Pediatrics Commons^™

Genetic And Epigenetic Features Of Bilateral Wilms Tumor Predisposition In Patients From The Children’S Oncology Group Aren18b5-Q, Andrew J Murphy, Changde Cheng, Justin Williams, Timothy I Shaw, Emilia M Pinto, Karissa Dieseldorff-Jones, Jack Brzezinski, Lindsay A Renfro, Brett Tornwall, Vicki Huff, Andrew L Hong, Elizabeth A Mullen, Brian Crompton, Jeffrey S Dome, Conrad V Fernandez, James I Geller, Peter F Ehrlich, Heather Mulder, Ninad Oak, Jamie Maciezsek, Carolyn M Jablonowski, Andrew M Fleming, Prahalathan Pichavaram, Christopher L Morton, John Easton, Kim E Nichols, Michael R Clay, Teresa Santiago, Jinghui Zhang, Jun Yang, Gerard P Zambetti, Zhaoming Wang, Andrew M Davidoff, Xiang Chen

Faculty, Staff and Student Publications

Developing synchronous bilateral Wilms tumor suggests an underlying (epi)genetic predisposition. Here, we evaluate this predisposition in 68 patients using whole exome or genome sequencing (n = 85 tumors from 61 patients with matched germline blood DNA), RNA-seq (n = 99 tumors), and DNA methylation analysis (n = 61 peripheral blood, n = 29 non-diseased kidney, n = 99 tumors). We determine the predominant events for bilateral Wilms tumor predisposition: 1)pre-zygotic germline genetic variants readily detectable in blood DNA [WT1 (14.8%), NYNRIN (6.6%), TRIM28 (5%), and BRCA-related genes (5%)] or 2)post-zygotic epigenetic hypermethylation at 11p15.5 H19/ICR1 that may require analysis of …

A Community-Based Participatory Research Approach To Addressing Pediatric Asthma Disparities In Douglas County, Nebraska, Aislinn Rookwood

Theses & Dissertations

Pediatric asthma is the leading chronic disease among children in the United States, and the incidence of pediatric asthma is increasing every year. Nebraska has an asthma prevalence comparable to the United States average; however, the state has significant disparities by county, race, and geographic location, with prevalence rates much higher than the national average. Douglas County is a hot spot for increased pediatric asthma rates, with further differences in rates by race and geographic location within the county. The University of Nebraska Medical Center, in partnership with Healthy Housing Omaha and the Nebraska Asthma Coalition, collaborated to conduct this …

Iron Oxide-Coupled Crispr-Ncas9-Based Genome Editing Assessment In Mucopolysaccharidosis Iva Mice, Andrés Leal, Betul Celik, Nidhi Fnu, Shaukat Khan, Shunji Tomatsu

Department of Pediatrics Faculty Papers

Mucopolysaccharidosis (MPS) IVA is a lysosomal storage disorder caused by mutations in the GALNS gene that leads to the lysosomal accumulation of keratan sulfate (KS) and chondroitin 6-sulfate, causing skeletal dysplasia and cardiopulmonary complications. Current enzyme replacement therapy does not impact the bone manifestation of the disease, supporting that new therapeutic alternatives are required. We previously demonstrated the suitability of the CRISPR-nCas9 system to rescue the phenotype of human MPS IVA fibroblasts using iron oxide nanoparticles (IONPs) as non-viral vectors. Here, we have extended this strategy to an MPS IVA mouse model by inserting the human GALNS cDNA into the …

A Multicenter Evaluation Of Pediatric Emergency Department Injury Visits During The Covid-19 Pandemic, Holly R Hanson, Margaret Formica, Danielle Laraque-Arena, Mark R Zonfrillo, Puja Desai, Joseph O O'Neil, Purnima Unni, Estell Lenita Johnson, Patricia Cobb, Maneesha Agarwal, Kristen Beckworth, Stephanie Schroter, Stephen Strotmeyer, Katie A Donnelly, Leah K Middelberg, Amber M Morse, James Dodington, Richard F Latuska, Brit Anderson, Karla A Lawson, Michael Valente, Michael N Levas, Andrew Waititu Kiragu, Kathy Monroe, Stephanie M Ruest, Lois K Lee, Tanya Charyk Stewart, Megan M Attridge, Maya Haasz, Mubeen Jafri, Alicia Mcintire, Steven C Rogers, Neil G Uspal, Ashley Blanchard, Max D Hazeltine, Teresa Riech, Charles Jennissen, Lynn Model, Quinney Fu, Lindsay D Clukies, David Juang, Michelle T Ruda, Jose M Prince, Stephanie Chao, Brian K Yorkgitis, Wendy J Pomerantz

Faculty, Staff and Student Publications

BACKGROUND: Injuries, the leading cause of death in children 1-17 years old, are often preventable. Injury patterns are impacted by changes in the child's environment, shifts in supervision, and caregiver stressors. The objective of this study was to evaluate the incidence and proportion of injuries, mechanisms, and severity seen in Pediatric Emergency Departments (PEDs) during the COVID-19 pandemic.

