Pediatrics Commons^™

Growth In Achondroplasia Including Stature, Weight, Weight-For-Height And Head Circumference From Clarity: Achondroplasia Natural History Study-A Multi-Center Retrospective Cohort Study Of Achondroplasia In The Us, Julie E Hoover-Fong, Kerry J Schulze, Adekemi Y Alade, Michael B Bober, Ethan Gough, S Shahrukh Hashmi, Jacqueline T Hecht, Janet M Legare, Mary Ellen Little, Peggy Modaff, Richard M Pauli, David F Rodriguez-Buritica, Maria E Serna, Cory Smid, Chengxin Liu, John Mcgready

Faculty, Staff and Student Publications

BACKGROUND: Achondroplasia is the most common genetic skeletal disorder causing disproportionate short stature/dwarfism. Common additional features include spinal stenosis, midface retrusion, macrocephaly and a generalized spondylometaphyseal dysplasia which manifest as spinal cord compression, sleep disordered breathing, delayed motor skill acquisition and genu varus with musculoskeletal pain. To better understand the interactions and health outcomes of these potential complications, we embarked on a multi-center, natural history study entitled CLARITY (achondroplasia natural history study). One of the CLARITY objectives was to develop growth curves (length/height, weight, head circumference, weight-for-height) and corresponding reference tables of mean and standard deviations at 1 month increments …

Biochemical Testing For The Diagnosis Of Wilson's Disease: A Systematic Review, Hafiz Muhammad Salman, Mahwish Amin, Javaria Syed, Zouina Sarfraz, Azza Sarfraz, Muzna Sarfraz, Maria Jose Farfán Bajaña, Miguel Felix, Ivan Cherrez-Ojeda

Department of Paediatrics and Child Health

Background: Wilson's disease (WD) is a rare inherited disorder that leads to copper accumulation in the liver, brain, and other organs. WD is prevalent worldwide, with an occurrence of 1 per 30,000 live births. Currently, there is no gold standard diagnostic test for WD. The objective of this systematic review is to determine the diagnostic accuracy for WD of three biochemical tests, namely hepatic copper, 24-hour urinary copper, and ceruloplasmin using the Leipzig criteria.
Methods: Adhering to PRISMA guidelines, databases including PubMed/MEDLINE, CINAHL Plus, Web of Science, and Cochrane were searched. Studies that comprised of confirmed or suspected WD along …

Evaluating The 0–10 Point Pain Scale On Adolescent Opioid Use In Us Emergency Departments, Michael T. Phan, Daniel M. Tomaszewski, Cody Arbuckle, Sun Yang, Brooke Jenkins, Michelle A. Fortier, Theodore Heyming, Erik Linstead, Candice Donaldson, Zeev N. Kain

Pharmacy Faculty Articles and Research

Objective: To evaluate trends in national emergency department (ED) adolescent opioid use in relation to reported pain scores. Methods: A retrospective, cross-sectional analysis on National Hospital Ambulatory Medical Care Survey (NHAMCS) data was conducted on ED visits involving patients aged 11–21 from 2008–2017. Crude observational counts were extrapolated to weighted estimates matching total population counts. Multivariate models were used to evaluate the role of a pain score in the reported use of opioids. Anchors for pain scores were 0 (no pain) and 10 (worst pain imaginable). Results: 31,355 observations were captured, which were extrapolated by the NHAMCS to represent 162,515,943 …

Eliminating Contamination In Umbilical Cord Blood Culture Sampling For Early-Onset Neonatal Sepsis., Vilmaris Quinones Cardona, Vanessa Lowery, David Cooperberg, Endla K Anday, Alison J Carey

Department of Pediatrics Faculty Papers

Introduction: Despite the advantages of umbilical cord blood culture (UCBC) use for diagnosis of early onset sepsis (EOS), contamination rates have deterred neonatologists from its widespread use. We aimed to implement UCBC collection in a level III neonatal intensive care unit (NICU) and apply quality improvement (QI) methods to reduce contamination in the diagnosis of early onset sepsis. Methods: Single-center implementation study utilizing quality improvement methodology to achieve 0% contamination rate in UCBC samples using the Plan-Do-Study-Act (PDSA) model for improvement. UCBC was obtained in conjunction with peripheral blood cultures (PBC) in neonates admitted to the NICU due to maternal …

