Medicine and Health Sciences Commons^™

Clinical Utility Of Anti-Mullerian Hormone In Pediatrics, Roopa Kanakatti Shankar, Tazim Dowlut-Mcelroy, Andrew Dauber, Veronica Gomez-Lobo

Pediatrics Faculty Publications

CONTEXT: Anti-Mullerian hormone (AMH) was originally described in the context of sexual differentiation in the male fetus but has gained prominence now as a marker of ovarian reserve and fertility in females. In this mini-review, we offer an updated synopsis on AMH and its clinical utility in pediatric patients.

DESIGN AND RESULTS: A systematic search was undertaken for studies related to the physiology of AMH, normative data, and clinical role in pediatrics. In males, AMH, secreted by Sertoli cells, is found at high levels prenatally and throughout childhood and declines with progression through puberty to overlap with levels in females. …

Stop The Beat To See The Rhythm: Excitation-Contraction Uncoupling In Cardiac Research., Luther M Swift, Matthew W. Kay, Crystal M Ripplinger, Nikki Gillum Posnack

Pediatrics Faculty Publications

No abstract provided.

Sports Bra Preferences By Age And Impact Of Breast Size On Physical Activity Among American Females, Katherine H. Rizzone, Bianca Edison, Nailah Coleman, Cordelia Carter, Ingrid Ichesco, Paige Cassidy, Jane Chung, Courtney Marie Cora Jones

Pediatrics Faculty Publications

No abstract provided.

Using Nature To Nurture: Breast Milk Analysis And Fortification To Improve Growth And Neurodevelopmental Outcomes In Preterm Infants, Katherine Marie Ottolini, Elizabeth V. Schulz, Catherine Limperopoulos, Nickie Andescavage

Pediatrics Faculty Publications

No abstract provided.

Randomized And Non-Randomized Designs For Causal Inference With Longitudinal Data In Rare Disorders., Rima Izem, Robert Mccarter

Pediatrics Faculty Publications

In the United States, approximately 7000 rare diseases affect 30 million patients, and only 10% of these diseases have existing therapies. Sound study design and causal inference methods are essential to demonstrate the therapeutic efficacy, safety, and effectiveness of new therapies. In the rare diseases setting, several factors challenge the use of typical parallel control designs: the small patient population size, genotypic and phenotypic diversity, and the complexity and incomplete understanding of the disorder's progression. Repeated measures, when spaced appropriately relative to disease progression and exploited in design and analysis, can increase study power and reduce variability in treatment effect …

Muc1 Ectodomain Is A Flagellin-Targeting Decoy Receptor And Biomarker Operative During Pseudomonas Aeruginosa Lung Infection., Avelino C Verceles, Pavan Bhat, Zain Nagaria, Destiny Martin, Harsh Patel, Afua Ntem-Mensah, Sang W Hyun, Andrea Hahn, Jean Jeudy, Alan S Cross, Erik P Lillehoj, Simeon E Goldblum

Pediatrics Faculty Publications

No abstract provided.

2021 Paces Expert Consensus Statement On The Indications And Management Of Cardiovascular Implantable Electronic Devices In Pediatric Patients: Executive Summary., Michael J Silka, Maully J Shah, Jennifer N Avari Silva, Seshadri Balaji, Cheyenne M Beach, Monica N Benjamin, Charles I. Berul, Bryan Cannon, Frank Cecchin, Mitchell I Cohen, Aarti S Dalal, Brynn E Dechert, Anne Foster, Roman Gebauer, M Cecilia Gonzalez Corcia, Prince J Kannankeril, Peter P Karpawich, Jeffery J Kim, Mani Ram Krishna, Peter Kubuš, Martin J Lapage, Douglas Y Mah, Lindsey Malloy-Walton, Aya Miyazaki, Kara S Motonaga, Mary C Niu, Melissa Olen, Thomas Paul, Eric Rosenthal, Elizabeth V Saarel, Massimo Stefano Silvetti, Elizabeth A Stephenson, Reina B Tan, John Triedman, Nicholas H Von Bergen, Philip L Wackel

Pediatrics Faculty Publications

No abstract provided.

All In The Family: Child And Adolescent Weight Loss Surgery In The Context Of Parental Weight Loss Surgery., Eleanor R. Mackey, Megan M York, Evan P. Nadler

Pediatrics Faculty Publications

No abstract provided.

