Pediatrics Commons^™

Next-Generation Sequencing In The Diagnosis Of Rare Pediatric Sinonasal Tumors., Atif A. Ahmed, Divya Vundamati, Midhat Farooqi, Elena Repnikova, Timothy Zinkus, Maxine Hetherington, Lorien Paulson

Manuscripts, Articles, Book Chapters and Other Papers

The diagnosis of desmoid fibromatosis or other spindle cell tumors in the sinonasal region is very rare in children and needs to be thoroughly confirmed with immunohistochemical and/or molecular tests. We report 2 patients with such rare tumors and describe the use of next-generation sequencing in their evaluation. A 3-year-old female had a 4.4-cm midline nasal cavity mass involving the bony septum and extending into the base of the skull bilaterally. The moderate cellular fibroblastic proliferation revealed areas of thick keloid-like collagen bands and other areas with myxoid edematous stroma. Deep targeted sequencing identified a novel G34V mutation in the

Covid-19 In Children-Not Just Little Adults., Jennifer E. Schuster, Annabelle De St Maurice

Manuscripts, Articles, Book Chapters and Other Papers

Comment on

• Underlying Medical Conditions Associated With Severe COVID-19 Illness Among Children. Kompaniyets L, Agathis NT, Nelson JM, Preston LE, Ko JY, Belay B, Pennington AF, Danielson ML, DeSisto CL, Chevinsky JR, Schieber LZ, Yusuf H, Baggs J, Mac Kenzie WR, Wong KK, Boehmer TK, Gundlapalli AV, Goodman AB.JAMA Netw Open. 2021 Jun 1;4(6):e2111182. doi: 10.1001/jamanetworkopen.2021.11182.PMID: 34097050 Free PMC article.

The Dietary Management Of Potassium In Children With Ckd Stages 2-5 And On Dialysis-Clinical Practice Recommendations From The Pediatric Renal Nutrition Taskforce., An Desloovere, José Renken-Terhaerdt, Jetta Tuokkola, Vanessa Shaw, Larry A. Greenbaum, Dieter Haffner, Caroline Anderson, Christina L. Nelms, Michiel J S Oosterveld, Fabio Paglialonga, Nonnie Polderman, Leila Qizalbash, Bradley A. Warady, Rukshana Shroff, Johan Vande Walle

Manuscripts, Articles, Book Chapters and Other Papers

Dyskalemias are often seen in children with chronic kidney disease (CKD). While hyperkalemia is common, with an increasing prevalence as glomerular filtration rate declines, hypokalemia may also occur, particularly in children with renal tubular disorders and those on intensive dialysis regimens. Dietary assessment and adjustment of potassium intake is critically important in children with CKD as hyperkalemia can be life-threatening. Manipulation of dietary potassium can be challenging as it may affect the intake of other nutrients and reduce palatability. The Pediatric Renal Nutrition Taskforce (PRNT), an international team of pediatric renal dietitians and pediatric nephrologists, has developed clinical practice recommendations …

Correlation Between Strain And Weight Status In Infants With A Univentricular Hear, Amulya Buddhavarapu, Mathew Warren, Lori Erickson, Christopher Mathis, Whitney Haas, Daniel Forsha

Posters

Background

Infants with univentricular physiology are at risk of poor weight gain and reduced ventricular function, both of which have been independently associated with worse outcomes. Since nutritional status has been correlated to ventricular function in other populations, we evaluated the relationship between nutritional status and ventricular function including speckle-tracking strain during this period.

