEGG annota- tion terms among transcripts increased in fro/fro mice included synaptogenesis, syn- apse development, synaptic signaling, axon development, and …

Cigarette Smoke-Induced Epithelial-To-Mesenchymal Transition: Insights Into Cellular Mechanisms And Signaling Pathways, Sarah Mohammed Alqithami, Amrita Machwe, David K. Orren

Markey Cancer Center Faculty Publications

This review delves into the molecular complexities underpinning the epithelial-to-mesenchymal transition (EMT) induced by cigarette smoke (CS) in human bronchial epithelial cells (HBECs). The complex interplay of pathways, including those related to WNT//β-catenin, TGF-β/SMAD, hypoxia, oxidative stress, PI3K/Akt, and NF-κB, plays a central role in mediating this transition. While these findings significantly broaden our understanding of CS-induced EMT, the research reviewed herein leans heavily on 2D cell cultures, highlighting a research gap. Furthermore, the review identifies a stark omission of genetic and epigenetic factors in recent studies. Despite these shortcomings, the findings furnish a consolidated foundation not only for the …

Cryo-Em Reconstruction Of Oleate Hydratase Bound To A Phospholipid Membrane Bilayer, Michael L. Oldham, M. Zuhaib Qayyum, Ravi C. Kalathur, Charles O. Rock, Christopher D. Radka

Markey Cancer Center Faculty Publications

Oleate hydratase (OhyA) is a bacterial peripheral membrane protein that catalyzes FAD-dependent water addition to membrane bilayer-embedded unsaturated fatty acids. The opportunistic pathogen Staphylococcus aureus uses OhyA to counteract the innate immune system and support colonization. Many Gram-positive and Gram-negative bacteria in the microbiome also encode OhyA. OhyA is a dimeric flavoenzyme whose carboxy terminus is identified as the membrane binding domain; however, understanding how OhyA binds to cellular membranes is not complete until the membrane-bound structure has been elucidated. All available OhyA structures depict the solution state of the protein outside its functional environment. Here, we employ liposomes to …

Nsd3::Nutm1 Fusion Sarcoma Mimicking Malignant Peripheral Nerve Sheath Tumor With Prolonged Survival, Jing Di, Ali M. Alhaidary, Chi Wang, Jinge Liu, Sainan Wei, Joseph Valentino, Therese J. Bocklage

Markey Cancer Center Faculty Publications

Nuclear Protein in Testis (NUT)-rearranged tumors comprise predominantly NUT car- cinoma but also include certain lymphomas, leukemias, skin appendage tumors, and sarcomas. Although histologically diverse, all are genetically identified by oncogenic rearrangement in the NUTM1 gene. Many fusion partners occur, and NSD3 is NUT carcinoma’s third most common partner. Herein, we present a case of a 26-year-old man with an NSD3::NUTM1 fusion sarcoma. The patient presented at the age of 13 months with a scalp nodule. Over the next 24 years, he experienced five local recurrences and ultimately expired of a rapidly progressive recurrence. His treatment included surgical resections, radiation, …

Variables Affecting Neurodevelopmental Outcomes In Infants With Critical Congenital Heart Disease, Elizabeth Loughman, Marcie G. Files, Julie Weiner

Posters

Background: The mortality of cardiac conditions in infancy is improving with advances in surgical planning and medical management. Neurologic morbidity remains significant, and clinically correlating an infant with their neurologic exam, MRI/EEG, and subsequent developmental testing remains a challenge. Counseling parents regarding neurologic outcomes after cardiac intervention is difficult due to the wide range of findings that can sometimes prove to be clinically irrelevant. Purpose: The goal of this study is to identify factors associated with poor neurodevelopmental outcomes in infants with critical congenital heart defects (CCHD) as assessed by the Bayley Scales of Infant and Toddler Development Edition IV …

Addendum: A Small Molecule Produced By Lactobacillus Species Blocks Candida Albicans Filamentation By Inhibiting A Dyrk1-Family Kinase, Jessie Macalpine, Martin Daniel-Ivad, Zhongle Liu, Junko Yano, Nicole M. Revie, Robert T. Todd, Peter J. Stogios, Hiram Sanchez, Teresa R. O'Meara, Thomas A. Tompkins, Alexei Savchenko, Anna Selmecki, Amanda O. Veri, David R. Andes, Paul L. Fidel, Nicole Robbins, Justin Nodwell, Luke Whitesell, Leah E. Cowen

School of Dentistry Faculty Publications

No abstract provided.

