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  Oral Surgical Needs in a Pediatric Hospital Dental Clinic

  Meredith Clark, Richard Nguyen, Kaitlin Carlson, Brenda S Bohaty, Amy Burleson, Robin Onikul, Neena Patel, and Jenna Sparks

  Purpose: The aim of this study is to evaluate and assess the oral surgery needs in a pediatric hospital dental clinic at Children’s Mercy Hospital in Kansas City. Methods: Following IRB approval, appointments over a 6-month time-period were reviewed and recorded when a referral was placed to an oral surgeon. The reason for referral, patient age and medical history were recorded and analyzed. Results: Half of the patients needing oral and maxillofacial procedures were between the ages of 8-10 years old. There was an equal number of patients needing extractions due to carious/non-restorable teeth with and without special health care needs. Two-thirds of the patients were referred from an outside provider to Children’s Mercy Hospital. Conclusion: Due to the oral surgery needs of patients that are seen in the Children’s Mercy dental clinic, it would be beneficial to the patient population to have an oral surgeon on staff. This would provide better access to care by eliminating barriers in the referral process and reducing time to treatment completion.

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  Physician Use of Progesterone in transgender adolescents: A survey

  Samhita Bhattarai and Michelle Knoll

  Objectives To evaluate the current perspective and clinical practices of physicians regarding the use of synthetic progestins as an adjunct to estrogen therapy in transfeminine adolescents. The survey focuses on perceived benefits, risks and overall efficacy in gender affirming hormone therapy. Methods A cross sectional survey was conducted among physicians who care for transfeminine patients. The survey included questions regarding their specialty, practice setting, age range of patients. Data was also collected regarding their indication for use, perceived benefits (e.g. breast development) and rationale if not using progestins for this purpose. Results Of 110 physicians who participated in the survey, 85 (77.2%) endorsed providing gender affirming care. Of those 85 respondents, 71(83.5%) have used progestins in some way, and 57 (67%) have used them as an adjunct to estrogen. Other uses included for contraception or amenorrhea in transmasculine patients, for suppression of endogenous puberty, and to induce patient requested hormone cycling. The most common form of progesterone used was oral progestin at 80.2% while depo medroxyprogesterone was used 21.1% Majority of the physicians (46/57, 80.7%) responded to being either somewhat satisfied or neutral to use of progestins. Of those who do not use progestins in transfeminine patients, most respondents noted lack of data on efficacy and side effects or lack of recommendations in guidelines. Others noted that it has not been necessary in achieving desired outcomes. Conclusions The survey indicates that while a significant number of physicians who practice gender affirming care incorporate progestins into hormone therapy for transgender youth, there was interest in more data on benefits and side effects on their use in transfeminine patients. Further research is needed to add to the current WPATH guideline to ensure more patient centric care along with ensuring its safety and effectiveness.

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  Primary Molar Loss and Resulting Space Outcomes: a Systematic Review

  Kaitlin Carlson, Meredith Clark, Richard Nguyen, Brenda S Bohaty, Amy Burleson, Robin Onikul, Neena Patel, and Jenna Sparks

  Premature loss of a primary molar can occur due to a multitude of reasons, including caries, infection, and trauma. When this occurs and restoration of the affected primary tooth is not possible, the AAPD recognizes space maintenance as a “Best Practice” to prevent loss of arch length, width, and perimeter after extraction. However, reports suggest that with proper intercuspation of first permanent molars, space loss from premature loss of a primary molar is minimal and a space maintainer in that area may not be needed. The clinical management for such cases has been controversial across pediatric dental providers, with factors such as timing, behavior, and age all playing large roles in making treatment planning complex. This systematic review is aimed at reviewing the current scientific literature to evaluate the need for a unilateral space maintainer after premature loss of a primary molar in the mixed dentition stage. For sake of completion, studies that looked at loss of first primary molars and/or second primary molars were included. Bilateral space maintenance, such as lower lingual holding arches or Nance appliances, is not investigated in this review due to its relative effectiveness across clinical cases. A systematic review is needed to help guide clinicians’ decision making and provide clarity for management in the currently ambiguous scenario of the premature loss of a primary molar in the mixed dentition stage.

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  Acceptability of a Brief Acceptance and Commitment Therapy Intervention for Caregivers of Children with Pediatric Feeding Disorder

  Emma Murray, Taylor P. Harris, Annie L. Ryder, Alexandra Zax, Taylor M. Datillo, Corey Schurman, Laura Slosky, Sarah T. Edwards, Delwyn Catley, Ann M. Davis, and Dana Bakula

  Introduction: Pediatric feeding disorder (PFD) affects 1 in 37 young children and is associated with heightened stress and anxiety among caregivers. Currently, there are no established interventions that target PFD caregiver stress, and traditional models of adult mental healthcare face feasibility issues in pediatric care settings. To address this gap, we developed a brief acceptance and commitment therapy (ACT) intervention (Clinical Trials #NCT06001398). We assessed caregiver-perceived acceptability to inform future efficacy testing. Method: Twenty caregivers participated in a NIMH-funded pilot study of a 2-session ACT intervention for parents of children with PFD. Caregivers with children aged 2 to 6 years with PFD were recruited and randomized to either the active (n = 14) or control intervention (n = 6). Intervention content included psychoeducation, ACT skills training, and application of ACT skills to mealtimes. Caregivers’ rated intervention acceptability at 3-month follow-up. Results: Eight caregivers who received the ACT intervention completed acceptability measures. Most (75%) found it “somewhat” or “very” helpful. 62% reported using the ACT strategies 3 months following intervention completion and 88% said they would recommend this program to other parents. Most (88%) reported the same level of confidence in managing their own stress as prior to the intervention. Descriptively, caregivers enjoyed the sessions (“I enjoyed the sessions and just being more mindful of how my own stress was impact[ing] our mealtimes”). Conclusions: Caregivers found this brief ACT intervention to be highly acceptable. Caregivers reported regular use of the strategies, positive perceptions of the program, and that they would recommend it to others. Interestingly, they did not report feeling that their confidence in managing their stress improved. It is possible that retrospective comparisons about stress management may be difficult to assess, making it critical that we complete rigorous efficacy testing with pre-post measures of stress. Further efficacy testing is needed.

