Medicine and Health Sciences Commons^™

High-Cost Drug Formulary Stewardship: Health System Evaluation Of Intravenous Immunoglobulin (Ivig) Utilization, Christine J. Hurteau Pharmd, Christopher Flores Pharmd, Pcscp, Rebecca Pulk Pharmd, Ms, Bsp

Posters

• Intravenous immunoglobulin (IVIG) therapy is essential for treating various immune mediated conditions
• Dosing and duration of IVIG varies based on indication, and there is lack of consensus for some indications on ideal dosing strategy
• IVIG is a high cost medication that is being used at higher rates in a five hospital health system compared to similarly sized institutions
• Given the high utilization rates, evaluation of IVIG prescribing patterns and clinical appropriateness was

A Puzzling Case: Complete Regression Of Pituitary Macroadenoma, Nebyat Mazeniga Md, Deepak R. Vangipuram, Sachin Majumdar

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We present a case of pituitary macroadenoma regression following childbirth and two months of cabergoline treatment.

Importance Of Tropical Medicine, Chia-Chi Angela Fu, A Clinton White, Miguel Cabada, Ryan Northup

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Overviews the importance of tropical medicine to society and global health.

Safe Sleep Education For Local Refugee And Immigrant Populations, Sara Jones

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Provide Safe Sleep education to local refugee and immigrant populations through their resettlement agency Jewish Vocational Services (JVS) with resources available in their first language.

Himalayan Health Exchange: Kargiakh Medical Expedition, Rachel Oldfather

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Medical expedition to remote Himalayan villages involving high-altitude treks, improvised clinics, and immersion in the local Buddhist culture.

Global Health Elective - Philippines, Hannah Ho

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Provides an overview of experiences working in the Philippines as a pediatric resident.

Expanding The Winter Warmth, Sarah Gwazdacz

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Reviews the efforts to expand collection of winter apparel for refugee families in Kansas City.

Healthcare In Under-Resourced Areas, Gabrielle Fleming, Ryan Northup, Bill Houseworth, Lori Housworth

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Describes pediatric resident's experience working in the inpatient unit, ER, PICU, and NICU in Cambodia.

Evaluation Of Current Monitoring Practices For Patients Receiving Infliximab, Yujin Hong Pharmd, Michael Schaedler Pharmd, Bcps, Bcop, Vincent Do Pharmd, Bcps, Bctxp

Posters

• Infliximab is used for a wide range of inflammatory conditions including Crohn’s disease, ulcerative colitis, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, and plaque psoriasis.
• Patients should be closely monitored for development of signs and symptoms of active tuberculosis and latent infection, hepatitis B virus, and human immunodeficiency virus (HIV) during treatment with infliximab following termination of therapy.
• Hematologic abnormalities have also been reported in patients receiving infliximab and caution should be exercised in patients being treated with infliximab who have ongoing or a history of significant hematologic abnormalities.
• Patients with symptoms of signs of liver dysfunction should be evaluated for …

Evaluation Of Compliance To Brivaracetam Restrictions Within A Large Multicenter Health System, Meghan Mcnulty Pharmd, Mahmoud Amir Pharmd, Fccm, Bcps, Bcccp, Paul Ahearn, Michele Moura Pharmd, Bcps, Bcccp

Posters

This evaluation aims to assess whether the health-system is adhering to brivaracetam prescribing restrictions and determine if there are any opportunities for improvement to the current ordering workflow.

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 …

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

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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 …

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 …

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

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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.

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

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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 …

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

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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, …

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

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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 …

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

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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 …

Understanding Macrophage Phagocytosis In Pediatric Leukemia, Molly Leyda, Jacqelyn Nemechek, John Szarejko, Fang Tao, Tykeem Manor, Douglas Myers, John M. Perry

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Macrophages are a diverse and widespread type of innate immune cell which play an important role in homeostasis and defense. In a process called phagocytosis, macrophages engulf dying cells, foreign substances, and pathogens. Importantly, macrophages can present antigens from phagocytosed cells allowing them to initiate an adaptive immune response against remaining cells of the same type. Despite the immunosuppressive nature of the tumor microenvironment, macrophages often infiltrate tumors where they can either promote or inhibit cancer development, though the specific conditions influencing their pro- vs anti-cancer activity remain enigmatic. Recently, macrophages have been brought into the spotlight of immunotherapy research …

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

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

Multicentric Reticulohistiocytosis: A Case Report With Molecular Analysis And Discussion, Casey Schukow, Katherine Burke, Arivarasan Karunmurthy, Jonhan Ho

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Multicentric reticulohistiocytosis (MRH) is a rare non-Langerhans cell histiocytosis that affects multiple organ systems, including the skin and joints. It commonly occurs in adult females and is diagnosed based on clinical presentation with associated radiologic and histologic findings. Histologic features include an infiltrate composed of multinucleated histiocytes and giant cells with a characteristic “ground glass” eosinophilic cytoplasm. Although the exact prevalence is unknown, current literature estimates that anywhere from 300 to 400 cases have been documented since being first reported in 1937. Here we present a case of MRH in a 45-year-old female who presented with eruptive red papules on …

Why Is It Blue And Squamous? A Report Of Cylindrocarcinoma With Squamous Differentiation, Casey Schukow, Timothy H. Mccalmont, Hongyu Henry Yang

