Higher Education and Teaching Commons^™

Gastrointestinal Fibromatosis: A Case Of Successful Oral Tyrosine Kinase Inhibitor Delivery To An Infant With Proximal Duodenal Obstruction, Darius Blanding

Research Days

Background: Infantile myofibromatosis (IM) is a rare disorder characterized by the fibrous proliferation of the skin, bone, muscle, and viscera that usually presents at birth, or soon after. Although IM is a benign condition, significantly higher morbidity and mortality have been reported when the viscera is involved. This case report is of a 6-week-old female who presented with poor feeding along with subcutaneous nodules that were diagnosed as IM. Upper and lower endoscopy were performed for worsening feeding intolerance showing marked nodularity throughout the gastrointestinal (GI) tract, including partial obstruction of the proximal duodenum by biopsy-proven myofibromas, confirming visceral IM …

Subclinical Status Epilepticus In The Setting Of An Acute Covid-19 Infection, Thomas Rieth

Research Days

Background: While COVID-19 commonly presents as an upper respiratory infection, acute COVID infections has the potential to have multisystem involvement. Neurological symptoms, which have mainly been reported in adults, are rare in children and are not well recognized. Better understanding of the neurological manifestations of COVID-19 in children can lead to earlier detection of COVID-19 and help with medical decision making and treatment.

Objectives/Goal: To describe an unusual presentation of COVID-19 in a previously healthy female and explore the neurological presentations of COVID-19.

Results: A 10 year old previously healthy female presented with a 1 day history of altered mental …

A Case Of Fibrous Hamartoma In Term Neonate, Elizabeth Perry

Research Days

Case report abstract: Fibrous hamartoma of infancy (FHI) is a rare, benign lesion characterized as a tumor of myofibroblastic origin that has characteristic features of triphasic histology. FHI was first described in 1956 by Reye and formally named by Enzinger in 1965 (1, 2). The lesion is defined as a hamartoma due to the histologic presentation of disorganized mesenchymal, fibrous, and adipose tissue with absence of mitotic figures; this combination of derived tissue without evidence of anaplasia is diagnostic for FHI. These lesions typically arise as a single, solitary mass, are most commonly located on the extremities, trunk, sacrum, or …

Early Onset Obesity In A 4-Year-Old Female Found To Have Variants In Both The Single-Minded Homolog 1 And Sh2b Adaptor Protein 1 Genes, Erica Zarse

Research Days

No abstract provided.

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 …

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 …

Characterization Of Comorbidities In Patients With A Dual Diagnosis Of Down Syndrome And Autism Spectrum Disorder Using Cerner Health Facts, Michael Slogic

Research Days

Background: Up to 19% of patients with Down syndrome (DS) meet diagnostic criteria for autism spectrum disorder (ASD) (Channell, et al, 2019). While the medical and psychological comorbidities for patients with DS or ASD are well characterized, comorbidities and outcomes for patients with a dual diagnosis (DS-ASD) are poorly understood. A large cohort of patients with DS-ASD, as well as those with ASD and DS alone, are needed to better understand this. Methods for grouping and analyzing complex diagnostic phenotypes are also needed.

Objectives/Goal: Our objective is to utilize Cerner Health Facts, a multi-institutional healthcare database, to identify large populations …

Effectiveness And Safety Of Repeat Dexamethasone For Bronchopulmonary Dysplasia, Christian Oliveros

Research Days

Background: Although a short course of steroids maybe beneficial in infants at high risk for bronchopulmonary dysplasia, response to treatment is variable. In infants with persistent lung disease despite initial treatment, repeat courses of steroids have been described but not well-studied. This 10-year observational study from a single tertiary referral center evaluates the effectiveness and safety of repeat steroid treatment for bronchopulmonary dysplasia.

Objectives/Goal: To describe effectiveness of repeat dexamethasone treatment for bronchopulmonary dysplasia (BPD) and to evaluate potential detrimental effects on growth and neurodevelopment.