METHODS: This multicenter, cross-sectional study from January 2019 through December 2020 examined visits to 40 PEDs for children < 18 years old. Injury was defined by at least one International Classification of Disease-10th revision (ICD-10) code for bodily injury (S00-T78). The main study outcomes were total and proportion of PED injury-related visits compared to all visits in March through December 2020 and to the same months in 2019. Weekly injury visits as a percentage of total PED visits were calculated for all weeks between January 2019 and December 2020.

RESULTS: The study included 741,418 PED visits for injuries pre-COVID-19 pandemic (2019) and during the COVID-19 pandemic (2020). Overall PED visits from all causes …

Postmortem Minimally Invasive Tissue Sampling In Communities: Exploring Perceptions Of Families, Funeral Workers, Religious And Community Leaders And Healthcare Providers From Pakistan, Nazia Ahsan, Fauzia Aman Malik, Waliyah Mughis, Rawshan Jabeen, Shaheen Mehboob, Raheel Allana, Syeda Quratulain, Saima Jamal Saima Jamal, Christina R. Paganelli, Abdul Momin Kazi

Department of Paediatrics and Child Health

Background: Minimally invasive tissue sampling (MITS) has increasingly been used to improve the diagnosis of disease and identification of the cause of death, particularly in underserved areas. However, there are multiple barriers to accessing those who die within the community, our study aimed to explore the perceptions and insights of community members and healthcare providers regarding the feasibility of implementing MITS in community settings.
Methods: A qualitative exploratory study was conducted. A total of twenty one in-depth interviews were conducted with deceased infants' parents, elders of the family, religious leaders, community leaders, and funeral workers. Focus group discussions were conducted …

Ablation Of Accessory Pathway From Right Atrial Appendage To Anatomic Left Ventricle In L-Transposition Of The Great Arteries, Shree Lata Radhakrishnan, Robert O. Drutel, Cody M. Williams, Raman Danrad, Kelly K. Gajewski, Paul A. Lelorier

School of Medicine Faculty Publications

No abstract provided.

Lessons Learnt In The First Year Of An Australian Pediatric Cardio Oncology Clinic., Claudia Toro, Ben Felmingham, Mangesh Jhadav, David S. Celermajer, Andre La Gerche, John O'Sullivan, Sanjeev Kumar, Marion K. Mateos, Joy M. Fulbright, Dinisha Govender, Lane Collier, Michael Cheung, David D. Eisenstat, Peter W. Lange, Julian Ayer, David A. Elliott, Rachel Conyers

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Modern oncological therapies together with chemotherapy and radiotherapy have broadened the agents that can cause cardiac sequelae, which can manifest for pediatric oncology patients while on active treatment. Recommendations for high-risk patients who should be monitored in a pediatric cardio-oncology clinic have previously been developed by expert Delphi consensus by our group. In 2022 we opened our first multidisciplinary pediatric cardio-oncology clinic adhering to these recommendations in surveillance and management.

OBJECTIVES: Our pediatric cardio-oncology clinic aimed to: (i) Document cardiovascular toxicities observed within a pediatric cardio-oncology clinic and. (ii) Evaluate the applicability of the Australian and New Zealand Pediatric …

Applying Occupation-Centered Coaching For Caregivers Of Children With Disabilities, Ali S. Gregory, Mary A. Smith Phd, Otr/L, Mary E. Godman Otd, Ms

Fall 2023 Virtual OTD Capstone Symposium

Statement of the problem: Caregivers of children with disabilities (CWD) experience stress, mental health conditions, sleep disruption, and fatigue leading to decreased occupational engagement and family community participation.

Purpose Statement: This project applied Occupation-Centered Coaching with a family-centered approach to promote caregiver empowerment, advocacy for mental health services, and family community participation.

