Medical Specialties Commons^™

Elucidating Human Milk Oligosaccharide Biosynthetic Genes Through Network-Based Multi-Omics Integration, Benjamin P Kellman, Anne Richelle, Jeong-Yeh Yang, Digantkumar Chapla, Austin W T Chiang, Julia A Najera, Chenguang Liang, Annalee Fürst, Bokan Bao, Natalia Koga, Mahmoud A Mohammad, Anders Bech Bruntse, Morey W Haymond, Kelley W Moremen, Lars Bode, Nathan E Lewis

Children’s Nutrition Research Center Staff Publications

Human Milk Oligosaccharides (HMOs) are abundant carbohydrates fundamental to infant health and development. Although these oligosaccharides were discovered more than half a century ago, their biosynthesis in the mammary gland remains largely uncharacterized. Here, we use a systems biology framework that integrates glycan and RNA expression data to construct an HMO biosynthetic network and predict glycosyltransferases involved. To accomplish this, we construct models describing the most likely pathways for the synthesis of the oligosaccharides accounting for >95% of the HMO content in human milk. Through our models, we propose candidate genes for elongation, branching, fucosylation, and sialylation of HMOs. Our …

Burnout In Pediatric Nephrology Fellows And Faculty: Lessons From The Sustainable Pediatric Nephrology Workforce Project (Superpower)., Susan M. Halbach, Kartik Pillutla, Patricia Seo-Mayer, Alan Schwartz, Darcy K. Weidemann, John D. Mahan

Manuscripts, Articles, Book Chapters and Other Papers

Physician well-being is an important contributor to both job satisfaction and patient outcomes. Rates of burnout among physicians vary by specialty, ranging from 35 to 70%. Among pediatric residents, longitudinal data demonstrates consistent rates of burnout around 50-60%, although little is known about burnout among pediatric subspecialty fellows. Specifically, the degree of burnout among pediatric nephrologists remains unknown, as does the impact faculty burnout may have on trainee burnout. We sought to evaluate prevalence and predictors of burnout among US pediatric nephrology fellows and faculty, and assess for interactions between groups. In this multi-center pilot survey of all United States …

Large Single Center Experience In Pediatric Oncology And Bone Marrow Transplant Patients On Ecmo: How Should We Decide Candidacy?, Jordan Marquess

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Background: ECMO is rarely utilized in pediatric oncology and bone marrow transplant (BMT) populations due to concerns surrounding poor prognoses and high rates of complications. Patients with malignancies are often not offered ECMO as an option. There is paucity of data to determine whether a pediatric oncology or bone marrow transplant patient is an appropriate candidate for ECMO.

Methods: This is a single center retrospective cohort study on pediatric patients with primary oncologic diagnosis or history of bone marrow transplant who required VA- or VV-ECMO from 2015-2020.

Results: Twelve patients were identified with a 58% …

Admission Pao2 And Mortality Among Pediatric Icu Patients And Critically Ill Subgroups, Cara Holton

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Background and Objectives: Studies analyzing the association between hyperoxia and mortality in critically ill pediatric patients and those admitted after cardiac arrest are conflicting and limited by small sample sizes. The aim of this study is to evaluate the association between PaO2 on admission to the pediatric ICU and mortality in a heterogeneous, multicenter cohort and among subgroups of critically ill patients.

Methods/Design: A retrospective cohort study was conducted using data from Virtual Pediatric Systems (VPS) database. VPS is a clinical pediatric critical care database with over 135 participating hospitals in North America. All VPS …

Safety, Timing And Outcomes Of Early Postoperative Cardiac Catheterization Following Congenital Heart Surgery, Karoline Krzywda

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Background: Early postoperative ( < 6weeks) cardiac catheterization (EPOCC) has been considered high risk and often delayed. Recently, the safety of EPOCC has been demonstrated. The optimal timing of catheterization and its outcomes in struggling postoperative patients remains uncertain.

Objective: To describe EPOCC in our center and its impact on length of stay (LOS), number of mechanical ventilation days, number of ECMO days, and catheter-based interventions.