Methods

Thirty term infants (median age 55 days, 13 females) with univentricular physiology prior to stage II palliation were included with data obtained at the time of their initial hospital discharge. Ventricular function was quantified using 2D global longitudinal strain (GLS) and strain rate, analyzed from …

Outpatient Antibiotic Handbook, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Clinical Characterization Of Pediatric Gastroparesis Using A Four-Hour Gastric Emptying Scintigraphy Standard, Sharon Wolfson, Zoe Wilhelm, Antone R Opekun, Robert Orth, Robert J Shulman, Bruno P Chumpitazi

Children’s Nutrition Research Center Staff Publications

Objectives: Four-hour gastric emptying scintigraphy (GES) is the recommended method to identify both adult and childhood gastroparesis (GP). Previous pediatric studies have, however, not used this standard. We sought to determine the characteristics and outcomes of children versus adolescents with GP using the 4-hour GES evaluation.

Methods: We performed a retrospective chart review of pediatric patients diagnosed with GP by 4-hour GES (>10% retention at 4 hours). Demographics, body mass index, GP-related symptoms, comorbidities, etiologies, therapies (eg, medications), healthcare utilization, and response to therapy were captured systematically. Symptoms were compared from the initial versus last gastroenterology visit. Outcomes were …

Neurosurgical Shunts, Trouble Shooting For Malfunction, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Neurosurgical Shunts, Trouble Shooting For Infection, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Clinical, Neuroimaging, And Molecular Spectrum Of Tecpr2-Associated Hereditary Sensory And Autonomic Neuropathy With Intellectual Disability, Sonja Neuser, Barbara Brechmann, Gali Heimer, Ines Brösse, Susanna Schubert, Lauren O'Grady, Michael Zech, Siddharth Srivastava, Shahnaz Ibrahim, Fatima Khan

Department of Paediatrics and Child Health

Bi-allelic TECPR2 variants have been associated with a complex syndrome with features of both a neurodevelopmental and neurodegenerative disorder. Here, we provide a comprehensive clinical description and variant interpretation framework for this genetic locus. Through international collaboration, we identified 17 individuals from 15 families with bi-allelic TECPR2-variants. We systemically reviewed clinical and molecular data from this cohort and 11 cases previously reported. Phenotypes were standardized using Human Phenotype Ontology terms. A cross-sectional analysis revealed global developmental delay/intellectual disability, muscular hypotonia, ataxia, hyporeflexia, respiratory infections, and central/nocturnal hypopnea as core manifestations. A review of brain magnetic resonance imaging scans demonstrated a …

Evaluating Motor Performance With The Bruininks-Oseretsky Test Of Motor Proficiency In Impoverished Pakistani Children, Zehra Habib Hasan, Fariha Shaheen, Arjumand Rizvi, Jelena Obradovic, Aisha Khan Yousafzai

Department of Paediatrics and Child Health

Objective: To evaluate the sensitivity of the modified Brief Form of Bruininks Oseretsky Test in identifying motor differences secondary to malnutrition and poverty.
Methods: This longitudinal cohort study was conducted at Nowshero Feroze, Sindh from 2013 to 2014 and comprised data drawn from children who participated in a randomised controlled trial, that assessed responsive stimulation and nutrition interventions in the first two years of life. Outcome measures included motor development assessed using Brief Form of Bruininks Oseretsky Test, child anthropometry and household economic and demographic information. Data was analysed using SPSS 15 and STATA 12.
Results: Of the 1058 children, …

Paediatric Pseudo Papillary Pancreatic Tumour: A Case Based Review, Mir Ibrahim Sajid, Ayesha Saleem, Muhammad Arshad

Medical College Documents

Solid pseudopapillary neoplasms are rare and represent approximately 4% of all cystic pancreatic tumours, and predominately affect females. They have an excellent prognosis, however 10-15% of the patients show metastasis at the time of surgery or recurrence of tumour during follow-up after pancreatectomy. Surgical resection is the recommended treatment. We present a rare case of a 15-year-old female, with a pseudopapillary tumour of the pancreas which was diagnosed pre-operatively.