The Similarities And Differences Between Glomerular Vs. Non-Glomerular Diagnoses On Intelligence And Executive Functions In Pediatric Chronic Kidney Disease: A Brief Report., Stephen R. Hooper, Rebecca J. Johnson, Marc Lande, Matthew Matheson, Shlomo Shinnar, Amy J. Kogon, Lyndsay Harshman, Joann Spinale, Arlene C. Gerson, Bradley A. Warady, Susan L. Furth

Manuscripts, Articles, Book Chapters and Other Papers

Pediatric chronic kidney disease (CKD) appears to be a heterogeneous group of conditions, but this heterogeneity has not been explored with respect to its impact on neurocognitive functioning. This study investigated the neurocognitive functioning of those with glomerular (G) vs. non-glomerular (NG) diagnoses. Data from the North American CKiD Study were employed and the current study included 1,003 children and adolescents with mild to moderate CKD. The G Group included 260 participants (median age = 14.7 years) and the NG Group included 743 individuals (median age = 9.0 years). Neurocognitive measures assessed IQ, inhibitory control, attention regulation, problem solving, working …

Post-Transplant Hepatic Graft Fibrosis In Pediatric Liver-Inclusive Transplant Recipients, Hanh D. Vo

Theses & Dissertations

Hepatic graft fibrosis is a common histologic finding following pediatric liver transplant (LT) that may affect the long-term graft outcome. Hence, it is essential to identify hepatic graft fibrosis at a stage where fibrosis is not yet clinically apparent but has the potential for progression to initiate appropriate intervention and prevent its progression. Liver biopsy is the gold standard for fibrosis staging, but it is invasive, requires general anesthesia in children, and has potential complications. Our long-term goal is to implement non-invasive tests to assess and monitor the progression of hepatic graft fibrosis in liver-inclusive transplant children. The work presented …

The Ecology Of Mental Health And The Impact Of Barriers On Mental Health Service Utilization, Alisha Aggarwal

Theses & Dissertations

Mental health has emerged as a major public health concern in recent times with several disparities and barriers related to adequate health service utilization. Different age groups experience diverse symptoms and face distinct barriers in accessing and utilizing healthcare. Policies like Mental Health Parity and Addiction Equity Act and the Patient Protection and Affordable Care Act were launched to improve insurance coverage and delivery of mental health services. However, a large gap of unmet needs exists for adequate mental health service utilization in the country. This dissertation aims to describe the financial and non-financial barriers to mental health service utilization …

Glad To Be Out. I Was Running Out Of Womb: A Study Of Omega-6 And Omega-3 Fatty Acid Metabolites At Delivery And Their Relationship To Infant Growth, Prematurity, And Admission To The Neonatal Intensive Care Unit, Maranda Thompson

Theses & Dissertations

Normal pregnancy relies on inflammation for implantation, placentation, and parturition, but uncontrolled it can lead to poor infant outcomes. Maternal diet is one modifiable factor that can impact inflammation. Omega-3 and omega-6 fatty acids have been shown to be metabolized into bioactive metabolites that effect inflammation. The presence and importance of these metabolites at the time of delivery are not well understood. Therefore, the purpose of this dissertation was to first quantify omega-3 and omega-6 metabolites in maternal plasma, cord plasma, and placental tissue. Then the relationships between metabolites and infant outcomes, including birth weight percentile, length percentile, head circumferences …

Does Digital Literacy Empower Adolescent Girls In Low- And Middle-Income Countries: A Systematic Review, Salima Meherali, Komal Abdul Rahim, Sandra Campbell, Zohra S. Lassi

School of Nursing & Midwifery

Background: The vast majority (90%) of the world's adolescents aged 10-19 live in low- and middle-income countries (LMICs); and in those resource-limited settings, girls face distinct challenges across multiple health, social, and economic domains. Gender equality and girls' empowerment are key goals in their own right and are central to all other development goals. Digital literacy is a great enabler for the empowerment of young girls. This systematic review aims to assess the range and nature of digital literacy interventions implemented to empower adolescent girls in LMICs and identify evidence about adolescent girls' access and use of digital technologies in …