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Cylindrocarcinoma is an uncommon adnexal carcinoma first described by Wieldman in 1929. It does not harbor distinctive clinical features and can present as a manifestation of Brooke-Spiegler syndrome. Histopathologically, cylindrocarcinoma shares some features in common with its benign analogue (cylindroma) but is recognizable on the basis of malignant features such as nuclear crowding, necrosis, and an elevated mitotic index. Historically, the immunophenotype associated with cylindrocarcinoma has been nonspecific, but SOX10 expression can now be used as a tool to distinguish the spiradenocylindrocarcoma spectrum (and also transformed adenoid cystic carcinoma) from other basaloid carcinomas. Squamous differentiation has been rarely documented within …

A Novel Case Report Of Balloon Cell Melanoma With Metastasis To The Ovary, Brain And Lymph Node, Casey Schukow, Antonina V. Kalmykova, Phillip Mckee

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Balloon cell melanoma (BCM) is a rare histologic variant (< 1%) of malignant melanoma characterized by large, foamy melanocytes with cytoplasmic vacuoles and pronounced nucleoli (“balloon cells”). Clinical presentation often varies and primary cutaneous lesions may be unknown in up to 47% of cases. Metastatic BCM tumors tend to display prominent balloon cell morphology, and cases have been reported involving organs of the gastrointestinal, urinary, ocular, and central nervous system tracts. However, ovarian involvement has not been well-reported. We present a novel biopsy-confirmed BCM case detected in a patient’s ovary with a primary cutaneous lesion previously found on the patient’s back in 2018. The ovarian lesion was initially thought to be a cyst, however the histology of the lesion was remarkable for metastatic balloon cells positive for melanocytic markers on immunostaining, including Melan-A, HMB45, SOX10, and tyrosinase. Brain and lymph node involvement was also detected, but unfortunately the patient was lost to follow-up due to wartime conflict and the remainder of the patient’s history is unknown. After conducting a thorough review of literature published on PubMed, the authors did not recognize previously reported cases of malignant BCM to the ovary to date. In this report, we recognize the importance of proper clinicohistologic and immunostain correlation when differentiating clear cell or xanthomatous lesions while briefly discussing how to differentiate these entities from BCM.

Calcified Chondroid Mesenchymal Neoplasm With An Fn1-Fgfr2 Translocation, Intralesional Fat, Diffuse Cd34 Positivity, And A Lack Of Giant Cells: A Case Report With Uncommon Findings, Casey Schukow, Christopher K. Hysell

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Calcified chondroid mesenchymal neoplasm (CCMN) is a term for an emerging group of soft tissue tumors that display cartilaginous matrix formation and harbor translocations involving the FN1 gene. These tumors are rare and behave indolently but can be mistaken for malignant neoplasms. Current literature indicates that these tumors frequently contain giant cells, and infrequently contain intralesional fat or stain for CD34. Henceforth, we present a case of CCMN with uncommon findings causing a soft tissue finger mass in a 74-year-old male with some erosion into the underlying bone. Microscopically, the mass was composed of a multinodular chondroid neoplasm with areas …

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

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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

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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.

Dexamethasone For Bpd While On Non-Invasive Positive Pressure Ventilation, Priya Tiwari, Jansynn Radford, Michael Norberg, Alain Cuna

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Background: Postnatal dexamethasone (DEX) has traditionally been reserved for preterm infants on prolonged mechanical ventilation, with reported rates of successful extubation at 47% to 76%. Recent emphasis on avoiding mechanical ventilation has resulted in preterm infants being increasingly supported with chronic non-invasive ventilation (NIV). The use of DEX for weaning preterm infants on chronic NIV remains largely unexplored. Objective: To determine efficacy of postnatal DEX in weaning infants on chronic NIV, and to identify factors and outcomes associated with successful weaning. Methods: Single-center retrospective study (2010-2022) of preterm infants ≤32 weeks gestation treated with postnatal DEX while on chronic NIV. …

Rumination Syndrome Diagnosis Supported By Colon Manometry In A Pediatric Patient, Alex Biller, John Rosen, Amornluck Krasaelap

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Introduction: Rumination syndrome is a disorder of gut-brain interaction characterized by effortless repetitive postprandial regurgitation, reswallowing, and/or spitting.(1) This condition is caused by increased intragastric pressure due to abdominal muscle contractions, leading to the opening of the lower esophageal sphincter and the return of gastric content into the esophagus.(1) Rumination is a clinical diagnosis after the exclusion of other etiologies of symptoms. The diagnosis of rumination is clinical, but supportive evidence can be obtained through esophageal or antroduodenal manometry.(2) We present a unique case report of rumination syndrome diagnosis supported by data obtained during colon manometry in a pediatric patient. …

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

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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 …

Increasing The Rate Of Infants Rooming In With Their Mothers With Low Acuity Congenital Heart Disease, Anna Nelson, Amy Marks, Ekta Patel

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Background The Children’s Mercy Fetal Health Center (FHC) was created to be able to deliver infants with complex fetal diagnoses including congenital heart disease. Mothers are referred from all over the Midwest region for their babies to have access to specialized care immediately after birth. Nearly all babies born in the FHC are admitted to our neonatal intensive care unit (NICU), as there are not well-established guidelines for keeping babies with their mothers if they have low-risk congenital heart disease that do not need intensive care continuous monitoring. Maternal infant bonding starts before an infant is born and becomes stronger …