Heterogeneity Of Magnitude, Allergen Immunodominance, And Cytokine Polarization Of Cockroach Allergen-Specific T Cell Responses In Allergic Sensitized Children., Ricardo Da Silva Antunes, Aaron Sutherland, April Frazier, Veronique Schulten, Anna Pomés, Jill Glesner, Agustin Calatroni, Matthew C Altman, Robert A Wood, George T O'Connor, Jacqueline A Pongracic, Gurjit K Khurana Hershey, Carolyn M Kercsmar, Rebecca S Gruchalla, Michelle Gill, Andrew H Liu, Edward Zoratti, Meyer Kattan, Paula J Busse, Leonard B Bacharier, Stephen J. Teach, Lisa M Wheatley, Alkis Togias, William W Busse, Daniel J Jackson, Alessandro Sette

Pediatrics Faculty Publications

Background: Characterization of allergic responses to cockroach (CR), a common aeroallergen associated with asthma, has focused mainly on IgE reactivity, but little is known about T cell responses, particularly in children. We conducted a functional evaluation of CR allergen-specific T cell reactivity in a cohort of CR allergic children with asthma.

Methods: Peripheral blood mononuclear cells (PBMCs) were obtained from 71 children, with mild-to-moderate asthma who were enrolled in a CR immunotherapy (IT) clinical trial, prior to treatment initiation. PBMC were stimulated with peptide pools derived from 11 CR allergens, and CD4+ T cell responses assessed by intracellular cytokine staining. …

Kawasaki Disease Shock Syndrome Vs Classical Kawasaki Disease: A Meta-Analysis And Comparison With Sars-Cov-2 Multisystem Inflammatory Syndrome., Loubna Lamrani, Cedric Manlhiot, Matthew D Elias, Nadine F Choueiter, Audrey Dionne, Ashraf S. Harahsheh, Michael A Portman, Brian W Mccrindle, Nagib Dahdah

Pediatrics Faculty Publications

No abstract provided.

Triglyceride-Glucose Index: A Potential New Biomarker For Lung Disease Associated With Metabolic Dysregulation., Xilei Xu Chen, Deepa Rastogi

Pediatrics Faculty Publications

No abstract provided.

Moving Beyond Size: Vorticity And Energy Loss Are Correlated With Right Ventricular Dysfunction And Exercise Intolerance In Repaired Tetralogy Of Fallot., Yue-Hin Loke, Francesco Capuano, Vincent Cleveland, Jason G. Mandell, Elias Balaras, Laura J. Olivieri

Pediatrics Faculty Publications

BACKGROUND: The global effect of chronic pulmonary regurgitation (PR) on right ventricular (RV) dilation and dysfunction in repaired Tetralogy of Fallot (rTOF) patients is well studied by cardiovascular magnetic resonance (CMR). However, the links between PR in the RV outflow tract (RVOT), RV dysfunction and exercise intolerance are not clarified by conventional measurements. Not all patients with RV dilation share the same intracardiac flow characteristics, now measurable by time resolved three-dimensional phase contrast imaging (4D flow). In our study, we quantified regional vorticity and energy loss in rTOF patients and correlated these parameters with RV dysfunction and exercise capacity.

METHODS: …

The Value Of Cognitive Pretesting: Improving Validity And Revealing Blind Spots Through The Development Of A Newborn Screening Parent Experiences Survey., Norma-Jean Simon, Anne Atkins, Brianne Miller, Natasha Bonhomme, Beth Tarini

Pediatrics Faculty Publications

Surveys are used to gather a range of data on newborn screening (NBS) processes. We describe the development of a survey about parents' NBS experiences, in the United States, informed by cognitive pretest interviews among parents with varying NBS test results (true-positive, false-positive, normal). Cognitive pretest interviews were conducted following a semi-structured script and notes were taken to identify problematic survey items. The study team met weekly to discuss pretest feedback, draft changes, and generate revised items. Pretests indicated that parent experiences with NBS are varied and NBS screening procedures are not well understood. Substantial modifications were made to survey …

Integrated Omics Endotyping Of Infants With Respiratory Syncytial Virus Bronchiolitis And Risk Of Childhood Asthma., Yoshihiko Raita, Marcos Pérez-Losada, Robert J. Freishtat, Brennan Harmon, Jonathan M Mansbach, Pedro A Piedra, Zhaozhong Zhu, Carlos A Camargo, Kohei Hasegawa

Pediatrics Faculty Publications

Respiratory syncytial virus (RSV) bronchiolitis is not only the leading cause of hospitalization in U.S. infants, but also a major risk factor for asthma development. While emerging evidence suggests clinical heterogeneity within RSV bronchiolitis, little is known about its biologically-distinct endotypes. Here, we integrated clinical, virus, airway microbiome (species-level), transcriptome, and metabolome data of 221 infants hospitalized with RSV bronchiolitis in a multicentre prospective cohort study. We identified four biologically- and clinically-meaningful endotypes: A) clinical