Defining The Transcriptional Control Of Pediatric Aml Highlights Rara As A Superenhancer-Regulated Druggable Dependency, Monika W Perez, Oscar Sias-Garcia, Alfred Daramola, Helen Wei, Maci Terrell, Raushan Rashid, Woojun D Park, Kevin Duong, Terzah M Horton, Feng Li, Nikitha Cherayil, Jost Vrabic Koren, Vincent U Gant, Jacob J Junco, Choladda V Curry, Alexandra M Stevens, Charles Y Lin, Joanna S Yi

Faculty, Staff and Students Publications

Somatic mutations are rare in pediatric acute myeloid leukemia (pAML), indicating that alternate strategies are needed to identify targetable dependencies. We performed the first enhancer mapping of pAML in 22 patient samples. Generally, pAML samples were distinct from adult AML samples, and MLL (KMT2A)-rearranged samples were also distinct from non-KMT2A-rearranged samples. Focusing specifically on superenhancers (SEs), we identified SEs associated with many known leukemia regulators. The retinoic acid receptor alpha (RARA) gene was differentially regulated in our cohort, and a RARA-associated SE was detected in 64% of the study cohort across all cytogenetic and molecular subtypes tested. RARA SE+ pAML …

Evaluation Of Pediatric Rheumatology Telehealth Satisfaction During The Covid-19 Pandemic, Lindsay N Waqar-Cowles, John Chuo, Pamela F Weiss, Sabrina Gmuca, Marianna Lanoue, Jon M Burnham

College of Population Health Faculty Papers

Background: During the Coronavirus disease 2019 pandemic, ambulatory pediatric rheumatology healthcare rapidly transformed to a mainly telehealth model. However, pediatric patient and caregiver satisfaction with broadly deployed telehealth programs remains largely unknown. This study aimed to evaluate patient/caregiver satisfaction with telehealth and identify the factors associated with satisfaction in a generalizable sample of pediatric rheumatology patients.

Methods: Patients with an initial telehealth video visit with a rheumatology provider between April and June 2020 were eligible. All patients/caregivers were sent a post-visit survey to assess a modified version of the Telehealth Usability Questionnaire (TUQ) and demographic and clinical characteristics. TUQ total …

Go Baby Go! Facilitating Play And Social Participation With Blind & Visually Impaired Children, Kennedy Wischmeyer, Angela Blackwell, Gina Benevente

Fall 2021 Virtual OTD Capstone Symposium

ABSTRACT NEEDED

Coping In Pediatric Burn Survivors And Its Relation To Social Functioning And Self-Concept, Mira D H Snider, Sarah Young, Paul T Enlow, Corrine Ahrabi-Nejad, Ariel M Aballay, Christina L Duncan

Department of Pediatrics Faculty Papers

Pediatric burn survivors experience increased risk for bullying, stigmatization, body image concerns, and problematic social functioning. Although coping behaviors are associated with engagement in social supports and positive self-concept in multiple pediatric illness populations, their relation has not been examined in pediatric burns. This study examined coping in relation to social functioning and self-concept in 51 pediatric burn survivors aged 7–17years (M=12.54; SD=2.65). Survivors and their caregivers completed the Child Coping Strategies Checklist (CCSC; youth report); the Burn Injury Social Questionnaire (BISQ; parent and youth report); and the Piers-Harris Children’s Self-Concept Scale-2 (PH-2; youth report). Associations between coping, social functioning, …

Generating Evidence From Contextual Clinical Research In Low- To Middle Income Countries: A Roadmap Based On Theory Of Change, Babar Hasan, Muneera Rasheed, Asra Wahid, Raman Krishna Kumar, Liesl Zuhlke

Department of Paediatrics and Child Health

Along with inadequate access to high-quality care, competing health priorities, fragile health systems, and conflicts, there is an associated delay in evidence generation and research from LMICs. Lack of basic epidemiologic understanding of the disease burden in these regions poses a significant knowledge gap as solutions can only be developed and sustained if the scope of the problem is accurately defined. Congenital heart disease (CHD), for example, is the most common birth defect in children. The prevalence of CHD from 1990 to 2017 has progressively increased by 18.7% and more than 90% of children with CHD are born in Low …

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.

