Medicine and Health Sciences Commons^™

The Similarities And Differences Between Glomerular Vs. Non-Glomerular Diagnoses On Intelligence And Executive Functions In Pediatric Chronic Kidney Disease: A Brief Report., Stephen R. Hooper, Rebecca J. Johnson, Marc Lande, Matthew Matheson, Shlomo Shinnar, Amy J. Kogon, Lyndsay Harshman, Joann Spinale, Arlene C. Gerson, Bradley A. Warady, Susan L. Furth

Manuscripts, Articles, Book Chapters and Other Papers

Pediatric chronic kidney disease (CKD) appears to be a heterogeneous group of conditions, but this heterogeneity has not been explored with respect to its impact on neurocognitive functioning. This study investigated the neurocognitive functioning of those with glomerular (G) vs. non-glomerular (NG) diagnoses. Data from the North American CKiD Study were employed and the current study included 1,003 children and adolescents with mild to moderate CKD. The G Group included 260 participants (median age = 14.7 years) and the NG Group included 743 individuals (median age = 9.0 years). Neurocognitive measures assessed IQ, inhibitory control, attention regulation, problem solving, working …

Tisagenlecleucel Immunogenicity In Relapsed/Refractory Acute Lymphoblastic Leukemia And Diffuse Large B-Cell Lymphoma., Karen Thudium Mueller, Stephan A. Grupp, Shannon L. Maude, John E. Levine, Michael A. Pulsipher, Michael W. Boyer, Keith August, Douglas Myers, Constantine S. Tam, Ulrich Jaeger, Stephen Ronan Foley, Peter Borchmann, Stephen J. Schuster, Edmund K. Waller, Rakesh Awasthi, Bernd Potthoff, Andy Warren, Edward R. Waldron, Fraser Mcblane, Andrea Chassot-Agostinho, Theodore W. Laetsch

Manuscripts, Articles, Book Chapters and Other Papers

Tisagenlecleucel is indicated for pediatric and young adult patients with relapsed/refractory (r/r) B-cell acute lymphoblastic leukemia (B-ALL) and adult patients with r/r diffuse large B-cell lymphoma (DLBCL). The tisagenlecleucel chimeric antigen receptor (CAR) contains a murine single-chain variable fragment domain; we examined the effects of humoral and cellular immune responses to tisagenlecleucel on clinical outcomes using 2 validated assays. Data were pooled from the ELIANA (registered at www.clinicaltrials.gov as #NCT02435849) and ENSIGN (#NCT02228096) trials in r/r B-ALL (N = 143) and the JULIET trial (#NCT02445248) in r/r DLBCL (N = 115). Humoral responses were determined …

Attenuation Of Autoimmune Phenomena In A Patient With Autoimmune Polyglandular Syndrome Type 1., Jill Jacobson, Julia Broussard, Courtney Marsh, Brandon D. Newell

Manuscripts, Articles, Book Chapters and Other Papers

Autoimmune polyglandular syndrome type 1 (APS1) is a progressive life-threatening illness with no known cure. Current treatments involve replacement of the hormone deficiencies that result from autoimmune destruction of multiple endocrine organs. We report on a girl whose disease was progressing rapidly until she began on immunosuppressive agents. A healthy 6-year-old girl with no remarkable medical history presented with new onset hypocalcemic seizures and primary hypoparathyroidism. Howell-Jolly bodies consistent with autoimmune hyposplenism were also noted. Genetic testing revealed compound heterozygosity for 2 disease-associated variants in the autoimmune regulator (AIRE) gene. She later developed elevated liver enzymes, primary adrenal insufficiency, and …

Multiplex Pcr Pathogen Detection In Acute Gastroenteritis Among Hospitalized Us Children Compared With Healthy Controls During 2011-2016 In The Post-Rotavirus Vaccine Era., Christopher J. Harrison, Ferdaus Hassan, Brian R. Lee, Julie Boom, Leila C. Sahni, Coreen Johnson, James Dunn, Daniel C. Payne, Mary E. Wikswo, Umesh Parashar, Rangaraj Selvarangan

Manuscripts, Articles, Book Chapters and Other Papers

Background: Despite vaccine-induced decreases in US rotavirus (RV) disease, acute gastroenteritis (AGE) remains relatively common. We evaluated AGE pathogen distribution in hospitalized US children in the post-RV vaccine era.

