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These posters have been presented at meetings in Children's Mercy and around the world. They represent research that was done at the time they were created, and may not represent medical knowledge or practice as it exists at the time viewers access these posters.

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  • Adults and Adolescents with High-Intensity Neurologic Impairment Hospitalized at Children’s Hospitals by Charles Maloy, Matt Hall, Joanna Thomson, Jean-Baptist LePichon, and Henry T. Puls

    Adults and Adolescents with High-Intensity Neurologic Impairment Hospitalized at Children’s Hospitals

    Charles Maloy, Matt Hall, Joanna Thomson, Jean-Baptist LePichon, and Henry T. Puls

    Introduction: Children with high-intensity neurologic impairment (HINI) account for a sizeable proportion of inpatient resources. As survival into adulthood for children with medical complexity increases, children’s hospitals will likely admit adults with HINI. Understanding adult HINI hospitalization patterns is necessary to develop effective care strategies for this population. Objective: Compare characteristics of adults and adolescents with HINI hospitalized at children’s hospitals and describe hospital-level variation in hospitalizations for adults with HINI. Methods: This retrospective cross-sectional study included discharges of patients with HINI aged 12-29 years from 41 children’s hospitals in the Pediatric Health Information System between 2022-24. Hospitals were excluded if birthing encounters were identified. Discharges were categorized by age: adolescents (12-17 years) and adults (18-29 years). Chi-square and Wilcoxon Rank-Sum tests compared hospitalization level characteristics (i.e., intensive care use, hospital length of stay, in-hospital mortality, and costs) across the two age groups. Hospital-level variation was described using the percentage of included HINI discharges that were adults and correlated with hospital volume of included HINI discharges using Pearson’s coefficient. Results: There were 136,290 discharges included; 23.6% were adults. Compared to adolescents, adults were more likely to have >1 HINI category (41.1% vs 33.9%), a co-morbid non-neurologic chronic complex condition (72.5% vs 58.5%), and technology use (49.2% vs 37.8%) (Table). Adults had slightly longer length-of-stay, similar ICU use, similar mortality, and higher hospitalization costs (Table). There was substantial hospital-level variation in the percentage of HINI hospitalizations that were adults (range 0.0% to 35.7%), which was correlated with hospital volume of all HINI discharges aged 12-29 years (p< 0.01). Conclusion: Adults with HINI were more complex and incurred longer hospitalizations and greater costs than their adolescent counterparts. Hospital-level variation in adults with HINI hospitalized at children’s hospitals was correlated with hospital volume of HINI discharges and may result from differences in the transition processes between institutions. Future studies should seek to understand optimal transition timing and care setting for adult HINI patients.

  • Building a Better Onboarding Process: The Addition of a 30-Minute 1:1 for New Faculty by Heather McNeill, Enam Haddad, Sarah Tsai, Ruba A. Abdelhadi, and Jacqueline Hill

    Building a Better Onboarding Process: The Addition of a 30-Minute 1:1 for New Faculty

    Heather McNeill, Enam Haddad, Sarah Tsai, Ruba A. Abdelhadi, and Jacqueline Hill

    Our faculty development office examined the addition of a 30-minute personalized 1:1 session to the broader faculty onboarding strategy at our pediatric teaching hospital. We surveyed 106 new faculty who participated in a session from 2023-2025 to assess its usefulness. Of the 69 (65.7%) respondents, a large majority (92%) “agreed” or “strongly agreed” that the session was a valuable use of their time. Several faculty commented that the most useful information was learning about career development resources (e.g., mentorship program, writing center), understanding how to prepare for academic promotion, and having time to ask questions. Most new faculty thought the 1:1 was valuable, justifying the use of time and staff resources to sustain this addition to the onboarding process.

  • Can Mentoring Improve Professional Skills for Mentors and Mentees? by Emilie Keeton, MSOD; Myles Chandler; Eric T. Rush; Susan B. Hathaway; and Jacqueline Hill

    Can Mentoring Improve Professional Skills for Mentors and Mentees?

    Emilie Keeton, MSOD; Myles Chandler; Eric T. Rush; Susan B. Hathaway; and Jacqueline Hill

    Introduction/Background: We implemented a structured 10-month mentoring program at our pediatric academic hospital designed to assist faculty in actualizing professional and personal goals through developing trusted, mutually rewarding relationships. Goals/Hypothesis: To evaluate if faculty participants in the 2 inaugural mentoring program cohorts improve scores on 6 behavioral competencies related to maintaining effective communication, aligning expectations, assessing understanding, addressing diversity, fostering independence, and promoting professional development. Methods/Approach We used pre- and post-program Mentoring Competency Assessments to assess changes in 26 skills across 6 competencies and gather feedback on program elements from participants. Results/Outcomes: 19/35 (54%) mentees and 15/29 (52%) mentors completed both assessments. 20/26 rated skills across 6 competencies significantly increased for mentees (p < 0.05), while 4/26 rated skills across 6 competencies significantly improved for mentors. Overall, 89% of mentors and 87% of mentees agreed the program was worth their time to participate. Conclusion/Impact: Program was successful in helping mentees improve most skills across all 6 competencies, whereas mentors saw minimal improvement in skills. Both mentors and mentees reported the program helped them build professional relationships and was worth their time to participate. Future efforts will focus on obtaining assessments and enrolling more participants.