Effects Of A Preventive Mental Health Curriculum Embedded Into A Scholarly Gaming Course On Adolescent Self-Esteem: Prospective Matched Pairs Experiment., Christopher Jenson, Sharon Fitzgerald Wolff, Libby Milkovich

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Positive self-esteem predicts happiness and well-being and serves as a protective factor for favorable mental health. Scholarly gaming within the school setting may serve as a channel to deliver a mental health curriculum designed to improve self-esteem.

OBJECTIVE: This study aims to evaluate the impact of a scholarly gaming curriculum with and without an embedded preventive mental health curriculum, Mental Health Moments (MHM), on adolescents' self-esteem.

METHODS: The scholarly gaming curriculum and MHM were developed by 3 educators and a school-based health intervention expert. The scholarly gaming curriculum aligned with academic guidelines from the International Society for Technology Education, …

Association Between Remote Monitoring And Interstage Morbidity And Death In Patients With Single-Ventricle Heart Disease Across Socioeconomic Groups., Bianca Cherestal, Lori A. Erickson, Janelle R. Noel-Macdonnell Phd, Girish S. Shirali, Hayley S. Hancock, Doaa Aly, Matthew Files, Sarah Clauss, Natalie Jayaram

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Despite improvements in survival over time, the mortality rate for infants with single-ventricle heart disease remains high. Infants of low socioeconomic status (SES) are particularly vulnerable. We sought to determine whether use of a novel remote monitoring program, the Cardiac High Acuity Monitoring Program, mitigates differences in outcomes by SES.

METHODS AND RESULTS: Within the Cardiac High Acuity Monitoring Program, we identified 610 infants across 11 centers from 2014 to 2021. All enrolled families had access to a mobile application allowing for near-instantaneous transfer of patient information to the care team. Patients were divided into SES tertiles on the …

Feasibility Of Electronic Medication Monitoring Among Adolescents And Emerging Adults With Sickle Cell Disease, Aimee Hildenbrand, Katherine Kidwell, Meghan Mcgrady, Constance Mara, Charles Quinn, Lori Crosby

Department of Pediatrics Faculty Papers

Purpose: To examine the feasibility of using MEMS® bottles to assess adherence among adolescents and emerging adults with sickle cell disease.

Patients and Methods: Eighteen non-Hispanic Black participants with HbSS (M = 17.8 years; 61% male) were given a MEMS® bottle to store hydroxyurea (n = 14) or deferasirox (n = 4).

Results: One hundred percent initiated MEMS® use and 61% sustained use through the 18-week study; at follow-up, only 11% returned their bottle on time. Barriers to MEMS® use included medication changes and transition to adult care; facilitators included tip sheets and reminders.

Conclusion: While MEMS® is acceptable to …

Recognizing Postural Orthostatic Tachycardia Syndrome In Primary Care, Brittney Hulsey

PA Faculty Publications

Postural orthostatic tachycardia syndrome (POTS) is a complex autonomic disorder characterized by abnormal tachycardia on standing. This disorder predominantly affects young women, with a peak incidence between ages 20 and 30 years. POTS often is associated with a variety of symptoms, including dizziness, palpitations, fatigue, generalized weakness, anxiety, and exercise intolerance, which can significantly impair patient quality of life. Primary care providers (PCPs) often are the first healthcare professionals to whom patients present. This article provides an overview of POTS, including diagnostic criteria and the importance of a thorough clinical evaluation to rule out other causes of symptoms. PCPs play …

Measuring Effects Of Screen Time On The Development Of Children In The Philippines: A Cross-Sectional Study, Angel Belle C. Dy, Alane Blythe C. Dy, Samantha Katrina Santos

Ateneo School of Medicine and Public Health Publications

Background

Screen time in young children is discouraged because of its negative effects on their development. However, excessive screen media use has been rising, particularly during the global pandemic when stay-at-home mandates were placed on young children in several countries. This study documents potential developmental effects of excessive screen media use.

Method

This is a cross-sectional study. Participants were 24 to 36 month old Filipino children recruited through non-probable convenience sampling from August to October 2021. Regression analyses were performed to test the association between screen time and changes in scaled scores for skills and behaviors determined from the Adaptive …

Bridging Gaps: A Qualitative Inquiry On Improving Paediatric Rheumatology Care Among Healthcare Workers In Kenya, Angela Migowa, Sasha Bernatsky, Anthony Ngugi, Helen Foster, Peterrock Muriuki, Roselyter Riang’A, Stanley Luchters

Paediatrics and Child Health, East Africa

Background: Due to the paucity of paediatric rheumatologists in Kenya, it is paramount that we explore strategies to bridge clinical care gaps for paediatric rheumatology patients in order to promote early diagnosis, prompt referral, and optimal management.