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  AHT Diagnoses: The Influences of Clinical Presentation and Evidence of Impact

  Ruairi Smith-Dewey, Angela Bachim, Colleen Bressler, Kristine Campbell, Lori Frasier, Christopher Greeley, Danielle Horton, Nicole Johnson, Jan Leonard, Megan Letson, Caitlin McNamara, Ashley K. Sherman, Shalon Nienow, Joanne Wood, and James Anderst

  In cases where Child Abuse Pediatricians (CAPs) diagnose abusive head trauma (AHT) in young children, some factors surrounding the diagnosis have been challenged including causative shaking, necessity of external signs of impact, or concomitant suspicious injuries. Evaluation of CAP diagnoses in children with subdural hemorrhage (SDH) can give insight into factors related to the frequency of abusive head trauma (AHT) diagnoses. Additionally, characterization of those with SDH+CSI without evidence of impact may inform controversies around AHT diagnoses. Objective We aim to compare CAP diagnoses in subjects with SDH across clinical presentations and in the context of evidence of head impact. We also aim to describe the subjects with SDH+CSI and no external signs of impact to the head. Design/Methods Retrospective case-control study using Child Abuse Pediatrics Research Network (CAPNET) data from 10 sites over 27 months. Subjects were < 24 months old with SDH and in-person CAP evaluation. Subjects were divided into groups based on study determined criteria: Ill SDH only, Non-ill SDH only, and SDH with CSI. Ill status was categorized by clinical signs of cardio-respiratory collapse and/or significantly altered consciousness. CSIs are injuries highly associated with abuse in the following categories: skin, bones, eyes, and abdomen. CAP diagnosis of abuse was defined as 5-7 on a 7-point scale. Abuse diagnoses were compared across study groups. Head impact signs included bruising to the head, scalp swelling, and skull fractures. Comparisons used unadjusted odds ratios with 95% confidence intervals. Results Of the 492 study subjects, 386 (78%) were diagnosed with AHT. The SDH+CSI subjects were more likely to be diagnosed with abuse than Ill-SDH (OR 2.7, 95% CI 1.3-5.6) and Non-ill SDH (OR 7.2, 95% CI 4.3-12.1). Of the 276 subjects without signs of head impact, 215 (78%) were diagnosed with abuse. In this subgroup, SDH+CSI were more likely to be diagnosed with abuse than Ill-SDH (OR 3.7, 95% CI 1.5-9.2) and Non-ill SDH (10.3, 95% CI 5.0-21.1). Of the 157 subjects with SDH+CSI and no signs of head impact, 88/157 (56%) had non-head/eye related CSIs, including 52/88 (59%) with high specificity fractures and/or multiple fractures, 48/88 (55%) with high specificity bruising. Multiple types of CSIs were in 22/88 (25%). Conclusion(s) Among children with SDH, AHT is more frequently diagnosed in the presence of CSIs, particularly in the absence of external signs of impact. The high prevalence of CSIs in cases with SDH and no external signs of impact indicates that AHT can occur absent external signs of head impact.

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  A Risk Score to Predict Recurrent High Intensity Inpatient Resource Utilization for Children with Medical Complexity.

  Charles Maloy, Matt Hall, Jessica L. Bettenhausen, YR Chan, Leah Jones, Kathryn Kyler, Laura Plencner, Jessica Markham, Jacqueline Walker, Smit Shah, Sian Best, Jonathan Ermer, Shelby Chesbro, Maria Newmaster, and Hank Puls

  Background

  Children with medical complexity (CMC) use a greater amount of inpatient resources and stand to benefit from unique care models and coordination. However, hospital resources can be limited, and a better understanding of which CMC are high resource users could improve hospital and staff planning.

  Objective:

  To develop a risk score predictive of recurrent high intensity inpatient utilization among CMC.

  Methods:

  We conducted a retrospective cohort study of children < 18 years old with ≥3 complex chronic conditions (CCC) discharged from one of 48 children’s hospitals in the Pediatric Health Information System during 2021 and 2022. To reflect CMC typically cared for by a general medical service, children with cardiac procedures, dialysis, oncology, or transplant CCCs, and index hospitalization mortality were excluded. We used multivariable logistic regression to determine factors present on the index hospitalization associated with ≥5 hospitalizations or ≥3 hospitalizations with one lasting ≥25 days within any 365-day period (high inpatient utilization) in 2021-2023. We developed risk scoring system using model coefficients and defined the optimal risk score cutoff to maximize sensitivity and specificity. We then applied the risk score cutoff to children discharged to determine (a) ratio of predicted:actual CMC with recurrent high resource use and (b) the total predicted daily volume on 50th, 75th, and 90th percentile days for each hospital.

  Results:

  Of 44,432 included children , 9,596 (21.6%) had high inpatient utilization (Table). Children under 1 year of age had the highest odds of high inpatient utilization (OR 2.26 [95% CI: 2.16, 2.37]) and all CCC and technology groups (except metabolic) met statistical significance (Figure 1). The optimal risk score cutoff was 29 and predicted 17,411 (39.2%) CMC as high inpatient resource utilizers; the predicted: actual ratio of CMC with high inpatient utilization was 1.81. The mean daily census of the 50th, 75th, and 90th percentiles across hospitals was 28 CMC with recurrent high utilization (± 17), 34 (±18), and 39 (±19; Figure2).

  Conclusions:

  It is feasible to develop a risk score predictive of recurrent high inpatient utilization among CMC. There is substantial variability in volume of CMC with high utilization between pediatric hospitals. The risk score cutoff values can be modified to suit different applications and institution-specific objectives. These applications could include a dedicated service line or staffing. Alternatively, this could identify patients who would benefit from more formal handoff from inpatient to outpatient providers.

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  Decreasing time to narcotic pain medication in patients with sickle cell disease presenting with acute pain crises in a pediatric emergency department: a quality improvement initiative

  Allison Adam, Lina Patel, Christopher Kaberline, Ibad Siddiqi, and Leslie Hueschen

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  Facilitators, Barriers, & Recommendations for Pediatric Research Sites Conducting Decentralized Clinical Trials (DCTs)

  Abby Kietzman

  The COVID-19 pandemic drastically altered the way clinical research is conducted through a widespread adoption of telemedicine practices to continue clinical trial procedures during peak periods of global COVID-19 spread. Using telehealth/telemedicine services allows researchers to conduct “decentralized clinical trials”, or DCTs, for all or portions of study protocol procedures while maintaining safety measures implemented for pandemic control. However, research sites are hesitant to adopt DCT policies due to concerns around digital health technology, data safety and quality, and limited information in the literature specifically assessing telemedicine use for pediatric clinical research via DCTs or how equipped pediatric research sites are for the transition away from the site. A cross-sectional, observational casestudy surveying clinical research professionals (CRPs) at a local pediatric institution in Kansas City, MO, was conducted to better understand pediatric site facilitators and barriers to implementation and utilization of DCTs from a site perspective.