  • Clinical and Non-Clinical Factors Associated with Early Discharge in Pediatric Non-Trauma Patients Transported by Rotor Wing by Jordon Mitzelfelt, Lisa Pruitt, Amy Papa, Ashley K. Sherman, David Juang, Sarah Brunner, Asdis Finnsdottir Wagner, and Jennifer Flint

    Clinical and Non-Clinical Factors Associated with Early Discharge in Pediatric Non-Trauma Patients Transported by Rotor Wing

    Jordon Mitzelfelt, Lisa Pruitt, Amy Papa, Ashley K. Sherman, David Juang, Sarah Brunner, Asdis Finnsdottir Wagner, and Jennifer Flint

    Objective: Helicopter emergency medical services (HEMS) are crucial for transporting critically ill children to definitive care, however, rising costs and relative risks of this type of transportation necessitate careful evaluation of potential over-utilization. Current literature primarily focuses on pediatric trauma patients, leaving a significant gap in data regarding non-trauma pediatric HEMS transports and the influence of non-clinical factors such as referral hospital distance, resources, and capabilities on transport decisions. This study aims to address these gaps by examining non-trauma pediatric HEMS patients and identifying associated clinical and non-clinical factors related to early hospital discharge (< 24 hours). Methods: This retrospective, single-center study includes pediatric patients with medical and non-trauma surgical diagnoses transported by HEMS between April 1, 2021 and March 31, 2024. Clinical data were extracted from transport team records and the hospital’s electronic health records. Analyses were performed to identify factors associated with discharge within 24 hours of admission. To identify predictors of early discharge, we analyzed clinical factors including chief complaint, pre-transport vascular access, transport crew interventions (medication administration and procedures), respiratory assistance, and Pediatric Age-Adjusted Shock Index (SIPA) scores. Additionally, we examined the impact of the initial admit unit, and medical support required within the first 24 hours of hospitalization. Non-clinical predictors included the referring hospital’s status (critical access and/or rural designation) and the time of transport. Results: A total of 568 patients were transported by HEMS, of whom 88 (15%) were discharged within 24 hours. Patients in the early discharge group were more likely to present with neurologic chief complaints (40%), whereas those remaining hospitalized beyond 24 hours more frequently presented with respiratory complaints (40%). Normal SIPA scores both prior to and at the conclusion of transport were significantly associated with early discharge (p=0.012 and p=0.011, respectively). Furthermore, patients requiring no medications or respiratory assistance during transport, or no respiratory support within the first 24 hours of admission, were significantly more likely to be discharged early (p< 0.001). Notably, all patients with central access prior to transport remained admitted for more than 24 hours (p=0.004). Regarding non-clinical factors, patients transported from designated rural hospitals were more likely to experience early discharge (p< 0.001). Conclusion: These results characterize the pediatric non-trauma HEMS population and identify key predictors of early hospital discharge. This data is essential for developing standardized dispatch criteria to mitigate over-utilization and ensure high-acuity transport is reserved for the most appropriate patients.

  • Initial Results of a Structured Coaching Program for Academic Promotion Preparation by Jacqueline Hill, Christine Sullivan, Tyler Smith, Margaret Gibson, Valerica Mateescu, and Wendell K. Clarkston

    Initial Results of a Structured Coaching Program for Academic Promotion Preparation

    Jacqueline Hill, Christine Sullivan, Tyler Smith, Margaret Gibson, Valerica Mateescu, and Wendell K. Clarkston

    Introduction: Applying for academic promotion can be a confusing and arduous process, often with little guidance to assure a successful experience, especially for junior faculty located at affiliate hospitals. Goals: We present initial results of a structured university mentorship program to prepare faculty to apply for academic promotion. Methods: Program was designed as a small working group of 5 structured sessions over a 9-month period where participants were coached by senior faculty from 3 university affiliate hospitals. Program culminated in the completion of an academic promotion application. Results: Since launch in 2019, we have completed 4 program cohorts and paused 2 cohorts due to the pandemic. 23 of 29 (79%) enrolled faculty successfully completed the program. Majority (24, 83%) were applying for Associate Professor. Of faculty who completed the program, 13 (57%) have successfully achieved promotion, 6 (26%) have applications currently under review, and 4 (17%) have not yet applied. Conclusion: Program was successful in coaching faculty to prepare applications for academic promotion. Through mentor feedback, some participants determined they did not yet have all necessary activities to successfully apply for promotion and instead created a plan to first bolster scholarly accomplishments. Our results suggest that investing in structured coaching sessions is a valuable strategy to increase the likelihood of faculty successfully achieving promotion.

  • Navigating Academic Promotion: Identifying the Winning Combination for Volunteer Faculty by Enam Haddad, Jacqueline Hill, Sarah Tsai, Ruba A. Abdelhadi, Sarah Braet, and Eric T. Rush

    Navigating Academic Promotion: Identifying the Winning Combination for Volunteer Faculty

    Enam Haddad, Jacqueline Hill, Sarah Tsai, Ruba A. Abdelhadi, Sarah Braet, and Eric T. Rush

    Introduction/Background: Academic promotion is an arduous and time-consuming process. Implementation of support resources is essential for faculty success. Supporting faculty through the process, we developed multiple promotion resources, including a robust online resource catalogue, peer coaching sessions, and detailed weekly email reminders. Goals/Hypothesis: To examine which resources faculty find most helpful in preparing their academic promotion application. Methods/Approach: We surveyed promotion applicants from 6 cycles (2020-2025). Faculty rated the usefulness of promotion resources, how prepared they felt at different points in the promotion timeline, and provided feedback about the overall promotion preparation process. Results/Outcomes: 258/354 (73%) applicants responded to the survey. Respondents agreed or strongly agreed that the following resources were most helpful: promotion strategy meetings with faculty development director (93%), conversations with promotion committee reviewer (93%), conversations with faculty leaders (90%), and weekly promotion communications (90%). 94% of respondents reported feeling prepared for initial application submission for promotion committee review and 96% felt prepared for the final submission to university. Conclusion/Impact: Majority of faculty applicants value promotion conversations with multiple faculty and promotion process leaders and weekly outlined communications as they navigate the academic promotion process.