Methods/Design: This was a 10-year single-center observational study of infants <30 >weeks’ gestational age at birth …

A Study Examining The Safety And Efficacy Of Ferric Carboxymaltose In A Large Pediatric Cohort, Chandni Dargan Md, David Simon Do

Research Days

Background: Iron deficiency anemia (IDA) is common in the pediatric population with varying high-risk factors. Intravenous (IV) iron supplementation has become more desirable in patients with moderate to severe anemia and in patients who are either unresponsive to or have adverse side-effects secondary to oral iron. Iron sucrose and Iron dextran have been traditionally used in pediatrics while ferric carboxymaltose (FCM) has only been FDA approved in adults. One of the major advantages of FCM is the ease of dosing and efficacy. Though FCM was approved for adults in 2013 and there have been no safety concerns, it is not …

Attitudes Of Pediatric Residents In The Care Of Transgender Youth, Michelle Knoll

Research Days

Background: Transgender individuals have health disparities compared to the general population and face several barriers to medical care. Prior studies indicate physician knowledge on transgender topics is poor and that transphobia is associated with poor knowledge even after educational interventions. Objectives/Goal: We sought to examine pediatric residents’ attitudes about transgender individuals and assess concerns related to the treatment of transgender youth. Methods: A survey was sent to 89 US Pediatric and combined (e.g. Med-Peds) residency programs and advertised on social media. Approximately 32 programs forwarded the survey to an estimated 1325 residents; 138 residents completed it. The survey consisted of …

Portable Low Field Strength Mri: Preliminary Experience In Neonates And Children, Johnston Fite

Research Days

Background: High field strength MRI (HF-MRI) is a pediatric imaging staple. However, HF-MRI access is limited by strong (1.5 – 3.0 T) magnetic fields with associated safety concerns, space requirements, and cost. To address these limitations, Hyperfine (Guilford, CT) developed a low magnetic field (0.064 T) portable MRI device, named Swoop. Preliminary data in adults shows benefits despite decreased image quality. In this study, initial evaluation of Swoop’s image quality in pediatric patients was assessed.

Objectives/Goal: The objective in this study was the initial evaluation of Swoop’s image quality in pediatric patients to serve as a baseline.

Methods/Design: The study …

Variability In Blood Gas Levels During Non-Invasive Ventilatory Support Following Planned Extubation And Association To 36-Week Bronchopulmonary Dysplasia In Preterm Neonates, Michelle Klueppelberg

Research Days

Background: Despite shifts to non-invasive support, as of 2012, 87% of preterm infants admitted to the neonatal intensive care unit (NICU) continued to receive some form of invasive mechanical ventilation1 . As a result, research around how prolonged respiratory support may impact the development of chronic lung disease, specifically bronchopulmonary dysplasia (BPD) has flourished. To date, literature has established associations between the time on invasive mechanical ventilation and incidence BPD2,3. Resulting in the development of standardized extubation strategies and bringing with them objective measures of extubation readiness4,5. Yet, due to their volatile conditions, some neonates will inevitably need to be …

A New Paradigm: Ecmo Therapy In Pediatric Hematology/Oncology Patients, Igor Areinamo

Research Days

Background: The use of extracorporeal membrane oxygenation (ECMO) for support of pediatric patients has significantly increased in the past years. Patients with known oncologic diseases, immunodeficiencies, or bone marrow transplants (BMT) have traditionally not been considered candidates for Extracorporeal membrane oxygenation (ECMO) due to perceived high risk for mortality. Ongoing advances have continued to push the boundaries of ECMO use and we report our recent experience with this patient population

Objectives/Goal: To evaluate survival to discharge of pediatric patients who require ECMO support and who also have an underlying hematologic or oncologic disease process. To determine specific patient characteristics that …

Low Dose Doxorubicin Inhibits Immune Checkpoint Upregulation In Acute Leukemias, Bradley C. Stockard

Research Days

Background: Evasion of drug and immune response in therapy-resistant leukemic stem cells (LSCs) is a major cause of relapse. A previous study has identified an alternative mechanism of action for low-dose doxorubicin (DXR) that inhibits upregulation of immune checkpoints (IC) in LSCs.

Objectives/Goal: The objective of this study is to establish the DXR dose range that will achieve the inhibition of immune checkpoint expression in leukemic cell lines.

Methods/Design: Cells were analyzed for expression of CTLA-4, LAG-3, PD-1, TIGIT, and TIM-3 via flow cytometry. Analysis was performed on days 3, 5, and 8 of treatment at concentrations identified as low, …

Posterior Reversible Encephalopathy Syndrome After Dinutuximab, Kayeleigh Higgerson Do, Chandni Dargan Md

Research Days

Background: Posterior reversible encephalopathy syndrome (PRES) is a reversible disorder associated with edema of subcortical white matter and cortical gray matter that usually involves the occipital and parietal lobes. Endothelial injury secondary to abrupt blood pressure changes breaks down the blood brain barrier leading to brain edema. Per the ANBL0032 Fall 2015 Study Progress Report, Dinutuximab was the probable cause of PRES in two patients. There have been published reports of PRES with other anti-GD2 antibody therapies but very few reports of Dinutuximab as the causative agent.

