Higher Education and Teaching Commons^™

Novel Rpl30 Variant In Diamond Blackfan Anemia Demonstrates Early Impact On Erythroid Differentiation With Downregulated Gata1-Hsp70, Alexandra Prosser-Dombrowski, Irina Pushel, Jacqelyn Nemechek, Priyanka Kumar, Jay L. Vivian, Jennifer Gerton, John M. Perry

Research Days

Background: Diamond Blackfan anemia (DBA) is an inherited bone marrow failure syndrome caused by ribosomal protein gene mutations leading to apoptosis of erythroid progenitors. We identified a novel heterozygous variant (c.167+769C>T) in the noncoding region of RPL30 in a patient diagnosed with DBA.

Objectives/Goal: We hypothesized that this variant stunts erythroid differentiation at the proerythroblast stage and is pathogenic for DBA.

Methods/Design: We developed an induced pluripotent stem cell (iPSC) model with a wild type (WT) and three CRISPR-Cas9 edited RPL30 mutant clones. iPSCs were differentiated into hematopoietic stem cells, which were assessed by flow cytometry and single cell …

Duration Of Labor And Umbilical Artery Acidosis In Critical Congenital Heart Disease, Sarah Gwazdacz Md, Teresa Nguyen Bs, Mary Donofrio Md, Faap, Facc, Fase, Timothy Bennett Md, Amanda Mcintosh Md, Faap, Facc.

Research Days

Standard delivery protocols for most prenatally diagnosed CCHD is induction of labor at 39 weeks with aims of a vaginal delivery (VD) near a tertiary care center. There is no benefit to planned Cesarean delivery (CD) over VD for most infants with CCHD. However, longer durations of labor (DOL) in CCHD have been described as a risk factor for umbilical artery (UA) acidosis. We aim to describe the relationship between DOL and UA pH and neonatal outcomes in those with prenatally diagnosed CCHD.

Retrospective chart review of prenatally diagnosed with CCHD born at Children’s Mercy from 2012 – 2023. Inclusion …

Opportunities For Solid Organ Donation In The Neonatal Intensive Care Unit, Sarah Pippin, Jotishna Sharma, Ashley Sherman, Julie Weiner

Research Days

This study aims to characterize the theoretical eligibility of neonates undergoing withdrawal of care in the NICU for donation after circulatory determination of death (DCDD), with a focus on warm ischemic time (WIT) ≤ 90 minutes.

Abusive Head Trauma (Aht) Diagnoses: The Influence Of Clinical Presentation And Evidence Of Impact, Ruairi Smith-Dewey, Angela N. Bachim, Colleen Bressler, Kristine Campbell, Lori Frasier, Christopher Greeley, Danielle Horton, Nicole Johnson, Jan Leonard, Megan Letson, Daniel M. Lindberg, Caitlin Mcnamara, Shalon Nienow, Ashley K. Sherman, Joanne Wood, James Anderst

Research Days

Background: In cases where Child Abuse Pediatricians (CAPs) diagnose abusive head trauma (AHT) in young children, some factors surrounding the diagnosis have been challenged including causative shaking, necessity of external signs of impact, or concomitant suspicious injuries. Evaluation of CAP diagnoses in children with subdural hemorrhage (SDH) can give insight into factors related to the frequency of abusive head trauma (AHT) diagnoses. Additionally, characterization of those with SDH+CSI without evidence of impact may inform controversies around AHT diagnoses.

Objectives/Goal: We aim to compare CAP diagnoses in subjects with SDH across clinical presentations and in the context of evidence of head …

In Search Of The Unknown Mimic Of Abusive Head Trauma., Ruairi Smith-Dewey, Angela N. Bachim, Colleen Bressler, Kristine Campbell, Lori Frasier, Danielle Horton, Nicole Johnson, Jan Leonard, Megan Letson, Caitlin Mcnamara, Shalon Nienow, Ashley K. Sherman, Stuart Sommers, Joanne Wood, James Anderst

Research Days

Background:

Some claim that Child Abuse Pediatricians (CAPs) are misdiagnosing a yet-to-be-identified mimic as abusive head trauma (AHT), particularly in ill children with isolated subdural hemorrhage (SDH). Though not diagnostic criteria, specific social stressors have been identified as AHT risk factors. If a yet-to-be-identified mimic is meaningfully present within a group being evaluated for AHT, that group should have fewer AHT risk factors than children with actual AHT. The existence of such a mimic can be assessed by comparing the prevalence of AHT risk factors across groups: CAP-identified mimics, non-ill SDH only, ill SDH only, and SDH plus concomitant suspicious …

Engineering A Novel Treg Population To Control Autoimmune Diabetes, Sofia Colon Guzman, Elly Puckett, Mary A. Markiewicz, Ryan T. Fischer

Research Days

We created a model to test the immunosuppressive capacity of Helios+FOXP3+ eTregs compared to nTregs against islet-specific autoreactive T cells. We also showed that nTregs decrease the response of these cytotoxic T cells when co-cultured with BetaLox5 cells.

Growth Hormone Deficiency In Childhood Cancer Survivors: Response To Growth Hormone Therapy And Aromatase Inhibitors Compared To Growth Hormone Therapy Alone., Haya Azouz, Sarah Tsai, Keith August, Jennifer W. Boyd

Research Days

With evolving technologies leading to better cancer cure rate, the number of childhood cancer survivors has increased. There has been increasing reports of later endocrine effects including growth hormone deficiency (GHD). Our aim is to investigate the influence of adding aromatase inhibitors (AI) to growth hormone (GH) therapy in childhood cancer survivors with growth hormone deficiency (GHD). A retrospective chart review was performed looking at children between ages 0 and 18 years with history of malignancy and subsequent diagnosis of GHD at our institution. Fifty-nine children were included in this study. Participants were classified into 3 groups: (1) the target …

Improving Adherence To Evidence Based Sti Care For Patients Who Tested Positive At Ed Index Visit Qi Project, Noelle Tran, Melissa Miller, Kim Barbee, Julie Jost, Christopher Kaberline, M Smith

Research Days

Problem Statement/Question:

Previous work improved clinician sexually transmitted infection (STI) testing behaviors, but overlooked treatment interventions needed to facilitate risk reduction, HIV and syphilis (RPR) testing, and STI retesting at three months—critical components that reduce morbidity and disease spread. We are not adhering to evidence-based STI care for adolescents who tested positive for STIs at the ED index visit.

Background/Project Intent (Aim Statement):

According to the Centers for Disease Control and Prevention (CDC), in 2023, over 2.4 million cases of RPR, gonorrhea (GC), and chlamydia (CT) were diagnosed; about half of reported cases were among adolescents and young adults aged …

Food Insecurity In Adolescent Athletes, Andrew Donaldson, Shannon Margherio, Jay Roberson, Kevin Latz

Research Days

Background: The prevalence of food insecurity among children in the United States is estimated at 21%. Food insecurity during childhood leads to worse school performance, more emotional and behavioral health concerns, and increased suicide attempts. Further, food insecurity has been noted to have multiple deleterious effects on the collegiate student athlete, including lower GPA, disordered eating, and substance use.

Hypothesis: Food insecurity is prevalent in the adolescent athlete population and exists across demographics.

Methods: Voluntary surveys were distributed to parents at all sports medicine clinics at our institution. Demographic data was collected including athlete age, race, school and school district, …

Variation In Blood Culture Testing And Outcomes In Children Hospitalized With Three Common Infections., Smit Shah, Jessica L. Markham, Jennifer Goldman, Matt Hall, Jillian Cotter, Samantha A. House, Matthew J. Molloy, Michelle Polich, Michael J. Steiner, John R. Stephens, Michael J. Tchou, Ronald J. Teufel Ii, Irma Ugalde, Andrew Yu

Research Days

Background: Prior studies of blood culture (BC) testing in uncomplicated community acquired pneumonia (CAP), skin and soft tissue infections (SSTI), and urinary tract infections (UTI) have demonstrated low utility of routine testing given similar rates of pathogen versus contaminant detection and little effect on clinical management. Understanding patterns and outcomes of BC testing can identify opportunities to curb unnecessary testing.