Methods: From December 2011 to June 2016, the New Vaccine Surveillance Network (NVSN) conducted prospective, active, population-based surveillance in hospitalized children with AGE. We tested stools from 2 NVSN sites (Kansas City, Houston) with Luminex x-TAG Gastrointestinal Pathogen Panels (Luminex GPP) and analyzed selected signs and symptoms.

Results: For 660 pediatric AGE inpatients and 624 age-matched healthy controls (HCs), overall organism detection was 51.2% and 20.6%, respectively (P < .001). Among AGE subjects, GPP polymerase chain reaction detected >1 virus …

Epidural Blood Patch For Pediatric Patients Only, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Pulmonary Embolism, Suspected, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Ambulatory Patients With A Central Line Presenting With A Fever, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Post-Dural Puncture Headache, Children's Mercy Kansas City

Clinical Pathways (Formally Known As Care Process Models)

No abstract provided.

Grading Of Improvement In Hypsarrhythmia With Standard Epileptic Spasms Treatment At A Large Pediatric Tertiary Care Center, Julie Grace Gianakon, Roha Khalid, Mohammed Ilyas

Posters

Background Epileptic spasm is a peculiar type of epileptic seizure, entailing the clinical spasms and a characteristic electroencephalogram (EEG) abnormality often called hypsarrhythmia or its variants. The main goal of epileptic spasm treatment with standard therapy is to suppress clinical spasms and abolish the hypsarrhythmia and its variant EEG pattern. This interictal EEG pattern frequently heralds developmental regression. The elimination of hypsarrhythmia is a principal goal of therapy and a key outcome measure in clinical trials. There have been several studies in the interpretation or grading of hypsarrhythmia (Watanabe et al.1993 & Jeavons & Bower et al.1961). Still, there have …

Elevated Stool Inflammatory Markers In Early Onset Polymorphic Post-Transplant Lymphoproliferative Disease Following Orthotopic Heart Transplant, Jeremy Stewart, Keith August, Thomas M. Attard

Posters

Acute gastrointestinal hemorrhage in the context of a recent organ transplant with immunosuppression, antibiotic exposure, drug exposures and other comorbidities is a challenging clinical scenario with coordinated multidisciplinary effort needed to optimize management and outcomes. We present a patient with recent solid organ transplant with severe acute gastrointestinal bleeding attributable to EBV associated post-transplant lymphoproliferative disease. The patient is a 13-year-old Female with Williams syndrome who is EBV negative and CMV positive who received an EBV/CMV positive orthotopic heart transplant 5.5 months prior to presentation. She presented with bloody diarrhea, anemia (7.8 gm/dL), hypovolemia, and acute kidney injury (BUN 25 …

Standardizing Resident Education On Gi Service, Jennifer Halma, Kenneth Schmidt

Posters

Introduction: The pediatric residency curriculum at Children’s Mercy Hospital is standardized to offer each trainee the same rotational experience. As a result, each resident spends one month on the inpatient gastroenterology (GI) service during training. This invites variability in the quantity and quality of GI education that residents receive. Therefore, there is a need to design a standard curriculum to provide comprehensive GI education. The aim of this project is to standardize resident education by identifying most requested topics and providing didactic education to increase resident general GI knowledge. Methods: Each month, residents were provided with a survey to assess …

Enterovirus D68-Associated Acute Respiratory Illness ─ New Vaccine Surveillance Network, United States, July-November 2018-2020., Melisa M. Shah, Ariana Perez, Joana Y. Lively, Vasanthi Avadhanula, Julie A. Boom, James Chappell, Janet A. Englund, Wende Fregoe, Natasha B. Halasa, Christopher J. Harrison, Robert W. Hickey, Eileen J. Klein, Monica M. Mcneal, Marian G. Michaels, Mary Moffatt, Catherine Otten, Leila C. Sahni, Elizabeth Schlaudecker, Jennifer E. Schuster, Rangaraj Selvarangan, Mary A. Staat, Laura S. Stewart, Geoffrey A. Weinberg, John V. Williams, Terry Fan Fei Ng, Janell A. Routh, Susan I. Gerber, Meredith L. Mcmorrow, Brian Rha, Claire M. Midgley

