Medicine and Health Sciences Commons^™

Something's Gnawing At Me, Catharine Kral, Brian Harvey

Posters

17-year-old female presented to Sports Medicine clinic with a chief complaint of bilateral hip pain and difficulty walking who was found to have bilateral ovarian teratoma.

Single Center Retrospective Comparison Of Bivalirudin And Heparin For Therapeutic Anticoagulation In Pediatric Patients, Clayton Habiger, Shannon L. Carpenter

Posters

Background: The pharmacological choice for continuous anticoagulation therapy in pediatric patients has classically been unfractionated heparin. However, with heparin there are risks of treatment resistance in pediatrics given differences in developmental hemostasis, and contemporary medications such as bivalirudin offer a valuable alternative. Bivalirudin binds to thrombin and has shown encouraging results compared to heparin in pediatric patients receiving ECMO, but no comparison has been made in patients receiving therapeutic anticoagulation. Three years ago, Children’s Mercy Pediatric Hematology department encouraged bivalirudin use over heparin use for therapeutic anticoagulation requiring a continuous infusion. Objectives: Compare clinical and laboratory outcomes in patients receiving …

Increasing Palliative Care Team Involvement In Pediatric Hematopoietic Stem Cell Transplant Patients, Meagan Vacek, Lisa Tarbell, Melissa List, Erin Hall, Jennifer Linebarger, Kelstan Ellis, Gina Jones, Joel Thompson

Posters

Background: Palliative care facilitates communication, helps with physical and psychological symptom management, and assists in goals of care and advance care planning discussions.1 Multiple organizations, including the American Academy of Pediatrics and American Society of Clinical Oncology encourage palliative care engagement and their involvement with hematopoietic stem cell transplant (HSCT) patients has been shown to be beneficial as HSCT is associated with a high degree of morbidity and possible mortality.2-3 Objective: To increase the number of PaCT consults for patients receiving HSCT for our targeted diagnoses from 48% to 75% by December 2023. Methods: Chart review was conducted for patients …

Early Cholestasis And Vitamin K Deficiency Secondary To Biliary Atresia Presenting As Coagulopathy And Thymic Hemorrhage, Abbey Elsbernd, Lauren Amos Md

Posters

Background Bleeding and progressive coagulopathy are established symptoms of early cholestasis, including extrahepatic biliary atresia. We present a patient with bleeding secondary to vitamin K deficiency and cholestasis-induced fat malabsorption with unusual presentation of mediastinal mass. Objective Describe a case of late-onset vitamin K deficiency and cholestasis ¬¬presenting as jaundice and coagulopathy with associated bleeding into thymic structures Design/Method Case report Results A 4-week-old term male infant was brought to the ED with fussiness and bruising. Prior to presentation, he was afebrile and followed regularly with his primary care provider for jaundice below phototherapy thresholds. Infant received vitamin K at …

Diagnosis Of Mosaic Rasopathy In A Child With Rhabdomyosarcoma, Meagan Vacek, Paige Johnson, Midhat S. Farooqi, Kristi M. Canty, Dihong Zhou, Brendan Lanpher, Wendy Allen-Rhoades, Erin M. Guest

Posters

Background: Phacomatosis pigmentokeratotica (PPK) is a subtype of epidermal nevus syndrome characterized by the co-existence of a sebaceous nevus and a speckled lentiginous nevus and described in approximately 30 cases in literature. PPK is now recognized as a mosaic RASopathy due a postzygotic mutation in the Ras-Raf-MEK-ERK pathway. RAS variants are also known to contribute to tumorigenesis, in some pediatric cancers, including rhabdomyosarcoma. Objective: Describe the presentation and evaluation of a child with pelvic rhabdomyosarcoma and evolving skin lesions found to have a rare mosaic-RASopathy. Design/Method: Case Report Results: The patient is a former 32-week premature female who presented to …