Gaba And Glutamate In The Preterm Neonatal Brain: In-Vivo Measurement By Magnetic Resonance Spectroscopy., Sudeepta K. Basu, Subechhya Pradhan, Adre J. Du Plessis, Yehezkel Ben-Ari, Catherine Limperopoulos

Pediatrics Faculty Publications

Cognitive and behavioral disabilities in preterm infants, even without obvious brain injury on conventional neuroimaging, underscores a critical need to identify the subtle underlying microstructural and biochemical derangements. The gamma-aminobutyric acid (GABA) and glutamatergic neurotransmitter systems undergo rapid maturation during the crucial late gestation and early postnatal life, and are at-risk of disruption after preterm birth. Animal and human autopsy studies provide the bulk of current understanding since non-invasive specialized proton magnetic resonance spectroscopy (

Observing Pediatric Residents' Communication Skills During Sick Visits: Do They Determine Concern And Their Reasons For Concern And Are Caregivers Satisfied?, Carter Guice, Kristine Schmitz, Annette Aldous, Larrie Greenberg

Pediatrics Faculty Publications

Fifty years ago, Dr. Barbara Korsch published her seminal work on pediatric resident communication skills, demonstrating that residents did not always ascertain the caregivers’ main concern for the visit was and specifically, why those caregivers were concerned.

Repeating her study using a direct observation methodology, the authors evaluated 103 pediatric sick visits at a large children’s hospital primary care clinic to determine 1) if residents elicited the caregiver’s main concern about their child’s acute illness, 2) why they were concerned, and 3) whether asking these questions was statistically associated with caregiver satisfaction, which was determined by an exit survey.

Results …

Prolonged Environmental Enrichment Promotes Developmental Myelination., Evan Z Goldstein, Vera Pertsovskaya, Thomas A Forbes, Jeffrey L Dupree, Vittorio Gallo

Pediatrics Faculty Publications

Postnatal neurodevelopment is profoundly influenced by environmental experiences. Environmental enrichment is a commonly used experimental paradigm that has uncovered numerous examples of experience-dependent plasticity in health and disease. However, the role of environmental enrichment in normal development, especially glial development, is largely unexplored. Oligodendrocytes, the myelin-forming glia in the central nervous system, provide metabolic support to axons and establish efficient saltatory conduction by producing myelin. Indeed, alterations in myelin are strongly correlated with sensory, cognitive, and motor function. The timing of developmental myelination is uniquely positioned to be influenced by environmental stimuli, as peak myelination occurs postnatally and continues into …

Sex-Specific Social Behavior And Amygdala Proteomic Deficits In, Maria Jesus Herrero, Li Wang, David Hernandez-Pineda, Payal Banerjee, Heidi Y. Matos, Meredith Goodrich, Aswini Panigrahi, Nathan Anthony Smith, Joshua G. Corbin

Pediatrics Faculty Publications

In humans, mutations in the transcription factor encoding gene, FOXP2, are associated with language and Autism Spectrum Disorders (ASD), the latter characterized by deficits in social interactions. However, little is known regarding the function of Foxp2 in male or female social behavior. Our previous studies in mice revealed high expression of Foxp2 within the medial subnucleus of the amygdala (MeA), a limbic brain region highly implicated in innate social behaviors such as mating, aggression, and parental care. Here, using a comprehensive panel of behavioral tests in male and female Foxp2 +/- heterozygous mice, we investigated the role Foxp2 plays …

Potential Consequences Of The Red Blood Cell Storage Lesion On Cardiac Electrophysiology, Marissa Reilly, Chantel D. Bruno, Tomas M. Prudencio, Nina Ciccarelli, Devon Guerrelli, Raj Nair, Manelle Ramadan, Naomi L C Luban, Nikki Gillum Posnack

Pediatrics Faculty Publications

No abstract provided.

Intraoperative Laser Speckle Contrast Imaging For Real-Time Visualization Of Cerebral Blood Flow In Cerebrovascular Surgery: Results From Pre-Clinical Studies., Antonella Mangraviti, Francesco Volpin, Jaepyeong Cha, Samantha I Cunningham, Karan Raje, M Jason Brooke, Henry Brem, Alessandro Olivi, Judy Huang, Betty M Tyler, Abhishek Rege

Pediatrics Faculty Publications

No abstract provided.