Methods: This is a retrospective cohort study of patients who underwent cardiac surgery between 01/01/2010 and 12/31/2019 and EPOCC within 30 days after surgery. Cohorts were early EPOCC (≤72 hr) vs late EPOCC ( >72 hr). Data on timing, indication, and complications of EPOCC; days of mechanical ventilation and ECMO; and CICU/total hospital LOS were collected. Data was analyzed using chi-square and Student’s t-test, …

Ekg Technology: It Is Time For A Change!, Megan Carroll

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Background: Technology for obtaining pediatric EKGs has not changed in over 70 years. Current guidelines recommend a 12-lead EKG, which takes approximately 7 minutes to complete. Newer adult technology utilizes between 1-6 leads for EKG analysis. There has not been a study in pediatrics to assess the minimum number of leads required to attain diagnostic accuracy.

Objectives/Goal: To determine the appropriate number of leads needed to read pediatric EKGs safely and accurately.

Methods/Design: This is a single center systematic review that included randomly selected EKGs from our hospital database (2017-2020) read by two independent pediatric …

Increased Prevalence Of Parenteral Nutrition-Associated Liver Disease In Neonates With Congenital Heart Disease, Yasasvhinie Santharam

Research Days

Background: A life-sustaining therapy, use of total parenteral nutrition (TPN) allows for meeting nutritional needs of low birth weight, premature, or otherwise complex infants with conditions that prevent feeding through the GI tract. However, 40-60% of infants on long-term TPN have been shown to develop parenteral nutrition-associated liver disease (PNALD)1 , a serious condition characterized by cholestatic jaundice, steatosis, and hepatic fibrosis. Given these risks, providers must account for risk of hepatic injury when prescribing TPN, including attributes of gestational age, infection, surgical and diagnosis history2-6 . Yet with a multifactorial pathogenesis, significant heterogeneity in PNALD incidence still occurs. Looking …

Pharmacometabolomics Profiling Of Preterm Infants Validates Patterns Of Metabolism Associated With Response To Dexamethasone Treatment For Bronchopulmonary Dysplasia, Bradley C. Stockard

Research Days

Background: Bronchopulmonary dysplasia (BPD) is one of the most common health complications of premature birth. Corticosteroids are commonly used for treatment of BPD, but their use is challenging due to variability in treatment response.

Objectives/Goal: Previous pharmacometabolomics study has established patterns of metabolite levels with response to dexamethasone. We obtained additional patient samples for metabolomics analysis to find associations between the metabolome and dexamethasone response in a validation cohort.

Methods/Design: 14 infants provided 15 plasma and 12 urine samples. The measure of treatment response was the calculated change in respiratory severity score (deltaRSS) from pre-to-post treatment. Each metabolite was assessed …

Patient-Facing Antihemophilic Factor Clinical Decision Support Tool, Chandni Dargan

Research Days

Background: Hemophilia represents a medical condition suited to individualization. Adherence with prophylactic antihemophilic factor regimens ranges from 6%-167%. Clinical decision support tools (CDS) have been shown to improve guideline compliance and enhance patient adherence.

Objectives/Goal: The objective of this study is to evaluate the usability, usefulness, and satisfaction of a pharmacology-driven, patient-facing antihemophilic factor CDS.

Methods/Design: A patient-facing CDS was created to allow patients and families to explore factor levels at any time during their regimen and with the administration of different dosing regimens. Development followed a user-centered design process to ensure development of a product targeted to the needs …

Association Of Patient Demographics, Code Characteristics, And Survival To Discharge After In-Hospital Cardiac Arrests In The Pediatric Intensive Care Unit, Nathan Lavoy

Research Days

Background: Lower socioeconomic status and minority background have been linked to worse outcomes after out-ofhospital and in-hospital cardiac arrests (IHCA). Despite this, there has not been a focus on IHCA within a pediatric intensive care unit (PICU) evaluating patient demographics and code characteristics.