An Increased Failure Rate Of Asparaginase Desensitization With Calaspargase Pegol, Jillian Fry, Erin M. Guest, Keith J. August

Posters

Background: In late 2022, pegaspargase (SS-PEG) became unavailable to patients younger than 22 years in the United States, leaving calaspargase pegol (SC-PEG) as the only available long-acting asparaginase formulation. SC-PEG has been compared to SS-PEG in two randomized, pediatric clinical trials and found to have similar rates of adverse events with comparable rates of event-free survival. Our institution uses a desensitization protocol for patients who experience a hypersensitivity reaction to asparaginase. Here we review our experience with desensitization since the transition to SC-PEG from SS-PEG. Objectives: To compare the rate of hypersensitivity reactions, success of desensitization protocols, and need for …

Anesthetic And Multidisciplinary Management Of A 2-Staged Liver Transplant​, Kasey Brooks, Lisa Conley, Brandon Layton

Posters

We present a case of a 2 staged liver transplant on a 12-month-old 8kg male with newly diagnosed mitochondrial depletion syndrome causing acute liver failure, refractory lactic acidosis, and cardiopulmonary collapse requiring ECMO. The patient’s past medical history at the time of presentation included history of prematurity at 32 weeks, developmental delay, and a brother with an undiagnosed pancreatic disorder. The patient was brought in by parents to the emergency department with a cough, minimal PO intake for 5 days, and increased fatigue who was found to be URI positive. Initial labs were concerning for anemia, elevated bilirubin, elevated AST/ALT, …

Anterior Hip Pain In Adolescent Female Wrestler, Melanie Kennedy

Posters

No abstract provided.

Transition Skills Lab: A Telehealth Intervention For Improving Healthcare Transition In Youth With Special Health Care Needs, Angela Combs Ma, Alaina Linafelter, Jordan Sevart, Michele H. Maddux

Posters

Introduction: Few interventions exist to provide hands-on practice of key health management tasks to youth with special health care needs (YSHCN). The Transition Skills Lab was adapted from Jerlym Porter’s work and piloted as a virtual intervention in YSHCN. Methods: Sixteen young adults participated in the virtual Transition Skills Lab, during which they practiced three key patient competencies pertaining to health care tasks: reading an insurance card, reading a pill bottle label, and scheduling an initial visit with an adult doctor. Participants were then asked to rate their confidence on skills taught within the intervention on a four-point scale from …

Telehealth Implementation Of A Young Adult Ibd Clinic: Uptake, Benefits To Patient Care, And Challenges, Angela Combs Ma, Alaina Linafelter, Jordan Sevart, Michele H. Maddux

Posters

Introduction: Since the COVID-19 pandemic, pediatric psychologists have been at the forefront of telemedicine expansion to ensure access for patients and families to needed mental health services. This has required adaptations across the care continuum – from scheduling to service delivery to documentation. This poster aims to summarize the telehealth (TH) application of a Young Adult IBD Clinic (YAC-IBD) that provides transition preparation to youth with Inflammatory Bowel Disease (IBD). Methods: In April 2020, the YAC-IBD was converted from in-person visits to a virtual visit format. Since then, 119 youth ages 17-21 (inclusive) have been seen in this clinic. This …

Double Aortic Arch With Atresia Of The Left Aortic Arch Proximal To The Left Common Carotid Artery In A Patient With Phace Syndrome – A Management Conundrum, Mohamed Aashiq Abdul Ghayum, Anmol Goyal, Aliessa P. Barnes, Sanket Shah

Posters

Background Cardiac anomalies in PHACE syndrome (P: posterior fossa anomalies, H: hemangiomas, A: arterial lesions, C: cardiac abnormalities, E: abnormalities of the eye) often consists of complex aortic arch anomalies with double aortic arch (DAA) being an uncommon association. Atresia of the proximal segment of the left arch in DAA is extremely rare and can pose a diagnostic and management conundrum due to formation of a loose vascular ring and asymptomatic nature as seen in our case. Case Description A 5-year-old male, diagnosed with PHACE syndrome, at a different center was noted to have an incidental finding on head imaging …

