Pediatrics Commons^™

Improving Nicu Discharge Readiness Through Standardization Of Preparatory Practices, David Carola

Department of Pediatrics Posters

Global Aim

Critical transitions of care for every newborn will meet the Institute of Medicine STEEEPS criteria: Safe, Timely, Efficient, Effective, Equitable, Patient and Family Centered1, and Socially Responsible

SMART Aim

We aim to increase our average discharge readiness score by 4 points by August 1st of 2020

Reflux, Apnea And The Impact On Discharge, Michele Savin, Dnp, Nnp-Bc, Kaitlin Kenaley, Md, Maureen Moffet, Msn, Rula Nassar, Md

College of Nursing Posters

Decisions made about timing of discharge can be subjective. Definitions of significant bradycardia are multiple, and interventions are variable.

This necessitates an informed discussion with parents about the natural course of apnea, bradycardia and reflux.

The physiology of gastroesophageal reflux and respiratory control have intermingled confounding variables.

Critical thinking is required to educate and support families, however a paucity of research exists. Risks and benefits of pharmacologic and nonpharmacologic interventions should be evaluated.

Nurses need increased confidence in understanding how to approach the preterm infant with apnea and/or reflux as well as how to interact with families to support discharge …

Development Of Substrate Degradation Enzyme Therapy For Mucopolysaccharidosis Iva Murine Model., Kazuki Sawamoto, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Mucopolysaccharidosis IVA (MPS IVA) is caused by a deficiency of the lysosomal enzyme N-acetylgalactosamine-6-sulfate sulfatase (GALNS). Conventional enzyme replacement therapy (ERT) is approved for MPS IVA. However, the fact that the infused enzyme cannot penetrate avascular lesions in cartilage leads to minimal impact on the bone lesion. Moreover, short half-life, high cost, instability, and narrow optimal pH range remain unmet challenges in ERT. Thermostable keratanase, endo-β-N-acetylglucosaminidase, has a unique character of a wide optimal pH range of pH 5.0-7.0. We hypothesized that this endoglycosidase degrades keratan sulfate (KS) polymer in circulating blood and, therefore, ameliorates the accumulation of KS in …

Effects Of The 2017 Clinical Practice Guidelines On Hypertension In Children And Adolescents: A Commentary, Bonita Falkner

Department of Medicine Faculty Papers

No abstract provided.

Biochemical And Clinical Response After Umbilical Cord Blood Transplant In A Boy With Early Childhood-Onset Beta-Mannosidosis., Troy C. Lund, Weston P. Miller, Julie B. Eisengart, Katrina Simmons, Laura Pollard, Deborah L. Renaud, David A. Wenger, Marc C. Patterson, Paul J. Orchard

Department of Neurology Faculty Papers

BACKGROUND: Deficiency in the enzyme β-mannosidase was described over three decades ago. Although rare in occurrence, the presentation of childhood-onset β-mannosidase deficiency consists of hypotonia in the newborn period followed by global development delay, behavior problems, and intellectual disability. No effective pharmacologic treatments have been available.

METHODS: We report 2-year outcomes following the first umbilical cord blood transplant in a 4-year-old boy with early childhood-onset disease.

RESULTS: We show restoration of leukocyte β-mannosidase activity which remained normal at 2 years posttransplant, and a simultaneous increase in plasma β-mannosidase activity and dramatic decrease in urine-free oligosaccharides were also observed. MRI of …

Promoting Adherence To Influenza Vaccination Recommendations In Pediatric Practice., Lloyd N. Werk, Maria Carmen Diaz, Adriana Cadilla, James P. Franciosi, Jobayer Hossain

Jefferson Health - Northeast Papers (formerly Aria Health Papers)

OBJECTIVES: In the United States, nonadherence to seasonal influenza vaccination guidelines for children and adolescents is common and results in unnecessary morbidity and mortality. We conducted a quality improvement project to improve vaccination rates and test effects of 2 interventions on vaccination guidelines adherence.

