Medicine and Health Sciences Commons^™

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., …

Infliximab As Rescue Therapy In Pediatric Severe Colitis, Kayla B. Briggs

Research Days

Background: Infliximab has been shown to be effective in achieving clinical remission in patients with ulcerative colitis (UC) refractory to conventional therapy. However, there is conflicting data in the literature regarding its effectiveness as rescue therapy in acute severe colitis. Furthermore, most studies were conducted in adults, and pediatric onset of inflammatory bowel disease (IBD) is associated with more severe disease that may be less amenable to rescue therapy.

Objectives/Goal: We reviewed our experience with pediatric severe colitis and report outcomes following attempted rescue therapy with infliximab.

Methods/Design: A retrospective review was conducted of patients with UC or indeterminate colitis …

Transgender Identity Among Adolescents With Pcos, Cintya Schweisberger

Research Days

Background: The estimated prevalence of transgender identity among US adolescents is 0.5% to 1.3%. Some studies have suggested an increased prevalence of PCOS in transmasculine adults before testosterone therapy. There is a paucity of data describing the relationship between gender dysphoria and PCOS in adolescents.

Objectives/Goal: The objective of our study was to describe the prevalence of transgender identity among adolescents with PCOS.

Methods/Design: A secondary data analysis was conducted in 169 adolescents (16.1 ±1.64 yrs) from our Multidisciplinary Adolescent medicine and Pediatric endocrine PCOS (MAPP) clinic who met NIH criteria for PCOS and had gender identity recorded. Variables analyzed …

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 …

Impact Of High Flow Nasal Cannula On Resource Utilization In Bronchiolitis, Scott Biggerstaff

Research Days

Background: Bronchiolitis is one of the most common diseases requiring hospitalization in children 1-24 months of age, but presently treatment is primarily supportive. High flow nasal cannula is a newer method of oxygen delivery pediatric hospitals have been rapidly adopting to treat respiratory insufficiency caused by viral bronchiolitis.

Objectives/Goal: We sought to compare resource utilization, including cost, length of stay (LOS), and readmission among children who were treated with HFNC versus those who were not.

Methods/Design: In this cross-sectional, multicenter study, we obtained clinical and resource utilization data from the Pediatric Health Information System (PHIS) database for otherwise healthy children …

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 …

Unique Evaluation And Management Considerations For Adolescents With Late Gynecologic And Colorectal Issues In The Setting Of Anorectal Malformations, Wendy Jo Svetanoff

Research Days

Background: Females born with anorectal malformations (ARMs) and bladder exstrophy have a greater incidence of Mullerian and genital anomalies requiring early gynecologic assessment and frequent surgical intervention. However, there is little guidance for management considerations of pubertally identified Mullerian anomalies in these patients

Objectives/Goal: We sought to assess the unique colorectal, gynecologic, and psychological issues present during the adolescent years.

Methods/Design: A retrospective review was performed of 10-25 year old female patients born with an ARM, cloaca, or exstrophy that presented to our multidisciplinary clinic between 2009 and 2019. Data abstracted included the presenting problem, history, imaging studies, psychological evaluation, …

Prevalence Of Mycoplasma Genitalium And Macrolide Resistance In Adolescent Females Receiving Care At A Pediatric Hospital, Kayla Barnes

Research Days

Background: Mycoplasma genitalium is an established sexually transmitted cause of nongonococcal urethritis in males and macrolide resistance is increasing. The pathogenic role is less well-defined in adolescent females and guidelines recommend M. genitalium testing only be considered in cases of persistent or recurrent cervicitis and pelvic inflammatory disease (PID). We lack understanding of the prevalence and macrolide resistance of M. genitalium among adolescent females.

Objectives/Goal: To determine the prevalence of M. genitalium and rate of detected macrolide resistance among adolescent females seeking care at a pediatric children’s hospital.