Manuscripts, Articles, Book Chapters and Other Papers

Enterovirus D68 (EV-D68) is associated with a broad spectrum of illnesses, including mild to severe acute respiratory illness (ARI) and acute flaccid myelitis (AFM). Enteroviruses, including EV-D68, are typically detected in the United States during late summer through fall, with year-to-year fluctuations. Before 2014, EV-D68 was infrequently reported to CDC (1). However, numbers of EV-D68 detection have increased in recent years, with a biennial pattern observed during 2014-2018 in the United States, after the expansion of surveillance and wider availability of molecular testing. In 2014, a national outbreak of EV-D68 was detected (2). EV-D68 was also reported in 2016 via …

Rotavirus Genotype Trends And Gastrointestinal Pathogen Detection In The United States, 2014-2016: Results From The New Vaccine Surveillance Network., Mathew D. Esona, M Leanne Ward, Mary E. Wikswo, Slavica M. Rustempasic, Rashi Gautam, Charity Perkins, Rangaraj Selvarangan, Christopher J. Harrison, Julie A. Boom, Janet A. Englund, Eileen J. Klein, Mary Allen Staat, Monica M. Mcneal, Natasha Halasa, James Chappell, Geoffrey A. Weinberg, Daniel C. Payne, Umesh D. Parashar, Michael D. Bowen

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Following the implementation of rotavirus vaccination in 2006, severe acute gastroenteritis (AGE) due to group A rotavirus (RVA) has substantially declined in US children. We report the RVA genotype prevalence as well as coinfection data from 7 US New Vaccine Surveillance Network sites during 3 consecutive RVA seasons, 2014-2016.

METHODS: A total of 1041 stool samples that tested positive for RVA by Rotaclone enzyme immunoassay were submitted to the Centers for Disease Control and Prevention (CDC) for RVA genotyping and multipathogen testing.

RESULTS: A total of 795 (76%) samples contained detectable RVA when tested at the CDC. Rotavirus disease …

Adding Family Digital Supports To Classroom-Based Physical Activity Interventions To Target In-And Out-Of-School Activity: An Evaluation Of The Stay Active Intervention Duringthe Covid-19 Pandemic, Bethany Forseth Hanson, Adrian Ortega, Paul R. Hibbing, Mallory Moon, Chelsea Steel, Mehar Singh, Avinash Kollu, Bryce Miller, Maurice Miller, Vincent S. Staggs, Hannah Calvert, Ann M. Davis, Jordan A. Carlson

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: Interventions targeting multiple levels of influence and settings may have a greater impact on children’s total daily physical activity than those targeting a single setting. This study evaluated the addition of family supports to a classroom-based physical activity intervention.

Methods: 6 schools were randomized to a remotely delivered classroom-based physical activity only (CBPA) or classroom-based physical activity plus family (CBPA+) arm. The latter added behavior change tools (e.g., self-regulation) delivered via text messages and newsletters to caregiver/child dyads. The intervention lasted for 7 weeks with a 1-week baseline and 1-week post-intervention assessment (9 weeks total). Children who enrolled in …

Demographic Characteristics Among Members Of Patient Family Advisory Councils At A Pediatric Health System., Amanda Montalbano, Sheryl Chadwick, Deejo Miller, Kathryn Taff, Evelyn Donis De Miranda, Kimberly Pina, Andrea Bradley-Ewing

Manuscripts, Articles, Book Chapters and Other Papers

Patient Family Advisory Councils (PFACs) are groups of patients, families, and clinical health system leaders collaborating to improve the quality, safety, and experience of care. Best practices encourage PFAC membership to reflect the diversity of the communities served. A cross-sectional survey was conducted from September 2019 to January 2020 collecting demographic characteristics of the members of a pediatric health system's 17 PFACs. Of the 93 respondents (71.5%), notable inequities were racial (4.8% adult PFAC members vs. 17.4% patients were Black) and financial (adult PFAC median annual income was >$100,000 compared to the community median $77,589). The governing family advisory board …

Implementation Of A Transition Readiness Assessment And Transition Discussion Documentation In A Type 1 Diabetes Clinic, Sonalee Ravi

Presentations

Background/Objective: Our institution utilizes a general transition readiness assessment to facilitate transition discussions. Patients rate six areas of knowledge, eight skills, and confidence in ability to transfer care successfully prior to age 22, then select a goal for their next visit. Discussions are documented in the medical record. Our aim was to implement this assessment with a 20% documentation rate after six months.