Transposition Of The Great Arteries With Intramural Left Main Coronary Artery: Salient Imaging Findings And Description Of Two Operative Techniques, Joshua Holbert, Manasa Gadiraju, Sanket Shah, Edo Bedzra

Posters

Background: Transposition of the great arteries (TGA) is a common cyanotic critical congenital heart disease, with a feasible prenatal diagnosis. Arterial switch operation (ASO) with/without VSD closure is the preferred surgical approach with added challenge when an intramural coronary artery (IMC) is present. ASO is technically quite demanding with IMC, and incidence of post-operative complications and mortality are potentially higher. We present our recent TGA-IMC experience focusing on salient features identified on echocardiography, CT and invasive angiograms, as well as variations in ASO surgical techniques for repair. Methods: Our echo imaging acquisition required high frequency transducers and shallow image depth …

A Mssic Study Reveals Non- Modifiable Risk Factors Were Associated With Post-Operative Hematoma Following Acdf, Richard Easton, Matthew Lipphardt, Matthew Easton, Nicholas Papakonstantinou, Lonni Schultz, Hsueh-Han Yeh, Cecile Pestano

Posters

Introduction

Post-operative hematoma (POH) following anterior cervical discectomy (ACDF) is uncommon . Although rare, POH is recognized as a potentially catastrophic post-operative complication . There is limited information as to who is vulnerable . From a large database, the authors sought to identify common modifiable and non-modifiable risk factors for developing POH and its associated clinical outcomes.

Materials and Methods

The Michigan Spine Surgery Improvement Collaborative (MSSIC) is a statewide quality improvement initiative . ACDF cases in MSSIC registry from 2014-2018 were identified . Patients who developed POH (POH) were compared to those who did not (non-POH) . Chi-square and …

Bowel Cleanout Prior To Intrathecal Baclofen Pump Implantation: Effects On Acute Post- Operative Gastrointestinal Complications, Amanda Lindenberg, Brittni Walton, Sathya Vadivelu, Matthew J. Mclaughlin

Posters

Background: Intrathecal baclofen (ITB) pumps have been used for management of spasticity and dystonia There is limited data on immediate post-operative complications such as GI adverse events after surgery for ITB pump related procedures. This study aimed to compare patients who underwent a pre-operative bowel cleanout to those who did not and the relationship with post-operative nausea, vomiting and constipation requiring an escalation of their bowel regimen. Design: Retrospective cohort study. Setting: Pediatric acute care hospital. Participants: All patients who underwent new ITB pump and/or catheter placement from January 2015-December 2022. Interventions: Preoperative bowel clean out. Main Outcome Measure: Quantity …

Ada Deficiency: Improvement In Immune Function After Enzyme Replacement Therapy, Esosa Adah, Nikita Raje

Posters

Introduction: Adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID) is characterized by severe lymphocytopenia affecting T-, and B-,lymphocytes, with non-immunological manifestations including neurodevelopmental deficits, sensorineural deafness, and skeletal abnormalities. Treatment modalities include enzyme replacement therapy(ERT) and/or autologous gene therapy or hematopoietic stem cell treatment. We report a 9-month-old female with ADA deficient T-B-NK+ SCID treated with ERT while awaiting stem cell treatment. Case Description: A 9-month-old female with a history of failure to thrive, hypotonia and loss of developmental milestones had genetic testing done that showed variants ADA c.911 T>G, c.302G>A. She was admitted for further work up. Lymphocyte …

A Case Report Of A 48-Year-Old Female With Cauda Equina Syndrome In The Setting Of Disseminated Mrsa Infection, Aimee Lambeth, Sarah Eickmeyer, Stephanie Fortune

Posters

To present an interesting case of cauda equina syndrome due to disseminated MRSA, where inpatient EMG was useful in diagnosis and treatment planning.

