Medicine and Health Sciences Commons^™

Pediatric Cardiovascular Care In Uganda: Current Status, Challenges, And Opportunities For The Future., Twalib Olega Aliku, Sulaiman Lubega, Judith Namuyonga, Tom Mwambu, Michael Oketcho, John O Omagino, Craig Sable, Peter Lwabi

Pediatrics Faculty Publications

In many developing countries, concerted action against common childhood infectious diseases has resulted in remarkable reduction in infant and under-five mortality. As a result, pediatric cardiovascular diseases are emerging as a major contributor to childhood morbidity and mortality. Pediatric cardiac surgery and cardiac catheterization interventions are available in only a few of Sub-Saharan African countries. In Uganda, open heart surgeries (OHSs) and interventional procedures for pediatric cardiovascular disease are only possible at the Uganda Heart Institute (UHI), having been started with the help of expatriate teams from the years 2007 and 2012, respectively. Thereafter, independent OHS and cardiac catheterization have …

Nasopharyngeal Microbiome Diversity Changes Over Time In Children With Asthma, Marcos Pérez-Losada, Lamia Alamri, Keith Crandall, Robert J. Freishtat

Pediatrics Faculty Publications

Background

The nasopharynx is a reservoir for pathogens associated with respiratory illnesses such as asthma. Next-generation sequencing (NGS) has been used to characterize the nasopharyngeal microbiome of infants and adults during health and disease; less is known, however, about the composition and temporal dynamics (i.e., longitudinal variation) of microbiotas from children and adolescents. Here we use NGS technology to characterize the nasopharyngeal microbiomes of asthmatic children and adolescents (6 to 18 years) and determine their stability over time.

Methods

Two nasopharyngeal washes collected 5.5 to 6.5 months apart were taken from 40 children and adolescents with asthma living in the …

"It Makes You Feel Like Someone Cares" Acceptability Of A Financial Incentive Intervention For Hiv Viral Suppression In The Hptn 065 (Tlc-Plus) Study., Elizabeth Greene, Allison Pack, Jill Stanton, Victoria Shelus, Elizabeth E Tolley, Jamilah Taylor, Wafaa M El Sadr, Bernard M Branson, Jason Leider, Natella Rakhmanina, Theresa Gamble

Pediatrics Faculty Publications

BACKGROUND: HPTN 065 (TLC-Plus) evaluated the feasibility and effectiveness of providing quarterly $70 gift card financial incentives to HIV-infected patients on antiretroviral therapy (ART) to encourage ART adherence and viral suppression, and represents the largest study to-date of a financial incentive intervention for HIV viral suppression. A post-trial qualitative substudy was undertaken to examine acceptability of the financial incentives among those receiving and implementing the intervention.

METHODS: Between July and October 2013, semi-structured interviews were conducted with 72 patients and 12 investigators from 14 sites; three focus groups were conducted with 12 staff from 10 sites. Qualitative data collection elicited …

Training Us Health Care Professionals On Human Trafficking: Where Do We Go From Here?, Clydette Powell, Kirsten Dickins, Hanni Stoklosa

Pediatrics Faculty Publications

Some 21 million adults and children are labor-trafficked or sex-trafficked through force, fraud, or coercion. In recognition of the interface between trafficking victims and the healthcare setting, over the last 10 years there has been a notable increase in training of health care professionals (HCPs) on human trafficking (HT) and its health implications. Many organizations have developed curricula and offered training in various clinical settings. However, methods and content of this education on trafficking vary widely, and there is little evaluation of the impact of the training. The goal of this study was to assess the gaps and strengths in …

Boiling Histotripsy Lesion Characterization On A Clinical Magnetic Resonance Imaging-Guided High Intensity Focused Ultrasound System., Avinash Eranki, Navid Farr, Ari Partanen, Karun Sharma, Hong Chen, Christopher T Rossi, Satya V V N Kothapalli, Matthew Oetgen, Aerang Kim, Ayele H Negussie, David Woods, Bradford J Wood, Peter C W Kim, Pavel S. Yarmolenko

Pediatrics Faculty Publications

PURPOSE: High intensity focused ultrasound (HIFU) is a non-invasive therapeutic technique that can thermally ablate tumors. Boiling histotripsy (BH) is a HIFU approach that can emulsify tissue in a few milliseconds. Lesion volume and temperature effects for different BH sonication parameters are currently not well characterized. In this work, lesion volume, temperature distribution, and area of lethal thermal dose were characterized for varying BH sonication parameters in tissue-mimicking phantoms (TMP) and demonstrated in ex vivo tissues.