Objectives: The objectives of this study were to: 1) Evaluate BC testing trends over time for children hospitalized for CAP, SSTI, and UTI; and 2) describe differences in clinical outcomes between hospital groups with low, moderate, or high testing rates. …

Association Between Neighborhood Opportunity And Youth Mental Health Emergency Department Visits, Shelby Chesbro Md, Henry T. Puls, Adrienne G. Deporre, Molly Krager, Laura Plencner, Shayla Sullivant, Matthew Hall Phd

Research Days

Background: Youth mental health emergency department visits are increasing and are a burden on the health care system. Prevalence of mental health disorders may be influenced by the neighborhood youths live in.

Objectives/Goal: Determine differences in rates of youth mental health ED visits across zip code levels of neighborhood opportunity.

Methods/Design: We performed a cross-sectional study using the Hospital Industry Data Institute dataset identifying ED visits with a primary mental health diagnosis in Kansas and Missouri for youths aged 5-19 years in 2022-2023. Mental health ED visits were then categorized at the zip code level by neighborhood opportunity using the …

Reducing Vancomycin Use In The Cicu, Karoline Krzywda, Ali Taber, Lauren Johnson

Research Days

Background/Aim Statement: In the CICU empiric vancomycin is frequently prescribed. This practice occurs without broad knowledge of the actual rates of methicillin resistant staph aureus (MRSA) and methicillin resistant staph epidermidis (MRSE) infections. Given the potential for harmful side effects of vancomycin as well as concern for growing antibiotic resistance, we sought to better characterize local unit prescribing practices and MRSA/MRSE infection rates to guide our QI project with an aim of reducing vancomycin use in the CICU.

Methods (include PDSA cycles): Patient data from January 2021 to January 2023 was analyzed. The number of monthly days spent on vancomycin …

A Risk Score To Predict Recurrent High Intensity Inpatient Resource Utilization For Children With Medical Complexity., Charles Maloy, Matthew Hall, Jessica L. Bettenhausen, Yui-Yee Chan, Leah Jones, Kathryn Kyler, Laura Plencner Md, Jessica Markham, Jacqueline M. Walker, Smit Shah, Sîan Best, Jonathan Ermer, Shelby Chesbro, Maria Newmaster, Henry T. Puls

Research Days

Background: Children with medical complexity (CMC) use a greater amount of inpatient resources and stand to benefit from unique care models and coordination. However, hospital resources can be limited, and a better understanding of which CMC are high resource users could improve hospital and staff planning.

Objectives/Goal: To develop a risk score predictive of recurrent high intensity inpatient utilization among CMC.

Methods/Design: We conducted a retrospective cohort study of children <18 years old with ≥3 complex chronic conditions (CCC) discharged from one of 48 children’s hospitals in the Pediatric Health Information System during 2021 and 2022. To reflect CMC typically cared for by a general medical service, children with cardiac procedures, dialysis, oncology, or transplant CCCs, and index hospitalization mortality were excluded. We used multivariable logistic regression to determine factors present on the index hospitalization associated with ≥5 hospitalizations or ≥3 hospitalizations with one lasting ≥25 days within any 365-day period (high inpatient utilization) in 2021-2023. We developed risk scoring system using model coefficients and defined the optimal risk score cutoff to maximize sensitivity and specificity. We then applied the risk score cutoff to children discharged to determine (a) ratio of predicted:actual CMC with recurrent high resource use and (b) the total predicted daily volume on 50th, 75th, and 90th percentile days for each hospital.

Results: Of 44,432 included children, 9,596 (21.6%) had high inpatient utilization (Table). Children under 1 year of age had the highest odds of high inpatient utilization (OR 2.26 [95% CI: …

Failure To Launch: Pediatric Colorectal Surgery Center Utilization By Patients Over 18 Years Of Age, Vatche Melkonian, Andres Gonzalez Salazar, Cory Nonnemacher, Seth Saylors, Margaret Martin-Mclain, Iris Lim-Beutel, Rebecca Rentea

Research Days

Background: As patients with childhood colorectal diseases transition into adulthood, many continue to seek care at pediatric colorectal centers rather than transitioning to adult hospital systems. This preference often stems from a sense of comfort and familiarity with their pediatric care teams. Additionally, patients often perceive inadequacies in adult healthcare systems in managing their specific conditions. As these numbers of patients continue to grow, pediatric colorectal centers may face increasing demands on their resources, potentially diverting attention away from pediatric patients.