Bile Acid Profiling Reveals Distinct Signatures In Undernourished Children With Environmental Enteric Dysfunction, Xueheng Zhao, Kenneth D R. Setchell, Rong Huang, Indika Mallawaarachchi, Lubaina Ehsan, Edward Dobrzykowski Iii, Junfang Zhao, Sana Syed, Najeeha Talat Iqbal, Junaid Iqbal, Kamran Sadiq, Sheraz Ahmed, Syed Asad Ali

Department of Paediatrics and Child Health

Background: Intestinal inflammation and malabsorption in environmental enteric dysfunction (EED) are associated with early childhood growth faltering in impoverished settings worldwide.
Objectives: The goal of this study was to identify candidate biomarkers associated with inflammation, EED histology, and as predictors of later growth outcomes by focusing on the liver-gut axis by investigating the bile acid metabolome.
Methods: Undernourished rural Pakistani infants (n = 365) with weight-for-height Z score (WHZ) < -2 were followed up to the age of 24 mo and monitored for growth, infections, and EED. Well-nourished local children (n = 51) were controls, based on consistent WHZ > 0 and height-for-age Z score (HAZ) > -1 on 2 consecutive visits at 3 and 6 mo. Serum bile acid (sBA) profiles were measured by tandem MS at the ages of 3-6 …

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.

Changing Perceptions Of Rural Frontline Workers And Caregivers About Management Of Childhood Diarrhea And Pneumonia Despite Several Inequities: The Nigraan Plus Trial In Pakistan, Fauziah Rabbani, Hyder Ali Khan, Suneel Piryani, Nousheen Pradhan, Natasha Shaukat, Anam Shahil Feroz, Shagufta Perveen

Community Health Sciences

Purpose: Diarrhea and pneumonia greatly contribute to high childhood mortality in Pakistan. Frontline community health workers or the Lady Health Workers (LHWs) provide care at the doorstep of over 60% of Pakistan's rural residents. Difficult terrain, lack of supplies, and inadequate supervision put these LHWs at an added disadvantage in the timely diagnosis and delivery of known treatment options to community caregivers (CCGs). This study aims to assess whether a supportive supervision intervention through Lady Health Supervisors (LHSs) using enhanced mentorship and written feedback cards have the potential to improve case management of childhood diarrhea and pneumonia.
Study setting and …

Placental Infarction And Intrauterine Growth Restriction Following Sars-Cov-2 Infection, Stephanie Moltner, Barbra De Vrijer, Harrison Banner

Paediatrics Publications

No abstract provided.

The Intranasal Dexmedetomidine Plus Ketamine For Procedural Sedation In Children, Adaptive Randomized Controlled Non-Inferiority Multicenter Trial (Ketodex): A Statistical Analysis Plan, Anna Heath, Juan David Rios, Eleanor Pullenayegum, Petros Pechlivanoglou, Martin Offringa, Maryna Yaskina, Rick Watts, Shana Rimmer, Terry P. Klassen, Kamary Coriolano, Naveen Poonai, Darcy Beer, Scott Sawyer, Maala Bhatt, April Kam, Quynh Doan, Vikram Sabhaney, Serena Hickes, Samina Ali, Karly Stillwell, Tannis Erickson, Chelsea Bowkett, Chelsea Bowkett, Carolyn Shimmin, Brendon Foot, Candace Mcgahern, Redjana Carciurmaruj, Jeannine Schellenberg

Paediatrics Publications

Background: Procedural sedation and analgesia (PSA) is frequently required to perform closed reductions for fractures and dislocations in children. Intravenous (IV) ketamine is the most commonly used sedative agent for closed reductions. However, as children find IV insertion a distressing and painful procedure, there is need to identify a feasible alternative route of administration. There is evidence that a combination of dexmedetomidine and ketamine (ketodex), administered intranasally (IN), could provide adequate sedation for closed reductions while avoiding the need for IV insertion. However, there is uncertainty about the optimal combination dose for the two agents and whether it can provide …