Objectives/Goal: The primary objective is to describe patient demographics, code characteristics, and survival to discharge of patients that suffer IHCA in a quaternary PICU. The goal of this descriptive, retrospective study is to identify possible differences in outcomes related to patient demographics, laying the foundation for further QI initiatives.

Methods/Design: Participants included patients between 24 hours and …

Maternal Antepartum Administration Of Lactoferrin Ameliorates Neonatal Infection By Bacteremia-Producing Escherichia Coli In Mice, Charity Dunlop

Research Days

Background: E. coli is a major cause of neonatal sepsis. Vaginal pathogenic E. coli strains ascend into the pregnant uterus infecting the offspring of colonized mothers. Lactoferrin (LF) is an antibacterial and immunomodulatory glycoprotein that has been given to preterm newborns to prevent lateonset sepsis. However, the effects of maternal LF administration to prevent neonatal E. coli invasive infection have not been studied.

Objectives/Goal: Our goal was to test the hypothesis that LF pre-treatment of pregnant mice administered prenatally will significantly reduce the bacterial load in their embryos following infection with E. coli.

Methods/Design: Human LF 100 mcg/mL or placebo …

Assessment Of Dental Care In Children With Congenital Heart Disease, Mollie Walton

Research Days

Background: The incidence of infective endocarditis (IE) is significantly higher in patients with congenital heart disease (CHD) compared to the general population. Studies have investigated the incidence of IE in adults with CHD to identify the factors that contribute to the development of IE. One such factor is suboptimal oral health. Several studies have shown that children with CHD have higher levels of untreated oral disease as compared to children without CHD.

Objectives/Goal: Identify factors that may affect compliance with routine dental care, including: age, sex, severity of CHD, repair/palliation, and qualification for prophylactic antibiotics.

Methods/Design: A retrospective chart review …

Prenatal Diagnosis Of Absent Aortic Valve With Continuous Mitral Regurgitation In Turner’S Syndrome: Too Many Things Gone Awry, Amulya Buddhavarapu

Research Days

Background: Congenitally absent aortic valve (AAoV) is a rare anomaly associated with high incidence of perinatal mortality.

Case: A female fetus of twin gestation underwent a fetal echocardiogram at 24 weeks gestation. Findings: rudimentary AoV leaflets with severe aortic insufficiency and aortic arch flow reversal, left ventricular (LV) non-compaction with severe dysfunction. Continuous moderate regurgitation across a dysplastic, functionally atretic mitral valve and borderline restrictive left to right flow through the foramen ovale (FO) was noted, setting for an inverse circular shunt: Aorta - LV- left to right atrium (FO) - right ventricle - pulmonary artery - ductus arteriosus - …

Neonatal Hypoxia Due To Pulmonary Artery Thrombus, Bianca Cherestal

Research Days

Background: Pulmonary artery thrombus is rare and should be considered in neonates with refractory hypoxia.

Case: Preterm female neonate had persistent hypoxia after birth despite intubation, high-frequency oscillatory ventilation (FiO2 100%), and inhaled nitric oxide. Delivery was via c-section at 33 weeks 4 days for new onset hydrops and poor biophysical profile. Echocardiogram revealed echo brightness in the lumen of the left pulmonary artery (LPA) with no color flow (figure 1a), concerning for occlusive thrombus. Chest CT angiogram confirmed the diagnosis (figure 1b, c). Hematology was consulted and recommended systemic thrombolysis (tissue plasminogen activator, tPA) with concomitant anticoagulation (Bivalirudin). Imaging …

Sigirr Variants Identified In Nec Infants Exaggerate Toll-Like Receptor Mediated Inflammation, Jennie Godwin

Research Days

Background: A key event underlying uncontrolled inflammation in necrotizing enterocolitis (NEC) is pathologic activation of Toll-like receptors (TLR). TLR4 recognizes lipopolysaccharide (LPS), from Gramnegative bacteria, and TLR2 peptidoglycans (PAM3Csyk4, PAM), from Gram-positive bacteria. TLR4 and TLR2 signaling events induce an inflammatory cascade through NF-kB, a cytokine inducing transcription factor. Single-immunoglobulin interleukin-1-related receptor (SIGIRR) is a major negative regulator of TLR-mediated NF-kB activation. Previous work from our lab suggests that loss of function in SIGIRR may predispose to NEC. Our lab has identified four variants in NEC patients predicted to alter function of SIGIRR. We hypothesized that identified SIGIRR variants will …