Methods: In March 2021, we trained providers and diabetes educators about the need for transition planning, the differences between transition planning and transfer, and the need for documentation. We added three diabetes-specific questions related to insulin adjustments, …

Optimizing Oral Glucose Tolerance Test Completion At A Pediatric Cystic Fibrosis Care Center: A 10-Year Continuing Quality Improvement Effort, Christopher M. Oermann, Paula Capel, Jessica Haynes, Michelle Fischer, Jill Kohmetscher

Posters

Background: Cystic fibrosis–related diabetes (CFRD) is a common comorbidity among people with CF (PwCF). It is associated with weight loss, protein catabolism, lung function decline, and increased mortality. Nutritional status and pulmonary function begin to decline in PwCF several years before the diagnosis of CFRD. Early CFRD detection and aggressive insulin therapy have been shown to reduce the mortality gap between PwCF who have CFRD and those who do not. The Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes recommend annual screening for people with CF starting at age 10 [1]. Methods: In 2011, team members at Children’s Mercy Kansas City …

Healthcare System-To-System Cost Variability In The Care Of Pediatric Abdominal Pain-Associated Functional Gastrointestinal Disorders., Michelle Livitz, Alec S. Friesen, Earl F. Glynn, Jennifer Verrill Schurman, Jennifer Colombo, Craig A. Friesen

Manuscripts, Articles, Book Chapters and Other Papers

The purpose of this study was to assess cost variability in the care of abdominal pain-associated functional gastrointestinal disorders (AP-FGIDS) in youth across health systems, races, and specific AP-FGID diagnoses. Patients, aged 8-17 years, with a priority 1 diagnosis corresponding to a Rome IV defined AP-FGID were identified within the Health Facts® database. Total costs were obtained across the continuum of care including outpatient clinics, emergency department, and inpatient or observation units. Cost variability was described comparing different health systems, races, and diagnoses. Thirteen thousand two hundred and fourteen patients were identified accounting for 17,287 encounters. Total costs were available …

Evaluation Of The Outcomes Of Trimethoprim-Sulfamethoxazole Oral Challenges In The Pediatric Population, Hannah N. Neuhaus, Salman Aljubran

Posters

Introduction: Trimethoprim-sulfamethoxazole allergy can complicate treatment for various infections. While trimethoprim-sulfamethoxazole hypersensitivity, desensitization/oral challenges are frequently reported in adults, data is limited on the outcomes/safety of oral challenges to trimethoprim-sulfamethoxazole in Pediatrics. The goal of this study was to characterize the outcomes and safety of trimethoprim-sulfamethoxazole oral challenges in Pediatrics.

Methods: An IRB-exempt retrospective chart review was performed of pediatric patients who underwent oral challenge to trimethoprim-sulfamethoxazole in Allergy Clinic over the last 12 years. We assessed characteristics including age, sex, reaction (IgE-mediated/non-IgE-mediated/indeterminate), skin testing, challenge outcome and complications to draw a conclusion regarding the overall safety of the procedure …

Impact Of Early Tracheostomy On Neurodevelopmental Outcome In Infants With Severe Bronchopulmonary Dysplasia Exposed To Postnatal Steroids, Amjad Taha, Gangaram Akangire, Janelle R. Noel-Macdonnell, Tiffany Willis, Winston Manimtim

Posters

No abstract provided.