METHODS: The following BH sonication parameters were varied using a clinical MR-HIFU system (Sonalleve V2, Philips, Vantaa, Finland): acoustic power, number of cycles/pulse, total …

Hypertension In The Pediatric Kidney Transplant Recipient., Olga Charnaya, Asha Moudgil

Pediatrics Faculty Publications

Hypertension after kidney transplant is a frequent occurrence in pediatric patients. It is a risk factor for graft loss and contributes to the significant burden of cardiovascular disease (CVD) in this population. The etiology of posttransplant hypertension is multifactorial including donor factors, recipient factors, medications, and lifestyle factors similar to those prevalent in the general population. Ambulatory blood pressure monitoring has emerged as the most reliable method for measuring hypertension in pediatric transplant recipients, and many consider it to be essential in the care of these patients. Recent technological advances including measurement of carotid intima-media thickness, pulse wave velocity, and …

Uhl’S Anomaly: A One And A Half Ventricular Repair In A Patient Presenting With Cardiac Arrest, Reginald Chonoune, Adam Lowry, Karthik Ramakrishnan, Gail D. Pearson, Jeffrey P. Moak, Dilip S. Nath

Pediatrics Faculty Publications

Uhl’s anomaly, first reported in 1952, is an extremely rare congenital cardiac defect characterized by partial or complete loss of the right ventricular myocardium and unknown etiology. Fewer than 100 cases have been described. The response to medical management is poor and there is no known ideal surgical approach or timing for treatment. We report the case of a previously active adolescent male presenting with cardiac arrest, who underwent successful bidirectional cavopulmonary anastomosis (“Glenn” anastomosis) with right atrial reduction and right ventricular free wall plication.

Malignant Peripheral Nerve Sheath Tumors State Of The Science: Leveraging Clinical And Biological Insights Into Effective Therapies., Aerang Kim, Douglas R Stewart, Karlyne M Reilly, David Viskochil, Markku M Miettinen, Brigitte C Widemann

Pediatrics Faculty Publications

Malignant peripheral nerve sheath tumor (MPNST) is the leading cause of mortality in patients with neurofibromatosis type 1. In 2002, an MPNST consensus statement reviewed the current knowledge and provided guidance for the diagnosis and management of MPNST. Although the improvement in clinical outcome has not changed, substantial progress has been made in understanding the natural history and biology of MPNST through imaging and genomic advances since 2002. Genetically engineered mouse models that develop MPNST spontaneously have greatly facilitated preclinical evaluation of novel drugs for translation into clinical trials led by consortia efforts. Continued work in identifying alterations that contribute …

Endotypes Of Difficult-To-Control Asthma In Inner-City African American Children., K R Brown, R Z Krouse, A Calatroni, C M Visness, U Sivaprasad, C M Kercsmar, E C Matsui, J B West, M M Makhija, M A Gill, H Kim, M Kattan, D Pillai, J E Gern, W W Busse, A Togias, A H Liu, G K Khurana Hershey

Pediatrics Faculty Publications

African Americans have higher rates of asthma prevalence, morbidity, and mortality in comparison with other racial groups. We sought to characterize endotypes of childhood asthma severity in African American patients in an inner-city pediatric asthma population. Baseline blood neutrophils, blood eosinophils, and 38 serum cytokine levels were measured in a sample of 235 asthmatic children (6-17 years) enrolled in the NIAID (National Institute of Allergy and Infectious Diseases)-sponsored Asthma Phenotypes in the Inner City (APIC) study (ICAC (Inner City Asthma Consortium)-19). Cytokines were quantified using a MILLIPLEX panel and analyzed on a Luminex analyzer. Patients were classified as Easy-to-Control or …