The Effects Of Necrotizing Enterocolitis And Short Chain Fatty Acids On The Developing Brain In Mice, Maribel Martinez, Venkatesh Sampath, Paula Nichols, Wei Yu, Suban Burale, Tianhua Lei

Research Days

Background: Necrotizing enterocolitis (NEC) is a devastating disease in premature infants characterized by acute intestinal inflammation and necrosis with a mortality of 15%-30%. Approximately 50% of infants that survive NEC will develop neurodevelopmental defects[1]. Studies on the effects of NEC on the neonatal brain are limited, however it’s postulated that neurodevelopmental damage is induced via cytokine activation of toll-like-receptor 4 (TLR4) by high mobility group box protein 1 (HMGB1) on the microglia Accumulation of reactive oxygen and demyelination contribute to neuronal damage. Therapies that limit the development of NEC can potentially also limit the neuroinflammatory changes seen in NEC[2]. We …

Identifying Predictive Factors For Patients Transferred From Floor To Picu Within 24 Hours Of Admission By A Pediatric Critical Care Transport Team, Gina Patel

Research Days

Background: Appropriate triage and disposition during transport can reduce the need for unplanned transfers to the pediatric intensive care unit (PICU) within 24 hours of admission. We sought to determine predictive factors of patients admitted to the inpatient ward that required PICU transfer within 24 hours following transport by Children’s Mercy Critical Care Transport (CMCCT) team. The pediatric early warning score (PEWS) is used to monitor changes in the level of acuity for inpatients at our hospital and has been utilized to measure acuity during transport. We hypothesized patients with respiratory illness and PEWS > 4 during interfacility transport are at …

Repeat Tracheal Aspirates In Pediatric Intensive Care Patients: Understanding Clinical Application, Edward Lyon

Research Days

Background: Tracheal aspirate cultures (TA) are regularly obtained in the pediatric intensive care unit (PICU) when clinical changes in intubated or tracheostomy dependent children occur. Positive TA results are often unable to distinguish infection from colonization. There is no data describing the frequency and impact of repeated TAs on patients in the PICU.

Goal: Our objectives were to describe the frequency of repeated TAs in PICU children and emergence of multidrug resistant organisms (MDRO), identify bacterial profile of TAs, and examine antibiotic prescribing patterns related to these cultures.

Methods: A retrospective chart review occurred on 15 patients in the PICU …

Iv Sotalol Use In Pediatric And Congenital Heart Patients: A Multicenter Registry Study., Lindsey Malloy-Walton, Nicholas H. Von Bergen, Seshadri Balaji, Peter S. Fischbach, Jason M. Garnreiter, S Yukiko Asaki, Jeffrey P. Moak, Luis A. Ochoa, Philip M. Chang, Hoang H. Nguyen, Akash R. Patel, Christa Kirk, Ashley K. Sherman, Jennifer N. Avari Silva, J Philip Saul

Manuscripts, Articles, Book Chapters and Other Papers

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age. In 79 patients (93%), IV sotalol was used to treat supraventricular tachycardia and 4 (5%) received it to treat ventricular arrhythmias. …

Iv Sotalol Use In Pediatric And Congenital Heart Patients: A Multicenter Registry Study, Lindsey E Malloy-Walton, Nicholas H Von Bergen, Seshadri Balaji, Peter S Fischbach, Jason M Garnreiter, S Yukiko Asaki, Jeffrey P Moak, Luis A Ochoa, Philip M Chang, Hoang H Nguyen, Akash R Patel, Christa Kirk, Ashley K Sherman, Jennifer N Avari Silva, J Philip Saul