Extracellular Superoxide Dismutase (Ec-Sod) Regulates Gene Methylation And Cardiac Fibrosis During Chronic Hypoxic Stress., Ayan Rajgarhia, Kameshwar R. Ayasolla, Nahla Zaghloul, Jorge M. Lopez Da Re, Edmund J. Miller, Mohamed Ahmed

Manuscripts, Articles, Book Chapters and Other Papers

Chronic hypoxic stress induces epigenetic modifications mainly DNA methylation in cardiac fibroblasts, inactivating tumor suppressor genes (RASSF1A) and activating kinases (ERK1/2) leading to fibroblast proliferation and cardiac fibrosis. The Ras/ERK signaling pathway is an intracellular signal transduction critically involved in fibroblast proliferation. RASSF1A functions through its effect on downstream ERK1/2. The antioxidant enzyme, extracellular superoxide dismutase (EC-SOD), decreases oxidative stress from chronic hypoxia, but its effects on these epigenetic changes have not been fully explored. To test our hypothesis, we used an in-vitro model: wild-type C57B6 male mice (WT) and transgenic males with an extra copy of human hEC-SOD (TG). …

Racial And Ethnic Disparities In Opioid Use For Adolescents At Us Emergency Departments, Michael T. Phan, Daniel M. Tomaszewski, Cody Arbuckle, Sun Yang, Candice D. Donaldson, Michelle A. Fortier, Brooke Jenkins, Erik Linstead, Zeev N. Kain

Pharmacy Faculty Articles and Research

Background

Racial/ethnic disparities in the use of opioids to treat pain disorders have been previously reported in the emergency department (ED). Further research is needed to better evaluate the impact race/ethnicity may have on the use of opioids in adolescents for the management of pain disorders in the ED.

Methods

This was a cross-sectional study using data from the National Hospital Ambulatory Medical Care Survey from 2006 to 2016. Multivariate models were used to evaluate the role of race/ethnicity in the receipt of opioid agonists while in the ED. All ED visits with patients aged 11–21 years old were analyzed. …

A Review Of Toolkits And Case Definitions For Detecting Enteric Fever Outbreaks In Asian And African Countries From 1965-2019, Asif Khaliq, Mohammad Tahir Yousafzai, Salman Haq, Rahima Yaseen, Sonia Qureshi, Fahad Rind, Zahra A. Padhani, Ayub Khan, Abdul Momin Kazi, Farah Naz Qamar

Department of Paediatrics and Child Health

Background: This review assessed the case definitions, diagnostic criteria, antimicrobial resistance, and methods used for enteric fever outbreaks and utilization of any unified outbreak score or checklist for early identification and response in Asia and Africa from 1965-2019.
Methods: We searched enteric fever outbreaks using PubMed, Google Scholar, and the Cochrane library. Studies describing a single outbreak event of enteric fever in Asia and Africa from 1965-2019 were reviewed. We excluded case reports, letter to editors, studies reporting typhoid in conjunction with other diseases, the Centers for Disease Control and Prevention (CDC) trip reports, the World Health Organization (WHO) bulletins …

Luspatercept Diminishes The Need For Red Blood Cell Replacement In Transfusion-Dependent Β-Thalassemia Patients, Joudeh B. Freij

Clinical Research in Practice: The Journal of Team Hippocrates

A clinical decision report appraising:

Cappellini MD, Viprakasit V, Taher AT, et al. A phase 3 trial of luspatercept in patients with transfusion-dependent β-thalassemia. N Engl J Med. 2020;382(13):1219-1231. https://doi.org/10.1056/NEJMoa1910182

for a patient with transfusion-dependent beta-thalassemia.