Objectives/Goal: To report a case of PRES after Dinutuximab

Methods/Design: Case Report

Results: …

Levofloxacin Versus Ciprofloxacin Prophylaxis In Pediatric Cancer Patients At High Risk Of Infection, Chandni Dargan Md, Amy Johnson Md, Mba

Research Days

Background: Patients with cancer and those undergoing chemotherapy are at risk of developing bacterial infections due to myelosuppression. Patients undergoing the most intensive chemotherapy regimens are at a higher risk for morbidity and mortality due to profound neutropenia. Antibacterial prophylaxis is given to reduce the incidence of infection in those at highest risk. Starting March 1, 2016 our institution used ciprofloxacin for antibacterial prophylaxis however recent literature, including the COG trial ACCL0934, supports using levofloxacin in certain high risk (HR) populations due to greater efficacy in reducing neutropenic fever (NF) and bacteremia. Therefore, we switched to this April 1, 2019. …

A Pediatric Case Of Treatment-Related Myelodysplastic Syndrome While On Therapy For Pre-B All, Sara Mcelroy

Research Days

Background: Treatment-related myelodysplastic syndrome (t-MDS) is a known, but rare, late effect of cancer therapy, specifically radiation, alkylating agents or topoisomerase II inhibitors. When secondary to treatment with alkylating agents, t-MDS typically occurs 4 to 7 years after therapy, and common cytogenetics include chromosomes 5 and 7 abnormalities. Treatment in children with t-MDS is typically allogeneic stem cell transplant, but the prognosis remains poor.

Objectives/Goal: To describe a rare case of a pediatric patient who developed t-MDS while receiving treatment for Pre-B Cell Acute Lymphoblastic Leukemia (pre-B ALL) and to outline the treatment regimen that he received.

Methods/Design: Case Report …

Therapeutic Plasma Exchange In Critically Ill Pediatric Patients With Leukemia, Sarah E. Mc Dermott, Chandni Dargan

Research Days

Background: Therapeutic plasma exchange (TPE) has well-documented applications in the adult population, outlined by the American Society of Apheresis (ASFA) 2019 guidelines. Limited data exists regarding the use of TPE in critically ill pediatric patients, however these reports rarely include patients with oncological diseases. Care for these patients poses certain clinical considerations including the safety of continuing chemotherapy, delayed clearance of chemotherapy secondary to end organ damage and mechanical clearance (i.e., continuous renal replacement therapy (CRRT) circuitry), and ability to tolerate chemotherapy once recovered. We aim to highlight the potential benefits of TPE in their acute management, so that it …

Outcomes Of Infants With Severe Rop At Risk Of Treatment With Avastin Compared To Laser Surgery, Stephen Doxey

Research Days

Background: Retinopathy of prematurity (ROP) is a well-known complication occurring in the most premature infants leading to visual impairment and in the most severe cases associated with retinal detachment and childhood blindness. Factors contributing the development of ROP include chronic hypoxia and disordered vascular genesis primarily through the vascular endothelial growth factor (VEGF) signaling pathway. Infants at risk of developing ROP are screened with serial eye exam and treated with either laser surgery or intraocular injects of bevacizumab (Avastin) to prevent progression of ROP and preserve vision. Bevacizumab, an inhibitor of VEGF signaling, may have positive effects on preventing ROP …

Long-Term Outcomes And Satisfaction Rates After Costal Cartilage Resection For Slipping Rib Syndrome, James Fraser

Research Days

Background/Objectives/Goal: Slipping rib syndrome (SRS) is a challenging and underdiagnosed condition that has proven to be an elusive diagnosis due to an extensive differential with presenting symptoms that mimic a myriad of alternative diagnoses of both thoracic and abdominal pathology. The diagnosis of SRS is recognized in a patient with consistent, progressive, often debilitating unilateral or bilateral chest wall and/or upper abdominal pain with intermittent clicking or rib slipping. SRS frequently does not resolve with conservative management, including physical therapy, rest, and NSAIDs, as well as intercostal injections, and even opioid medications. Workup can be extensive including multiple subspecialty referrals …

Multimodal Pain Control In Common Neonatal Surgeries: Post-Operative Pain Protocol Reduces Opiate Exposure And Side Effects, Jamesia Donato

Research Days

Background: Opioids are the primary post-operative (post-op) analgesic in neonates. Side effects include hypotension, apnea and ileus. Previous studies show IV acetaminophen decreases opiate need in specific populations including cleft palate repair, cardiac surgery. The effect of IV acetaminophen as part of a post-op pain algorithm is not known in common neonatal surgical procedures.