METHODS: We conducted a cluster randomized control trial with 11 primary care practices (PRACTICE) that provided care for 11 293 individual children and adolescents in a children's health care system from September 2015 through April 2016. Practice sites (with their clinicians) were randomly assigned to 4 arms (no intervention [Control], computerized clinical decision support system [CCDSS], …

Quantitative Sensory Testing In Children With Sickle Cell Disease: Additional Insights And Future Possibilities., Robin E. Miller, Dawn S. Brown, Scott W. Keith, Sarah E. Hegarty, Yamaja Setty, Claudia M. Campbell, Suzanne M. Mccahan, Suhita Gayen-Betal, Hal Byck, Marie Stuart

Department of Pharmacology and Experimental Therapeutics Faculty Papers

Quantitative sensory testing (QST) is used in a variety of pain disorders to characterize pain and predict prognosis and response to specific therapies. In this study, we aimed to confirm results in the literature documenting altered QST thresholds in sickle cell disease (SCD) and assess the test-retest reliability of results over time. Fifty-seven SCD and 60 control subjects aged 8-20 years underwent heat and cold detection and pain threshold testing using a Medoc TSAII. Participants were tested at baseline and 3 months; SCD subjects were additionally tested at 6 months. An important facet of our study was the development and …

Biomarkers In Patients With Mucopolysaccharidosis Type Ii And Iv., Honoka Fujitsuka, Kazuki Sawamoto, Hira Peracha, Robert W. Mason, William Mackenzie, Hironori Kobayashi, Seiji Yamaguchi, Yasuyuki Suzuki, Kenji Orii, Tadao Orii, Toshiyuki Fukao, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Glycosaminoglycans (GAGs), dermatan sulfate (DS), heparan sulfate (HS), and keratan sulfate (KS), are the primary biomarkers in patients with mucopolysaccharidoses (MPS); however, little is known about other biomarkers. To explore potential biomarkers and their correlation with GAGs, blood samples were collected from 46 MPS II patients, 34 MPS IVA patients, and 5 MPS IVB patients. We evaluated the levels of 8 pro-inflammatory factors (EGF, IL-1β, IL-6, MIP-1α, TNF-α, MMP-1, MMP-2, and MMP-9), collagen type II, and DS, HS (HS0S, HSNS), and KS (mono-sulfated, di-sulfated) in blood. Eight biomarkers measured were significantly elevated in untreated MPS II patients, compared with those …

Whole Genome Metagenomic Analysis Of The Gut Microbiome Of Differently Fed Infants Identifies Differences In Microbial Composition And Functional Genes, Including An Absent Crispr/Cas9 Gene In The Formula-Fed Cohort, Matthew D. Di Guglielmo, Karl Franke, Courtney Cox, Erin L. Crowgey

Department of Pediatrics Faculty Papers

Background: Advancements in sequencing capabilities have enhanced the study of the human microbiome. There are limited studies focused on the gastro-intestinal (gut) microbiome of infants, particularly the impact of diet between breast-fed (BF) versus formula-fed (FF). It is unclear what effect, if any, early feeding has on short- term or long-term composition and function of the gut microbiome.

Results: Using a shotgun metagenomics approach, differences in the gut microbiome between BF (n = 10) and FF (n = 5) infants were detected. A Jaccard distance principle coordinate analysis was able to cluster BF versus FF infants based on the presence …

Health Insurance Coverage Of Recommended Gender-Affirming Health Care Services For Transgender Youth: Shopping Online For Coverage Information, Nadia L. Dowshen, Julie Christensen, Siobhan Gruschow

Student Papers, Posters & Projects

We assessed online health insurance plan indication of coverage and accessibility of information for recommended services for transgender youth (TY). Content analysis was performed for plans used at a pediatric Gender Clinic by reviewing information about coverage of puberty blockers, hormones, masculinizing chest surgery, and counseling. Transgender-specific exclusions and the time required for the research assistant to review each plan's online information were noted. No plan (0%; n=36) indicated coverage of all four categories of recommended services online. Forty-nine percent indicated ≥1 transgender-specific exclusion. The median time required for a research assistant to review online coverage information for each insurance …

Histological Chorioamnionitis Induces Differential Gene Expression In Human Cord Blood Mononuclear Leukocytes From Term Neonates., Suhita Gayen Nee’ Betal, Swati Murthy, Michael Favara, Gina Fong, Joanna Chan, Sankar Addya, Thomas H. Shaffer, Jay S. Greenspan, Vineet Bhandari, Irfan Rahman, Zubair H. Aghai