Objectives/Goal: The objective of this study was to assess the utilization of our pediatric colorectal center by patients aged …

Patterns And Growth Of A Dedicated Pediatric Coagulation Consult Service In A Freestanding Children's Hospital, Nicole Demarco, Shannon Carpenter, Brian Lee

Research Days

Background: Hospital-acquired thrombosis remains a significant concern in pediatric care, contributing to morbidity and extended hospital stays. At Children’s Mercy, a dedicated inpatient coagulation consultation service has existed for many years. Efforts have been made to implement prophylactic measures aimed at reducing the incidence of hospital-acquired thrombosis. With the recent COVID pandemic we saw an influx in prophylactic anticoagulation due to recommended guidelines.

Objectives: To characterize the impact increased efforts in thromboprophylaxis during and after the COVID-19 pandemic on care delivery and the development of thrombosis among pediatric hospitalized patients.

Methods: This retrospective review included all pediatric inpatients with a …

Multi-Omic Analysis Of Kmt2a-Rearranged Pediatric Acute Myeloid Leukemia Reveals Novel Pathways Of Interest, Trenton Edwards, Irina Pushel, Thomas Gremminger, Lisa Lansdon, Byunggil Yoo, Michaella Rekowski, Michael Washburn, Midhat Farooqi

Research Days

Objectives/Goals

Acute Myeloid Leukemia (AML) is the second most common leukemia among pediatric populations. Approximately 15% of pediatric AML cases have KMT2A gene rearrangements (KMT2A-r), which carry a worse prognosis. Our goal is to better characterize the biologic landscape of KMT2A-r pediatric AML. We hypothesize that a multi-omic approach will reveal mechanisms underlying the aggressive nature of KMT2A-r AML.

Methods/Study Population

This study makes use of deidentified peripheral blood and/or bone marrow samples banked in the Children’s Mercy Tumor Bank Biorepository. For this study, we used samples at diagnosis and remission from four KMT2A-r pediatric AML …

No Suspicion For Nocarh: A Cautionary Tale Of Anchoring Bias, Nicole Demarco, Shannon Haines, Julia Allyson Hays, Bonnie Sullivan, Michael Price, Kimberly A. Horii

Research Days

Neonatal onset of cytopenia autoinflammation rash and hemophagocytic lymphohistiocytosis syndrome (NOCARH), a subset of hemophagocytic lymphohistiocytosis (HLH), is a rare and life-threatening condition characterized by uncontrolled immune response. It presents a diagnostic challenge in neonates due to the overlap of clinical features with other conditions, particularly congenital infections.

Nrp And Pals In Icu Code Events At A Quaternary Care Pediatric Hospital: A ‘Compressed’ Analysis, Deepa Kumar, Mitch Kinkor, Stephen Pfeiffer, Allison Taber, Janelle R. Noel-Macdonnell Phd, Jessica Brunkhorst, Danielle Reed

Research Days

This is a retrospective observational study comparing code events in children less than 18 months of age who were resuscitated in the NICU, PICU, and CICU using neonatal resuscitation program (NRP) and pediatric advance life support (PALS).

The Impact Of Race-Corrected Spirometry Results On Eligibility Into An Asthma Clinical Trial, Carina H. Perdew, Bridgette L. Jones, Wumi V. Akinjole

Research Days

BACKGROUND: Spirometry is a widely used pulmonary function test that assesses lung function by examining airflow and capacity. Spirometry measurements assist in the diagnosis, severity classification, and management of asthma, and can affect one’s eligibility into various events (e.g., clinical trials, high-risk jobs, sports). Reference equations are used to establish predicted values to account for biological variables such as age, sex, and height; however past clinical guidelines recommended utilizing race-correction. The incorporation of race may negatively impact people of color due to misrepresentation of one's lung function, consequently impacting future medical management. OBJECTIVES: To characterize differences in spirometry values across …

Emergency Department Utilization For Educational Prioritization For Children By Levels Of Community Advantage, Katherine Randolph, Chris Kennedy, Jennifer Watts, Matt Hall

Research Days

Background: Children with higher social deprivation have higher emergency department (ED) utilization. The Childhood Opportunity Index (COI) is a validated multidimensional measure of community advantage which includes access to resources and healthy neighborhood factors. Educational priorities are frequently not tied to risk data.