Objectives/Goal: To determine if (a) a post-op pain algorithm including IV acetaminophen and (b) IV acetaminophen exposure reduces cumulative opioid requirements in neonates undergoing common surgeries in the NICU.

Methods/Design: This is a retrospective cohort study comparing cumulative post-operative opiate exposure between infants with common surgeries …

Improving Thromboprophylaxis In Hospitalized Pediatric Inflammatory Bowel Disease Patients- A Quality Improvement Project, Amy Issa, Panam Kaur

Research Days

Background/Project Intent (Aim Statement): Pediatric inflammatory bowel disease (IBD) patients in acute flare have 1.5 to 3-fold higher risk of thromboembolism compared to the general population. Anticoagulation therapy is recommended for pediatric IBD patients who have ≥ 1 risk factors for venous thromboembolic events. At Children’s Mercy Hospital (CMH), approximately 80 patients/year are admitted for acute flare, of which, roughly 30% receive appropriate thromboprophylaxis. Lack of standardization of thromboprophylaxis and an educational gap amongst providers are significant barriers. Our goal is to standardize and improve the rates of thromboprophylaxis in IBD patients admitted at CMH from 30% to 60% by …

Umbilical Access In Laparoscopic Surgery In Infants Less Than 3 Months: Single Institution Retrospective Review, James Fraser

Research Days

Background/Objectives/Goal: Umbilical access in laparoscopic surgery has been cited as a potential factor for increased complications in low-birth-weight infants and those less than three months old. Previous series noted a self-reported complication rate of 10.6% among 329 pediatric surgeons via anonymous survey, citing carbon dioxide (CO2) embolism as the most common complication. We report four-year outcomes with blunt transumbilical laparoscopic access to examine the safety of this technique.

Methods/Design: Following IRB approval, a retrospective database of patients less than three months of age who underwent laparoscopic pyloromyotomy or inguinal hernia repair from 2016-2019 at a tertiary care academic center was …

Pediatric Pulmonary Artery Aneurysms Causing Pulmonary Hemorrhage: A Case Of Transcatheter Intervention While On Va Ecmo Support, Igor Areinamo

Research Days

Background: The incidence of pulmonary artery aneurysm (PAA) in children remains unknown. There are few reports of a transcutaneous interventional approach to address this type of pathology. Management of PAAs while on VA ECMO has not been described in pediatrics.

Objectives/Goal:

Methods/Design:

Case: 9-year-old previously healthy male presented with MRSA associated ARDS and sepsis requiring urgent VA ECMO cannulation. Chest CT done on ECMO day 22 showed development of right sided PAAs. Patient had an episode of significant pulmonary hemorrhage. Angiogram demonstrated two fusiform right PAAs, one of which had ruptured and was bleeding into the lung parenchyma. Patient had …

Not-So-Apparent Mixing Lesions: Late Presentation Of Cardioembolic Stroke, Amulya Buddhavarapu

Research Days

Background: Multiple levels of inter-atrial shunting can rarely present late in life with dyspnea and embolic stroke from effects of bidirectional shunting.

Case: A 55-year-old male with history of a patent foramen ovale, atrial fibrillation and embolic stroke presented with worsening fatigue and dyspnea at rest. Echocardiogram showed mildly reduced ventricular function with severe right heart dilation. On transesophageal echo, the coronary sinus (CS) was severely dilated with a persistent left superior vena cava (LSVC). A stress test showed no perfusion defects. CT angiography showed an absence of right SVC and a single LSVC connecting to the CS. There was …

Congenital Mitral Valve Regurgitation, The Dilemma Of Repair Vs Replacement., Bianca Cherestal

Research Days

Background: Congenital mitral regurgitation is a rare condition and can be challenging to manage when presenting in the neonatal period