  • Neonatal Outcomes Associated with Maternal SSRI Use throughout the COVID Pandemic by Carlie G. Gatlin, Megan Tucker, Angela Bolton, Mary Anne Kucera, Deanna McPherson, Kerry Kohrs, Staci S. Elliott, Hung-Wen Yeh, and Venkatesh Sampath

    Neonatal Outcomes Associated with Maternal SSRI Use throughout the COVID Pandemic

    Carlie G. Gatlin, Megan Tucker, Angela Bolton, Mary Anne Kucera, Deanna McPherson, Kerry Kohrs, Staci S. Elliott, Hung-Wen Yeh, and Venkatesh Sampath

    Background: Maternal antenatal selective serotonin reuptake inhibitor (SSRI) use has been suggested as a risk factor for neonatal complications, though evidence remains inconclusive. Mental health conditions increased during the COVID-19 pandemic, raising questions about SSRI prescription trends. This study examines associations between maternal SSRI use and neonatal outcomes across three time periods: before, during, and after the pandemic. Objectives: Primary: Determine if SSRI use in pregnancy is associated with NICU admission, respiratory support, hospital stay length, hypoglycemia, formula feeding, and/or delivery room resuscitation. Secondary: Assess SSRI prescription prevalence across pandemic phases. Methods: Retrospective chart review of term maternal-infant dyads born in Jan/June during each of the years of 2019–2022 in a level III NICU. Data included maternal demographics, SSRI use, comorbidities, and neonatal outcomes. Exclusions: congenital anomalies, multiple gestations, and preterm birth < 37 weeks. N=1,246. Categorical variables were compared using Chi-squared or Fisher’s exact test; continuous variables via Wilcoxon or Kruskal-Wallis tests. Regression models adjusted for COVID phase and covariates. Missing data (>5%) were imputed using MICE and random forest, with pooled results via Rubin’s rule. Results: Maternal SSRI use rates did not differ significantly across the 3 time periods (pre-Covid 54/572 9.5% vs. during Covid 31/338 9.3% vs. post-Covid 36/336 11%) (Table 1). SSRI use was highest among those with pre-pregnancy use (p=< 0.001), and white mothers (White 108/890 12% vs. Non-white 13/354 3.7 %, p= < 0.001) (Table 2). Infants exposed to SSRIs were more likely to require delivery room resuscitation (OR 2.51, 95% CI 1.19-5.26, p= 0.015) (Table 3B). Mothers using SSRIs were more likely to formula or combo feed rather than exclusively breastfeed (OR 2.86, 95% 1.68-4.85, p=0.000) (OR 1.91, 95% 1.05-3.47, p= 0.034) (Table 3F). No significant associations were found between maternal SSRI use and neonatal hypoglycemia, NICU admission, respiratory support, or length of stay (Table 3A,C,D,E). Conclusion: In our study, we did not find an increase in maternal SSRI use during the COVID pandemic. Maternal SSRI use was associated with increased delivery room resuscitation and non-exclusive breastfeeding, but not other adverse neonatal outcomes. These findings highlight the continued concerns of maternal SSRI use on neonatal outcomes, and advocate for careful consideration and discussion of its benefits vs. risks in pregnant mothers as expressed by recent FDA and ACOG statements.

  • Reducing Waste and Cost by Utilizing Tap Water for Laceration Irrigation in the Emergency Department: A Quality Improvement Approach by Alison L. Fowler; Rohan Akhouri; Josaih Galdean; Isabella Dunt; Christopher Kaberline; Viktoriya Stoycheva MHA, RN, CPN; Sarah Nienhaus; and Leslie Hueschen

    Reducing Waste and Cost by Utilizing Tap Water for Laceration Irrigation in the Emergency Department: A Quality Improvement Approach

    Alison L. Fowler; Rohan Akhouri; Josaih Galdean; Isabella Dunt; Christopher Kaberline; Viktoriya Stoycheva MHA, RN, CPN; Sarah Nienhaus; and Leslie Hueschen

    Purpose/Objective: Lacerations requiring closure make up 8 million emergency department (ED) visits annually. Irrigating prior to closure with potable tap water decreases cost and waste without increasing rates of infection compared to sterile fluids. Currently, only 3.5% of lacerations closed in our ED are irrigated with tap water. This project aims to increase the percent age of lacerations closed by ED providers irrigated with tap water from 3.5% to 60% by March 2026 in a freestanding pediatric emergency department across two sites (ED 1, ED 2). Design/Methods: A multidisciplinary team (physicians, nurses, QI consultant, parent advisors) formed in November 2024. Using driver diagram, fishbone, and PICK charts, interventions were identified and implemented in Plan-Do-Study-Act (PDSA) cycles (Table 1). The outcome measure was percentage of lacerations irrigated with tap water. Process measures were the number of sterile fluid bottles ordered for ED 2, order set usage, and rate of irrigation by nurses. The balancing measure was the rate of return visits for infection within 3 days. Fifty randomized charts were reviewed monthly. IRB deemed this study QI, not human subjects research. Results: Of 3,288 eligible encounters, 985 were randomized and included. Tap water irrigation increased from 3.5% to 64.6% (Fig 1). The average number of sterile bottles ordered monthly for ED 2 decreased from 102 to 16, representing a monthly savings of at least $430 and a reduction in plastic waste by at least 9 kg per month, reducing carbon emissions equivalent to 26 miles driven in a gas-powered car. There were no differences in irrigation by nurses or order set usage. There were no return visits for infection. Conclusion/Discussion: Altering the environment by removing sterile irrigation supplies from quick access locations was the most impactful intervention, allowing the team to meet their goal of increased tap water irrigation and decreased cost and waste. Future interventions include patient/family education and a new clinical pathway for lacerations. Limitations include inability to update order sets and limited monitoring of return visits. With the success of this project, the team plans to expand tap water irrigation to urgent care and subspecialist laceration repairs.