Faculty, Staff and Student Publications

Background There is limited information regarding the clinical use and effectiveness of IV sotalol in pediatric patients and patients with congenital heart disease, including those with severe myocardial dysfunction. A multicenter registry study was designed to evaluate the safety, efficacy, and dosing of IV sotalol. Methods and Results A total of 85 patients (age 1 day-36 years) received IV sotalol, of whom 45 (53%) had additional congenital cardiac diagnoses and 4 (5%) were greater than 18 years of age. In 79 patients (93%), IV sotalol was used to treat supraventricular tachycardia and 4 (5%) received it to treat ventricular arrhythmias. …

Rates Of Physical Abuse Screening And Detection In Infants With Brief Resolved Unexplained Events (Brues), Angela Doswell

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Background: “Apparent Life-Threatening Events” (ALTEs) have been associated with child physical abuse (CPA). In 2016, “Brief Resolved Unexplained Event” (BRUE) and development of its clinical guidelines and risk-stratification replaced ALTE. It is unknown if there is a similar association between BRUEs and CPA.

Objectives: To determine the rate of CPA in infants presenting with a BRUE, examine differences between infants with/without CPA, and examine rates of diagnostic testing used to detect CPA.

Methods/Design: This study was part of the BRUE Research and Quality Improvement Network, composed of 15 academic and community hospitals. Subjects were infants …

A Mixed-Methods Examination Of Transgender Youth Desistence, Mirae J. Fornander

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Background: Access to gender-affirming medical care (GAMC) for transgender/gender diverse (TGD) individuals is one of the most pressing social justice issues of our time. Research has begun to identify the benefits of GAMC. However, little is known about the healthcare needs of TGD youth, especially those who discontinue GAMC. Available literature lacks clear and consistent terms to define TGD youth who stop care. Further, a significant criticism of current research is the assumption that discontinuing care is equivalent to detransitioning. There are no studies, to our knowledge, that differentiate between TGD youth who choose to …

Healthcare Utilization Of Victims Of Commercial Sexual Exploitation Compared To Non-Victims, Cree Kachelski

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Background: Approximately 17,000 US children are victims of Commercial Sexual Exploitation of Children (CSEC) annually. Nearly 75% of adolescent CSEC victims received medical care within 6 months prior to identification. Screening tools have been developed based on known characteristics of CSEC victims; however, little is known about how CSEC victims compare to non-CSEC adolescents in healthcare utilization. Thus, appropriate medical CSEC screening settings are unknown.

Objectives/Goal: We aimed to identify where and how often adolescents presented to medical care in the 12 months prior to CSEC identification. We hypothesized that compared to controls, CSEC positive …

Self-Reported Sexual Behavior In A Pediatric Gender Clinic Sample, Mirae J. Fornander

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Background: Previous studies of transgender/gender diverse (TGD) youth indicate differences in rates of sexual behaviors between TGD youth and their cisgender peers. However, few studies have reported the sexual behavior and preferences of TGD youth in a large clinic sample, and no studies, to our knowledge, have utilized questionnaires that do not assume the sex or gender of one’s sexual partner.

Objectives/Goal: The current study aims to explore the self-reported sexual behavior of TGD youth presenting for gender-affirming medical care.

Methods/Design: Retrospective chart review of 589 patients (ages 12-22, M= 15.92, SD= 1.54; 72.9% assigned …

Behavioral Parent Training For Adhd And Sleep: A Dream Pair, Whitney Rolling

Research Days

Background: Estimates for children with attention-deficit/hyperactivity disorder (ADHD) and comorbid sleep disorders range from 25-75% (4,5,10). Among childhood sleep disorders, behavioral insomnia is one of the most common, with the treatment of choice being behavioral parent training (BPT) (4). BPT therefore is a common treatment element for both sleep disturbance and ADHD and presents a unique opportunity for more holistic pediatric care.

Objectives/Goal: Our objective was to implement and evaluate a behavioral sleep intervention in an ADHD behavioral parent training (BPT) program. Goals of the intervention were to decrease behavioral sleep symptoms, increase sleep duration, and decrease ADHD impairment.