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 (

Adolescent And Young Adult Oncology Patient And Provider Perspectives In Philadelphia (Aya-4p), Christoher Terry, Md, Miriam Pomerantz, Lsw, Jodi Sandos, Lcsw, Lindsay Wilde, Md

Health Equity and Quality Improvement (HEQI) Summit

Primary Objectives

• To assess the current perspective of AYA patients regarding medical services and psychosocial support delivered to AYAs in Philadelphia
• To assess the current knowledge and perspective of healthcare providers at the SKCC about the care and services needed by AYA patients

Impact Of Local Terminology On The Design Of A Community-Based Diagnostic And Management Algorithm For Postpartum Sepsis: Findings Of Formative Research, Shabina Ariff, Fatima Mir, Wafa Aftab, Farrukh Raza, Shujaat Zaidi, Sheraz Memon, Amnesty Lefevre, Linda A. Bartlett, Peter Winch, Sajid Bashir Soofi, Zulfiqar Ahmed Bhutta

Department of Paediatrics and Child Health

Background: Postpartum sepsis is one of the leading causes of maternal mortality and morbidity in developing countries. This formative research elicits local terms used for postpartum illnesses and symptoms of postpartum sepsis with the aim of improving postpartum diagnosis and management in Pakistan.
Methods: We conducted 34 in-depth interviews with recently delivered women (RDW), traditional birth attendants (TBAs), healthcare providers and family members of RDW from rural Sindh to explore local Sindhi terms used to describe postpartum sepsis and related symptoms. During interviews, all participants were asked to orally free list common symptoms of postpartum illnesses; those who were aware …

Plasma Adenosine Deaminase (Ada)-1 And -2 Demonstrate Robust Ontogeny Across The First Four Months Of Human Life., Oludare A. Odumade, Alec L. Plotkin, Jensen Pak, Olubukola T. Idoko, Matthew A. Pettengill, Tobias R. Kollmann, Al Ozonoff, Beate Kampmann, Ofer Levy, Kinga K. Smolen

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Background: Human adenosine deaminases (ADAs) modulate the immune response: ADA1

Methods: In a subgroup of the EPIC002-study, clinical data and plasma samples were collected from 540 Gambian infants at four time-points: day of birth; first week of life; one month of age; and four months of age. Concentrations of total extracellular ADA, ADA1, and ADA2 were measured by chromogenic assay and evaluated in relation to clinical data. Plasma cytokines/chemokine were measured across the first week of life and correlated to ADA concentrations.

Results: ADA2 demonstrated a steady rise across the first months of life, while ADA1 concentration significantly decreased 0.79-fold …

Sunscreen Use And Screening In College Athletes: An Evidence-Based Pilot Project, Nicole Brustkern Bsn, Rn, Cpn, Sharon Boothe-Kepple Phd, Msn, Fnp-C, Phn (Chickasaw)

Doctor of Nursing Practice Final Manuscripts

Background: Melanoma is the third most common cancer in individuals ages 15 – 29. The greatest risk for melanoma is ultraviolet (UV) radiation from the sun. Collegiate athletes are exposed to 1,000 more sun hours per year than the average adult, placing them at higher risk for melanoma. Evidence supports sunscreen education and screening to promote protective behaviors in young adults.

Objective: This evidence-based pilot project implements the standardized screening of sunscreen use and sun protective behaviors in women’s soccer players ages 18 to 21 years old at the University of San Diego.

Methods: The Sun Exposure and Protection Index …

Treatment Anxiety Education For Pediatric Cardiac Neurodevelopmental Patients, An Evidence Based Project, Mary Nagel

Doctor of Nursing Practice Final Manuscripts

Abstract

Background

Congenital heart disease (CHD) is the most common birth defect and affects almost 1% of all live births. Treatment anxiety is common among children with CHD who undergo multiple procedures early in their lifetime. Dramatically increased life spans for this cardiac population has led to a shift from focusing on survival to their long-term health outcomes.