Investigating The Association Between Mast Cell Activation Syndrome And Irritable Bowel Syndrome, Ishaan Jakhar, Maggie Urschler, Medha Singh, Suman Sahil, Hassan Ahmad

Posters

Investigating the Association between Mast Cell Activation Syndrome and Irritable Bowel Syndrome Introduction Mast Cell Activation Syndrome (MCAS) involves the inappropriate release of chemical mediators resulting in varied episodic symptoms. Irritable Bowel Syndrome (IBS) is a common functional abdominal disorder where mucosal immune alterations play a key role. Since mast cells function as gatekeepers for the intestinal interface, we examined what proportion of patients diagnosed with MCAS also had IBS along with comorbid risk factors. Methods All patients in our study were 18 years of age or older. We used Cerner Healthfacts to gather our cohorts of patients with MCAS, …

Increasing Annual Eye Exam Completion For Children With Cystic Fibrosis Receiving Cftr Modulators, Paula Capel, Claire Elson, Stephanie Duehlmeyer, Jessica Haynes, Amanda Conway, Christopher M. Oermann

Posters

Background: CFTR modulators are life-changing therapy for people with CF. During preclinical studies of ivacaftor, cataracts were identified in juvenile rat pups. Subsequently, noncongenital cataracts have been reported in children receiving CFTR modulator therapy [1], so baseline and annual slit lamp eye examinations for cataract development are recommended for children with CF (aged <18). At Children’s Mercy Kansas City (CMKC), our standard practice is to conduct baseline and annual ophthalmologic examinations to assess for cataracts in children with CF prescribed CFTR modulator therapy, but there was no formal process for tracking adherence to this standard and ensuring that examinations were completed. A quality improvement project was undertaken in May 2022 to determine the rate of completion of annual eye exams and increase adherence to our annual monitoring standard.

Methods: To improve adherence to annual monitoring standards, a quality improvement taskforce including CF care team nurses and pharmacists was developed. A database was used to document baseline and follow-up eye examinations. A standardized communication process was developed and deployed that included an eye exam brochure (Figure …

Evaluating Patients With Recurrent Fever And Elevated Igd, Thao Le, Nikita Raje

Posters

Introduction: Hyperimmunoglobulinemia D with periodic fever syndrome (HIDS) often presents within the first year of life. Symptoms include fevers, chills, headache, lymphadenopathy, and abdominal pain. Patients may be misdiagnosed as other autoinflammatory syndromes, resulting in delay of treatment. Case: A 16-year-old female presented for evaluation of recurrent fever. Her fevers started at two months of age, occurred one to two times a month, and were associated with headache, abdominal pain, and fatigue . At 18-months, she was diagnosed with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA). She was treated with prednisone and an adenotonsillectomy but her fevers persisted. . At …

Evaluating A Newborn Infant With Pancytopenia For Whim Syndrome, Shayan Sazdar, Thao Le, Nikita Raje

Posters

Introduction Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare immunodeficiency caused by a gain-of-function mutation in CXCR4. A complication of WHIM syndrome is severe neutropenia, which can result in fatal bacterial infections. Given the rarity of the disease and variability of clinical presentations, WHIM syndrome can be difficult to diagnose and manage. Case Description A full term male infant was hospitalized for hypoxic ischemic encephalopathy after delivery. Physical exam was unremarkable. During his hospitalization, he was found to have progressive pancytopenia. Initial lymphocyte subsets showed a significant T cell lymphopenia of 1325 mm3 CD3+, 920 mm3 CD4+, 390 …

Privacy Preserving Self-Service Degauss Based Geomarker Portal, Harpreet Gill, Kushal Vallambhatla, Mark A. Hoffman, Kevin Power, Catherine Jackson

Posters

DeGAUSS offers a privacy-preserving capability to annotate a list of addresses with geomarker information that is useful for social determinants and other research. The standard delivery of DeGAUSS requires command line proficiency, potentially limiting the group of users. We provide a self-service portal that enables researchers to load a file and receive a downloadable annotated output file. In our pilot phase, the system has processed 481 calls for more than 45,000 addresses at Children’s Mercy.