Training Us Health Care Professionals On Human Trafficking: Where Do We Go From Here?, Clydette Powell, Kirsten Dickins, Hanni Stoklosa

Pediatrics Faculty Publications

Some 21 million adults and children are labor-trafficked or sex-trafficked through force, fraud, or coercion. In recognition of the interface between trafficking victims and the healthcare setting, over the last 10 years there has been a notable increase in training of health care professionals (HCPs) on human trafficking (HT) and its health implications. Many organizations have developed curricula and offered training in various clinical settings. However, methods and content of this education on trafficking vary widely, and there is little evaluation of the impact of the training. The goal of this study was to assess the gaps and strengths in …

The Current Consensus On The Clinical Management Of Intracranial Ependymoma And Its Distinct Molecular Variants., Kristian W Pajtler, Stephen C Mack, Vijay Ramaswamy, Christian A Smith, Hendrik Witt, Amy Smith, Eugene Hwang, +Several Additional Authors

Pediatrics Faculty Publications

Multiple independent genomic profiling efforts have recently identified clinically and molecularly distinct subgroups of ependymoma arising from all three anatomic compartments of the central nervous system (supratentorial brain, posterior fossa, and spinal cord). These advances motivated a consensus meeting to discuss: (1) the utility of current histologic grading criteria, (2) the integration of molecular-based stratification schemes in future clinical trials for patients with ependymoma and (3) current therapy in the context of molecular subgroups. Discussion at the meeting generated a series of consensus statements and recommendations from the attendees, which comment on the prognostic evaluation and treatment decisions of patients …

Plasmodium Falciparum Histidine-Rich Protein-2 Plasma Concentrations Are Higher In Retinopathy-Negative Cerebral Malaria Than In Severe Malarial Anemia., Gregory S Park, Robert O Opoka, Estela Shabani, Alexis Wypyszynski, Benjamin Hanisch, Chandy C John

Pediatrics Faculty Publications

Background

Malaria retinopathy has been proposed as marker of “true” cerebral malaria (CM), ie, coma due to Plasmodium falciparum vs coma due to other causes, with incidental P falciparum parasitemia. Plasma P falciparum histidine-rich protein-2 (PfHRP2) concentrations distinguish retinopathy-positive (RP) from retinopathy-negative (RN) CM but have not been compared between RN CM and other forms of severe malaria or asymptomatic parasitemia (AP). Methods

We compared plasma PfHRP2 concentrations in 260 children with CM (247 examined for retinopathy), 228 children with severe malarial anemia (SMA), and 30 community children with AP. Results

Plasmodium falciparum HRP2 concentrations were higher in children with …

Transvenous Nonfluoroscopic Pacemaker Implantation During Pregnancy Guided By 3-Dimensional Electroanatomic Mapping, Jacob Hartz, Bradley C. Clark, Seiji Ito, Elizabeth D Sherwin, Charles I Berul

Pediatrics Faculty Publications

Patients with congenital heart disease are at ongoing risk of developing both bradyarrhythmias and tachyarrhythmias decades after surgical repair. Rarely, arrhythmias can be exacerbated during pregnancy and require emergent intervention. Here, we report unique experience with nonfluoroscopic pacemaker implantation during pregnancy. Ionizing radiation, even in low doses, is associated with an increased risk of malignancy, and a fetus may be at particularly increased risk.1, 2 Over the past 2 decades, the use of fluoroscopy in cardiac ablation procedures has become nearly obsolete with the development of 3-dimensional (3D) electroanatomic mapping software such as CARTO (Biosense-Webster, Diamond Bar, CA) …

Characteristics Of Multi-Institutional Health Sciences Education Research: A Systematic Review, Jocelyn Schiller, Gary L. Dallaghan, Terry Kind, Heather Mclauchlan, Joseph Gigante, Sherilyn Smith

Pediatrics Faculty Publications

Objectives: Multi-institutional research increases the generalizability of research findings. However, little is known about characteristics of collaborations across institutions in health sciences education research. Using a systematic review process, the authors describe characteristics of published, peer-reviewed multi-institutional health sciences education research to inform educators who are considering such projects.