Methods:

Administration of the Peds QL Cardiac Module 3.0 assesses health related quality of life (HRQOL) during annual visits to a Cardiac Neurodevelopmental (ND) Clinic by evaluating the level of Treatment Anxiety a patient experiences during healthcare appointments. The questionnaire scores are …

Providing High Reliability Care For Picu Patients With Pulmonary Hypertension: A Quality Improvement Project, Anjali Subbaswamy, Nicholas Tan, Iric Guthrie, Kelsey Fath, Cole Linzmeyer, Elizabeth Yakes Jimenez

Quality Improvement/Patient Safety Symposium

Providing High Reliability Care for PICU Patients with Pulmonary Hypertension: A Quality Improvement Project

Anjali Subbaswamy, MD; Nicholas Tan, DO; Iric Guthrie, MD; Kelsey Fath, MD; Cole Linzmeyer, DO; Elizabeth Yakes Jimenez, PhD, RDN, LD

Purpose: Pediatric patients with pulmonary hypertension (PHTN) are a high acuity, low frequency population, and are often admitted to the pediatric intensive care unit (PICU). To mitigate the risk of precipitating a potentially life-threatening pulmonary hypertensive crisis, health care staff need to take specific precautions. This quality improvement (QI) project aims to increase the number of inpatient PHTN patients for whom PHTN precautions are applied …

A Multicenter Evaluation Of Viral Bloodstream Detections In Children Presenting To The Emergency Department With Suspected Systemic Infection., Christina A. Rostad, Neena Kanwar, Jumi Yi, Claudia R. Morris, Jennifer Dien Bard, Amy Leber, James Dunn, Kimberle C. Chapin, Anne J. Blaschke, Judy A. Daly, Leslie Hueschen, Matthew Jones, Elizabeth Ott, Jeffrey Bastar, Kevin M. Bourzac, Rangaraj Selvarangan

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Fever is a common symptom in children presenting to the Emergency Department (ED). We aimed to describe the epidemiology of systemic viral infections and their predictive values for excluding serious bacterial infections (SBIs), including bacteremia, meningitis and urinary tract infections (UTIs) in children presenting to the ED with suspected systemic infections.

METHODS: We enrolled children who presented to the ED with suspected systemic infections who had blood cultures obtained at seven healthcare facilities. Whole blood specimens were analyzed by an experimental multiplexed PCR test for 7 viruses. Demographic and laboratory results were abstracted.

RESULTS: Of the 1114 subjects enrolled, …

Perceptions Of Family-Level Social Factors That Influence Health Behaviors In Latinx Adolescents And Young Adults At High Risk For Type 2 Diabetes, Erica G Soltero, Neeku Navabi, Felipe G Castro, Stephanie L Ayers, Jenny Mendez, Deborah I Thompson, Gabriel Q Shaibi

Children’s Nutrition Research Center Staff Publications

Given that health behaviors occur within the context of familial social relationships, a deeper understanding of social factors that influence health behaviors in Latinx families is needed to develop more effective diabetes prevention programming. This qualitative study identified perceived family-level social factors that influence health behaviors in Latinx adolescents (12–16 years; N = 16) and young adults (18–24 years; N = 15) with obesity and explored differences in perceptions across sex and age. Participants completed an in-depth interview that was recorded, transcribed, and coded using thematic content analysis. Emergent themes central to health behaviors included: perceived parental roles and responsibilities, …

Automated Assessment Of Thoracic-Abdominal Asynchrony In Patients With Morquio Syndrome., Madhavi V Ratnagiri, Yan Zhu, Tariq Rahman, Mary Theroux, Shunji Tomatsu, Thomas H Shaffer

Department of Pediatrics Faculty Papers

Morquio syndrome is a rare disease caused by a disorder in the storage of mucopolysaccharides that affects multiple organs, including musculoskeletal, respiratory, cardiovascular, and digestive systems. Respiratory failure is one of the leading causes of mortality in Morquio patients; thus, respiratory function testing is vital to the management of the disease. An automated respiratory assessment methodology using the pneuRIP device and a machine-learning algorithm was developed. pneuRIP is a noninvasive approach that uses differences between thoracic and abdominal movements (thoracic-abdominal asynchrony) during respiration to assess respiratory status. The technique was evaluated on 17 patients with Morquio (9 females and 8 …

Use Of Artificial Intelligence To Identify Predictors Of Functional Outcomes In Patients With Facioscapulohumeral Muscular Dystrophy, Natalie K. Katz