National Prescribing Practices For Dystonia Among Providers In The United States, Sarah Paige Davis, Natalie J. Kane, Haley Botteron, Rose N. Gelineau-Morel

Posters

Objective: While multiple oral medications are used to treat dystonia, limited information exists on current prescribing practices. This study analyzes prescribing practices for dystonia in the United States, evaluating variations in dosing and impact of co-morbidities. Methods: Querying the Cerner Real World database from 2014 to 2019 for children age 0-18 with an ICD-10 diagnosis containing “dystonia” resulted in 11,300 inpatient and outpatient encounters. Information extracted included current dystonia medications (baclofen, clonidine, carbidopa-levodopa, gabapentin, tetrabenazine, trihexyphenidyl, and benzodiazepines including diazepam, clonazepam, midazolam, and lorazepam), medication dosing, and co-morbid diagnoses of cerebral palsy, epilepsy, or spasticity. Encounters without current weight were …

Impact Of Fetal Health Center On Mode Of Neonatal Death, Erin Bolen, Ashley Sherman, Julie Weiner, Jotishna Sharma

Posters

Introduction There has been significant innovation and technological advancement in prenatal diagnosis, fetal therapeutic intervention, and establishment of fetal health centers (FHC) at children’s hospitals with dedicated delivery services for high-risk neonates and expedited access to level IV neonatal intensive care units (NICU). Morality trends have been examined on individual bases and within diagnostic groups, but there have been few studies that examine overall modes of mortality in the NICU population across diagnoses after the implementation of a dedicated FHC. This study aims to compare modes of death at a level IV children’s hospital NICU between infants born at the …

Sangrado Del Tubo Digestivo Alto Secundario A Infección De Adenovirus, Moises Alatorre-Jimenez, Sarah T. Edwards, Thomas M. Attard

Posters

Titulo: Sangrado del Tubo Digestivo Alto Secundario a Infección de Adenovirus Introducción: El Sangrado del tubo digestivo alto (STDA) es causado por múltiples etiologías incluyendo gastritis, varices, esofagitis, anomalidades vasculares, cuerpos extraños, y ulceras (1). Dentro de estas causas se conoce que infecciones virales pueden producir ulceras causando sangrado los más comunes son el virus de Epstein- Barr, Covid-19, Herpesvirus-6, Herpesvirus-7 y Citomegalovirus (1,2,3,4,5). Presentamos el caso de un paciente con STDA severo a causa de infección por adenovirus. Reporte de Caso: Paciente de 22 meses de edad, previamente sano que se presenta al servicio de Urgencias debido a un …

Factors Associated With Respiratory Pathogen Panel Utilization In Children Hospitalized With Acute Respiratory Illness — New Vaccine Surveillance Network, Kansas City, 2017–2021, Edward Lyon, Brian R. Lee, Benjamin Clopper, Heidi L. Moline, Rangaraj Selvarangan, Jennifer Schuster

