Medical Specialties Commons^™

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 …

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 …

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 …

Potential Role Of Methotrexate Polyglutamates In Therapeutic Drug Monitoring For Pediatric Inflammatory Bowel Disease., Ryan Morrow, Ryan Funk, Mara Becker, Ashley K. Sherman, Leon Van Haandel, Taina Hudson, Rebecca Casini, Valentina Shakhnovich

Manuscripts, Articles, Book Chapters and Other Papers

Inside cells, the immunomodulator methotrexate (MTX) undergoes the addition of glutamates to form methotrexate polyglutamates (MTX-Glu)-promising biomarkers of systemic exposure and treatment response to MTX in rheumatology. MTX-Glu are underexplored in Inflammatory Bowel Disease (IBD), with no data in pediatrics. In this cross-sectional secondary analysis, we assessed the relationships between MTX-Glu and MTX dose and treatment response in pediatric IBD. Twenty-one children with IBD, receiving maintenance therapy with infliximab (IFX) and MTX, had MTX-Glu1-6 concentrations and IFX troughs/antibodies measured and disease activity assessed for comparison in remission vs. active IBD using non-parametric tests, with associations explored using Spearman's correlation (ρ) …

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 …

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 …

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, …

Psychosocial Factors Affecting Quality Of Life In Patients With Anorectal Malformations And Hirschsprung’S Disease – A Qualitative Systematic Review, Wendy Jo Svetanoff

Research Days

Background: While great strides have been made in surgical techniques and bowel management therapies for patients with anorectal malformations (ARM) and Hirschsprung’s disease (HSCR), little is known about psychosocial and behavioral factors that impact the quality of life at each stage of development.

Objectives/Goal: We aimed to perform a qualitative literature review to highlight the psychosocial, emotional, and behavioral themes that affect the quality of life as patients born with congenital colorectal disease.

Methods/Design: A systematic literature review of all articles published between 1980-2019 was performed in the PubMed and CINAHL databases. Inclusion criteria included articles that reported on the …

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 …

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 …

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 …

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 …

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 …

Impact Of Covid-19 On Families With A Child In Cancer Treatment, Mirae J. Fornander

Research Days

Background: A new diagnosis of pediatric cancer diagnosis is a sudden and tremendous stressor to families (Long & Marsland, 2011). The COVID-19 pandemic presented an acute, universal stressor that impacted daily life around the world (Brooks et al., 2020). The impact of the COVID-19 pandemic on children with cancer and their families is not yet known.

Objectives/Goal: The current study is a pilot investigation of COVID-19 exposure and impact among a sample of families with a child being treated for cancer.

Methods/Design: Primary caregivers (N=22) of a child (M age=7.4, SD=4.6) who was diagnosed with cancer within the last 18 …

Pharmacogenetic Testing In Patients With Autism Spectrum Disorder Evaluated In The Children’S Mercy Hospital Goldiloks© Clinic, Rachel Goodson

Research Days

Background: Autism Spectrum Disorder (ASD) affects 1 in 54 children in the United States. Children with ASD are more likely to be diagnosed with co-occurring mental health disorders. There is currently little research guiding medication choice and dosing in patients with ASD. Children with ASD are at increased risk for reduced clinical response and adverse reaction (including due to polypharmacy). Pharmacogenomics is an approach for medication decision making that leverages an individual’s genetic information and clinical presentation to make informed choices, but no studies have specifically investigated the outcomes of PGX for patients with ASD. Understanding the clinical and genetic …

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 …

Hyperglycemia Requiring Insulin Among Pediatric Patients Diagnosed With Hemophagocytic Lymphohistiocytosis, Cintya Schweisberger

Research Days

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder marked by massive cytokine release due to macrophage and T-cell activation. Hallmarks of the diagnosis include fever, splenomegaly, cytopenias, hypertriglyceridemia, hypofibrinogemia, and elevations in ferritin and soluble IL-2 receptor. Given HLH is associated with critical illness, elevation in inflammatory markers, and treated with glucocorticoids, the development of hyperglycemia during its course is not unexpected. However, detailed descriptions of the severity of hyperglycemia and strategies in insulin management among HLH patients are lacking. We describe 10 years’ experience at a single tertiary pediatric health center with HLH patients who developed insulin dependent …

Long-Term Safety Of Growth Hormone Treatment In Childhood: Two Large Observational Studies: Nordinet Ios And Answer., Lars Sävendahl, Michel Polak, Philippe Backeljauw, Joanne C Blair, Bradley S Miller, Tilman R Rohrer, Anita Hokken-Koelega, Alberto Pietropoli, Nicky Kelepouris, Judith L. Ross

Department of Pediatrics Faculty Papers

CONTEXT: Growth hormone (GH) treatment has a generally good safety profile; however, concerns about increased mortality risk in adulthood have been raised.