Delayed-Onset Anaphylactic Reaction With High Fever After Amoxicillin Oral Challenge And Negative Penicillin Skin Testing, Jordan Pitt, Paul J. Dowling, Christopher Miller, Aarti Pandya

Posters

Introduction:  Immunologic adverse drug reactions can be categorized based on Gell and Coombs’s classification system. Anaphylaxis is generally considered a type I, immediate, IgE-mediated reaction and typically occurs independent of other immunologic reactions. However, the child presented here reacted after amoxicillin challenge with features of type I and type III or IV hypersensitivity reactions. Case Description:  A 12-year-old female presented for amoxicillin allergy evaluation after treatment for scarlet fever with amoxicillin. After the second dose she developed rash with varied features, fatigue, edema, and joint swelling. Labs included a persistently low C4 level, eosinophilia, normal inflammatory markers, and normal tryptase …

Optic Disc Pit Maculopathy Leading To Vision Loss In A Pediatric Patient, Allyson Hall

Posters

This case will review optic disc pit maculopathy in children and treatments available. An emphasis in vision rehabilitation will be discussed as any visual insult during this development period can lead to long-term visual consequences.

A Case Of An Elevated Tryptase, Sonya Parashar, Nikita Raje

Posters

Hereditary Alpha Tryptasemia (HαT) is an autosomal dominant disorder characterized by an elevated baseline tryptase that occurs up to 3% of the population and clinically resembles mast cell activation syndrome.

Vancomycin Auc Monitoring In Individuals With Cystic Fibrosis At A Pediatric Institution, Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier, Claire Elson

Posters

Vancomycin AUC monitoring in individuals with cystic fibrosis at a pediatric institution S. Duehlmeyer1, C. Oermann1, E. Meier1, E. Elson1. 1Pulmonology, Children’s Mercy Kansas City, Kansas City, USA Background: Antibiotic therapy is essential for the treatment of cystic fibrosis (CF) lung infections. Methicillin-resistant Staphylococcus aureus (MRSA) infects 20% to 25% of people with CF (PwCF) and is associated with increased morbidity. Treatment of pulmonary exacerbations (PEs) often requires hospitalization including respiratory treatments and intravenous (IV) antimicrobials. IV vancomycin, which is commonly used for MRSA infections, requires serum concentration monitoring to ensure efficacy and minimize toxicity. Previous guidelines recommended trough concentrations …

Covid-19 Vaccination In Individuals With Cystic Fibrosis At A Pediatric Cystic Fibrosis Center, Christopher M. Oermann, Claire Elson, Ellen Meier, Paula Capel, Jessica Haynes, Michelle Fischer, Stephanie Duehlmeyer

Posters

Background: Observational data suggest that most people with cystic fibrosis (PwCF) who contract COVID-19 have outcomes similar to those of the general population, although PwCF who are older or have CF-related diabetes, poor lung function, or a history of lung transplantation may be at greater risk for more severe disease. Therefore, the CF Foundation advocates for PwCF to discuss vaccination with care teams. At present, the FDA has authorized emergency use of 3 COVID-19 vaccines. ACIP/CDC guidance allows each state to determine vaccine distribution based on an individual’s exposure and risk for severe disease. This study describes the attitudes of …

Prenatal Delineation Of Coronary Anatomy In Dextro-Transposition Of Great Arteries, Geetha Haligheri, Chandrakant Patel, Rukmini Komarlu

Manuscripts, Articles, Book Chapters and Other Papers

Background: Dextro-transposition of the great arteries (D-TGA) is the second-most common cyanotic congenital heart disease with variable coronary artery anatomy. The arterial switch procedure has revolutionized outcomes for this defect, with coronary anatomy being a key determinant of both short- and long-term outcomes following surgical repair. The assessment of coronary anatomy is usually undertaken in the postnatal period by transthoracic echocardiography, with assessment prenatally not being well studied. We sought to assess the feasibility of delineating the coronary arteries on fetal echocardiograms in a small cohort of patients followed prenatally. Methods: This was a retrospective review of fetuses with D-TGA …