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  In Search of the Unknown Mimic of Abusive Head Trauma (AHT)

  Ruairi Smith-Dewey, Angela Bachim, Colleen Bressler, Kristine Campbell, Lori Frasier, Danielle Horton, Nicole Johnson, Jan Leonard, Megan Letson, Caitlin McNamara, Shalon Nienow, Ashley K. Sherman, Stuart Sommers, Joanne Wood, and James Anderst

  Background Child Abuse Pediatricians (CAPs) are accused of misdiagnosing a yet-to-be-identified mimic as abusive head trauma (AHT), particularly in ill children with an isolated subdural hemorrhage (SDH). Though not diagnostic criteria, specific social stressors are identified as AHT risk factors. The existence of a mimic can be assessed by comparing the prevalence of AHT risk factors across study groups (See Table 1). Objective We hypothesize that the distribution of AHT risk factors will be: fewer in the CAP-identified mimic group versus the CAP-diagnosed abuse group, no difference between ill SDH only and SDH+CSI groups as these are likely the same condition, and fewer in the non-ill SDH only versus SDH+CSI groups as there are likely undocumented known mimics in the non-ill SDH only group. Design/Methods Retrospective case-control study using Child Abuse Pediatrics Research Network (CAPNET) data from 10 sites over 27 months. As proof of concept, the prevalence of AHT risk factors was compared between CAP identified mimics and CAP-diagnosed abuse groups. Social risks were compared using unadjusted odds ratios with 95% confidence intervals. For social risk comparison across SDH groups, subjects were < 24 months old and evaluated by a CAP. Ill subjects had cardio-respiratory collapse and/or significantly altered consciousness. CSIs were injuries that are highly associated with abuse in the skin, bones, eyes, and abdomen. CAP diagnosis was not considered for SDH group determination. Prevalence of social risk, demographics, and trauma history were compared between groups using two one-sided equivalence tests with a 20% equivalence margin. Results In proof of concept, 1058 subjects with known medical mimics had statistically significantly fewer AHT social risk factors than 548 abused subjects with ICH/fracture in 5/7 domains. In the main study, when comparing the 350 SDH+CSI subjects and 46 Ill SDH subjects, the two groups were statistically equivalent in 5/7 social risks. When comparing the 350 SDH+CSI and 96 Non-Ill SDH subjects, they were statistically equivalent in 2/7 social risks. Provision of trauma history was equivalent across all three groups (RD –0.01-0.10). Conclusion(s) Social risk distribution differentiates CAPNET subjects with medical mimics from those without. Social risk distribution largely did not differ between SDH+CSI and Ill-SDH groups, indicating that a yet-to-be-identified mimic is not widely prevalent in the Ill-SDH group. Non-ill appearing subjects with SDH appear to have fewer AHT risks compared to SDH+CSI, supporting possible medical causes in some of these children.

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  Maternal Guilt Following Pediatric Burn Injury

  Sabrina Ung, Abby Dugan, Casey Lawless, Mallory Netz, Alec Bernstein, and Carisa Parrish

  Background: Previous research has shown that parental guilt is a risk factor for poor parental adjustment (e.g., depression, posttraumatic stress) following a pediatric burn injury (Hawkins et al., 2019). However, few studies have examined risk factors for parental guilt. Objectives/Goal: The current study aimed to examine relationships among demographic variables, burn characteristics, maternal mental health, with maternal guilt following their child’s burn injury. Methods/Design: Mothers of 147 children who experienced a burn injury (mean child age = 6.28 years, 46% female, 44.7% White, 19.2% Black or African American, 9.6% Hispanic, 1.3% Asian, 11.9% multiracial, total body surface area range .10 – 15%) were screened using questionnaires assessing parent posttraumatic stress, depression, anxiety, and guilt during their children’s outpatient burn clinic visit. Clinical and demographic variables were retrieved retrospectively from patients’ medical charts. Descriptive statistics and linear regression analyses were conducted. Several demographic factors (e.g., child’s sex, race, insurance type) and burn injury characteristics (e.g., length of hospital stay, burn injury mechanism) were found to be nonsignificant in bivariate correlational and regression analyses and therefore were excluded in final analyses. Results: Maternal depressive symptoms (β = .23, p = .04) and posttraumatic stress related to their child’s burn injuries (β = .49, p = < .001) significantly predicted maternal guilt. Mothers of younger children were observed to experience higher levels of guilt (β = -.15, p = .03). Lastly, burn size (β = -.11, p = .14) and maternal anxiety (β = .00, p = .99) were not significant predictors of maternal guilt. Conclusions: Following a burn injury, maternal posttraumatic stress, depressive symptoms, and child age contribute to maternal guilt related to the injury regardless of burn size. Findings support integration of routine screening of caregivers’ guilt and mental health following pediatric burn injuries, regardless of burn size, to inform appropriate interventions.

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  Opportunities for Solid Organ Donation in the Neonatal Intensive Care Unit

  Sarah Pippin, Ashley Sherman, Julie Weiner, and Jotishna Sharma

  Background: Pediatric patients are at an increased risk of mortality when waiting for an organ transplant. Approximately 50% of pediatric patients wait for> a year for a solid organ transplant and infants < 1 year of age have the highest death rate while awaiting transplantation. There are two pathways for organ donation (OD), donation after death by neurologic criteria (DDNC) or donation after circulatory determination of death (DCDD). Neonates rarely meet brain death criteria, and since most deaths in the NICU occur aftera planned extubation, DCDD with organ procurement after a planned extubation from mechanical ventilation can be a consideration especially with kidney and liver donations. Organ procurement may be limited by warm ischernic time (WI1); usually :'.S 90 minutes. The question remains which neonates would meet criteria for DCDD.