Research Days

Background: Facioscapulohumeral muscular dystrophy (FSHD) is one of the most common forms of muscular dystrophy characterized by slowly progressive muscle weakness. Symptoms initially involve the facial and shoulder girdle muscles, with later involvement of the distal lower extremities, pelvic girdle and proximal lower extremities. FSHD type 1 (FHSD1) is the most common form (~95% of individuals) and is caused by deletion of microsatellite repeats in the D4Z4 region on chromosome 4 (4q35). Normal individuals have >10 repeats whereas individuals with FSHD1 have 1-10 repeats. There is significant variability in regards to disease severity, rates of progression and functional outcomes, but …

Financial Outcomes Of High Flow Nasal Cannula Use For Bronchiolitis On The General Pediatric Floor Across Children's Hospitals, Jonathan Hartley

Research Days

Background: Bronchiolitis is a leading cause of hospitalization for infants and results in over 1.7 billion dollars in yearly hospital charges (Hasegawa). The use of high flow nasal cannula (HFNC) on a general pediatrics floor may reduce hospital costs and length of stay (LOS, Kalburgi). However, the financial impact of HFNC use is not welldescribed.

Objective: The primary objective are the financial outcomes of HFNC utilization on the general pediatric floor. The secondary objective included financial outcomes stratified by All Patients Refined Diagnosis Related Groups (APRDRG) severity of illness (SOI) and payor type. We hypothesize that costs outweigh reimbursement for …

Prevalence Of Adolescent Sexual Health Outcomes At Children’S Hospitals During Covid-19, Margarita Abella

Research Days

Background: The Coronavirus Disease 2019 (COVID-19) pandemic mitigation strategies, including social distancing, decreased structured environments, and decreased sexual and reproductive health (SRH) care access, could negatively impact adolescent SRH behaviors and related outcomes. To date, little is known regarding changes in adolescent SRH outcomes during COVID-19.

Objectives/Goal: Our goals were to assess changes in adolescent SRH outcomes, including sexually transmitted infections (STIs), pregnancy, and sexual assault in the hospital (emergency department [ED] and inpatient [IP]) during COVID-19 compared to pre-COVID-19.

Methods/Design: We conducted a retrospective cohort study of adolescent (11-18 years old) IP or ED hospital visits using the Pediatric …

Factors Associated With Suicide Screenings For An Autism Spectrum Disorder Population In A Pediatric Medical Setting, Cheng Qian, Dawn Greathouse

Research Days

Background: Individuals with autism spectrum disorder (ASD) are at least 3 times more likely to attempt suicide than those without ASD (Kolves et al., 2021; Richa et al., 2014). Core features of ASD (e.g., deficits in communicating emotions, difficulty with change) are associated with known suicide attempt antecedent events, and rates of co-occurring mental health concerns (e.g., depression) that increase suicide risk are higher than in the general population (Richa et al., 2014). However, mental health clinicians perceive patients with ASD to be at lower risk for suicide, and report lower self-efficacy in screening in this population (Jager-Hyman et al., …

Bmi And Associated Variables In A Pediatric Gender Clinic Sample, Mirae J. Fornander

Research Days

Background: Studies of transgender/gender diverse (TGD) youth indicate a high prevalence of overweight/obesity and concern for unhealthy weight control behavior.

Objectives/Goal: Describe BMI and the association of medication use, well-being, and recreational activities in treatmentnaïve pediatric TGD patients.

Methods/Design: Chart review of 302 patients (age 3-19, 73.5% sex assigned at birth (SAB) female; 85.8% white) from 2017-20. BMI was calculated by age and SAB using CDC growth charts. Parents reported medication use. Parent and self-reported Pediatric Quality of Life (PEDS-QL) Well-Being and activities were surveyed electronically.

Results: By BMI category, 3.3% were underweight (BMI85% and 29.5% fell >95%. Overweight and …