  • Variation in dexamethasone prescribing practices for pediatric asthma exacerbations in acute care settings by Hannah Reilly, Natalie J. Kane, Sian Best, Mark A. Hoffman, and Kathryn Kyler

    Variation in dexamethasone prescribing practices for pediatric asthma exacerbations in acute care settings

    Hannah Reilly, Natalie J. Kane, Sian Best, Mark A. Hoffman, and Kathryn Kyler

  • Clinical validation of targeted somatic structural variant detection in pediatric cancer via long-read sequencing by Aravindh Nagarajan, Byunggil Yoo, Emily Farrow, John Herriges, Lei Zhang, Elena Repnikova, Tomi Pastinen, Carol J. Saunders, Midhat Farooqi, and Lisa Lansdon

    Clinical validation of targeted somatic structural variant detection in pediatric cancer via long-read sequencing

    Aravindh Nagarajan, Byunggil Yoo, Emily Farrow, John Herriges, Lei Zhang, Elena Repnikova, Tomi Pastinen, Carol J. Saunders, Midhat Farooqi, and Lisa Lansdon

    Introduction: Structural variants (SVs) leading to gene fusions are one of the most common classes of driver mutations in pediatric cancer. Clinical SV detection relies on a combination of techniques including karyotyping, FISH, microarray and RNA fusion testing, and, more recently, optical genome mapping. SV detection via long-read genome sequencing (lr-GS) offers the advantage of breakpoint and complex event resolution; however, its implementation has been limited by the lack of an optimized somatic SV caller, among other factors. Here we describe the clinical validation of lr-GS for targeted somatic SV detection in pediatric cancer using Severus, a newly available somatic SV caller for lr-GS. Methods: DNA was isolated from fresh or fresh frozen samples from 34 pediatric cancers (26 heme and 8 solid) previously tested clinically for SVs, 28 of which were positive for clinically relevant (i.e., oncogenic) SVs (crSVs) expected to result in a gene fusion. The estimated tumor content for these cases, per pathology review, was 25-98%. Briefly, libraries were prepared using PacBio’s SMRTbell Prep Kit 3.0, and Revio sequencing was performed to an average depth of 30x. Reads were aligned to GRCh38 and processed with Severus v1.6.0 in ‘tumor-only’ mode. Data filtering parameters included a pediatric crSV gene-focused panel , a minimum supporting read count of 4, and the location of the breakpoint relative to the nearest gene. All crSVs detected by Severus were inspected in IGV and compared with orthogonal clinical results for each patient. To establish the assay’s limit of detection, DNA from a neoplastic sample with a crSV was diluted at different ratios with DNA from the same patient’s germline sample. Assay precision was assessed by sequencing the same sample with a crSV across two different runs performed on different days. All resulting data was analyzed as above. Results: Severus detected 25 out of 25 crSV-positive cases that were above the limit of detection established for this assay (30% tumor); the results were concordant with prior laboratory testing, leading to an accuracy of 100%. In some cases, the breakpoint resolution offered by lr-GS identified additional rearrangement complexity that helped clarify orthogonal test results. Across the 34 samples (28 positive + 6 negative), no false positive SVs were identified, yielding an analytical specificity of 100%. In the dilution sample (84% tumor), the SV remained detectable down to an ∼13% observed VAF, leading to a conservative reporting cutoff at ~15% VAF (supportive of the 30% tumor minimum requirement). The assay demonstrated 100% precision, as a re-sequenced sample processed with different technologists, reagent lots, and SMRT cell detected the expected crSV consistently across both runs. Conclusion: Reliable detection of crSVs is crucial for supporting or refining diagnoses, predicting the prognosis, and determining effective treatment strategies for pediatric cancer. The validation performance characteristics presented here demonstrate that Severus can be successfully implemented in clinical settings for effective targeted crSV detection. This approach also enables precise breakpoint resolution for improved interpretation of complex or cryptic crSV events, with the potential to expand from a targeted assay to a genome-wide SV assessment tool. Furthermore, lr-GS offers the benefits of SNV and methylation information that, although not explored in the present study, hold great future potential for additional profiling of these samples from a single data source.

  • Lessons learned: Targeted RNA sequencing as a tool for resolving variants of uncertain significance by Allison Kalinousky, Vitoria Paolillo, Florencia Del Viso, Elena Repnikova, Ana S A Cohen, Joseph Alaimo, Emily Farrow, Carol J. Saunders, and Isabelle Thiffault

    Lessons learned: Targeted RNA sequencing as a tool for resolving variants of uncertain significance

    Allison Kalinousky, Vitoria Paolillo, Florencia Del Viso, Elena Repnikova, Ana S A Cohen, Joseph Alaimo, Emily Farrow, Carol J. Saunders, and Isabelle Thiffault

    Our study demonstrated the utility of RNA studies in providing sufficient evidence to reinterpret VUS in a small cohort of clinically diverse pediatric patients who received ES/GS-based testing. Fifteen patients (68%) received an updated clinical report. Notably, in 59% the RNAseq data resulted in a molecular diagnosis. Moreover, ~47% of our positive variants were not located at splicing junctions, and therefore, would most likely not be flagged as a likely candidate in ES/GS analysis pipelines. While most variants with low TPM in blood failed, two positive variants had TPM less than 0.1, indicating that while TPM values can help narrow down which variants could be reflexed to targeted RNAseq, these values and in silico splicing predictions have significant limitations. As the cost of sequencing continues to decrease, findings of this diagnostic study demonstrate that the ability to perform targeted RNAseq concurrently with DNA sequencing represents an important advancement in genetic testing by improving classification of variants, therefore, improving the diagnostic yield.