Department of Pediatrics Faculty Papers

Histological chorioamnionitis (HCA) is an infection of fetal membranes and complicates 5.2% to 28.5% of all live births. HCA is associated with increased mortality and morbidity in both premature and term neonates. Exposure to HCA may have long-term consequences, including an increased risk for allergic disorders and asthma later in childhood, the mechanism(s) of which are still not yet well understood. The objective of this study was to determine the mRNA transcriptome of cord blood mononuclear leukocytes from term neonates to identify key genes and pathways involved in HCA. We found 366 differentially expressed probe IDs with exposure to HCA …

Maternal And Gestational Influences On Childhood Blood Pressure., Bonita Falkner

Department of Medicine Faculty Papers

Exposures that contribute to a sub-optimal intrauterine environment can have an effect on the developing fetus. Impaired fetal growth that results in low birth weight is an established risk factor for cardio-metabolic disorders later in life. Recent epidemiologic and prospective cohort studies that include the maternal and gestational period have identified maternal and gestational conditions that confer increased risk for subsequent cardio-metabolic disorders in the absence of low birth weight. Maternal pre-conception health status, including chronic obesity and type 2 diabetes, increase risk for childhood obesity and obesity-related higher blood pressure (BP) in child offspring. Maternal gestational exposures, including gestational …

A Closer Look At Arsa Activity In A Patient With Metachromatic Leukodystrophy., Kathleen Doherty, S. Barron Frazier, Matthew Clark, Anna Childers, Sumit Pruthi, David A. Wenger, Jessica Duis

Department of Neurology Faculty Papers

Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease mainly caused by a deficiency of arylsulfatase A activity. The typical clinical course of patients with the late infantile form includes a regression in motor skills with progression to dysphagia, seizures, hypotonia and death. We present a case of a 4-year-old female with rapidly progressive developmental regression with loss of motor milestones, spasticity and dysphagia. MRI showed volume loss and markedly abnormal deep white matter. Enzymatic testing in one laboratory showed arylsulfatase A activity in their normal range. However, extraction of urine showed a large increase in sulfatide excretion in …

Early Progression Of Krabbe Disease In Patients With Symptom Onset Between 0 And 5 Months., Maria L. Beltran-Quintero, Nicholas A. Bascou, Michele D. Poe, David A. Wenger, Carlos A. Saavedra-Matiz, Matthew J. Nichols, Maria L. Escolar

Department of Neurology Faculty Papers

BACKGROUND: Krabbe disease is a rare neurological disorder caused by a deficiency in the lysosomal enzyme, β-galactocerebrosidase, resulting in demyelination of the central and peripheral nervous systems. If left without treatment, Krabbe disease results in progressive neurodegeneration with reduced quality of life and early death. The purpose of this prospective study was to describe the natural progression of early onset Krabbe disease in a large cohort of patients.

METHODS: Patients with early onset Krabbe disease were prospectively evaluated between 1999 and 2018. Data sources included diagnostic testing, parent questionnaires, standardized multidisciplinary neurodevelopmental assessments, and neuroradiological and neurophysiological tests.

RESULTS: We …

Pharmacological And Non-Pharmacological Treatments For The Neonatal Abstinence Syndrome (Nas)., A. K. Mangat, G. M. Schmölzer, W. K. Kraft

Department of Pharmacology and Experimental Therapeutics Faculty Papers

Neonatal abstinence syndrome is defined by signs and symptoms of withdrawal that infants develop after intrauterine maternal drug exposure. All infants with documented in utero opioid exposure, or a high pre-test probability of exposure should have monitoring with a standard assessment instrument such as a Finnegan Score. A Finnegan score of >8 is suggestive of opioid exposure, even in the absence of declared use during pregnancy. At least half of infants in most locales can be treated without the use of pharmacologic means. For this reason, symptom scores will drive the decision for pharmacologic therapy. Nevertheless, all infants, regardless of …

Minimally Invasive Surgery In Neonates With Congenital Anomalies: Experience From The Nsqip-P, Emily Sagalow, Katerina Dukleska, Md, Courtney Devin, Md, Erin Teeple, Md, Scott W. Cowan, Md, Charles D. Vincour, Md, Loren Berman, Md

Phase 1

Background: Congenital diaphragmatic hernias (CDH) and tracheoesophageal fistulas (TEF) are managed with minimally invasive surgery (MIS) or open surgery. Little is known about the patient populations and outcomes for those treated by each approach. Hypothesis/Specific Aims: We expect that there will be fewer complications, better outcomes, and longer operative times for the MIS group versus the open group.