Effectiveness Of Pfizer-Biontech Mrna Vaccination Against Covid-19 Hospitalization Among Persons Aged 12-18 Years - United States, June-September 2021., Samantha M. Olson, Margaret M. Newhams, Natasha B. Halasa, Ashley M. Price, Julie A. Boom, Leila C. Sahni, Katherine Irby, Tracie C. Walker, Stephanie P. Schwartz, Pia S. Pannaraj, Aline B. Maddux, Tamara T. Bradford, Ryan A. Nofziger, Benjamin J. Boutselis, Melissa L. Cullimore, Elizabeth H. Mack, Jennifer E. Schuster, Shira J. Gertz, Natalie Z. Cvijanovich, Michele Kong, Melissa A. Cameron, Mary A. Staat, Emily R. Levy, Brandon M. Chatani, Kathleen Chiotos, Laura D. Zambrano, Angela P. Campbell, Manish M. Patel, Adrienne G. Randolph, Overcoming Covid-19 Investigators

Manuscripts, Articles, Book Chapters and Other Papers

Pfizer-BioNTech COVID-19 vaccine is authorized for use in children and adolescents aged 12-15 years and is licensed by the Food and Drug Administration (FDA) for persons aged ≥16 (1). A randomized placebo-controlled trial demonstrated an efficacy of 100% (95% confidence interval [CI] = 75.3%-100%) in preventing outpatient COVID-19 in persons aged 12-15 years (2); however, data among adolescents on vaccine effectiveness (VE) against COVID-19 in real-world settings are limited, especially among hospitalized patients. In early September 2021, U.S. pediatric COVID-19 hospitalizations reached the highest level during the pandemic (3,4). In a test-negative, case-control study at 19 pediatric hospitals in 16 …

Lipomatous Infiltration In Tuberous Sclerosis Complex - A Case Series And Literature Review., Mohammed Ilyas, Julio Quezada, Erin K. Opfer

Manuscripts, Articles, Book Chapters and Other Papers

Diffuse Lipomatosis is a dermatological lesion consisting of a poorly circumscribed, infiltrative overgrowth of mature adipose tissue that usually affects the trunk and the extremities. The lesions in the Tuberous Sclerosis Complex (TSC) are usually hamartomatous in nature, but lesions arising from adipocytes are rare. There are only three previous reports of association of TSC with diffuse lipomatosis. Herein we present a case series of diffuse lipomatosis in three subjects with TSC and proceed to review the literature for any other reported cases. On the basis of the three index cases and identification of three more cases in the literature, …

A Prospective Observational Study Of Antihemophilic Factor (Recombinant) Prophylaxis Related To Physical Activity Levels In Patients With Hemophilia A In The United States (Space)., Barbara A. Konkle, Doris V. Quon, Leslie Raffini, Michael Recht, Vlad C. Radulescu, Shannon L. Carpenter, Amy L. Dunn, Mei Lu, Maureen Watt

Manuscripts, Articles, Book Chapters and Other Papers

Introduction: High collision-risk physical activity can increase bleeding risk in people with hemophilia A, as can increasing the time between factor VIII (FVIII) administration and physical activity. FVIII prophylaxis may be tailored to planned activities to prevent activity-related bleeding.

Aim: To explore the relationship between physical activity levels, FVIII infusion timing, and occurrence of bleeding in patients with severe/moderately severe hemophilia A without FVIII inhibitors receiving antihemophilic factor (recombinant) (rAHF; ADVATE®; Baxalta US Inc., a Takeda company, Lexington, MA, USA).

Methods: SPACE was a 6-month, prospective, multicenter, observational outcomes study (NCT02190149). Enrolled patients received an eDiary application and …

Knowledge About Juvenile Idiopathic Arthritis-Associated Uveitis: More Frequent Reminders Are Associated With Higher Patient And Family Uveitis Knowledge., Ashley M. Cooper, Elaine R. Flanagan, Tova Ronis, Baruch Goldberg, Ashley K. Sherman, Chelsey Smith, Gary N. Holland, Carra Uveitis Workgroup

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Chronic anterior uveitis is a sight-threatening complication of juvenile idiopathic arthritis (JIA) and a primary contributor to long-term morbidity in people with JIA. Levels of knowledge about uveitis among JIA patients and their parents are unknown. A survey of JIA patients and parents was conducted to assess knowledge about uveitis complications and recommended screening.

METHODS: A survey was developed consisting of six demographic questions, six arthritis/uveitis history questions, and nine uveitis knowledge questions. The survey was administered to JIA patients age 14 and older and parents of patients with JIA at three pediatric rheumatology practices and online through the …