  • Prospective Validation Of Selective Serotonin Reuptake Inhibitor Pharmacokinetic Models For Precision Dosing In Children And Adolescents by Ethan A. Poweleit; Whitney Sparks MS; Catherine Koertje; Addison Pritchett MS; Gail Robertson; Lindsey Malloy-Walton; Stephani Stancil; Jeffrey R. Strawn; Alexa L. Pagano; Kathryn Kyler; Jonathan B. Wagner; Brandon Retke; Paul C. Toren; J Steven Leeder PharmD, PhD; and Laura B. Ramsey

    Prospective Validation Of Selective Serotonin Reuptake Inhibitor Pharmacokinetic Models For Precision Dosing In Children And Adolescents

    Ethan A. Poweleit; Whitney Sparks MS; Catherine Koertje; Addison Pritchett MS; Gail Robertson; Lindsey Malloy-Walton; Stephani Stancil; Jeffrey R. Strawn; Alexa L. Pagano; Kathryn Kyler; Jonathan B. Wagner; Brandon Retke; Paul C. Toren; J Steven Leeder PharmD, PhD; and Laura B. Ramsey

    Background: Selective serotonin reuptake inhibitors (SSRIs) are commonly prescribed to children and adolescents with anxiety and depression. However, their clinical use is complicated by marked interindividual variability in exposure, which contributes to differences in treatment response and side effects. Further, SSRIs are commonly dosed using a trial-and-error approach. Model-informed precision dosing could improve treatment outcomes, yet existing SSRI population pharmacokinetic (PK) models have not been prospectively validated and may not generalize to pediatric patients. This study aims to evaluate how accurately these models predict exposure for three SSRIs—escitalopram (ESC), sertraline (SRT), and fluoxetine (FLX)—and refine them to develop tools for dose individualization. Methods: This is an open-label prospective study with dense PK sampling in participants (6-18 years old) prescribed ESC, SRT, or FLX at steady state. Following two weeks of adherence monitoring, participants complete a 24-hour PK study visit where ≤18 blood samples are collected across the dosing interval to measure SSRI plasma concentrations by liquid chromatography mass spectrometry. Published models are implemented into NONMEM to generate individual predicted concentrations from observed trough concentrations to mimic therapeutic drug monitoring. The models are considered validated if the mean ratios of predicted-to-observed Cmax, AUC24, and clearance were within 0.8-1.25. Results: A total of 8 ESC, 8 SRT, and 3 FLX/norfluoxetine (NFLX) models were included for validation. There were 4 ESC, 2 SRT, and 11 FLX/NFLX participants with measured concentrations from interim analysis. Model validation showed 5/8 (63%) of ESC, 0/8 of SRT, and 1/3 (33%) FLX/NFLX models (both parent and metabolite) met full acceptance criteria for all three endpoints, with some models meeting partial acceptance criteria (Figure). Conclusions: Prospective external validation shows several models met the a priori validation criteria for the ESC and FLX/NFLX cohorts. Additional participants in each drug cohort are needed to verify the models are fit for purpose. These findings provide a foundation for future model refinement and suggest that model-informed precision dosing could reduce trial-and-error prescribing and improve SSRI tolerability and efficacy in youth.

  • Asthma Phenotypes and Control in 22q11.2 Deletion Syndrome: A Single-Center Retrospective Study by Cody Shopper, Katey Campbell, and Nikita S. Raje

    Asthma Phenotypes and Control in 22q11.2 Deletion Syndrome: A Single-Center Retrospective Study

    Cody Shopper, Katey Campbell, and Nikita S. Raje

    Background: Patients with 22q11.2 deletion syndrome have increased rates of atopy and immune dysregulation and may experience a higher burden of asthma; however, real-world data describing asthma control, severity, and treatment patterns in this population are limited. We hypothesized that asthma control and severity patterns in patients with 22q11.2 deletion syndrome differ from those observed in the general asthma population. Objectives/Goal: To characterize asthma control, severity, and treatment patterns among patients with 22q11.2 deletion syndrome and to compare observed patterns with those typically reported in the general asthma population. Methods/Design: We conducted a retrospective observational cohort study of patients with 22q11.2 deletion syndrome and a provider-diagnosed history of asthma at a single tertiary care center. Data was extracted from electronic medical records and included demographics, asthma severity classification, Asthma Control Test (ACT) scores, oral corticosteroid (OCS) use, inhaled controller therapies, comorbid pulmonary conditions, hospitalizations, biologic therapy use, and asthma-related mortality. The primary outcome was asthma control, defined as a mean ACT score < 20 and/or receipt of two or more OCS courses in the preceding 12 months. When available, the three most recent ACT scores were averaged from the most recent assessment. Results: Of our 512 patient cohort, 92 patients (18%) with 22q11.2 deletion syndrome were diagnosed with asthma. Overall, 20% met criteria for uncontrolled asthma at their most recent evaluation. Among the cohort, 23% had moderate or severe persistent asthma. Eight percent required two or more courses of oral corticosteroids in the prior 12 months. ACT scores were available for 50% of patients; among those with documented ACT data, 28.3% had a mean ACT score < 20. Patients were more frequently treated with medium-dose inhaled corticosteroids alone (n=24) compared with combination inhaled corticosteroid–long-acting beta agonist (ICS–LABA) therapy (n=12). No patients received biologic therapy, and there were no asthma-related deaths. Conclusions: In this single-center cohort of patients with 22q11.2 deletion syndrome, nearly one in five were diagnosed with asthma. Further, one in five individuals with asthma demonstrated uncontrolled disease, and nearly one-quarter had moderate to severe persistent asthma. Despite this, combination ICS–LABA therapy and biologic agents were infrequently utilized. These findings suggest a potential treatment gap and raise important questions regarding asthma phenotype, pulmonary comorbidities, and optimal controller strategies in this population. Future prospective studies incorporating pulmonary function testing, inflammatory biomarkers, and standardized asthma assessments are needed to better characterize disease mechanisms and improve asthma outcomes in patients with 22q11.2 deletion syndrome.