Methods: National Surgical Quality Improvement Program-Pediatric Participant Use Files (NSQIP-P PUFs) from 2012-2015 were used to identify neonates (up to 30 days old) who underwent CDH and TEF repair. The patient characteristics, post-operative complications, and 30-day mortality were analyzed using multivariable logistic …

Management Of Retinoblastoma In Older Children (>5 Years) Using Intra-Arterial Chemotherapy: Comparison Of Outcomes To Pre-Chemotherapy And Intravenous Chemotherapy Eras, Evan Selzer, Ms, R. Joel Welch, Md, Pascal Jabbour, Md, Ann Leahey, Md, Carol L. Shields, Md

Phase 1

Introduction: Intra-arterial chemotherapy (IAC) has emerged as an effective treatment for retinoblastoma (RB), but little information exists regarding its use in older patients (>5 years). We evaluate the use of IAC (2008-2018) for RB in older patients and compare outcomes to those in the pre-chemotherapy (<1994) and intravenous chemotherapy (IVC) (1994-2007) eras.

Objective: To evaluate the hypothesis that IAC is effective in managing RB in older patients.

Methods: We performed a retrospective analysis of all patients older than 5 years who were treated with IAC for RB from 2008-2018 on the Ocular Oncology Service at the Wills Eye Hospital. Comparisons were made to historic, …

Cross-Sectional Analysis Of Sound Levels In The Neonatal Intensive Care Unit (Nicu) At Thomas Jefferson University Hospital, Caroline Komlo, Martin Morris, Robert Ries, Michael Velez

Phase 1

Introduction . Infants in the NICU are considered at greater risk of developmental delay. It is now known that excessively loud noise can have a negative impact on parameters such as blood pressure, breathing, heart beat and oxygen saturation. Previous research has concluded that the optimal decibel (dB) level for proper growth of neonate hair cells rests around 45dB. Consequently, the American Academy of Pediatrics recommends that noise levels in the NICU be maintained to a maximum of 45dBA. However, little research has focused on designing new noise-altering products and their impact on neonatal outcomes.

Methods. This was a cross …

Common Practice For Behavioral Health Screening In Pediatric Primary Care Settings, Joseph A. Menand, Mario Cruz, Md

Phase 1

Mental health disorders in the United States are highly prevalent, affecting 14-20% of children yearly. Only 25-35% receive treatment after on average a six-plus year delay from symptom onset. Current recommendations suggest primary care physicians/providers (PCP) manage mental health conditions, however, PCPs are wary, citing lack of time, training and resources.This study evaluated pediatric/adolescent PCPs using a self-report questionnaire assessing knowledge/skill, clinical practice (e.g. screening tool) and perceived barriers when managing attention deficit hyperactivity disorder (ADHD), anxiety and depression (N = 11). Self-reported knowledge/skill diagnosing, treating and referring each disorder were recorded on a 5-point Likert scale and evaluated using …

Feasibility Of Using A Commercial Fitness Tracker As An Adjunct To Family-Based Weight Management Treatment: Pilot Randomized Trial., Thao-Ly Tam Phan, Nadia Barnini, Sherlly Xie, Angelica Martinez, Lauren Falini, Atiera Abatemarco, Maura Waldron, Jane M. Benton, Steve Frankenberry, Cassandra Coleman, Linhda Nguyen, Cindy Bo, George A. Datto, Lloyd N. Werk

Department of Pediatrics Faculty Papers

BACKGROUND: Fitness trackers can engage users through automated self-monitoring of physical activity. Studies evaluating the utility of fitness trackers are limited among adolescents, who are often difficult to engage in weight management treatment and are heavy technology users.

OBJECTIVE: We conducted a pilot randomized trial to describe the impact of providing adolescents and caregivers with fitness trackers as an adjunct to treatment in a tertiary care weight management clinic on adolescent fitness tracker satisfaction, fitness tracker utilization patterns, and physical activity levels.