Methods/Design: We collected 200 salvaged urogenital samples (56 urine and 144 vaginal) …

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 …

Antibiotic Durations For Skin And Soft Tissue Infections In Pediatric Urgent Care Clinics, Megan Hamner

Research Days

Background: Skin and soft tissue infections (SSTIs) are the second most common diagnosis leading to pediatric antibiotic prescriptions in the outpatient setting after respiratory diagnoses. However, most antibiotic stewardship programs have mainly focused on the latter. Children seen in the ambulatory setting for SSTIs often receive >7 days of antibiotics, although current society guidelines recommend 5-7 days for most diagnoses.

Objectives/Goal: To determine the baseline percentage of patients receiving antibiotic prescriptions for >7 days for SSTIs in Children’s Mercy urgent care clinics (UCC)s and to evaluate factors that influence providers towards longer durations.

Methods/Design: We built a report that extracted …

A Case Series Of Hyperglycemic Hyperosmolar State During The Global Covid-19 Pandemic, Benjamin Hoag

Research Days

Background: Hyperglycemic hyperosmolar state (HHS) is rare in pediatrics, particularly in patients with antibody positive diabetes mellitus (DM). Recent literature has implicated COVID-19 in the reported increase in new onset DM cases, as well as mixed diabetic ketoacidosis (DKA) and HHS cases; however, a rise in HHS cases alone has not been well reported [1,2]. We noted an anecdotal increase in the frequency of HHS cases in our pediatric tertiary care center following the onset of the global COVID-19 pandemic.

Objectives/Goal: To investigate further, a retrospective chart review evaluating all patients with DM admitted in the first 6 months of …

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 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 …

Neonatal Arch Obstruction: Not Always A Coarctation, Karoline Krzywda

Research Days

Background: Spontaneous neonatal arch thrombosis is a rare, highly lethal cause of aortic arch obstruction.

Case: A 1-day-old male infant presented failing CCHD screen, on prostaglandin infusion for echocardiographic concerns of aortic coarctation. Repeat echocardiogram revealed severe arch obstruction due to a large, 7.5x5mm echogenic mass, at the aortic isthmus (Fig1A) with right to left systolic PDA flow. CT angiography confirmed presence of a large aortic arch thrombus (Fig1B). Bivalirudin infusion was initiated without change in thrombus size after 2 days. Tissue plasminogen activator (tPA) was subsequently administered with significant reduction in thrombus size to 3x4mm (Fig1C) on day 1 …

Germline Variants Associated With Cancer Predisposition And Bone Marrow Failure Are Common In Kmt2a-R Infant Acute Lymphoblastic Leukemia Patients, Sarah E. Mc Dermott

Research Days

Background: Infant acute lymphoblastic leukemia (ALL), is a particularly aggressive subtype of leukemia with an early onset and unfavorable clinical outcome. Most (~70%) cases of infant ALL involve chromosomal rearrangement of KMT2A (KMT2A- r) on chromosome 11q23, the strongest independent predictor of a poor prognosis. To date, genomics studies have consistently demonstrated KMT2A-r infant ALL to have a strikingly silent landscape of DNA mutations. Germline mutations in cancer predisposition genes are found in 8.6% of pediatric malignancies and 4.4% of pediatric leukemias, compared to 1.1% in persons in the 1000 Genomes Project.

Objectives/Goal: We hypothesized that germline variants may contribute …

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 …

Bleeding Disorder Referrals To Hematology Clinic: A Single Institution Experience, Zuri Hudson

Research Days

Background: Our center receives hundreds of referrals yearly for bleeding disorder evaluation both due to bleeding symptoms and secondary to routine preoperative laboratory testing. The evaluation for a bleeding disorder can be challenging due to the wide variability of symptoms as well as the need for accurately interpreting lab results. Bhasin et al showed that 4% of patients referred to hematology based on a preoperative coagulation evaluation had a clinically relevant bleeding disorder. Currently there is little published about the referral patterns to pediatric hematology and the outcomes of these referrals.

