Posters

Follow

• 

  Unclear Source of Gastrointestinal Bleeding: Utilizing Small Bowel Capsule to Detect an Enteric Duplication Cyst

  Victoria Sarata, Kirby Lampe, and Kathryn Clarkston

  Introduction Enteric duplication cysts (EDCs) are rare congenital malformation formed during embryonic development of the gastrointestinal tract. Symptoms may include vomiting, abdominal distension, palpable abdominal mass, or bleeding but may vary based on location of the lesion [1]. We present a case of a 9-year-old-male with presented with recurrent rectal bleeding and had abnormalities on small bowel capsule which were diagnosed as enteric duplication cyst on exploratory laparoscopy. Case This is a 9 year old male with a history of persistent iron deficiency anemia, gallstones s/p cholecystectomy and recurrent hematochezia. Patient was initially admitted for recurrent red bloody stools and fatigue. His hemoglobin on admission was 8.1. Fecal calprotectin was elevated to 927. Meckel’s scan negative. Esophagogastroduodenoscopy and colonoscopy completed which showed mild superficial chronic gastritis but were otherwise normal. Small bowel capsule was initiated and normal but was not completed. He was discharged home on high dose proton pump inhibitor (PPI) and iron supplement. Hemoglobin on discharge was 8.4 He had follow up with GI about 1 month after this admission. He was doing well with no recurrent bloody stools. Hemoglobin was 12.7. PPI was decreased to once daily. About one month after clinic visit, he was re-admitted for recurrent bloody stools and fatigue. Hemoglobin on admission was 8.7. During this admission, magnetic resonance enterography (MRE) was negative. Nuclear medicine gastrointestinal blood loss imaging was negative with no active bleeding. EGD and colonoscopy repeated and visually normal. No biopsies obtained. Repeat small bowel capsule placed. Hemoglobin on discharge 10.3. Small bowel capsule now showing an area of narrowing, mucosal disruption, and ulceration concerning for a short-segment stricture. There is erythema/mucosal disruption without active bleeding in the distal 1/3 of the small bowel. There is a double lumen appearance with surface ulceration potentially concerning for a Meckel’s diverticulum. There are scattered areas of erythema in the distal small bowel. Dilated lacteals noted in the distal 2/3 of the small bowel. Double balloon enteroscopy vs surgery consult was recommended for further evaluation of these findings. He was again admitted about 1 month later for melena. Hemoglobin 9.6. Exploratory laparoscopy completed and visualized a large conglomeration of hypervascular bowel with small bowel coming in and out of it and adherence to the mesentery proximal to terminal ileum. This was identified as enteric duplication cyst. Patient tolerated the procedure and recovered well. Pathology identified it as benign enteric cystic lesion with mucosal ulceration consistent with an enteric duplication cyst. There was focal presence of oxyntic type gastric mucosa. At surgery follow up visit, he was doing well with stable hemoglobin. Discussion EDCs are congenital malformations of the GI tract that may cause a variety of abdominal symptoms including pain, vomiting, or bleeding. They are typically found in the distal ileum with an incidence of 4500-12500 but may occur anywhere along the GI tract [2]. EDC must have epithelial lining containing the mucosa of the GI tract, an envelope of smooth muscle and an attachment to the GI tract [1]. Most EDCs are diagnosed prenatally but if undetected, they can gradually increase in size and cause worsening obstruction, bleeding or ulceration. Ultrasound is the imaging technique of choice and will show the presence of a cyst with a “double-wall” or “muscular rim” sign. Advanced imaging such as computed tomography (CT) or magnetic resonance can also be used but these are less preferred given cost, radiation exposure or need for sedation. In a study by Okur et al., (2014), out of 32 pediatric cases of EDCs described, 28 were diagnosed with ultrasound and CT was performed in 21 patients [2]. In our case, MRE did not show evidence of an EDC.