Methods: Two medical librarians searched MEDLINE, the Education Resources Information Center (ERIC), EMBASE, and CINAHL databases for English-language studies published between 2004 and 2013 using keyword terms related to multi-institutional systems and health sciences education. Teams of two authors reviewed each study and resolved coding discrepancies through consensus. Collected data points …

Visual Neurons In The Superior Colliculus Innervated By Islet2+ Or Islet2− Retinal Ganglion Cells Display Distinct Tuning Properties, Rachel Kay, Jason W. Triplett

Pediatrics Faculty Publications

Throughout the visual system, different subtypes of neurons are tuned to distinct aspects of the visual scene, establishing parallel circuits. Defining the mechanisms by which such tuning arises has been a long-standing challenge for neuroscience. To investigate this, we have focused on the retina’s projection to the superior colliculus (SC), where multiple visual neuron subtypes have been described. The SC receives inputs from a variety of retinal ganglion cell (RGC) subtypes; however, which RGCs drive the tuning of different SC neurons remains unclear. Here, we pursued a genetic approach that allowed us to determine the tuning properties of neurons innervated …

Mycn Amplification Is Associated With Repressed Cellular Immunity In Neuroblastoma: An In Silico Immunological Analysis Of Target Database, Peng Zhang, Xiaofang Wu, Moushumi Basu, Chen Dong, Pan Zhang, Yang Liu, Anthony D. Sandler

Pediatrics Faculty Publications

Purpose: RNA and DNA sequencing data are traditionally used to discern intrinsic cellular pathways in cancer pathogenesis, their utility for investigating the tumor microenvironment (TME) has not been fully explored. This study explores the use of sequencing data to investigate immunity within the TME.

Experimental design: Here, we use immune cell fraction estimation analysis to determine the immune profiles in the microenvironment of neuroblastoma (NB) based on RNA-seq data in the TARGET database. The correlation between immune cell transcripts and prognosis in pediatric NB is also investigated.

Results: In silico analysis revealed a strong inverse correlation between MYCNamplification and …

Non-Invasive Placental Perfusion Imaging In Pregnancies Complicated By Fetal Heart Disease Using Velocity-Selective Arterial Spin Labeled Mri, Zungho Zun, Greg Zaharchuk, Nickie Niforatos Andescavage, Mary T. Donofrio, Catherine Limperopoulos

Pediatrics Faculty Publications

The placenta is a vital organ for fetal growth and development during pregnancy. Congenital heart disease (CHD) is a leading cause of morbidity and mortality in newborns. Despite the parallel development of the placenta and fetal heart early in pregnancy, very few studies suggested an association between placental dysfunction and fetal CHD. In this study, we report placental perfusion of healthy pregnancies and pregnancies complicated by fetal CHD measured using advanced fetal MRI techniques. We studied forty-eight pregnant women (31 healthy volunteers and 17 with fetal CHD) that underwent fetal MRI during their second or third trimester of pregnancy. Placental …

Lysosomal Storage Diseases, Carlos Ferreira, William Gahl

Pediatrics Faculty Publications

Lysosomes are cytoplasmic organelles that contain a variety of different hydrolases. A genetic deficiency in the enzymatic activity of one of these hydrolases will lead to the accumulation of the material meant for lysosomal degradation. Examples include glycogen in the case of Pompe disease, glycosaminoglycans in the case of the mucopolysaccharidoses, glycoproteins in the cases of the oligosaccharidoses, and sphingolipids in the cases of Niemann-Pick disease types A and B, Gaucher disease, Tay-Sachs disease, Krabbe disease, and metachromatic leukodystrophy. Sometimes, the lysosomal storage can be caused not by the enzymatic deficiency of one of the hydrolases, but by the deficiency …

Medical Genetics And Genomic Medicine In The United States. Part 2: Reproductive Genetics, Newborn Screening, Genetic Counseling, Training, And Registries, Debra S. Regier, Carlos Ferreira, Suzanne Hart, Donald Hadley, Maximilian Muenke

Pediatrics Faculty Publications

eview of genetics in the United States with emphasis on the prenatal, metabolic, genetic counseling, and training aspects of the field.