  Purpose: The aim of this study is to characterize theoretical eligibility of DCDD in infants undergoing withdrawal of care in the NICU based on WIT :'.S 90 minutes

  Method: Retrospective study of a cohort of infants 2:36 weeks at the time of death who died at a level IV NICU from January 1, 2012, to December 31, 2022. Demographic information was obtained. Mode of death was categorized as withdrawal of care, withholding of care, or CPR. The withdrawal of care was further examined for eligibility for DCDD based on WIT :'.S 90 minutes.

  Results: During the study period, there were 337 infants 2': 36 weeks at the time of death and of those, 67% (226/337) infants had withdrawal of care. The most co=on cause of death was congenital anomalies. Those who met DCDD criteria were found to have significantly higher birth weight (p=< 0.01), older GA at birth (p=0.01), and older PMA at death (p=

  Conclusion: One third ofNICU deaths 2::36 weeks undergoing withdrawal of mechanical ventilation could be potential solid organ donor after DCDD with WIT ::S 90 minutes. Increased organ donation opportunities in this age group could have an impact for pediatric patients waiting for an organ transplant. Establishing a DCDD protocol in level IV NICUs should be strongly considered. Further studies are needed to determine factors that predict success of neonatal solid organ donation through DCDD.

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  Reducing Antibiotic Duration for Pneumonia and Sinusitis in the Emergency Department: A Quality Improvement Approach

  Joshua Saucedo, Leslie Hueschen, Amanda Nedved, Marsha Dannenberg, Patricia Phillips, Jennifer Floyd, Alaina N. Burns, and Rana El Feghaly

  Background: Antibiotic duration is one of the pillars of antimicrobial stewardship programs (ASP) as it helps to limit unnecessary antibiotic exposure while continuing to provide appropriate care for infections. In our emergency department (ED), clinicians are using first-line guideline-recommended antibiotics as an appropriate choice for most patients (per our ASP report tracking), however, duration continues to be prolonged especially for community acquired pneumonia (CAP) and acute bacterial rhinosinusitis (ABRS). National guidelines recommend shortening antibiotic durations to 3-5 days for CAP and 5-7 days for ABRS, however currently only 22% of patients discharged from our ED receive the recommended duration. Objective: Our aim is to increase percentage of children discharged home from the ED with a diagnosis of CAP or ABRS treated with an optimal antibiotic duration (5 days or less for CAP, 7 days or less for ABRS) from 22% to 70% by July 2025, while assessing for differences in care based on demographic and socioeconomic factors. Design/Methods: A multidisciplinary team (infectious diseases physicians, ED and urgent care physicians, advanced practice providers, nurses, and pharmacy) formed in December 2023. Outcome metric is the percentage of patients receiving recommended antibiotic duration for CAP or ABRS; data trends analyzed monthly and process changes implemented according to the SPC Out of Control Process Western Electric rule set. Process metrics include use of order set, quick discharge order set, and QR code. Balancing metric is the number of return visits with the same discharge diagnosis within 14 days. Utilizing cause-and-effect diagram, driver diagram (Figure 1), and PICK chart (Figure 2), we identified possible interventions. Plan-Do-Study-Act cycles so far have included implementation of new internal CAP, updates to electronic medical record and quick discharge order sets. A survey of prescriber comfort with shorter duration identified barriers and provided education, which has led to posting evidence for lower duration treatment to prescribers in their workrooms and developing new ABRS CPG. Results: We have shifts in the central line of our overall outcome in August 2023, May and July 2024 to 65.4% with no change in balancing metric (Figures 3A-CQBS, annotated with PDSA cycles). Discussion: We have seen a reduction in the duration of antibiotics for CAP and ABRS with electronic medical record and prescriber education interventions. Future cycles will focus on barrier mitigation and increased data transparency, with huddle board reminders and positive encouragement to providers.

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  Reducing Vancomycin in a CICU

  Karoline Krzywda, Lauren Johnson, and Allison Taber

  Introduction: In the cardiac intensive care unit (CICU) empiric vancomycin is frequently prescribed. This practice occurs without wide knowledge of the true rates of methicillin resistant staph aureus (MRSA) and methicillin resistant staph epidermidis (MRSE) infections. Given the potential for harmful side effects of vancomycin as well as concern for growing antibiotic resistance we sought to better characterize local unit prescribing practices and MRSA/MRSE infection rates to guide our quality improvement project with an aim of reducing vancomycin use in our CICU. Methods: This project is taking place at Children’s Mercy Kansas City CICU, a 24 bed unit with an average of 274 pump cases per year. Patient data from January 2021-January 2023 was analyzed. There were approximately 20-60 vancomycin days per month with a center line of 0.1, indicating that over that time frame, 10% of the patient days were vancomycin days. In 2021 there were 111 orders for Vancomycin. Using a Pareto chart, we found that a majority (52) of Vancomycin orders were for fever alone. We analyzed all positive cultures in 2021 and of the 111 vancomycin orders there were only 11 positive MRSA/MRSE infections for which Vancomycin was prescribed as a full treatment course. We aim to reduce the number of vancomycin days from 0.1 per 1000 patient days to 0.075 per 1000 patient days by May 2025. Given the mismatch between prescribing habits and true infection rates, our first test of change will be to provide education regarding MRSA/MRSE infection rates in the CICU and current prescribing habits. Additionally, we will ask that providers not order vancomycin if a patient has a fever alone without hemodynamic instability, without high or increasing inflammatory markers, has not had a heart transplant, is not on extracorporeal membrane support or a ventricular assist device or is not immunocompromised. As a balancing measure we will track MRSA/MRSE positive cultures that did not receive vancomycin prior to culture results. Results: Insufficient time has passed to evaluate the project’s first test of change. Conclusions: Vancomycin is frequently empirically prescribed with low rates of MRSA/MRSE infections in our CICU. Our first test of change is to provide education on rates of MRSA/MRSE infections and vancomycin prescribing habits in our CICU. We will be monitoring for decrease in the number of vancomycin days as well MRSA/MRSE infections in which empiric therapy with vancomycin was missed.