• 

  Circulation of Respiratory Viruses from Air and Human Specimens in a Pre-kindergarten-12th Grade School District in Kansas City, MO

  Nicole Neeley, Brian Lee, Brittney Fritschmann, Luke Gard, Nibha Sagar, Dithi Banerjee, Anjana Sasidharan, Rangaraj Selvarangan, Olivia Almendares, Hannah L. Kirking, Jennifer Goldman, and Jennifer E. Schuster

  Background: Air sampling with respiratory virus testing in community settings may be a potential non-invasive, scalable method to monitor respiratory viruses. We performed an ecological analysis to describe the correlation between respiratory virus detections in air and human samples across all schools to evaluate the potential for air sampling as a tool for community-level surveillance. Methods: Knowledge of Infectious Diseases in Schools (School KIDS) is a voluntary respiratory virus surveillance program in a pre-kindergarten-12th grade public school district in Kansas City, MO. In 10 schools, students and staff collected anterior nasal swabs while at school, with optional on-demand swabbing when experiencing acute respiratory illness. Simultaneously, air samples were collected biweekly using AerosolSense samplers (ThermoFisher Scientific) in 2-4 (total n=32) locations (e.g., classrooms and nurse’s office) per school for 6-8 hours during school. All specimens were tested by multiplex PCR for adenovirus, human metapneumovirus (hMPV), influenza (Flu) A and B, parainfluenza viruses (PIV) 1-4, respiratory syncytial virus (RSV), rhinovirus/enterovirus (RV/EV), SARS-CoV-2, and seasonal coronaviruses ([sCoV] OC43, HKU1, NL63, 229E). Positivity was defined as detection of ≥1 virus. District-wide weekly virus positivity rates from human and air samples were compared using Spearman’s rank correlation. Results: During September 18, 2024–March 31, 2025, 839 participants (Table) submitted 3851 specimens, with 970 (25.2%) testing positive. Of 353 air samples, 312 (88.4%) were positive. Air sample positivity was higher than human specimen positivity in all 22 weeks when both human and air samples were collected (Figure). Strong positive correlations were observed for RSV (0.87), sCoV OC43 (0.87), PIV-4 (0.73); moderate for sCoV NL63 (0.61), Flu A (0.58), and SARS-CoV-2 (0.53); and weak for hMPV (0.29) and RV/EV (0.12). Conclusion: Air sampling in schools demonstrated moderate-to-strong correlation with human respiratory virus detections for several viruses, suggesting possible utility for non-invasive public health monitoring. Further studies are needed to evaluate the relationship between air and human virus detection.

• 

  Monitoring The Laboratory Frequency For Musculoskeletal Infections At A Tertiary Care Children's Hospital: A Diagnostic Stewardship Qi Project

  Kedar Tilak, Douglas Swanson, Christine Symes, Alaina N. Burns, Ann L. Wirtz, and Rana El Feghaly

  Patients with musculoskeletal infections (MSKI) undergo several laboratory tests. To promote laboratory stewardship, our Infectious diseases (ID) division recommended the following frequencies for labs: 1) C-reactive protein (CRP) every 2-3 days until >50% reduction while >3mg/dL, otherwise weekly until normal, 2) Sedimentation rate (ESR) at the start of therapy and end if > 20mm/hr initially, 3) Complete blood cell count (CBC) at the start of therapy and when transitioning to oral therapy if WBC is initially elevated. Baseline data showed that of 42 children evaluated between July 2023-May 2024, only 15 (35.7%) had a CBC and 13 (30.95%) had a CRP and ESR obtained at the recommended frequency. Our aim was to increase appropriately ordered monitoring labs to 65% by October 2025. Methods We formed a quality improvement team (QI) of ID specialists (fellow, advanced practice provider, pharmacists, physician) and consulted with orthopedics and hospital medicine physicians. We followed QI methodology to create a cause-and-effect analysis (Figure 1) and prioritization matrix (Figure 2). Our Plan-Do-Study-Act (PDSA) cycles included: 1) An electronic health record (EHR) template to use in notes and emphasizing verbal communication with team; 2) Daily huddle discussion of MSKI and performing time-out before entering patients’ rooms. Our outcome measures were the percentage of patients with MSKI having laboratory frequency in accordance with our consensus, our process measure was the percentage of time the laboratory frequency is recommended in the ID consultation note, and our balancing measure was hospital readmission rate for patients with MSKI. We used control charts to display data and followed Shewhart rules to shift center lines. Results We saw a shift in appropriate ESR frequency testing in November 2023 from 27% to 68% shortly after the team formed (Figure 3 A). We have not observed a shift in the frequency of CBC or CRP (Figure 3B, 3C). We saw an increase in our process measure from 0% to 60%. We saw no change in our balancing measure. Conclusion Our QI initiative aimed at enhancing the frequency of laboratory monitoring for patients with MSKI has shown promising trends. Continued efforts and adjustments to our strategies are essential to achieve our goal of 65% by October 2025.