Purpose: To identify proposed interventions which can improve the ability of non-specialist healthcare workers to care for paediatric rheumatology patients across Kenya.

Methods: We conducted 12 focus group discussions with clinical officers (community physician assistants), nurses, general practitioners and paediatricians across six regions in Kenya. Interviews were conducted, audio-recorded, transcribed verbatim, and analysed using MAXQDA 2022.2 software.

Results: A total of 68 individuals …

Implementing Community Health Workers To Improve The Management Of Chronic Non-Communicable Diseases In Children, Julia L. Newell

Doctor of Nursing Practice (DNP) Projects

Background: Childhood obesity, asthma, and untreated mental health conditions are three examples of chronic non-communicable diseases (CNDs) that pose a host of negative consequences later in life. Minority children from low-income families, especially those with environmental disadvantages, face additional risk factors for the development of these diseases. Community health workers (CHWs) are in a unique position to help address these negative health externalities by being attuned with the linguistic, cultural, and socioemotional needs of members of their communities. Research has demonstrated success in CHW-led interventions curtailing the negative impacts associated with these diseases in low-income, minority communities.

Methods: A literature …

Sars-Cov-2 Epidemiology And Covid-19 Mrna Vaccine Effectiveness Among Infants And Children Aged 6 Months-4 Years - New Vaccine Surveillance Network, United States, July 2022-September 2023., Ayzsa Tannis, Janet A. Englund, Ariana Perez, Elizabeth J. Harker, Mary Allen Staat, Elizabeth P. Schlaudecker, Natasha B. Halasa, Laura S. Stewart, John V. Williams, Marian G. Michaels, Rangaraj Selvarangan, Jennifer E. Schuster, Leila C. Sahni, Julie A. Boom, Geoffrey A. Weinberg, Peter G. Szilagyi, Benjamin R. Clopper, Yingtao Zhou, Meredith L. Mcmorrow, Eileen J. Klein, Heidi L. Moline

Manuscripts, Articles, Book Chapters and Other Papers

SARS-CoV-2 infection in young children is often mild or asymptomatic; however, some children are at risk for severe disease. Data describing the protective effectiveness of COVID-19 mRNA vaccines against COVID-19-associated emergency department (ED) visits and hospitalization in this population are limited. Data from the New Vaccine Surveillance Network, a prospective population-based surveillance system, were used to estimate vaccine effectiveness using a test-negative, case-control design and describe the epidemiology of SARS-CoV-2 in infants and children aged 6 months-4 years during July 1, 2022-September 30, 2023. Among 7,434 children included, 5% received a positive SARS-CoV-2 test result, and 95% received a negative …

Rare Tumors: Opportunities And Challenges From The Children's Oncology Group Perspective., Kris Ann P. Schultz, Murali Chintagumpala, Jin Piao, Kenneth S. Chen, Rachana Shah, Robyn D. Gartrell, Emily Christison-Lagay, Farzana Pashnakar, Jesse L. Berry, Allison F. O'Neill, Lauren M. Vasta, Ashley Flynn, Sarah G. Mitchell, Brittani Kn Seynnaeve, Jeremy Rosenblum, Samara L. Potter, Junne Kamihara, Carlos Rodriguez-Galindo, Douglas S. Hawkins, Theodore W. Laetsch

Manuscripts, Articles, Book Chapters and Other Papers

While all childhood cancers are rare, tumors that are particularly infrequent or underrepresented within pediatrics are studied under the umbrella of the Children's Oncology Group Rare Tumor committee, divided into the Retinoblastoma and Infrequent Tumor subcommittees. The Infrequent Tumor subcommittee has traditionally included an emphasis on globally rare tumors such as adrenocortical carcinoma, nasopharyngeal carcinoma, or those tumors that are rare in young children, despite being common in adolescents and young adults, such as colorectal carcinoma, thyroid carcinoma, and melanoma. Pleuropulmonary blastoma, gonadal stromal tumors, pancreatic tumors including pancreatoblastoma, gastrointestinal stromal tumor, nonmelanoma skin cancers, neuroendocrine tumors, and desmoplastic small …