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  Detection of Chromosomal Mosaicism- the Importance of Karyotyping and FISH

  Allison Kalinousky, Jennifer Roberts, Madeeha Alikhan, John Herriges, Lei Zhang, and Elena Repnikova

  Introduction: The use of whole-exome/whole-genome sequencing for the detection of sequence-level variants and copy number variations (CNV) is increasingly being adopted as a primary testing method for genetic disease diagnosis by many institutions. Additionally, long-read sequencing is a promising technique to capture not only sequence level variants and CNVs but also structural chromosomal abnormalities. Significant progress has been achieved in this field; however, the detection of chromosomal mosaicism, including numerical gains and losses, rings, markers, and structural rearrangements – particularly those that are lineage-specific or exist at low levels – might still pose challenges with these emerging technologies. Furthermore, differentiating various mosaic cell lines may not be feasible. Literature review shows that chromosomal mosaicism is estimated to occur in up to 70% of human preimplantation embryos. Clinically significant chromosomal mosaicism is reported in ~3% of products of conceptions (POC), 1-2% of chorionic villus sampling (CVS), 0.1-0.3% of amniotic fluids (AF) samples, and 0.05-0.1% postnatal samples when assessed using conventional cytogenetic methods. To date, there are no studies available that provide data on the accuracy and detection levels of chromosomal mosaicism using third of fourth generation sequencing technologies. Methods: We conducted a retrospective review of patients who underwent conventional cytogenetic testing (chromosome analysis via Giemsa staining and fluorescence in situ hybridization (FISH)) for various clinical indications at Children’s Mercy Hospital from 2003 and 2023. Within this period, we received 20,866 constitutional cases of with 4,414 were cytogenetically abnormal. We also evaluated 762 cases submitted for exome sequencing with copy number analysis in 2023. We then examined all mosaic cases. Results: We found that the average number of mosaic cases each year was 29 (range: 9-39), accounting for ~3.1% of all clinical cases studied and ~14% of all abnormal cases per year. Levels of mosaicism were variable, ranging from 4 to 95%. Of all cases (both normal and abnormal) across all years, 4.7% of POCs, 4.3% of AF samples, and 2.7% of peripheral blood (PB) samples had chromosomal mosaicism. In comparison to the 762 PB exome cases, only three were mosaic (two with trisomy 9 and a ring(X)) totaling ~0.4% of postnatal cases. Numerical abnormalities are the most common form of mosaicism in our cohort, accounting for ~79% of mosaic cases. Of the numerical abnormalities, sex chromosomes are the most frequently implicated, with an average involvement of 44% in POCs, 40% in AF samples, and 79% in PB. The most common numerical abnormality of the autosomes in POCs were affecting chromosomes 22 (7%), 15 (6%), and 16 (5%); in AF samples affecting chromosomes 21 (20%), 18 (7.5%), and 22 (5%); and in PBs affecting chromosomes 21 (11%) and 18 (4%). Approximately 14% of mosaic cases harbored a structural abnormality of the autosomes. The most common abnormality in PBs involved the Y chromosome (12%), and in POCs involved chromosome 13 (19%). The structural abnormalities in AF samples affected chromosomes 3, 7, 9, 12, 14, 16, 18, and 22 at relatively equal prevalence. Discussion: Review of our data indicates a higher prevalence of chromosomal mosaicism identified through conventional cytogenetic methods across various specimen types compared to existing literature. Furthermore, our limited genomic sequencing data suggests that conventional cytogenetic technologies may detect chromosomal mosaicism more frequently than genomic technologies. These findings underscore the importance of employing karyotyping and FISH for the detection of chromosomal mosaicism, particularly at low levels, which may be missed by genomic sequencing. Additionally, a thorough examination of clinical phenotypes can enhance the suspicion of mosaicism, ultimately enhancing diagnostic rates.

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  Development of Mobility Scales for Infants and Children on ECMO

  Johanna I. Orrick, Debra E. Newton, and Caroline Holton

  Extracorporeal Membrane Oxygenation (ECMO) is a life-saving intervention commonly used in pediatric and neonatal intensive care; however, it presents unique challenges for maintaining patient mobility. Early mobility contributes to better long-term outcomes. Existing mobility scales by the Extracorporeal Life Support Organization (ELSO) are limited to patients aged 8 years and older, leaving a gap for younger populations. This study aimed to develop and validate mobility scales specifically for infants (< 2 years) and children aged 2-8 years undergoing ECMO therapy. Through a collaborative effort involving pediatric ECMO specialists, physicians, and critical care nurses, we adapted ELSO’s mobility criteria to be developmentally appropriate for these younger age groups. For infants, mobility benchmarks include passive and active limb movements, head control, and supported sitting. The criteria for children aged 2-8 is expanded to include sitting, supported standing, and age-appropriate gross motor activities. This scale provides graded levels of mobility that accommodate ECMO-specific safety considerations, allowing practitioners to chart interventions according to each child’s developmental and physiological capacity. The resulting scales are being pilot-tested at our large ECMO center, with data showing preliminary effectiveness at capturing the degree of mobility achieved during ECMO runs. This mobility scale fills a critical need by providing structured charting guidelines for mobilizing infants and young children on ECMO, promoting functional recovery and potential improvements in long-term developmental outcomes. Further studies are recommended to establish consistency across institutions and confirm the scale's impact on mobility outcomes in pediatric ECMO patients.

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  Effect of Social Determinants of Health on Clinic Visit Attendance in Patients with Hemophilia

  Shailly Gaur; Brian Lee PhD, MPH; James Anderst MD, MSCI; Katie Foote LSCSW, LCSW, OSW-C; Andrea Bradley-Ewing MPA, MA; and Shannon L. Carpenter