Disorders Of Metal Metabolism, Carlos Ferreira, William A. Gahl

Pediatrics Faculty Publications

Trace elements are chemical elements needed in minute amounts for normal physiology. Some of the physiologically relevant trace elements include iodine, copper, iron, manganese, zinc, selenium, cobalt and molybdenum. Of these, some are metals, and in particular, transition metals. The different electron shells of an atom carry different energy levels, with those closest to the nucleus being lowest in energy. The number of electrons in the outermost shell determines the reactivity of such an atom. The electron shells are divided in sub-shells, and in particular the third shell has s, p and d sub-shells. Transition metals are strictly defined as …

Relationship Of Common Vascular Anatomy To Cannulated Catheters., Paul Gagne, Karun Sharma

Pediatrics Faculty Publications

Superficial veins of the upper extremity are the primary location for placement of peripheral IV catheters (PIVC). It is believed that a significant portion of PIVCs placed may cross or abut valves and branching veins or occlude a significant portion of the vein, limiting the ability to aspirate blood from the PIVC. Two separate clinical investigations using ultrasound were performed to understand the potential interaction between PIVCs and the vein lumen and the venous valves and branches of the superficial veins of the upper extremity. One study with 35 adult volunteers interrogated 210 vein segments where a PIV would likely …

Genetic Variation In The Histamine Production, Response, And Degradation Pathway Is Associated With Histamine Pharmacodynamic Response In Children With Asthma, Bridgette L. Jones, Catherine M.T. Sherwin, Xiaoxi Liu, Hongying Dai, Carrie A. Vyhlidal

Pediatrics Faculty Publications

Introduction: There is growing knowledge of the wide ranging effects of histamine throughout the body therefore it is important to better understand the effects of this amine in patients with asthma. We aimed to explore the association between histamine pharmacodynamic (PD) response and genetic variation in the histamine pathway in children with asthma.

Methods: Histamine Iontophoresis with Laser Doppler Monitoring (HILD) was performed in children with asthma and estimates for area under the effect curve (AUEC), maximal response over baseline (Emax), and time of Emax (Tmax) were calculated using non-compartmental analysis and non-linear mixed-effects model with a linked effect PK/PD …

Predicting Tacrolimus Concentrations In Children Receiving A Heart Transplant Using A Population Pharmacokinetic Model, Joseph E. Rower, Chris Stockmann, Matthew W. Linakis, Shaun S. Kumar, Xiaoxi Liu, E. Kent Korgenski, Catherine M.T. Sherwin, Kimberly M. Molina

Pediatrics Faculty Publications

Objective Immunosuppressant therapy plays a pivotal role in transplant success and longevity. Tacrolimus, a primary immunosuppressive agent, is well known to exhibit significant pharmacological interpatient and intrapatient variability. This variability necessitates the collection of serial trough concentrations to ensure that the drug remains within therapeutic range. The objective of this study was to build a population pharmacokinetic (PK) model and use it to determine the minimum number of trough samples needed to guide the prediction of an individual’s future concentrations.

Design, setting and patients Retrospective data from 48 children who received tacrolimus as inpatients at Primary Children’s Hospital in Salt …

Necrotizing Fasciitis Secondary To Aeromonas Infection Presenting With Septic Shock, Nikhil Bhatia, Manuel Castro-Borobio, John N. Greene, Sowmya Nanjappa

Pediatrics Faculty Publications

This report describes a case of necrotizing fasciitis presenting with septic shock due to an Aeromonas infection. The patient cut his foot while mowing the lawn and then spent time in a pool with black mold. He began feeling ill and developed swelling and a quarter-sized black area on his right lower extremity. Despite being hemodynamically unstable with systolic blood pressure in the low 70s, the patient was transferred to our facility from outside hospital 100 miles away. Upon arriving to facility, the patient appeared to be septic and the infected area of skin had grown. Irrigation and debridement were …