Posters

Background: Respiratory pathogen panels (RPP) are multiplex PCR platforms that detect several respiratory viruses from one specimen. For most children hospitalized with acute respiratory illness (ARI), management is supportive, and detection of a specific virus from RPP does not impact clinical care. Therefore, clinical RPP use is not standardized, and ordering is at the discretion of the clinician. We sought to understand factors associated with RPP utilization among pediatric patients hospitalized with ARI. Methods: From October 2017 to September 2021, participants < 18 years hospitalized with ARI were enrolled at a single site in the New Vaccine Surveillance Network (NVSN). Eligible patients were residents of Jackson County, MO, had one or more ARI symptoms (e.g., cough, fever, nasal congestion) lasting < 14 days, and were enrolled within 48 hours of admission. Parent interviews and medical chart reviews were conducted. All participants had a research RPP, but results were not available to the clinical providers. Clinical providers were able to order a clinical RPP (cRPP), for which they received test results. Characteristics of NVSN enrollees hospitalized with ARI with and without a cRPP are described. Lastly, medical complexity was assessed via the pediatric complex chronic conditions classification system (CCC) then analyzed via chi- square test between groups. Findings: During the study period, 1,038 participants were enrolled, and 555 (53.4%) received a cRPP. Most, 299 (53%), cRPPs were ordered in the emergency department or urgent care before admission. Age was a significant factor associated with cRPP use (Table 1). cRPP participants were more likely to have complex chronic conditions, and/or technology dependence. No difference in cRPP use was observed by race/ethnicity, payer status, or sex. More participants were enrolled in 2020-2021, but the overall usage of cRPP is similar between years (Table 2). Conclusions: In this large cohort of children hospitalized with ARI, medical complexity, technological dependence, and age < 2 months were associated with increased utilization of cRPPs. Understanding the impact of cRPP on clinical care requires further investigation to better understand the utility of these tests.

Latency Time From Prior Authorization Submission To Initiation Of Biologic Therapy In Inflammatory Bowel Disease, Shannon Capraro, Janelle R. Noel-Macdonnell Phd, Lauren Disselhoff, Layne Sieve, Rachel Chevalier

Posters

Background For pediatric patients with a diagnosis of inflammatory bowel disease (IBD), initiation of biologic therapy can be essential to attain disease control. Unfortunately, patients often experience delays in receiving the first dose of these medications due to the need for insurance companies to approve medication prior authorizations (PA). Physicians and support staff spend many working hours collating and transferring information regarding urgency to payors. To our knowledge, no studies exist to evaluate the factors that contribute to how quickly a medication is approved in pediatric patients. We set out here to evaluate which demographic and disease factors contribute to …

The Implementation Of A Pectus Bar Insertion Eras Pathway, Todd A. Glenski, Emily Weisberg, Christian M. Taylor, Brandon Layton, Andrea Melanson, Sameer Lalani

Posters

No abstract provided.

Candida Chorioretinitis In Immunocompromised Patient With Candida Tropicalis Fungemia Secondary To Chemotherapy, Jourdan Valkner Krause

Posters

Summary: An immunocompromised patient with candida tropicalis fungemia secondary to chemotherapy presented for follow up of candida chorioretinitis. This infection puts the patient at high risk for CNVM formation and must be followed closely. Case History: • 3-year-old Hispanic female • CC: Candida chorioretinitis follow up • POH: Bilateral orbital chloromas and optic nerve atrophy • PMH: Acute myeloid leukemia, Candida tropicalis fungemia • Meds: o Amphotericin B liposomal 85 mg 42.5 mL, IV, q24hr o Flucytosine 500 mg Capsule *NF* 1,000 mg 2 capsule, PO/NG, q6hr o Micafungin 100 mg 100 mL, IV, q24hr *Plus numerous chemotherapy, pain and …

Teaching And Tomorrow: Children's Mercy Hospital Pediatric Gastroenterology Fellowship Program, Children's Mercy Kansas City

Posters

No abstract provided.

Oscillometry In Term Neonates Without Respiratory Disease, Anna Nelson, Cheri Gauldin, Brooke Smith, Venkatesh Sampath, Winston M. Manimtim

Posters

Background Bronchopulmonary dysplasia (BPD) is one of the most common complications of prematurity. While severity of BPD is defined by need and type of respiratory support at 36 weeks, it does not distinguish between endotypes of disease. Recent studies suggest that infants with severe BPD have parenchymal, airway and pulmonary vascular disease. Determining the BPD phenotype could help guide future individualized therapy. Oscillometry is a bedside tool that measures the resistance of the airways and the reactance of the pulmonary parenchyma. Normative values of oscillometry need to be established to serve as a comparison prior to establishing oscillometry characteristics of …