  • Improvements in EEG tracing following initiation of cannabidiol: a case report by Katherine Horman, Ara Hall, and Kailash Pawar

    Improvements in EEG tracing following initiation of cannabidiol: a case report

    Katherine Horman, Ara Hall, and Kailash Pawar

  • Perioperative anaphylaxis in a healthy adolescent after first lifetime surgery by Shayan Sazdar and Salman Aljubran

    Perioperative anaphylaxis in a healthy adolescent after first lifetime surgery

    Shayan Sazdar and Salman Aljubran

    Introduction: Perioperative anaphylaxis is a rare life-threatening event. Its evaluation poses complex considerations inherent to the use of multiple medications. Patient presentation: A healthy 11-year-old boy developed anaphylaxis shortly after discharge home from first lifetime surgery for orchiopexy. Perioperative medications included rocuronium, propofol, fentanyl, bupivacaine, sugammadex, ondansetron, dexamethasone, dexmedetomidine, and ketorolac. No antibiotics. After surgery, he consumed fast food dinner and ice cream. Within 2 hours after discharge home, he returned to our Emergency Department (ED) with facial and tongue angioedema, hives, and respiratory distress. He received multiple doses of intramuscular epinephrine and briefly required epinephrine drip in the ED. Tryptase was 3.9. He was admitted to the general pediatric service for 24-hour observation. Due to uncertain recall of general tree nut consumption, tree nut panel was obtained which was non-contributory. He had skin prick and intradermal (ID) testing to all suspected medications used intraoperatively except for ketorolac and dexmedetomidine. ID testing was positive for rocuronium, otherwise negative. Since his event, he tolerated ibuprofen, acetaminophen, methylprednisolone and ondansetron. Additionally, he passed in clinic challenge to oral ketorolac. Discussion: In perioperative anaphylaxis, the most common offending agents are antibiotics and neuromuscular blocking agents (NMBAs). With positive intradermal testing, rocuronium is the most suspected agent for this patient and will need to be avoided along with other NMBAs. Challenges to other medications with negative testing were recommended to be done in the operating room. Patient outcomes: This case highlights the importance of careful review of history and appropriate medication skin testing in evaluating perioperative anaphylaxis.

  • Quantitative Echocardiography Markers to Denote Severity of Tricuspid Regurgitation Compared to Cardiac MRI in Single Right Ventricle Variants by Tony Zunica, Daniel Forsha, Janelle Noel-Macdonnell, and Anmol Goyal

    Quantitative Echocardiography Markers to Denote Severity of Tricuspid Regurgitation Compared to Cardiac MRI in Single Right Ventricle Variants

    Tony Zunica, Daniel Forsha, Janelle Noel-Macdonnell, and Anmol Goyal

    Background: Single right ventricle and Hypoplastic Left Heart Syndrome (HLHS) are congenital heart diseases that require multiple palliative surgeries to create a single right ventricular system. 25-30% of these patients develop significant tricuspid regurgitation (TR), which is associated with worse outcomes among all stages of palliation. Echocardiography remains the cornerstone imaging modality for serial evaluation in these patients. However, estimating tricuspid regurgitation is highly subjective in the pediatric population, necessitating need for reliable and reproducible quantitative echocardiography markers. Currently, cardiac magnetic resonance (CMR) is considered the gold standard for quantification of tricuspid regurgitation with good accuracy and reproducibility. Objectives/Goal: Our primary aim was to identify quantitative markers of determining TR severity using echocardiography that correlated with CMR between the final two palliative stages. Methods/Design: This was a single-center, retrospective study performed at Children’s Mercy Hospital. Inclusion criteria included patients with single systemic right ventricle with a systemic tricuspid valve who have undergone an echocardiogram within 6 months of their CMR. All other congenital heart diseases were excluded. Patient characteristics included age, sex, body surface area, diagnosis, heart rate with echocardiography and CMR and time between echocardiography and CMR. Multiple echocardiographic variables were recorded including but not limited to Nyquist limit, TR continuous wave doppler density, shape and volume time integral (VTI), vena contract width, jet number, proximal isovelocity surface (PISA) radius, leaflet morphology and right ventricular functional indices. Tricuspid valve regurgitation fraction (TRF) by CMR was recorded and categorized as mild (≤15%) or greater than mild (>15%). Each quantitative echocardiographic variable was correlated with the two groups as estimated by CMR. P value < 0.05 was considered statistically significant. Results: 27 patients met inclusion criteria. Mean age was 3.8 ± 0.7 years. Majority were male (20, 74%) with HLHS being the most common diagnosis (21, 78%). Average heart rate was 89 bpm. Average time between echocardiography and CMR was 31 days (0-112 days). Nyquist limit ranged between 42 to 123 cm/s, with mean of 82.4 cm/s. 20 patients (74%) were characterized as mild based on CMR TRF. Vena contracta width (0.61-0.76 for greater than mild vs 0.17-0.35 for mild, p< 0.001), TR continuous wave VTI (76.8-143.9 for greater than mild vs 15.7-69.3 for mild, p = 0.021) and PISA radius (0.51-0.89 for greater than mild vs 0.21-0.40 for mild, p = 0.006) were statistically significantly higher in the greater than mild category. Calculated indices such as effective regurgitation orifice area and regurgitation volume were statistically significantly higher in greater than mild category (p=0.016 and 0.007, respectively). Conclusions: Although sample size is small, our study highlights echocardiographic markers such as vena contracta width, PISA radius, and associated calculated indices that can potentially be used to better estimate severity of TR in HLHS variants. Future large-scale studies are needed to further evaluate this correlation, which may lead to more objective methods of estimating TR using echocardiography, leading to improved management decisions and outcomes.