Choosing Wisely Canada – Pediatric Otolaryngology Recommendations, Mitchell Mcdonough, Kalpesh Hathi, Gerard Corsten, Christopher J. Chin, Paolo Campisi, Jonathan Cavanagh, Neil Chadha, M. Elise Graham, Murad Husein, Liane B. Johnson, Jodi Jones, Bruce Korman, John Manoukian, Lily H.P. Nguyen, Doron D. Sommer, Julie Strychowsky, Trina Uwiera, Warren Yunker, Paul Hong

Paediatrics Publications

The Choosing Wisely Canada campaign raises awareness amongst physicians and patients regarding unnecessary or inappropriate tests and treatments. Using an online survey, members of the Pediatric Otolaryngology Subspecialty Group within the Canadian Society of Otolaryngology – Head & Neck Surgery developed a list of nine evidence based recommendations to help physicians and patients make treatment decisions regarding common pediatric otolaryngology presentations: (1) Don’t routinely order a plain film x-ray in the evaluation of nasal fractures; (2) Don’t order imaging to distinguish acute bacterial sinusitis from an upper respiratory infection; (3) Don’t place tympanostomy tubes in most children for a single …

Parents’ Report Of Canadian Elementary School Children’S Physical Activity And Screen Time During The Covid-19 Pandemic: A Longitudinal Study, Emma Ostermeier, Patricia Tucker, Andrew Clark, Jamie A. Seabrook, Jason Gilliland

Paediatrics Publications

COVID-19 public health protocols have altered children’s daily routines, limiting their physical activity opportunities. The purpose of this study was to examine how the COVID-19 pandemic affected children’s (ages 10–12 years) physical activity and screen time, and to explore the impact of gender, socioeconomic status (SES), and public health constraints (i.e., facility use and social interaction) on the changes in children’s health behaviors. Online surveys were disseminated to parents at two time points: before COVID-19 (May 2019 to February 2020) and during COVID-19 (November to December 2020). Wilcoxon signed-rank tests were used to assess changes in physical activity and screen …

Neonatal Outcomes And Congenital Anomalies In Pregnancies Affected By Hypothyroidism, Zareen Kiran,, Aisha Sheikh, Khadija N Humayun, Najmul Islam

Section of Diabetes, Endocrinology and Metabolism

Background: Maternal hypothyroidism has been reported to have concerns over neonatal outcomes, not only in the context of neurocognitive development but also in the short term as birth weight and neonatal jaundice.
Patients and methods: We conducted a cross-sectional retrospective study on 638 cases who delivered live births in the Aga Khan University Hospital after ethical approval. Data were collected on hypothyroid pregnant females who were diagnosed before conception or during their antenatal visits during the year 2008-2016. Neonatal outcomes were noted for birth weight, maturity, and neonatal jaundice, neonatal hypothyroidism, neonatal respiratory distress syndrome, sepsis, hypocalcaemia, congenital anomalies, need …

Qualitative Exploration Of Feasibility And Acceptability Of The Modified Atkins Diet Therapy For Children With Drug Resistant Epilepsy In Kenya, Pauline Samia, Violet Naanyu, J. Helen Cross, Richard Idro, Paul Boon, Jo Wilmshurst, Stanley Luchters

Paediatrics and Child Health, East Africa

Purpose: Approximately one-third of children with epilepsy have clinical syndromes characterized by drug resistance. Modified Atkins dietary therapy (MADT) can reduce seizures and improve health out- comes for these children. This intervention is yet to be consistently offered as standard of care in sub- Saharan Africa.

Objectives: This study aimed to assess feasibility and acceptability of MADT for children with drug- resistant epilepsy and identify enabling strategies for implementation and adherence to the MADT. Methods: This qualitative inquiry utilized in-depth interviews with purposively selected caregivers and adolescent patients having used MADT for drug-resistant epilepsy. A qualified team consisting …

Grading Of Improvement In Hypsarrhythmia With Standard Epileptic Spasms Treatment At A Large Pediatric Tertiary Care Center, Julie Grace Gianakon, Roha Khalid, Mohammed Ilyas