Objectives/Goal: Two week old male presented with poor weight gain, murmur and cardiomegaly on chest X-ray. Echocardiogram showed moderate to severe mitral regurgitation (MR) and suprasystemic pulmonary hypertension (PHN) (fig 1 a, b). The mitral valve (MV) leaflets were thickened and tethered with failure of central coaptation. PHN was classified as WHO I and II (due to persistent PHN of newborn and MR respectively). Inhaled nitric oxide, Enalapril and Furosemide were initiated. Cardiac catheterization revealed PVRi of 8.9 WU x …

Online Echocardiography Modules For Pediatric Cardiology Fellows, Sarah Studyvin, Doaa Aly, Tyler Johnson, Laura Kuzava, Alison Samrany, Nitin Madan, Sanket Shah

Research Days

Background: Adequate echocardiographic (echo) education is integral to successful cardiology training. Online education is proven to be effective in other specialties, but pediatric echo training data are lacking.

Objectives/Goal: Our aim was to design and assess the role of a novel online module-based curriculum to enhance pediatric cardiology fellows’ echo education.

Methods/Design: Four interactive modules, focused on principles and interpretation of a normal echo, were delivered to fellows. Content included high quality 2D and 3D echo clips as well as interactive quizzes and could be accessed from a phone, tablet, or computer. All fellows completed pre- and post-tests, validated by …

The Impact Of Early Tracheostomy On Neurodevelopmental Outcome Of Infants With Severe Bronchopulmonary Dysplasia Exposed To Postnatal Steroids, Amjad Taha

Research Days

Background: BPD is associated with long-term neurodevelopmental impairment. Tracheostomy is performed in 5-12% of severe BPD for prolonged ventilation. There is evidence that chronic ventilation with tracheostomy in severe BPD may facilitate neurodevelopment and lead to improved outcome. However, there is no consensus on the optimal timing of tracheostomy. A large multicenter study of infants with tracheostomy performed at <120 days of life had better neurodevelopmental outcomes at 18-22 months of age. Use of steroids postnatally to ameliorate the severity of BPD had been controversial due to its negative effect on neurodevelopment. To date, no data has specifically evaluated the impact of early tracheostomy on neurodevelopmental outcome of infants with severe BPD who are exposed to postnatal steroids.

Objectives/Goal: To compare the cognitive, language and motor scores among 3 groups of severe BPD infants who received early vs late vs no tracheostomy. Secondly, evaluate if postnatal steroids had an additive negative effect on neurodevelopmental outcomes.

Methods/Design: IRB approved retrospective …

Constrictive Pericarditis After Repair Of A Ruptured Sinus Of Valsalva, Sarah Studyvin, Laura Kuzava

Research Days

Background: Constrictive pericarditis is an uncommon complication of cardiac surgery. We report a patient who developed constrictive pericarditis after ruptured sinus of Valsalva (RSOV) repair.

Methods: A 23-year-old male presented with exertional dyspnea one year after RSOV repair. TTE showed a small, circumferential effusion with thickened pericardium, ventricular septal bounce, left atrial enlargement, diastolic hepatic flow reversal, and trivial mitral regurgitation without stenosis. He underwent cardiac catheterization, which revealed elevated filling pressures (RVEDP 16 mmHg, LVEDP 18 mmHg), RVEDP/RVSP ratio < 0.5, and a low cardiac index (1.65 L/min/m2). Cardiac MRI confirmed pericardial thickening with paradoxic septal motion, dilated pulmonary veins and retrograde flow in the SVC.

Results: Pericardiectomy of thickened and adherent pericardium was performed. The central venous pressure decreased from 23 to 7 mmHg and …

Local Implementation And Standardization Of The Pediatric Severe Traumatic Brain Injury Guidelines, Michelle Brown

Research Days

Problem Statement/Question: Severe pediatric Traumatic Brain Injury (TBI) remains a significant cause of morbidity and mortality. Evidence-based guidelines leave gaps in how to best implement and deliver standardized care on the local hospital level.

Background/Project Intent (Aim Statement): We aim to improve clinical outcomes for severe TBI patients (Glasgow Coma Scale ≤8) with standardization of care according to updated evidence-based guidelines. We will target early guideline adherence (initial 72 hours of PICU admission) with a focus on 1) targeted temperature management (TTM40mmHg).

Methods (include PDSA cycles): The Children’s Mercy Hospital Pediatric ICU Severe TBI Guidelines were updated after the 3rd …