Optocardiography And Electrophysiology Studies Of Ex Vivo Langendorff-Perfused Hearts., Luther M. Swift, Rafael Jaimes Iii, Damon Mccullough, Morgan Burke, Marissa Reilly, Takuya Maeda, Hanyu Zhang, Nobuyuki Ishibashi, Jack M. Rogers, Nikki Gillum Posnack

Pediatrics Faculty Publications

No abstract provided.

Plasticizer Interaction With The Heart: Chemicals Used In Plastic Medical Devices Can Interfere With Cardiac Electrophysiology., Rafael Jaimes, Damon Mccullough, Bryan Siegel, Luther Swift, Daniel Mcinerney, James Hiebert, Erick A Perez-Alday, Beatriz Trenor, Jiansong Sheng, Javier Saiz, Larisa G Tereshchenko, Nikki Gillum Posnack

Pediatrics Faculty Publications

BACKGROUND: Phthalates are used as plasticizers in the manufacturing of flexible, plastic medical products. Patients can be subjected to high phthalate exposure through contact with plastic medical devices. We aimed to investigate the cardiac safety and biocompatibility of mono-2-ethylhexyl phthalate (MEHP), a phthalate with documented exposure in intensive care patients.

METHODS: Optical mapping of transmembrane voltage and pacing studies were performed on isolated, Langendorff-perfused rat hearts to assess cardiac electrophysiology after MEHP exposure compared with controls. MEHP dose was chosen based on reported blood concentrations after an exchange transfusion procedure.

RESULTS: Thirty-minute exposure to MEHP increased the atrioventricular node (147 …

Lights, Camera, Path Splitter: A New Approach For Truly Simultaneous Dual Optical Mapping Of The Heart With A Single Camera., Rafael Jaimes, Damon Mccullough, Bryan Siegel, Luther Swift, James Hiebert, Daniel Mclnerney, Nikki Gillum Posnack

Pediatrics Faculty Publications

Background: Optical mapping of transmembrane voltage and intracellular calcium is a powerful tool for investigating cardiac physiology and pathophysiology. However, simultaneous dual mapping of two fluorescent probes remains technically challenging. We introduce a novel, easy-to-use approach that requires a path splitter, single camera and excitation light to simultaneously acquire voltage and calcium signals from whole heart preparations, which can be applied to other physiological models - including neurons and isolated cardiomyocytes.

Results: Complementary probes were selected that could be excited with a single wavelength light source. Langendorff-perfused hearts (rat, swine) were stained and imaged using a sCMOS camera outfitted with …

Dual-Display Laparoscopic Laser Speckle Contrast Imaging For Real-Time Surgical Assistance, Jaepyeong Cha, Corey Zheng, Lung Wai Lau

Pediatrics Faculty Publications

Laser speckle contrast imaging (LSCI) utilizes the speckle pattern of a laser to determine the blood flow in tissues. The current approaches for its use in a clinical setting require a camera system with a laser source on a separate optical axis making it unsuitable for minimally invasive surgery (MIS). With blood flow visualization, bowel viability, for example, can be determined. Thus, LSCI can be a valuable tool in gastrointestinal surgery. In this work, we develop the first-of-its-kind dual-display laparoscopic vision system integrating LSCI with a commercially available 10mm rigid laparoscope where the laser has the same optical axis as …

Results From A 78-Week, Single-Arm, Open-Label Phase 2 Study To Evaluate Ux007 In Pediatric And Adult Patients With Severe Long-Chain Fatty Acid Oxidation Disorders (Lc-Faod)., Jerry Vockley, Barbara Burton, Gerard T Berry, Nicola Longo, John Phillips, Pranoot Tanpaiboon, +Several Additional Authors

Pediatrics Faculty Publications

Long-chain fatty acid oxidation disorders (LC-FAOD) are rare disorders characterized by acute crises of energy metabolism and severe energy deficiency that may present with cardiomyopathy, hypoglycemia, and/or rhabdomyolysis, which can lead to frequent hospitalizations and early death. An open-label Phase 2 study evaluated the efficacy of UX007, an investigational odd-carbon medium-chain triglyceride, in 29 subjects with severe LC-FAOD. UX007 was administered over 78 weeks at a target dose of 25-35% total daily caloric intake (mean 27.5%). The frequency and duration of major clinical events (hospitalizations, emergency room visits, and emergency home interventions due to rhabdomyolysis, hypoglycemia, and cardiomyopathy) occurring during …

Frequency Of Cd19, Qianzi Zhao, Lawrence K Jung

Medicine Faculty Publications

BACKGROUND: To understand the relationship between regulatory B cells (Bregs) and juvenile idiopathic arthritis (JIA), we analyzed the percentages of Bregs and their function in peripheral blood (PB) and synovial fluid (SF) of JIA patients.