• 

  Neonatal Early-Onset Sepsis: It’s Not Always What You Think

  Gabriela M. Henriquez, Kedar Tilak, Douglas Swanson, and Susana Chavez-Bueno

• 

  Scleral Lens For Recurrent Corneal Ulcer in a Pediatric Patient with Blepharokeratoconjunctivitis

  Hannah Walleck and Christina Twardowski

• 

  A Novel “Stairstep” Approach to the Management of Extreme Hypernatremia Using Continuous Kidney Replacement Therapy and Hypertonic Saline

  Bahar Barani, Xuexin Lu, Darcy Weidemann, Judith Vansickle, Vimal Chadha, and Nathan Beins

  Hypernatremia is defined as a serum sodium greater than 145 mEq/L and is due to either a deficit of free water or excess sodium intake. Extreme hypernatremia (sodium level > 190 mEq/L) is immediately life–threatening with mortality rates > 60%. It is especially challenging to avoid complications, such as permanent neurological injury, respiratory failure, and profound electrolyte derangement, when children are in kidney failure. Established guidelines are lacking for correcting such extreme levels due to a paucity of information in the literature, although general recommendations are to decrease levels by no more than 10-12 mEq/L per day to prevent neurological complications. We report a case series of two infants with extreme hypernatremia due to salt intoxication with anuric kidney failure that were successfully managed with continuous kidney replacement therapy (CKRT) with modification of dialysate sodium composition, in conjunction with careful titration of intravenous fluid therapy. The first case involved a 3-month-old with end stage kidney disease, with home peritoneal dialysis use, presenting with a sodium of 222 mEq/L due to iatrogenic salt intoxication. Complications included multisystem organ failure, subdural hemorrhage, cardiac arrest with subsequent myocardial dysfunction requiring multiple inotropes, respiratory failure, and severe liver dysfunction. Sodium levels normalized over 10 days of “stairstep” CKRT with sodium additives with fine-tuning hypertonic saline and normal saline (See Figure 1). One year later, he has no neurologic sequelae and continues to meet all age-appropriate developmental milestones. The second case was a 9- month-old with congenital heart disease who presented with sodium of 196 mEq/L due to iatrogenic salt intake, severe dehydration, and anuric kidney failure. Complications included pulmonary embolism. Her sodium was corrected after 5 days with the same strategy as above (See Figure 2). At 4-month follow-up, no adverse neurological or cardiac sequelae were noted. Both CKRT prescriptions utilized low clearance of 500 ml/min/1.73m2 and every 12-24 hours CKRT dialysate bags were changed to achieve a sodium reduction of about 10 meq/L per day. In summary, this presents a novel approach to severe hypernatremia in patients with acute or chronic kidney failure through individualized CKRT strategies and judicious fluid therapy.

• 

  Does a “True Bovine Arch” Exist? Prenatal Diagnosis of a Unique Arch Anomaly in a Neonate with Absent Ductus Venosus

  Jenna Schermerhorn, Mohamed Aashiq Abdul Ghayum, Laura Kuzava, Sanket Shah, and Anmol Goyal

  Introduction: The origin of all head and neck vessels from a single aortic root – “True Bovine Arch” - is an extremely rare arch anomaly that has been rarely reported in literature. Clinical Presentation: A 28-year-old G2P1 woman was referred at 27-weeks-gestation for concerns of absent ductus venosus and suspected common atrioventricular valve defect. Fetal echocardiogram revealed bilateral Superior Vena Cava (SVC), dominant left SVC, absent ductus venosus, moderate sized posterior muscular Ventricular Septal Defect (VSD), biventricular dilation with preserved systolic function and a unique arch anomaly with all head and neck vessels arising from a single trunk off the aorta. Postnatal transthoracic echocardiography and CT angiogram confirmed the diagnosis of a true bovine arch with no concerns for coarctation or persistence of a 5th aortic arch. Further systemic imaging revealed dysgenesis of corpus callosum and butterfly vertebrae. A whole exome sequencing was non-diagnostic. Serial follow up echocardiograms revealed the VSD had become restrictive and smaller in size, however, there has been a serial increase in aortic root and ascending aorta dimension. Imaging Findings: Figure 1a shows a sagittal view of the aortic arch with unique branching pattern of all head and neck vessels arising from a single aortic root. Figure 1b and 1c shows post-natal confirmation of the unique aortic arch anomaly via TTE and CTA respectively. Role of Imaging in Patient Care: Accurate prenatal identification of cardiac and aortic arch anomalies is paramount for prenatal counseling, genetic diagnosis, delivery planning and postnatal management. CTA can complement pre and postnatal suspicion for appropriate surgical and endovascular procedural planning. Discussion: The origin of all head and neck vessels from a single aortic root is an extremely rare arch anomaly that has been seldom reported in literature with no documented prenatal identification. A “true bovine arch” refers to a single arterial trunk off the aortic arch that trifurcates into both subclavian arteries and a common bi-carotid trunk—mirroring the configuration found in cattle. Although a common brachiocephalic trunk is considered a normal variant, it has been shown to have a higher likelihood of developing ascending aortic aneurysm and embolic cerebrovascular accidents, a possibility even more likely to happen in our patient with unique arch branching. This could be secondary to hemodynamic variations and sheer stress from an altered angle of branching of the great vessels making identification of these variations vital for risk assessment prior to procedures. Accurate pre-natal delineation of the head and neck vessels can aid in appropriate prenatal counseling, genetic diagnosis, delivery planning and postnatal management especially when associated with critical aortic abnormalities. CT angiogram can complement pre and postnatal suspicion for appropriate surveillance guidelines and pre-procedural planning for surgical and endovascular procedures.