Increased Risk Of Cutaneous T-Cell Lymphoma Development After Dupilumab Use For Atopic Dermatitis, Jenna Mandel, Jaanvi Mehta, Ramsay Hafer, Mahaa Ayub, Faria Nusrat, Henry Yang, Pierluigi Porcu, Neda Nikbakht

Department of Dermatology and Cutaneous Biology Faculty Papers

There have been several reports of dupilumab use and the development of CTCL; however, the risk of CTCL development has not been adequately evaluated at the population level. The objective of this study is to determine whether dupilumab administration for AD is associated with an increased risk of developing CTCL and to identify at-risk populations within this group. This retrospective cohort study used TriNetX, a deidentified medical record database including over 107 million patients, to identify eligible patients. Treatment and control groups were evaluated for the development of CTCL. Patients of any age with a documented diagnosis of AD were …

Assessment Of Ferritin And Lead Levels In Children Living In An Informal Urban Settlement In Kenya, Geoffrey Omuse, Daniel Maina, Maureene Ondayo, Stanley Onyango, Syama Sinuff, Alex Mugo, Pauline Samia, William Macharia

Pathology, East Africa

Background: Childhood lead exposure from recycling used lead-acid batteries (ULAB) in unregulated areas is a significant cause of lead toxicity, particularly in low and middle-income countries. This study aimed to assess the proportion of children with increased blood lead levels (BLLs) in an informal settlement where ULAB recycling occurs.

Methodology: This was a descriptive study conducted in Nairobi county, Kenya. Children aged 6 months to 7 years were recruited after obtaining written informed consent, completion of a questionnaire and collection of samples for complete blood count, ferritin, and lead analysis.

Results: Out of 215 children, 17 (8.0%) had BLLs ≥ …

A Lexicon For First-Trimester Us: Society Of Radiologists In Ultrasound Consensus Conference Recommendations, Shuchi K Rodgers, Mindy M Horrow, Peter M Doubilet, Mary C Frates, Anne Kennedy, Rochelle Andreotti, Kristyn Brandi, Laura Detti, Sarah K Horvath, Aya Kamaya, Atsuko Koyama, Penelope Chun Lema, Katherine E Maturen, Tara Morgan, Sarah G Običan, Kristen Olinger, Roya Sohaey, Suneeta Senapati, Lori M Strachowski

Faculty, Staff and Students Publications

The Society of Radiologists in Ultrasound convened a multisociety panel to develop a first-trimester US lexicon based on scientific evidence, societal guidelines, and expert consensus that would be appropriate for imagers, clinicians, and patients. Through a modified Delphi process with consensus of at least 80%, agreement was reached for preferred terms, synonyms, and terms to avoid. An intrauterine pregnancy (IUP) is defined as a pregnancy implanted in a normal location within the uterus. In contrast, an ectopic pregnancy (EP) is any pregnancy implanted in an abnormal location, whether extrauterine or intrauterine, thus categorizing cesarean scar implantations as EPs. The term …

Sinus Arrest Related To Dexmedetomidine Infusion In An Infant; A Case Report And Review Of Current Literature, Alexandra Dennee, Thomas P Fogarty, Taylor S Howard, Ryan Brandon Hunter

Faculty, Staff and Students Publications

Background: Dexmedetomidine, an alpha 2 agonist, has emerged as a desirable sedative agent in the pediatric intensive care unit due to its minimal effect on respiratory status and reduction in delirium. Bradycardia and hypotension are common side effects, however there are emerging reports of more serious cardiovascular events, including sinus arrest and asystole. These case reports have been attributed to high vagal tone or underlying cardiac conduction dysfunction.

Objectives: To describe the development of sinus arrest during sedation with dexmedetomidine in a patient without clinical features of high vagal tone, underlying cardiac conduction dysfunction, or intervening episodes of bradycardia.

Case …

Sinus Arrest Related To Dexmedetomidine Infusion In An Infant; A Case Report And Review Of Current Literature, Alexandra Dennee, Thomas P Fogarty, Taylor S Howard, Ryan Brandon Hunter

Faculty, Staff and Students Publications

Background: Dexmedetomidine, an alpha 2 agonist, has emerged as a desirable sedative agent in the pediatric intensive care unit due to its minimal effect on respiratory status and reduction in delirium. Bradycardia and hypotension are common side effects, however there are emerging reports of more serious cardiovascular events, including sinus arrest and asystole. These case reports have been attributed to high vagal tone or underlying cardiac conduction dysfunction.

Objectives: To describe the development of sinus arrest during sedation with dexmedetomidine in a patient without clinical features of high vagal tone, underlying cardiac conduction dysfunction, or intervening episodes of bradycardia.