Posters

Background Epileptic spasm is a peculiar type of epileptic seizure, entailing the clinical spasms and a characteristic electroencephalogram (EEG) abnormality often called hypsarrhythmia or its variants. The main goal of epileptic spasm treatment with standard therapy is to suppress clinical spasms and abolish the hypsarrhythmia and its variant EEG pattern. This interictal EEG pattern frequently heralds developmental regression. The elimination of hypsarrhythmia is a principal goal of therapy and a key outcome measure in clinical trials. There have been several studies in the interpretation or grading of hypsarrhythmia (Watanabe et al.1993 & Jeavons & Bower et al.1961). Still, there have …

Central Line Associated Bloodstream Infection Caused By Kodamaea Ohmeri In A Young Child, Amier Haidar, Farhana Khaja, Brian Simms, Amr Issam Elgehiny, Tracy Omoegbele, Nikita Khetan

Faculty, Staff and Student Publications

INTRODUCTION:

CASE REPORT: The patient is a 5-year-old male with a history of short gut syndrome, and total parenteral nutrition (TPN) dependence who presented to the emergency room with a two-day history of productive-cough, rhinorrhea, and fever. Antibiotic therapy was initiated with cefepime and vancomycin for suspected CLABSI. However, within the first twenty-four hours of his admission, his initial blood culture from his central venous catheter became positive for yeast so fluconazole was added due to suspicion of candidemia. During his admission, his initial central line and peripheral blood culture were later speciated as

CONCLUSIONS: This case report further acknowledges …

Alterations In Excitatory And Inhibitory Synaptic Development Within The Mesolimbic Dopamine Pathway In A Mouse Model Of Prenatal Drug Exposure, Taylor Boggess, James C. Williamson, Ethan B. Niebergall, Hannah Sexton, Richard D. Egleton, Larry Grover, Chris Risher

Biomedical Sciences

The rise in rates of opioid abuse in recent years in the United States has led to a dramatic increase in the incidence of neonatal abstinence syndrome (NAS). Despite improved understanding of NAS and its acute symptoms, there remains a paucity of information regarding the long-term effects of prenatal exposure to drugs of abuse on neurological development. The primary goal of this study was to investigate the effects of prenatal drug exposure on synaptic connectivity within brain regions associated with the mesolimbic dopamine pathway, the primary reward pathway associated with drug abuse and addiction, in a mouse model. Our secondary …

Disparities In Delaware Caregiver Beliefs About The Covid-19 Vaccine For Their Children, Thao-Ly Phan, Paul T Enlow, Michael K. Wong, Amanda M. Lewis, Anne E Kazak, Jonathan M. Miller

Department of Pediatrics Faculty Papers

Objective: To describe sociodemographic disparities in caregiver beliefs about the COVID-19 vaccine for their children. Methods: This was a cross-sectional study, linking caregiver-reported data to geocoded sociodemographic data from child EHRs. Caregivers of children receiving care in a Delaware pediatric healthcare system were invited to complete a survey about COVID-19 vaccine beliefs from March 19 to April 16, 2021. Results: 1499 caregivers participated (18% Black, 11% Hispanic, 32% public insurance, 12% rural). 54% of caregivers intended to vaccinate their children, while 34% were unsure and 12% would not. Caregivers of younger children (aOR 3.70, CI 2.36-5.79), Black children (aOR 2.11, …

Identifying The Molecular Cause Of Extreme Endoplasmic Reticulum Dilation In Pediatric Osteosarcoma And Its Relationship To The Disease, Rachael Wood

Theses and Dissertations (ETD)

Pediatric osteosarcoma tumors are characterized by an unusual abundance of grossly dilated endoplasmic reticulum and an immense genomic instability that has complicated identifying new effective molecular therapeutic targets. Here we report a novel molecular signature that encompasses the majority of 108 patient tumor samples, PDXs and osteosarcoma cell lines. These tumors exhibit reduced expression of four critical COPII vesicle proteins that has resulted in the accumulation of procollagen-I protein within ‘hallmark’ dilated ER. Using CRISPR activation technology, increased expression of only SAR1A and SEC24D to physiologically normal levels was sufficient to restore both collagen-I secretion and resolve dilated ER morphology …

Epidural Blood Patch For Pediatric Patients Only, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.