METHODS: Adolescents were randomized to 1 of 2 groups (adolescent or dyad) at their initial weight management clinic …

Prenatal Tobacco And Marijuana Co-Use: Impact On Newborn Neurobehavior., Laura R. Stroud, George D. Papandonatos, Meaghan Mccallum, Tessa Kehoe, Amy L. Salisbury, Marilyn A. Huestis

Institute of Emerging Health Professions Faculty Papers

Tobacco and marijuana are some of the most common prenatal substance exposures worldwide. The social acceptability and political landscape of marijuana and its potency have changed dramatically in the last two decades leading to increased use by pregnant women. Despite evidence for increasing marijuana use and high rates of co-use of tobacco (TOB) and marijuana (MJ) during pregnancy, the impact of prenatal exposure to each substance is typically studied in isolation. We investigated the influence of co-exposure to TOB and MJ on infant neurobehavioral development over the first postnatal month. Participants were 111 mother-infant pairs from a low-income, diverse sample …

Three Years Of Growth Hormone Therapy In Children Born Small For Gestational Age: Results From The Answer Program, Robert Rapaport, Peter Lee, Judith L. Ross, Paul Saenger, Vlady Ostrow, Giuseppe Piccoli

Department of Pediatrics Faculty Papers

Growth hormone (GH) is used to treat short stature and growth failure associated with growth disorders. Birth size and GH status variably modulate response to GH therapy. The aim of this study was to determine the effect of birth size on response to GH therapy, and to determine the impact of GH status in patients born small for gestational age (SGA) on response to GH therapy. Data from the prospective, non-interventional American Norditropin® Studies: Web-Enabled Research (ANSWER) Program were analyzed for several growth outcomes in response to GH therapy over 3 years. GH-naïve children from the ANSWER Program were included …

Pediatric Obesity: Influence On Drug Dosing And Therapeutics, Barbara Ameer Pharmd, Mba, Bcps, Fcp, Michael Weintraub Md

Department of Medicine Faculty Papers

Obesity is an ongoing global health concern and has only recently been recognized as a chronic disease of energy homeostasis and fuel partitioning. Obesity afflicts 17% of US children and adolescents. Severe obesity (³120% of the 95^th percentile of BMI-for-age, or a BMI of ³35 kg/m²) is the fastest growing subgroup and now approaches 6% of all US youth. Health consequences (e.g., type 2 diabetes, coronary heart disease) are related in a dose-dependent manner to severity of obesity. Since therapeutic interventions are less effective in severe obesity, prevention is a high priority.

Treatment plans involving combinations of …

Clinical Perspective On Passive Audio Vocal Measurement In The Evaluation Of Selective Mutism., Helen Y Xu, Jacob Stroud, Renee K. Jozanovic, Jon Clucas, Jake Jungwoo Son, Bonhwang Koo, Juliet Schwarz, Arno Klein, Rachel Busman, Michael P. Milham

Student Papers, Posters & Projects

Selective Mutism (SM) is an anxiety disorder often diagnosed in early childhood and characterized by persistent failure to speak in certain social situations but not others. Diagnosing SM and monitoring treatment response can be quite complex, due in part to changing definitions of and scarcity of research about the disorder. Subjective self-reports and parent/teacher interviews can complicate SM diagnosis and therapy, given that similar speech problems of etiologically heterogeneous origin can be attributed to SM. The present perspective discusses the potential for passive audio capture to help overcome psychiatry's current lack of objective and quantifiable assessments in the context of …

Evaluating Comfort Measures For Commonly Performed Painful Procedures In Pediatric Patients., Sana Dastgheyb, Keith Fishlock, Constantine Daskalakis, Jami Kessel, Paul Rosen

Department of Pharmacology and Experimental Therapeutics Faculty Papers

Introduction: Management of pediatric pain from medical procedures is of great importance for improving both patient care and experience. In this study, we investigated methods of managing acute pain in infants and children by studying the correlation between the number of attempts to complete painful procedures, given different comfort measures.