Objectives/Goal: To characterize our hematology referrals for bleeding disorder …

Do Males With 45,X/46,Xy Mosaicism Have Turner Syndrome, Michelle Knoll

Research Days

Background: Patients with 45,X/46,XY mosaicism can have genitalia spanning the range from typical female to typical male, and the sex assigned at birth is often based on the appearance of the genitalia. According to current nomenclature, only the subgroup assigned female are diagnosed with Turner syndrome (TS) (Gravholt et al, 2017). Clinical practice guidelines recommend that girls with TS be screened for several comorbidities, including short stature, cardiac and renal anomalies, and autoimmune conditions, but there are no recommendations for screening of boys with 45,X/46,XY mosaicism.

Objectives/Goal: We sought to determine if boys with 45,X/46,XY mosaicism exhibited a similar rate …

Understanding Illness Encouragement And Pain Acceptance In Pediatric Patients With Abdominal Pain, Scott Wagoner

Research Days

Background: Parents can respond to their child’s abdominal pain in a variety of ways, and parental illness encouragement (e.g., spoiling, letting child stay home from school) is known to relate to longer periods of illness. Modeling and reinforcement of the “sick role” has been used to explain these processes. Many pediatric patients with abdominal pain will continue to experience symptoms in adulthood, so having a full understanding of what pain management techniques patients adopt is crucial. This study explored the impact of parental illness encouragement on how patients engage in valued activities (i.e., activity engagement) and prioritize controlling their pain …

Changes In Family Functioning During The First Year Of Pediatric Cancer Treatment: A Mixed-Method Pilot Study, Carolyn Bates

Research Days

Background: A new diagnosis of pediatric cancer presents a sudden and tremendous stressor for families of all backgrounds and circumstances (Long & Marsland, 2011; Bemis et al., 2015). Although many families are resilient in the context of new cancer diagnosis, approximately 33% are at risk for negative outcomes due to psychosocial distress and difficulties with effective coping (Kazak et al., 2020; Pai et al., 2008), supporting the need for systematic assessment of psychosocial health as a standard of care in pediatric oncology (Kazak et al., 2015). Alongside heightened stress within the first year of diagnosis, parents may relax family rules …

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 …

Atrial Standstill In A Pediatric Patient With Scn5a Mutation Following Procainamide Challenge, Anmol Goyal

Research Days

Background: Atrial standstill (AS) is a rare arrhythmia characterized by absence of electrical and mechanical atrial activity associated with SCN5A channelopathy.

Case: An 18 year old male with structurally normal heart, frequent sinus pauses, nonsustained atrial tachycardia and high-grade block was found to have SCN5A mutation c.3823G>A (p.Asp1275Asn). An electrophysiology study (EPS) with high density voltage mapping of the right atrium was done (Fig 1a). Nonsustained multifocal atrial tachycardia was induced without ablative targets (Fib 1b). Procainamide challenge was negative for Brugada, however induced AS (Fig 1c-d). No atrial capture could be achieved at maximal output. Empiric atrial lead …

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: …

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 …

Reconsidering Perioperative Antibiotic Use In Elective Laparoscopic Cholecystectomy, Kayla B. Briggs

Research Days

Background: As rates of antimicrobial resistance increase, the use of prophylactic perioperative antibiotics (PPA) has been questioned in cases with a low risk of surgical site infection (SSI). In laparoscopic cholecystectomy performed for biliary dyskinesia, hyperkinetic gallbladder, and gallbladder polyps, the use of PPA varies with no widely accepted practice pattern.

Objectives/Goal: We examined institutional PPA usage and SSI rates for elective laparoscopic cholecystectomy to determine if PPAs are indicated.

Methods/Design: Following IRB approval, children/2010 and 8/2020 for symptomatic cholelithiasis, biliary dyskinesia, hyperkinetic gallbladder, and/or gallbladder polyps were included. Several surgeons changed practice to selective PPA use in 2016, decreasing …