Objective: This study aimed to identify zip codes with higher-than-expected ED utilization by diagnoses to prioritize the development of educational initiatives for hospitals, emergency medical services, pediatrician offices, and the community.

Methods: We performed a population-based study of children aged 0-17 years who presented to an ED in Kansas or Missouri in 2023 using census data via …

Variables Affecting Neurodevelopmental Outcome In Infants With Critical Congenital Heart Disease, Elizabeth Loughman, Julie Weiner Do, Marcie Files Md

Research Days

Background: The mortality of cardiac conditions in infancy is improving with advances in surgical planning and medical management. Neurologic morbidity remains significant. Clinically correlating an infant with their neurologic exam, MRI/EEG, and subsequent developmental testing remains a challenge. Counseling regarding neurologic outcomes after cardiac intervention is difficult due to the wide range of findings that can sometimes be clinically irrelevant.

Purpose: The goal of this study is to identify factors associated with poor neurodevelopmental outcomes in infants with critical congenital heart defects (CCHD) as assessed by the Bayley Scales of Infant and Toddler Development Edition IV (Bayley).

Methods: This is …

Implementation Of A Weaning Protocol For Inhaled Nitric Oxide In A Level Iv Neonatal Intensive Care Unit, Elizabeth Loughman, Julie Weiner Do, Maryam El Tawil, Chris Nitkin Md

Research Days

Background: Decrease time to begin iNO weaning from 13 hours to 9.75 hours by 31 December 2024 in patients with persistent pulmonary hypertension of the newborn (PPHN).

Methods: Countermeasures include (1) provider education at each disciplinary level, (2) badge reminder cards and laminated copies of the protocol available at the bedside, (3) order set modifications, (4) abnormality tracking at huddles. Data collection will be automated through weekly Business Objects report. The main outcome measure will be time to first wean of iNO after meeting protocol criteria. Feedback regarding the ease of weaning will be generated via Gemba Rounds.

Results: These …

Effect Of Social Determinants Of Health On Clinic Visit Attendance In Patients With Hemophilia, Shailly Gaur Md, Brian Lee Phd, Mph, James Anderst Md, Msci, Katie Foote Lscsw, Lcsw, Osw-C, Andrea Bradley-Ewing Mpa, Ma, Shannon Carpenter Md

Research Days

Background

Social determinants of health (SDOH) create barriers to seeking care regularly, especially for patients with chronic disease. Patients with moderate-severe hemophilia A and B (factor 8 and 9 deficiencies respectively) have a life-long higher risk of bleeding and require chronic therapies. Specialized care is offered through hemophilia treatment centers (HTCs); however, these can be difficult to access for some individuals. Previous SDOH research in this patient population has been limited; therefore, it can be difficult to identify the barriers to care that exist. There is a need to examine SDOH more thoroughly to create patient-directed interventions to improve access …

Intraspinal Anomalies In Presumed Ais Does Not Increase The Risk Of Intraoperative Neuromonitoring Changes During Posterior Spinal Fusion, Connor Mathes, Bill Woodhams, Aaron Shaw, John Anderson, Michael Benvenuti, Richard Schwend

Research Days

This study aims to identify the association between intraoperative neuromonitoring changes during posterior spinal fusion surgery among patients with adolescent idiopathic scoliosis and those found to have intraspinal anomalies on standard preoperative imaging.