Methods: The study is a retrospective review of 74,276 procedures performed at two pediatric hospitals in an integrated academic children's health system between 2013 and 2016. We compared three comfort measures most frequently offered: positions of comfort (POC), distraction (DIST), and pharmacological (PHARM). These methods were compared in the setting …

Early Gestational Mesenchymal Stem Cell Secretome Attenuates Experimental Bronchopulmonary Dysplasia In Part Via Exosome-Associated Factor Tsg-6., Sushma Chaubey, Sam Thueson, Devasena Ponnalagu, Mohammad Afaque Alam, Ciprian P. Gheorghe, Zubair Aghai, Harpreet Singh, Vineet Bhandari

Department of Pediatrics Faculty Papers

BACKGROUND: Mesenchymal stem cells (MSCs) are promising tools for the treatment of human lung disease and other pathologies relevant to newborn medicine. Recent studies have established MSC exosomes (EXO), as one of the main therapeutic vectors of MSCs in mouse models of multifactorial chronic lung disease of preterm infants, bronchopulmonary dysplasia (BPD). However, the mechanisms underlying MSC-EXO therapeutic action are not completely understood. Using a neonatal mouse model of human BPD, we evaluated the therapeutic efficiency of early gestational age (GA) human umbilical cord (hUC)-derived MSC EXO fraction and its exosomal factor, tumor necrosis factor alpha-stimulated gene-6 (TSG-6).

METHODS: Conditioned …

The Childhood Role In Development Of Primary Hypertension., Bonita Falkner

Department of Medicine Faculty Papers

Primary hypertension is not just an adult disorder. Current US population data on children and adolescents demonstrate a prevalence of elevated blood pressure (BP) and hypertension combined of over 10%. Recent reports from prospective cohort studies describe an association of high BP in childhood with hypertension in young adulthood. Excess adiposity is strongly associated with higher BP in childhood and increases risk for hypertension in adulthood. In addition to overweight/obesity, other exposures that raise the risk for high BP include low birthweight, dietary sodium, and stress. Using intermediate markers of cardiovascular injury, studies on hypertensive children report findings of cardiac …

Hey, What’S The Delay? An Evaluation Of Reporting Time For Pediatric Eegs, Aparna Polavarapu, Md, Rachit Patil, Md, Maromi Nei, Md

House Staff Quality Improvement and Patient Safety Conference (2016-2019)

Aim

To determine if there is a reduction in time to EEG reporting after institution of the Pediatric Epilepsy fellowship track.

Predicting Risk Of Hospitalisation: A Retrospective Population-Based Analysis In A Paediatric Population In Emilia-Romagna, Italy., Daniel Z. Louis, Clara A. Callahan, Mary Robeson, Mengdan Liu, Jacquelyn Mcrae, Joseph S. Gonnella, Marco Lombardi, Vittorio Maio

College of Population Health Faculty Papers

OBJECTIVES: Develop predictive models for a paediatric population that provide information for paediatricians and health authorities to identify children at risk of hospitalisation for conditions that may be impacted through improved patient care.

DESIGN: Retrospective healthcare utilisation analysis with multivariable logistic regression models.

DATA: Demographic information linked with utilisation of health services in the years 2006-2014 was used to predict risk of hospitalisation or death in 2015 using a longitudinal administrative database of 527 458 children aged 1-13 years residing in the Regione Emilia-Romagna (RER), Italy, in 2014.

OUTCOME MEASURES: Models designed to predict risk of hospitalisation or death in …

Associations Between Feeding Practices And Growth And Neurodevelopmental Outcomes, Barbara T. Do, Nellie I. Hansen, Carla Bann, Rebecca L. Lander, Shivaprasad S. Goudar, Omrana Pasha, Elwyn Chomba, Sangappa M. Dhaded, Vanessa R. Thorsten, Jan L. Wallander, Fred J. Biasini, Richard Derman, Robert L. Goldenberg, Waldemar A. Carlo

Global Health Articles

Background

Feeding practices over the first several years of a child’s life can critically influence development. The purpose of this study was to examine associations between feeding practices and growth and neurodevelopmental outcomes at 36 months of age among children from low- and low-middle-income countries (LMIC).

Methods

We conducted a secondary analysis using data collected from children in India, Pakistan, and Zambia who were enrolled in a randomized controlled trial of a home-based early development intervention program called Brain Research to Ameliorate Impaired Neurodevelopment Home-based Intervention Trial. Qualitative dietary data collected at 36 months was used to assess the modified …