METHODS: Twenty-one JIA patients and 11 children with growing pain but without known rheumatic diseases as controls were included. The B cell phenotype and intracellular production of IL-10 of Bregs were assessed by flow cytometry. Mononuclear cells from PB and SF were stimulated to produce IL-10 in vitro for the identification of IL-10- producing regulatory B cells.

RESULTS: The percentage of CD24

CONCLUSIONS: The inability …

Economic Evaluation Of Patient Navigation Programs In Colorectal Cancer Care, A Systematic Review., Chloé Gervès-Pinquié, Anne Girault, Serena Phillips, Sarah Raskin, Mandi Pratt-Chapman

Pediatrics Faculty Publications

Patient navigation has expanded as a promising approach to improve cancer care coordination and patient adherence. This paper addresses the need to identify the evidence on the economic impact of patient navigation in colorectal cancer, following the Health Economic Evaluation Publication Guidelines. Articles indexed in Medline, Cochrane, CINAHL, and Web of Science between January 2000 and March 2017 were analyzed. We conducted a systematic review of the literature using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The quality assessment of the included studies was based on the Consolidated Health Economic Evaluation Reporting Standards (CHEERS) checklist. Inclusion criteria …

Lung Cancer Risk And Low (≤50 Μg/L) Drinking Water Arsenic Levels For Us Counties (2009⁻2013)-A Negative Association., Steven H Lamm, Isabella J Boroje, Hamid Ferdosi, Jaeil Ahn

Pediatrics Faculty Publications

While epidemiologic studies clearly demonstrate drinking water with high levels of arsenic as a significant risk factor for lung cancer, the evidence at low levels (≤50 μg/L) is uncertain. Therefore, we have conducted an ecological analysis of recent lung cancer incidence for US counties with a groundwater supply of <50 μg/L, the historical limit for both the EPA and WHO. Data sources used included USGS for arsenic exposure, NCI for lung cancer outcome, and CDC and US Census Bureau forcovariates. Poisson log-linear models were conducted for male, female, and total populations using for exposure median county arsenic level, maximum arsenic level ≤50 μg/L, and ≥80% population groundwater dependency. Statistically significant negative associations were found in each of the six models in which the exposure was limited to those who had major exposure (≥80% dependency) to low-levels of arsenic (≤50 μg/L). This is the first large ecological study of lung cancer risk from drinking water arsenic levels that specifically examined the dose-response slope for populations whose exposure was below the historical limit of ≤50 μg/L. The models for each of the three populations (total; male; female) demonstrated an association that is both negative and statistically significant.

First-Pass Cyp3a-Mediated Metabolism Of Midazolam In The Gut Wall And Liver In Preterm Neonates., Janneke M Brussee, Huixin Yu, Elke H J Krekels, Berend De Roos, Margreke J E Brill, Johannes N Van Den Anker, Amin Rostami-Hodjegan, Saskia N De Wildt, Catherijne A J Knibbe

Pediatrics Faculty Publications

To predict first-pass and systemic cytochrome P450 (CYP) 3A-mediated metabolism of midazolam in preterm neonates, a physiological population pharmacokinetic model was developed describing intestinal and hepatic midazolam clearance in preterm infants. On the basis of midazolam and 1-OH-midazolam concentrations from 37 preterm neonates (gestational age 26-34 weeks) receiving midazolam orally and/or via a 30-minute intravenous infusion, intrinsic clearance in the gut wall and liver were found to be very low, with lower values in the gut wall (0.0196 and 6.7 L/h, respectively). This results in a highly variable and high total oral bioavailability of 92.1% (range, 67-95%) in preterm neonates, …

Admission Eeg Findings In Diverse Paediatric Cerebral Malaria Populations Predict Outcomes., Douglas G Postels, Xiaoting Wu, Chenxi Li, Peter W Kaplan, Karl B Seydel, Terrie E Taylor, Youssef A Kousa, Richard Idro, Robert Opoka, Chandy C John, Gretchen L Birbeck

Pediatrics Faculty Publications

BACKGROUND: Electroencephalography at hospital presentation may offer important insights regarding prognosis that can inform understanding of cerebral malaria (CM) pathophysiology and potentially guide patient selection and risk stratification for future clinical trials. Electroencephalogram (EEG) findings in children with CM in Uganda and Malawi were compared and associations between admission EEG findings and outcome across this diverse population were assessed. Demographic, clinical and admission EEG data from Ugandan and Malawian children admitted from 2009 to 2012 with CM were gathered, and survivors assessed for neurological abnormalities at discharge.

RESULTS: 281 children were enrolled (Uganda n = 122, Malawi n = 159). …