  • Current practices of anticoagulant utilization in pediatric patients with acute myeloid leukemia (AML) by Abbey Elsbernd, N Grills, J Antoon, Kathryn Kyler, L Orth, T Savage, M Hall, Jennifer Goldman, and Shannon Carpenter

    Current practices of anticoagulant utilization in pediatric patients with acute myeloid leukemia (AML)

    Abbey Elsbernd, N Grills, J Antoon, Kathryn Kyler, L Orth, T Savage, M Hall, Jennifer Goldman, and Shannon Carpenter

  • Evaluation for non-accidental trauma (NAT) and bleeding disorders in infants referred to pediatric hematology by Abbey Elsbernd, Lyndsey Hultman, and James Anderst

    Evaluation for non-accidental trauma (NAT) and bleeding disorders in infants referred to pediatric hematology

    Abbey Elsbernd, Lyndsey Hultman, and James Anderst

  • A Case Report: Ansa Pancreatica as a Cause of Recurrent Pancreatitis by Chase Branstetter, Elizaveta Khenner, Victoria Sarata, and Nadia Ibrahimi

    A Case Report: Ansa Pancreatica as a Cause of Recurrent Pancreatitis

    Chase Branstetter, Elizaveta Khenner, Victoria Sarata, and Nadia Ibrahimi

  • A Rare Case of Ogilvie’s Syndrome in a Pediatric Patient with a Complex Medical History by Kirby Lampe, Victoria Sarata, and Aileen Har

    A Rare Case of Ogilvie’s Syndrome in a Pediatric Patient with a Complex Medical History

    Kirby Lampe, Victoria Sarata, and Aileen Har

    Acute colonic pseudo-obstruction (ACPO) or Ogilvie’s syndrome is an acute dilatation of the small or large bowel without an obstructive lesion. Symptoms include abdominal distension, nausea and vomiting, constipation and occasional dyspnea. The disorder most commonly involves the cecum and right colon and is most frequently seen in male patients above the age of 60 (1). It is rare for Ogilvie’s to occur in the pediatric population though some infrequent instances have been reported. Here, we report a case of a patient with significant abdominal distension and gaseous distension seen on x-ray, which was diagnosed and treated as Ogilvie’s syndrome. He was unresponsive to conservative and medical management and ultimately required endoscopic decompression.

  • Intravenous sotalol in pediatric cardiac intensive care patients compared to other antiarrhythmics: insights from a multicenter database by Rohit S. Loomba, Emily R. Backes, Juan Farias Torres, Alejandro Borquez, Lindsey Malloy-Walton, and Saul Flores

    Intravenous sotalol in pediatric cardiac intensive care patients compared to other antiarrhythmics: insights from a multicenter database

    Rohit S. Loomba, Emily R. Backes, Juan Farias Torres, Alejandro Borquez, Lindsey Malloy-Walton, and Saul Flores

    Background: Sotalol is an antiarrhythmic for which an intravenous (IV) form became available in 2015. Studies suggest it is safe and effective for treating pediatric arrhythmias, with fewer effects on physiologic parameters and cardiac output than amiodarone, and it does not appear to be associated with its long-term risks. However, data in pediatric patients remains limited. This study aimed to characterize the use of intravenous sotalol in pediatric intensive care admissions and compare its use and outcomes with intravenous amiodarone and procainamide. Methods: Data were drawn from the Pediatric Health Information System across six hospitals with the highest IV sotalol use (October 2015 to December 2019). Included were admissions of pediatric patients who received intravenous sotalol, amiodarone, or procainamide. Collected variables included demographics, arrhythmias, congenital heart disease, length of stay, and mortality. Trends were analyzed by year. Multivariable regressions were conducted to model length of stay and mortality with various clinical variables and antiarrhythmic medications included as independent variables. Results: Of 927 admissions, 128 (14%) received sotalol, 660 (71%) amiodarone, and 139 (15%) procainamide. Cardiac surgery was least common in the sotalol group (p< 0.01). Sotalol and amiodarone use both increased over time (p=0.03 and p< 0.01) (Figure 1). Supraventricular tachycardia was the most common arrhythmia (Table 1). Mean length of stay (days) was shortest with procainamide (21.8), followed by sotalol (22.7), and amiodarone (29.5) (Figure 2). Inpatient mortality was lowest with sotalol (5.4%) and highest with amiodarone (13.1%) (p=0.02) (Figure 3). Regression analysis demonstrated no independent association between sotalol and length of stay (β = -0.2, p=0.83), while amiodarone was independently associated with increased length of stay (β = 8.4, p< 0.01). Sotalol was linked to decreased mortality (OR = 0.95, p=0.04) and amiodarone with increased mortality (OR = 1.1, p< 0.01). Procainamide showed no significant associations. Conclusion: Intravenous sotalol use in pediatric intensive care patients was associated with lower inpatient mortality and comparable length of stay compared to amiodarone and procainamide after regression analyses. These results support that intravenous sotalol is a safe alternative for pediatric arrhythmia management, meriting further prospective studies.

  • Juvenile Polyp and Lymphoid Hyperplasia “Leads” to Intussusception by Rasheed Ansari, Kirby Lampe, and Ding-You Li

    Juvenile Polyp and Lymphoid Hyperplasia “Leads” to Intussusception

    Rasheed Ansari, Kirby Lampe, and Ding-You Li

    OP is an 8-year-old female who presented to the ED with 1 day of NBNB emesis and abdominal pain. • The CT abdomen showed colocolonic intussusception at the hepatic flexure with a possible polypoid lesion serving as a lead point. • Radiology did not recommend air enema for reduction given atypical age, atypical location, and visualization of lead point. • GI consulted for a possible colonoscopy to reduce intussusception. Colonoscopy contraindicated due to polypoid mass causing obstruction and inability for patient to tolerate cleanout. • Diagnostic laparoscopy performed. Noted Intussusception in the distal ascending colon/proximal transverse colon. Chronic inflammatory changes noted and significant lymphadenopathy. • Surgeon performed right hemicolectomy with ileocolic anastomosis. OP is an 8-year-old female who presented to the ED with 1 day of NBNB emesis and abdominal pain. • The CT abdomen showed colocolonic intussusception at the hepatic flexure with a possible polypoid lesion serving as a lead point. • Radiology did not recommend air enema for reduction given atypical age, atypical location, and visualization of lead point. • GI consulted for a possible colonoscopy to reduce intussusception. Colonoscopy contraindicated due to polypoid mass causing obstruction and inability for patient to tolerate cleanout. • Diagnostic laparoscopy performed. Noted Intussusception in the distal ascending colon/proximal transverse colon. Chronic inflammatory changes noted and significant lymphadenopathy. • Surgeon performed right hemicolectomy with ileocolic anastomosis.