  Background Social determinants of health (SDOH) create barriers to seeking care regularly, especially for patients with chronic disease. Patients with moderate-severe hemophilia A and B (factor 8 and 9 deficiencies respectively) have a life-long higher risk of bleeding and require chronic therapies. Specialized care is offered through hemophilia treatment centers (HTCs); however, these can be difficult to access for some individuals. Previous SDOH research in this patient population has been limited; therefore, it can be difficult to identify the barriers to care that exist. There is a need to examine SDOH more thoroughly to create patient-directed interventions to improve access to care. Objectives To identify common SDOH affecting patients with moderate-severe hemophilia and determine how each impacts clinical utilization within a single HTC. Methods A retrospective chart review of patients age < 18 years old with a diagnosis of moderate or severe hemophilia A or B seen at Children’s Mercy Hemophilia Treatment Center over the last five years. Dependent variables included no-show visits, which were any missed visit since the patient established care, and missed yearly comprehensive visits, of which non-parametric distributions were calculated. Rates were standardized to an assumed 10-year follow-up period and then compared across independent variables including race, ethnicity, language spoken, insurance type, rural vs urban home, food insecurity rate, poverty level, distance to an ER and to the HTC using the Kruskal-Wallis and Wilcoxon rank-sum tests, where appropriate. Significant findings were determined using a p-value < 0.05. Results Patients with the following SDOH had a statistically significant difference in rates of no-show visits: distance >60min from the HTC (p=0.009), urban vs rural home county (p=0.033), race of patient (p< 0.001), and insurance status (p=0.007). [Figure 1] Further demographic data and analysis included with confidence intervals provided in Fig 1. Conclusions The rate of no-show visits was most affected by patient race, insurance type, distance from HTC, and urban vs rural living conditions. No statistically significant correlations existed within the yearly comprehensive visits, which may be indicative of the shared importance of keeping regularly scheduled appointments between patient and provider when caring for a chronic disease. Interestingly, though previous data in this field has demonstrated rurality as a limitation to care, these findings suggest that an urban setting closer to the HTC presented more of an issue. These findings also help support the benefits of outreach clinics in providing specialized care directly in rural environments, likely reducing no-show rates in this population. This may inspire a similar approach to localized outreach within the urban neighborhoods affected by SDOH limitations to offer clinics where patients are most vulnerable.

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  Engineering A Novel Treg Population to Control Autoimmune Diabetes

  Sofia Colon Guzman, Elly Puckett, Ryan T. Fischer, and Mary Markiewicz

  Purpose Type 1 diabetes (T1D) in an autoimmune disorder where T cells attack  islet cells, which are the pancreatic cells responsible for insulin production. T1D affects about 1 in 500 children in the United States and is one of the most common chronic diseases in pediatrics. Currently, insulin is the most effective treatment for T1D However, it does not change the underlying disease, and patients still experience many complications throughout their lifetimes. With a significant increase in the prevalence of T1D in children and adolescence in the past two decades, it’s important to explore therapies that alter the immune response as a possible way to reverse or even prevent the progression and development of this disease. One approach being investigated is to use regulatory T cells (Tregs), which can suppress the function of autoreactive T cells, as treatment for autoimmune disease. However, using a patient’s own naturally occurring Tregs (nTregs) as therapy poses several challenges. Therefore, we developed a novel way to generate human engineered Tregs (eTregs) from conventional T cells (Tconvs) by expressing both FOXP3 and HELIOS in both CD4+ and CD8+ Tconvs. We hypothesize that these novel eTregs will be as, or more, effective in their suppressive abilities against islet-specific effector T cells and be more stable under inflammatory conditions compared to naturally occurring Tregs. Methods We isolated CD8+ T cells from healthy human donors through negative selection using magnetic beads. From these cells we created cytotoxic T cells (Tconvs) through lentiviral transduction of a vector that expressed a TCR specific for an islet-specific antigen (IGRP) presented in HLA-A2:01 and mCherry as a fluorescent marker of transduction. The presence of IGRP-specific TCR was confirmed using dextramer staining. These Tconvs were co-cultured with BetaLox5 cells, an immortalized human cell line derived from islet cells. The response of the CD8+ Tconvs was determined by measuring effector cytokine release. We also created autologous natural Tregs (nTregs) and tested the ability of these cells to reduce the CD8+ Tconv response against BetaLox5Unpaired T test analysis was used to measure the statistical difference in cytokine production between co-culture conditions. Summary of Results IGRP-specific T cells responded to the human islet cell line BetaLox5 in vitro, demonstrated by elevated IFN-γ and TNF- production after co-culture. This response was decreased when nTregs were present in co-culture, as the amount of IFN-γ and TNF- in co-culture supernatant was significantly lower. Conclusions We were able to create a model to test the immunosuppressive capacity of Helios+FOXP3+ eTregs compared to nTregs against islet-specific autoreactive T cells. We also showed that nTregs decrease the response of these cytotoxic T cells when co-cultured with BetaLox5 cells. We are now comparing the immunosuppressive ability of our eTregs to nTregs in this assay, as well as comparing their stability under inflammation.

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  The Pediatric ECMO Diary: Strengthening Communication, Family Coping, and Staff Engagement

  Sarah P. Jimenez, Johanna I. Orrick, and Kari L. Davidson

  Bedside diaries have been used in Adult Intensive Care Units (ICUs) in Europe since the 1970s. These journals encourage clinicians to record a patient’s progress, illness narrative, and daily occurrences. Adult ICUs in America are beginning to implement and study the benefits of ICU journals. Bedside journals have been shown to increase staff engagement and aid in psychological recovery for patients and families following their illness. These diaries improve communication between the healthcare team and families, and help patients make sense of ICU stays by filling in memory gaps. Studies on ICU diaries have shown that a bedside diary kept throughout an ICU stay can decrease anxiety levels, decrease depression, decrease symptoms of post-traumatic stress disorder (PTSD), and improve an ICU survivor’s quality of life. This project examines the impact of bedside diaries on families with patients on Extracorporeal Membrane Oxygenation (ECMO) support and the staff that cares for them. The hospital legal team and Family Advisory Board were consulted in the diary development. A survey was sent to ECMO staff to evaluate existing knowledge of ICU diaries. Education was provided through department meetings. Diary champions were recruited to audit diaries. Diaries included non-digital photographs. Family members completed a survey after their loved one’s ECMO run and completion of the diary. Common themes from family surveys included: “loved,” “great,” “perspective,” “roller coaster,” “traumatic time,” “amazing,” “encouraging,” “awesome,” and “cute.” Families expressed that the diaries helped humanize the ECMO staff and care providers, fostering a more personal connection. A follow-up survey was conducted with ECMO staff to assess their perceptions. Common themes from the staff post survey included: “enjoyment,” “coping," “useful,” and “value.” Patient diaries have a positive psychological effect on family members, serving as a powerful rapport-building tool. Families and staff provided positive feedback, noting increased engagement and improved communication.