Implementation Of An Enhanced Recovery After Surgery Pathway In Adolescent Patients Undergoing Laparoscopic Evaluation Of Endometriosis, Lauren Roth Md, Madeline Ross Md, Todd Glenski Md, Emily Weisberg Md, Ashli Lawson

Research Days

This study evaluates the implementation of an Enhanced Recovery After Surgery (ERAS) pathway for adolescent patients undergoing diagnostic laparoscopy for endometriosis at a tertiary children's hospital. The retrospective reviewed included 53 patients (20 pre-ERAS, 33 post-ERAS) aged 12-20 years old, comparing preoperative compliance, intraoperative adherence, and postoperative outcomes. Findings showed 81% compliance with preoperative medication, 76% received appropriate preoperative medications, and 39% had recommended carbohydrate loading. Intraoperative compliance was 87%, with 61% adherence to postoperative medications. Post-anesthesia care unit (PACU) stay time increased (44 vs 53 minutes, p=0.21), but pain scores, postoperative prescriptions, and hospital readmissions remained unchanged. This study …

Spinal Deformity Characteristics Associated With Intraspinal Anomalies In A Population Of Presumed Ais, Connor Mathes, Aaron Shaw, Bill Woodhams, John Anderson, Michael Benvenuti, Richard Schwend

Research Days

This study aims to identify an association between pre-operative radiographic criterion and the presence of an underlying intraspinal anomaly in patients presenting with presumed adolescent idiopathic scoliosis undergoing posterior spinal fusion.

Adherence To Oral Iron In Adolescent Females With Iron Deficiency Secondary To Heavy Menstrual Bleeding, Kennedy Sheedy, Lauren Amos Md, Ms

Research Days

Iron deficiency is an important, but often minimized issue among adolescent females. While treat with oral iron is generally first-line treatment, IV iron has been shown to be safe and effective. Our study looks at adherence and barriers to oral iron treatment and assesses current use of IV iron for iron deficiency at Children's Mercy, in hopes of helping move towards utilizing the most beneficial treatment for each individual patient.

Decreasing Time To Narcotic Pain Medication In Patients With Sickle Cell Disease Presenting With Acute Pain Crises In A Pediatric Emergency Department: A Quality Improvement Initiative., Allison Adam, Leslie Hueschen, Lina Patel, Christopher Kaberline, Ibad Siddiqi

Research Days

Background: Guidelines published by the National Heart, Lung and Blood Institute recommend that patients with sickle cell disease who present in acute pain crisis receive narcotic medication < 60 minutes from arrival. Multi-center studies indicate approximately 50% of institutions are adherent to this level 1a recommendation. From October 2022-October 2023, our institutional median time to narcotic was 61 minutes.

Objective: Our aim was to decrease the time to narcotic administration by 10% (61 min to 55 min) in patients with Sickle Cell Disease presenting to the emergency department for acute pain crisis while simultaneously decreasing disparities in our patient population by Oct 2024. Additionally, we sought to increase the percentage of patients within this population to receive their first narcotic in 60 min from 49% to 70%, by Oct 2024.

Methods: We …

A Single Center Qualitative Study To Improve Coordination Of Follow-Up Care For Patients Diagnosed With Abusive Head Trauma, Ruairi Smith-Dewey, Elise Wright, Daniella Donofrio, Hannah Goebel, Sara Kilbride

Research Days

Background/Project Intent: Review of cases identified 16 possible follow-up needs. A single patient had a maximum of 12 potential needs. Needs not being addressed included written documentation for need of follow-up without subsequent referral placed/appointment scheduled or need not addressed at all. The goal of this project was to have all follow-up needs addressed for at least 50% of children diagnosed with AHT by January 2025.

Methods: All children admitted to CMH from January 2024-December 2024 and diagnosed with AHT by the Safety, Care & Nurturing (SCAN) team were included. Interventions were designed and implemented by a multidisciplinary team to …

Implementing Balanced Fluid Resuscitation For Sepsis Patients In A Tertiary Care Pediatric Emergency Department, Grace E. Arends Md, Christopher Kaberline, Ibad Siddiqi, Sarah Nienhaus, Viktoriya Stoycheva Mha, Rn, Cpn, Leslie Hueschen

Research Days

Background

National data supports balanced fluid during volume resuscitation in the setting of sepsis as it improves morbidity and mortality, particularly preventing acidosis and kidney injury. Despite this recommendation, the CMH ED still has not implemented balanced fluid resuscitation.

Aim

The aim of the project is to increase the percentage of patients with positive sepsis screen treated in the CMH Adele Hall Emergency Department receiving lactated ringer’s fluid resuscitation from 4.6% to 15% by June 2024.

Methods

Primary outcome measures were percentage of patients with suspected sepsis who received at least one LR fluid bolus, as well as percentage of …