Case …

Developmental Delay Can Precede Neurologic Regression In Early Onset Metachromatic Leukodystrophy, Laura Ann Adang, Samuel Groeschel, Chloe Grzyb, Russell D'Aiello, Francesco Gavazzi, Omar Sherbini, Nowa Bronner, Akshilkumar Patel, Ariel Vincent, Anjana Sevagamoorthy, Sylvia Mutua, Kayla Muirhead, Johanna Schmidt, Amy Pizzino, Emily Yu, Danielle Jin, Florian Eichler, Jamie L Fraser, Lisa Emrick, Keith Van Haren, Jean-Martin Boulanger, Maura Ruzhnikov, Michel Sylvain, Cam-Tu Émilie Nguyen, Ana Potic, Stephanie Keller, Ali Fatemi, Eloise Uebergang, Michele Poe, Pouneh Amir Yazdani, John Bernat, Kristen Lindstrom, Joshua L Bonkowsky, Genevieve Bernard, Chloe A Stutterd, Paul Orchard, Ashish O Gupta, Merete Ljungberg, Sabine Groenborg, Alberto Zambon, Sara Locatelli, Francesca Fumagalli, Saskia Elguen, Christiane Kehrer, Ingeborg Krägeloh-Mann, Justine Shults, Adeline Vanderver, Maria L Escolar

Faculty, Staff and Students Publications

Objective: Metachromatic leukodystrophy (MLD) is a rare neurodegenerative disorder. Emerging therapies are most effective in the presymptomatic phase, and thus defining this window is critical. We hypothesize that early development delay may precede developmental plateau. With the advent of presymptomatic screening platforms and transformative therapies, it is essential to define the onset of neurologic disease.

Methods: The specific ages of gain and loss of developmental milestones were captured from the medical records of individuals affected by MLD. Milestone acquisition was characterized as: on target (obtained before the age limit of 90th percentile plus 2 standard deviations compared to a normative …

Sensory Symptoms Across The Lifespan In People With Cerebral Palsy, Ariel M Lyons-Warren, Danielle Guez-Barber, Sruthi P Thomas, Evelyne K Tantry, Aditya Mahat, Bhooma Aravamuthan

Faculty, Staff and Students Publications

Background: To estimate the prevalence of sensory symptoms in people with cerebral palsy (CP) across the lifespan.

Methods: In this cross-sectional study, the self-reported Sensory Processing Scale Inventory (SPS-I) was administered via Research Electronic Data Capture (REDCap) between February 1, 2022, and August 15, 2022, to people with CP or their caregivers enrolled in the online MyCP Community Registry. We determined the association between SPS-I scores and age (Pearson correlation) and functional status as assessed using five validated functional classification systems for CP (analysis of variance [ANOVA]). We hypothesized that sensory symptoms would differ between younger and older individuals with …

Editorial Comment: Diversity And Disparities In Orthopaedic Surgery: Update 2024, Kimberly J Templeton, Melvyn A Harrington

Faculty, Staff and Students Publications

No abstract provided.

Factors Associated With Failed Closed Reduction In Flexion And Gartland Type Iii Pediatric Supracondylar Humerus Fractures, Pablo Coello, Nathaniel Grey Loyd, David A Hsiou, Rachel S Silverstein, Scott B Rosenfeld

Faculty, Staff and Students Publications

Background: Most displaced supracondylar humerus fractures (SCHFs) are treated with closed reduction and percutaneous pinning. While there are only a few possible indications for converting to an open reduction, a failed closed reduction is a common cause. This study aims to elucidate possible risk factors for failed closed reductions of SCHF.

Methods: A retrospective review of SCHF from 2010 to 2020 at a pediatric tertiary medical center, which underwent operative fixation, was conducted. Exclusion criteria were open fractures and reasons for open reduction other than failed closed reduction. Rates of open reduction were assessed by preoperative fracture classification and assessed …

Risk Factors For Loss To Follow Up In Pediatric Supracondylar Humerus Fractures, Nathaniel Grey Loyd, David Hsiou, Armando Martinez, Pablo Coello, Lon Kai Pang, Muhammad Hamza Shamim, Jessica Mcgraw-Heinrich, Scott B Rosenfeld

Faculty, Staff and Students Publications

Background: This study seeks to analyze risk factors associated with loss to follow up (LTF) after pin pull in pediatric patients with operatively treated supracondylar humerus fractures (SCHF).

Methods: A retrospective cohort study of patients under 18 years with operative SCHF from 2010 to 2020 was conducted. Factors of interest included LTF, age, race, language, distance to the hospital, Gartland fracture type, and Social Deprivation Index (SDI) by ZIP code. Univariate logistic regression was performed for each independent variable and significant variables were additionally analyzed with multivariate logistic regression analysis.

Results: Six hundred ninety-eight patients were included in the study. …