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  The Role of the Apheresis Tandem Safety Timeout in ECMO and Apheresis Therapy

  Johanna I. Orrick, Debra E. Newton, Kari L. Davidson, and Karen Murray

  The Apheresis Tandem Safety Timeout was implemented in 2017 at Children’s Mercy Hospital (CMH) to ensure safe, coordinated care when performing therapeutic plasma exchange (TPE) alongside Extracorporeal Membrane Oxygenation (ECMO). The first tandem procedure at CMH was performed in 2013, introducing a valuable tool for treating the sickest patients in the region. However, when patients are placed on two extracorporeal circuits simultaneously, the risk of complications increases significantly. These include electrolyte imbalances, anticoagulation challenges, hemodynamic instability, and the potential for either circuit to be compromised. The ECMO and Apheresis team recognized that even minor miscommunication could lead to life-threatening complications in these complex tandem therapies. Checklists are widely recognized in healthcare as effective tools to promote safety and identify human error before critical events occur. A checklist was developed to mitigate the increased risk of abnormalities in electrolytes, anticoagulation levels, and hemodynamics by ensuring that each team member understands the potential issues identified and addresses them before initiating apheresis on ECMO patients. The Apheresis Tandem Safety Timeout checklist facilitates closed-loop communication, defining roles, verifying system readiness, and identifying possible adverse events that may arise during the procedure. Given the high likelihood of technical challenges, such as maintaining blood flow in two circuits simultaneously, avoiding pressure differentials, and managing the risk of air emboli, the checklist ensures that the ECMO and Apheresis team are thoroughly prepared for each tandem procedure. By standardizing communication, the checklist enhances team coordination and reduces the likelihood of system failures or patient complications. The ongoing refinement of the checklist, based on quantitative and qualitative data from each procedure, has led to safer, more efficient tandem therapies. The checklist remains a vital tool in managing the complexity of these cases, allowing multiple teams to provide optimal care to critically ill patients who require simultaneous extracorporeal therapies.

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  Viral Detection in Anterior Nasal Swabs Versus Nasopharyngeal Swabs in Children

  Nicole Neeley, Abby Kietzman, Rangaraj Selvarangan, Dithi Banerjee, Jennifer Goldman, and Jennifer Schuster

  Background: Respiratory viruses are a significant cause of pediatric illness and healthcare utilization. Diagnostic testing for respiratory viruses is generally performed using nasopharyngeal (NP) specimens obtained by trained medical staff, limiting testing to medical settings. Evaluating additional sampling methods in children, such as anterior nasal swab (NS) specimens may expand access to diagnostic testing and aid public health surveillance. Methods: Eligible children hospitalized at Children’s Mercy Hospital in Kansas City who had a standard of care NP specimen collected for nucleic acid amplification testing for respiratory viruses were enrolled. Research NS specimens were obtained through self, caregiver, or staff collection. Specimens were tested on QIAstat-Dx-Analzyer using QIAstat-Dx Respiratory SARS-Cov-2 Panel and included: adenovirus, seasonal coronavirus (229E, HKU1, NL63, OC43), SARS-CoV-2, human metapneumovirus (hMPV) A+B, influenza A/A H1/A H3/A H1N1/pdm09, influenza B, parainfluenza virus 1/2/3/4, rhinovirus/enterovirus (RV/EV), and respiratory syncytial virus (RSV) A+B. Statistical analysis for sensitivity with 95% confidence intervals was calculated with NP as the gold standard. Further sub-analysis evaluating time to NS collection and test results (NP specimens as time zero and NS specimens grouped from 1-24 hours, 25-48 hours, and 49+ hours) was performed. Pairs with both NP and NS specimens having viruses detected or the same virus(es) detected were considered completely concordant. Specimens with multiple viruses detected on NP swab but only a single virus detected on NS swab, or vice versa, were considered partially concordant. Specimens with one virus detected on NS or NP and none detected on its pair were considered discordant. Results: One hundred and forty-seven pairs, each including one NP and one NS specimen, were obtained. Of the 147 pairs, 114 (77.5%) had complete concordance – 86 (58.5%) had viruses detected and 28 (19%) had no viruses detected. Fourteen (9.5%) pairs were partially concordant, and 19 (13%) were discordant. Sensitivity of NS specimens collected within 48 hours of their NP pair was ≥80% for all viruses except seasonal coronavirus (sensitivity 42.9%) (Table 1). NS specimens for adenovirus, influenza, parainfluenza, RSV, and SARS-CoV-2 were 100% sensitive when collected within 24 hours of NP specimens. Conclusion: Overall, NS are a potential alternative method for respiratory virus detection in the pediatric population. This less invasive collection method allows for broader use outside of medical settings, including respiratory virus surveillance in community settings. Future studies with closely time-matched NS and NP collection are needed to evaluate individual virus type detections.

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  A Case of Heterotaxy and Common Atrioventricular Canal with Bradycardia

  Mohamed Aashiq Abdul Ghayum, Jenna Schermerhorn, Hayley S. Hancock, Kelsey Brattrud, Melanie Kathol, and Maria Kiaffas