Methods/Design: …

Improving Adolescent Sexual And Mental Health Care Access Through Community Outreach, Anna Bitely

Research Days

Background: In the pandemic’s wake, marginalized adolescents face exacerbated barriers to accessing sexual and reproductive health (SRH) and mental health (MH) care. Telemedicine access for SRH/MH care has increased, but disparities remain.

Objectives/Goal: We assessed the impact of our intervention (AccessKCTeen) on care access.

Methods/Design: We trained four peer leaders to share SRH/MH information and mobilize their networks to attend AccessKCTeen outreach events. We partnered with community organizations to host events where we demonstrated telemedicine, shared resources for SRH/MH care, and discussed and distributed free health items (e.g. emergency contraception (EC), condoms, pregnancy tests, hand sanitizer). Teens completed surveys prior …

Cvst As A Rare Complication Of Acute Pediatric Covid-19, Erin Bolen

Research Days

Background: A previously healthy 13-year-old male was admitted to the hospital for a bacterial superinfection in the setting of recent COVID-19, including orbital cellulitis and pansinusitis with subgaleal, epidural, and subdural empyema. Further workup identified an occult, non-occlusive cerebral venous sinus thrombosis (CVST) along the length of the superior sagittal sinus.

Objectives/Goal: To evaluate the reported cases of CVST as a potential complication of COVID-19 in the pediatric population and identify meaningful trends in demographics, presentation, and diagnostic course.

Methods/Design: A literature search was run with various combinations of the following terms, always including “COVID”: “pediatric,” “adolescent,” “CVST,” “cerebral venous …

Evaluation Of A Hirschsprung Disease Systemwide Enterocolitis De-Escalation Strategy, Albert Aickareth

Research Days

Problem Statement/Question: Components of systems that identify, treat and prevent enterocolitis are not well described. In addition, for patients with HSCR both pre and post pull-through, questions regarding a child’s clinical status occur through several different medical access points and may occur outside of colorectal center clinic hours.

Background/Project Intent (Aim Statement): Hirschsprung associated enterocolitis (HAEC) is a severe and potentially life-threatening complication of Hirschsprung Disease (HSCR) that can present with a variety of symptoms from mild abdominal pain to fulminant colitis. We evaluated our system-wide enterocolitis de-escalation strategy for both hospital staff and patient caregivers. Our primary analysis was …

Impact Of A Mental Health Screening Process In A Pediatric Ed, Bryan Stocker Do, Shobhit Jain Md, Kathryn Worland Msw, Celeste Tarantino Md, Lina Patel Md, Shayla Sullivant Md

Research Days

Background: Up to 20% of children in the United States experience a mental health disorder and suicide is the second leading cause of death among adolescents. Children presenting with mental health concerns to the Emergency Department (ED) can be challenging due to lack of access to trained professionals and long lengths of stay. Specialized teams have shown to be beneficial, but little is known about their impact in the ED.

Objectives/Goal: This study explored the impact of specialized social workers partnering with emergency medicine providers to provide care during an acute mental health crisis.

Methods/Design: We performed this work at …

X-Linked Nephrogenic Syndrome Of Inappropriate Antidiuresis Secondary To Vasopressin Receptor 2 Mutation, Erica Zarse

Research Days

Background: X-linked nephrogenic syndrome of inappropriate antidiuresis (NSIAD) is a rare cause of hyponatremia with a similar biochemical evaluation to the syndrome of inappropriate antidiuretic hormone secretion but with suppressed vasopressin (AVP) levels.

Objectives: We present a case of hyponatremia due to NSIAD.

Results: A 25-month-old male with developmental delay became unresponsive 30 minutes after falling off a couch onto a hardwood floor. He presented with tonic-clonic movements and eye deviation requiring antiepileptics. Serum laboratories noted hyponatremia (123 mmol/L), hypoosmolality (262 mOsm/kg) and hypochloremia (90 mmol/L) prompting endocrine consult. Head imaging normal. Urine dilute with specific gravity 1.004, osmolality 160 …