  • Refractory Benign Esophageal Strictures Complicated by Esophageal Perforation and Treated with Serial Dilations and Stent Placement in a Pediatric Patient by Kirby Lampe, Victoria Sarata, and Nadia Ibrahimi

    Refractory Benign Esophageal Strictures Complicated by Esophageal Perforation and Treated with Serial Dilations and Stent Placement in a Pediatric Patient

    Kirby Lampe, Victoria Sarata, and Nadia Ibrahimi

    Patient is a 2-year-old male who was originally referred to GI for failure to thrive and recurrent vomiting following feeds. Upper GI showed long-segment stricture with mid-thoracic tapering and initial EGD revealed 2mmx5cm distal esophageal stricture that was balloon dilated with serial dilations and triamcinolone injections. He presented one week after his 3rd dilation with fever, vomiting and respiratory distress and was found to have cardiac tamponade, left pleural effusion, septic shock and esophageal perforation. He was transferred to the ICU and started on broad-spectrum antibiotics, IV fluids and chest tube placement. He required intubation and vasopressors. After stabilization, esophogram showed marked proximal esophageal dilation and no contrast passage into the stomach. Repeat EGD showed recurrence of the severe stenosis but no contrast extravasation. A 10 mm esophageal stent was placed to keep the esophagus patent. Serial EGDs were completed, with mitomycin C application and stents replacements for larger diameters, up to 16 mm, for a total of 3 months. Congenital esophageal stenosis (CES) is a rare but significant cause of dysphagia, aspiration and feeding difficulties in infants and toddlers. Management typically begins with endoscopic therapy with dilation being first line. Other endoscopic modalities in management of CES include endoscopic incisional therapy, esophageal stenting, intralesional steroid injection and Mitomycin C application. Esophageal perforation rates for dilation of CES are reported to be as high as 44.4%. Traditional management of esophageal perforation includes NPO, antibiotics and chest drainage in the setting of large fluid in the chest; which were effective in our patient. Other modalities include esophageal stenting and endoscopic vacuum assisted closure. Esophageal stents can also serve as a bridge to surgery or as long-term management for recurrent strictures though success rate in treating stricture has been variable and ranges from 0-86%. Stents are also associated with numerous complications such as migration, bleeding, perforation, tracheal compression and erosion into adjacent structures. Ultimately this case underscores the need for a multidisciplinary approach, close monitoring and flexible management in managing complex pediatric esophageal strictures.

  • Symptomatic Severe Hyponatremia: A Rare Complication of Double Balloon Enteroscopy by Victoria Sarata, Kirby Lampe, and Thomas M. Attard

    Symptomatic Severe Hyponatremia: A Rare Complication of Double Balloon Enteroscopy

    Victoria Sarata, Kirby Lampe, and Thomas M. Attard

    Previously healthy 11-year-old female, newly diagnosed Peutz-Jeghers Syndrome (PJS) confirmed by genetic testing STK11 PV (c.464+1dupG) underwent double balloon enteroscopy (DBE) after small bowel capsule showed multiple small intestinal polyps including >1.5 cms diameter. Pre-procedure, she completed a two-day MiraLAX clean out with >10 x 17g doses of MiraLAX each mixed in 6-8oz of water and other clear liquids. DBE completed with resection of 17 total small intestinal (SI), jejunal and ileal polyps. Procedure lasted approximately 4hrs and 5L of sterile water was used for underwater technique and irrigation.Sterile water was used for copious irrigation needed for direct visualization, and increased buoyancy of SI polyps in compliance with scope manufacturer recommendations (IFU). Patient was admitted secondary to number and size of polyps removed. Six hours post-procedure patient had hematemesis followed by a generalized tonic-clonic seizure. Abortive medications given and severe hyponatremia noted with serum sodium of 119mEq/L . Patient transferred to the ICU and treated with 3% hypertonic saline and sodium normalized over several hours. Patient discharged home with several stable sodium levels and no neurologic sequelae. This case represents the rare but potentially life- threatening complication of post-endoscopic symptomatic hyponatremia in a previously healthy patient. In adults, this is typically associated with excessive free water intake, older age, or in the context of unknown underlying comorbidities.

  • The Use of Omalizumab For Idiopathic Isolated Angioedema In A Pediatric Patient by Manali Shah and Thao Le

    The Use of Omalizumab For Idiopathic Isolated Angioedema In A Pediatric Patient

    Manali Shah and Thao Le

    Introduction Angioedema manifests as localized, asymmetric, nonpruritic cutaneous and/or mucosal swelling. The two main clinical forms are histaminergic and bradykinin mediated angioedema. Histaminergic is more common and presents with hives, and responds to antihistamines, steroids, and epinephrine. Bradykinin mediated angioedema lacks hives and does not respond to these therapies. Ten percent of patients with recurrent angioedema have isolated angioedema without urticaria. Case Description A previously healthy 8-year-old male presented initially to the ED evaluation of isolated lip swelling and no other systemic symptoms. There was no exposure to new foods, medications, or illness symptoms. He received anti-histamines and steroids with improvement in his symptoms and referred to Allergy clinic for further evaluation. Lab evaluation with CBCD, BMP, LFTs, inflammatory markers, thyroid studies, ANA, hereditary angioedema, and tryptase were within normal limits. He continued to have recurrent episodes of swelling despite high dose second-generation antihistamines and H2 antagonist. He was started on omalizumab for idiopathic isolated angioedema, with resolution of his symptoms after the second dose. He has been able to stop all anti-histamines without recurrence of symptoms. Discussion This case highlights the off-label use of omalizumab for idiopathic isolated angioedema and demonstrates improvement in symptom control. Isolated angioedema in the absence of urticaria is rare but still warrants a thorough evaluation as management may differ. Isolated angioedema can be difficult to treat as it may not respond to standard therapy. Omalizumab may be effective for these patients and can be considered as a treatment option for recurrent idiopathic isolated angioedema.

 

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