  Background: Heterotaxy-polysplenia syndrome (otherwise known as left atrial isomerism) should be suspected in the presence of complete atrioventricular canal defect (CAVC) with interruption of the inferior vena cava (IVC), atrioventricular block (AVB), and viscero-cardiac heterotaxy. The prognosis remains dismal for the fetus with such entity, especially in the presence of major structural heart disease and high-grade atrioventricular block. Early diagnosis, close follow up, and appropriate delivery planning, allows for effective stabilization of the baby after birth and might improve survival. Methods: A 26-year-old mother at 26 weeks gestational age was referred for cardiac evaluation of cardiomegaly, suspected CAVC and bradycardia. Maternal SSA and SSB antibodies were negative. Fetal echocardiogram showed moderate cardiomegaly, bradycardia with 3° AVB with ventricular rate of 64 beats per minute. Anatomy was consistent with heterotaxy {S/A,D,S}, hypoplastic left atrioventricular valve, unbalanced, right-dominant CAVC, dysplastic pulmonary valve, interrupted IVC with azygous continuation to a persistent left superior vena cava draining to a dilated coronary sinus. Partial anomalous pulmonary venous connection and coarctation of the aorta were suspected. Biventricular function was low normal with ventricular non-compaction. Ductus venosus Doppler was notable for a-wave reversal. Umbilical artery and middle cerebral artery waveforms were normal. Biweekly follow-up revealed worsening extracardiac Dopplers and decreasing ventricular function. Results: Given the prematurity, bradycardia, complex cardiac anatomy, non-compaction and ventricular dysfunction, prognosis was considered unfavorable. An integrated consult involving fetal cardiology, neonatology, Electrophysiology (EP) and Palliative care teams was undertaken. The family was counseled for guarded prognosis and high risk for demise, need for postnatal permanent pacemaker and surgical repair leading to a single ventricle pathway with possibility for heart transplant. A 2.79 kg female was delivered by c-section at 35 weeks 5 days; Apgar score was 4,4,and 8. The newborn was immediately transferred for placement of temporary pacing wires with subsequent transfer to the cardiac intensive care unit for stabilization and further management. Coarctation repair, PDA ligation and permanent pacemaker placement were performed at 4 weeks of age. Progressive biventricular dysfunction, bilateral outflow tract obstruction and pacemaker dependency resulted in transplant listing of the patient with a successful orthotopic heart transplantation taking place at 4 months of life and has continued to do well on follow up. Conclusions: Heterotaxy polysplenia syndrome should be suspected in cases with CAVC with interruption of the IVC, and AVB. The presence of major structural heart disease and high-grade AV block carries a guarded prognosis, with survival to discharge from the hospital after delivery at term or near term reported to be less than 50%; in cases of prematurity, survival is expected to be even lower. In our patient, early diagnosis, appropriate counseling, close follow-up and meticulous delivery planning allowed successful perinatal stabilization with timely pacing and effective supportive management. Timely listing and eventual heart transplantation resulted in the survival of this patient.

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  Common Pulmonary Vein Atresia: A Diagnostic and Management Distinction

  Adenike Adenikinju, Jenna Schermerhorn, Anmol Goyal, William Douglas, and Sanket Shah

  Common pulmonary vein atresia (CPVA) is an extremely rare congenital heart disease (CHD) characterized by a pulmonary vein confluence with no connection to the left atrium (LA) and no large decompressing vessel. We present a rare case of CPVA prognosticated with multimodal imaging.

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  D-Transposition of the Great Arteries with Right Aortic Arch, Bilateral Ductus with Ductal Origin of the Left Subclavian Artery and Abnormal Aortic Origin of the Left Coronary Artery

  Jenna Schermerhorn, Anmol Goyal, Param Vora, Braidyn Wells, Edo Bedzra, and Sanket Shah

  We present a case of prenatally diagnosed d-transposition of the great arteries (d-TGA) with intact ventricular septum (IVS), postnatally found to have bilateral patent ductus arteriosus (PDA) with potential for isolation of the left subclavian artery (LSA) and an abnormal left coronary artery (LCA) course. Isolation of the LSA is a rare occurrence and defined as an anomalous origin from the pulmonary artery via PDA and apparent discontinuation from the arch following PDA closure. It is exceedingly rare in association with d-TGA. Coronary artery anatomy is particularly important in d-TGA given most patients undergo the arterial switch operation (ASO).

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  Duration of Labor and Umbilical Artery Acidosis in Critical Congenital Heart Disease

  Sarah Gwazdacz, Teresa Nguyen, Henry Yeh, Mary T. Donofrio, Timothy Bennett, and Amanda McIntosh

  • Background (83 words): Standard delivery protocols for most prenatally diagnosed CCHD is induction of labor at 39 weeks with aims of a vaginal delivery (VD) near a tertiary care center. There is no benefit to planned Cesarean delivery (CD) over VD for most infants with CCHD. However, longer durations of labor (DOL) in CCHD have been described as a risk factor for umbilical artery (UA) acidosis. We aim to describe the relationship between DOL and UA pH and neonatal outcomes in those with prenatally diagnosed CCHD. • Methods (150 words): Retrospective chart review of prenatally diagnosed with CCHD born at Children’s Mercy from 2012 – 2023. Inclusion criteria was CCHD defined as anticipated neonatal intervention or prostaglandin dependence, single ventricle (SV) circulation, or risk of hemodynamic instability with placental separation. Excluded fetal demise, multiple gestations, prematurity, post-maturity, and comfort cares. Divided subjects into 3 groups: those who had VD, CD after a trial of labor (TOL), or CD without a TOL. The primary outcome was UA pH. Secondary outcomes were delivery room intubation, perinatal brain injury, and peak lactate, cardiac arrest, death, or inotropic support in the first 24 hours of life. Collected demographic and clinical information for the maternal-fetal dyad. Divided CCHD diagnoses into 4 groups based on SV versus biventricular anatomy and the presence of systemic outflow obstruction. Welch 2-sample test or ANOVA were used for inter-group comparisons. For associations, multivariable linear regression modeling was performed. • Results: (150 words) Analyzed 201 VD, 32 CD after a TOL, and 90 scheduled CD without a TOL patients. Between the groups, there was no difference in birth weight (BW), gestational age (GA), intrauterine growth restriction (IUGR), and UA pH. Mothers with planned CD were older and delivered fetuses with higher rates of genetic syndromes. On univariate analysis, DOL in VD group was associated with lower UA pH (estimate -0.0006707 (95% CI: [-0.00130, -0.00005], P-value: 0.037). This remained significant on multivariable analysis controlling for GA, BW, hydrops, genetic syndromes, and IUGR (P-value = 0.011). Looking at CCHD subgroups, DOL and UA pH only had a significant association in neonates with SV anatomy and systemic obstruction (P-value= 0.009). DOL and lactate had a significant relationship on univariate (estimate 0.01479 (95% CI: [0.00307,0.02651], P-value= 0.014) and multivariate analysis (p-value = 0.015). There was not a significant relationship between DOL and other secondary outcomes. • Conclusion (51 words) In this single center retrospective study of fetuses with CCHD, longer duration of labor was associated with higher neonatal lactate levels. It was associated with lower UA pH in neonates with single ventricle anatomy and systemic obstruction. This may be a modifiable risk factor in certain subsets of prenatally diagnosed CCHD.

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  OCD Lesions of the Lateral Femoral Condyle and the Impact of the Location on Recovery

  Catharine Kral, Joshua Cummings, Ellie Helton, Shannon Margherio, Brian S. Harvey, and Kevin Latz

  Descriptive study based on Zhou, Liang, et al. we categorized the LFC OCD lesions into weight bearing and non-weight bearing lesions to assess clinical and radiographic variables and evaluate the need for progression to operative management.