The Modified Clinical Progression Scale For Pediatric Patients: Evaluation As A Severity Metric And Outcome Measure In Severe Acute Viral Respiratory Illness., Shannon B. Leland, Steven J. Staffa, Margaret M. Newhams, Robinder G. Khemani, John C. Marshall, Cameron C. Young, Aline B. Maddux, Mark W. Hall, Scott L. Weiss, Adam J. Schwarz, Bria M. Coates, Ronald C. Sanders, Michele Kong, Neal J. Thomas, Ryan A. Nofziger, Melissa L. Cullimore, Natasha B. Halasa, Laura L. Loftis, Natalie Z. Cvijanovich, Jennifer E. Schuster, Heidi Flori, Shira J. Gertz, Janet R. Hume, Samantha M. Olson, Manish M. Patel, David Zurakowski, Adrienne G. Randolph, Pediatric Acute Lung And Sepsis Investigator’S Network Pediatric Intensive Care Influenza Study Group (Palisi Picflu) Investigators And Overcoming Covid-19 Investigators

Manuscripts, Articles, Book Chapters and Other Papers

OBJECTIVES: To develop, evaluate, and explore the use of a pediatric ordinal score as a potential clinical trial outcome metric in children hospitalized with acute hypoxic respiratory failure caused by viral respiratory infections.

DESIGN: We modified the World Health Organization Clinical Progression Scale for pediatric patients (CPS-Ped) and assigned CPS-Ped at admission, days 2-4, 7, and 14. We identified predictors of clinical improvement (day 14 CPS-Ped ≤ 2 or a three-point decrease) using competing risks regression and compared clinical improvement to hospital length of stay (LOS) and ventilator-free days. We estimated sample sizes (80% power) to detect a 15% clinical …

Obesity And Outcomes Of Kawasaki Disease And Covid-19-Related Multisystem Inflammatory Syndrome In Children., Michael Khoury, Ashraf S. Harahsheh, Geetha Raghuveer, Nagib Dahdah, Simon Lee, Marianna Fabi, Elif Seda Selamet Tierney, Michael A. Portman, Nadine F. Choueiter, Matthew Elias, Deepika Thacker, Frédéric Dallaire, William B. Orr, Tyler H. Harris, Kambiz Norozi, Dongngan T. Truong, Manaswitha Khare, Jacqueline R. Szmuszkovicz, Joseph J. Pagano, Cedric Manlhiot, Pedrom Farid, Brian W. Mccrindle, International Kawasaki Disease Registry

Manuscripts, Articles, Book Chapters and Other Papers

IMPORTANCE: Obesity may affect the clinical course of Kawasaki disease (KD) in children and multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19.

OBJECTIVE: To compare the prevalence of obesity and associations with clinical outcomes in patients with KD or MIS-C.

DESIGN, SETTING, AND PARTICIPANTS: In this cohort study, analysis of International Kawasaki Disease Registry (IKDR) data on contemporaneous patients was conducted between January 1, 2020, and July 31, 2022 (42 sites, 8 countries). Patients with MIS-C (defined by Centers for Disease Control and Prevention criteria) and patients with KD (defined by American Heart Association criteria) were included. Patients with …

Acute And Chronic Kidney Injury During Therapy For Pediatric Acute Leukemia: A Report From The Leukemia Electronic Abstraction Of Records Network (Learn), Wendy Hsiao, Yimei Li, Kelly Getz, Lusha Cao, Edward Krause, Mark Ramos, Judy Lee, Maria Monica Gramatges, Karen R Rabin, Michael E Scheurer, Richard Aplenc, Michelle Denburg, Tamara P Miller

Center for Medical Ethics and Health Policy Staff Publications

Children with acute leukemia are at increased risk of kidney injury. Using electronic health record data from three centers between 2010 and 2018, this study retrospectively described acute kidney injury (AKI) and chronic kidney disease (CKD) prevalence in children with acute lymphoblastic or myeloid leukemia (ALL, AML) using Common Terminology Criteria for Adverse Events (CTCAE) and Kidney Disease Improving Global Outcomes (KDIGO) definitions. AKI during therapy was 25% (ALL) and 32% (AML) using CTCAE, versus 84% (ALL) and 74% (AML) using KDIGO. CKD prevalence was low and Grade 1/Stage 2. Further investigation is needed to optimally define kidney injury in …