OBJECTIVE: This work aims to assess the long-term safety of GH treatment in clinical practice.

METHODS: Data were collected from 676 clinics participating in 2 multicenter longitudinal observational studies: the NordiNet International Outcome Study (2006-2016, Europe) and ANSWER Program (2002-2016, USA). Pediatric patients treated with GH were classified into 3 risk groups based on diagnosis. Intervention consisted of daily GH treatment, and main outcome measures included incidence rates (events/1000 patient-years) of adverse drug reactions (ADRs), serious adverse events …

The Power Of Outpatient Botulinum Injections In Preventing Hirschsprung Associated Enterocolitis, Wendy Jo Svetanoff

Research Days

Background: Hirschsprung associated enterocolitis (HAEC) is a potentially life-threatening complication, where the child often presents with obstructive symptoms. Failure of the internal anal sphincter (IAS) to relax is thought to play a role in these symptoms. Botulinum toxin (BT) injections assist in temporarily relieving obstructive symptoms due to IAS achalasia in patients with Hirschsprung’s disease following surgical pull-through. The use of BT may play a role in preventing HAEC episodes related to IAS dysfunction.

Objectives/Goal: Our aim was to determine the effect of scheduled outpatient Botulinum injections on the development of HAEC.

Methods/Design: A retrospective review between July 2010 – …

Iv Amiodarone Vs Iv Sotalol Use In Postoperative Junctional Ectopic Tachycardia (Jet): A Randomized Study, Tanner Isaacson

Research Days

Background: Junctional ectopic tachycardia (JET) is one of the most common post-operative arrhythmias encountered following congenital heart surgery. For many, amiodarone is the pharmacologic treatment of choice. While effective, amiodarone has been associated with dose-related adverse effects. Sotalol has been approved for intravenous use in pediatric patients in the United States to treat both supraventricular and ventricular arrhythmias, although pediatric data remains limited. Sotalol has class III antiarrhythmic properties with some mild β-adrenergic blocking effects, similar to amiodarone. Data shows a lower rate of cardiovascular collapse and adverse events with sotalol in patients with congenital heart disease. At present, it …

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 …

The Effect Of Covid-19 On Pediatric Orthopaedic Trauma Volume At A Level 1 Pediatric Trauma Center In The Midwestern United States, Kirsten Norrell

Research Days

Background: School and playground closures and the cancellation of extracurricular activities in the Kansas City region on March 17, 2020 due to the COVID-19 pandemic created an unprecedented situation for children and parents. Early reports in the literature on adult and pediatric trauma volume suggested a decrease in orthopaedic injuries during the pandemic. This is the first study to report how the pandemic affected orthopaedic trauma volume at a Level 1 pediatric trauma center during the entire five months since school and activity closures went into effect.

Objectives/Goal: The aim of this study was to compare the volume and type …

Pre-Hospital Traumatic Cardiopulmonary Arrest In Children At A Level 1 Pediatric Trauma Center, Kayla B. Briggs

Research Days

Background: The survival of traumatic cardiopulmonary arrest (TCA) requiring pre-hospital cardiopulmonary resuscitation (P-CPR) is abysmal across age groups.

Objectives/Goal: We aim to describe the patterns of injury, pre-hospital interventions, and outcomes of children suffering from TCA leading to P-CPR at our institution to support standardized guidelines addressing the termination of prolonged resuscitation attempts in children.

Methods/Design: Following IRB approval, retrospective review was conducted to identify children ages 0-17 years who suffered TCA leading to P-CPR at our institution between 5/1/2009–3/1/2020. For analysis, patients were stratified into those still undergoing CPR at arrival and those who attained pre-hospital return of spontaneous …