• 

  Epidemiology and Microbiology of Admission Surveillance Nasal Swabs in a Level IV Neonatal Intensive Care Unit (NICU) and Their Association with Subsequent Infections ​

  Kedar Tilak, Neena Kanwar, Rangaraj Selvarangan, and Venkatesh Sampath

  Background Newborns, especially preterm infants, are highly susceptible to sepsis, a major cause of neonatal morbidity and mortality. Known risk factors include prematurity, low birth weight, prolonged NICU stay, and use of central venous catheters. The link between early nasal colonization (within the first week of life) and later infections is unclear. Although many NICUs screen for Staphylococcus aureus using nasal swabs, the predictive value of early colonization by other Gram-positive and Gram-negative bacteria is not well understood. In our NICU, admission nasal swabs were sent for aerobic cultures to detect these organisms, highlighting the need for further research into their clinical significance and associated risk factors. Objectives The primary aim of this study is to identify epidemiological risk factors associated with early nasal colonization by Gram-positive and Gram-negative bacteria, as detected by aerobic cultures from surveillance swabs in neonates. The secondary aim is to examine the relationship between initial nasal colonization and the development of subsequent infections caused by these organisms during the NICU stay. Design/Methods We conducted a retrospective chart review of neonates admitted to our Level IV NICU from July 2021 to June 2023 with positive aerobic cultures from admission nasal swabs. Only infants under one week old on admission were included. Data collected included demographics, birth details, hospitalization course, and episodes of culture-positive sepsis. A total of 250 neonates met inclusion criteria. We are analyzing associations between maternal and neonatal risk factors (e.g., race, maternal age, prematurity, low birth weight, antenatal infections) and nasal colonization, as well as links between colonizing organisms and later infections. Statistical methods include chi-square tests and logistic regression. Results will be available by July 2025.

• 

  High-Frequency and Conventional Ventilation in Very Low Birth Weight Neonates During Interfacility Transport

  Trevor Cabrera, Janelle R. Noel-Macdonnell, and Emily McNellis

  Background: Very low birth weight (VLBW) neonates (≤1500 grams) transported across neonatal facilities often require advanced respiratory support, including high-frequency ventilation (HFV). While HFV use in this setting has increased, data evaluating its safety and efficacy in the VLBW population remain limited. We hypothesized that HFV will provide a safe modality to transport sick VLBW infants. Objectives: To present a comparative experience of safety and efficacy of HFV and CV in VLBW neonates during interfacility transport. Methods: We retrospectively reviewed neonates ≤1500g who received invasive ventilation during interfacility transport by our transport team between January 2019 and December 2022. HFV was delivered using ……, while conventional ventilation (CMV) was delivered via ………Demographic, clinical, transport characteristics, ventilator settings, and respiratory variables were collected. We compared primary outcomes under three categories: (1) ventilatory stability assessed by hypocarbia (pCO2 < 35) or hypercarbia (pCO2 >55) at the end of transport; (2) respiratory complications during transport; and (3) transport mortality. Results: A total of 122 neonates met inclusion criteria; 37 (30%) received HFV and 85 (70%) received CV. Neonates transported on HFV were more likely to have been born at younger gestation, transferred at ≥24 hours old, have a history of PROM or PPROM, require higher FiO2 and have higher pCO2 levels at the transferring facility (Table 1). Upon arrival at the accepting facility, both groups had comparable admission pCO₂, pH, blood pressure, and oxygenation, despite higher FiO₂ and more frequent pressor use in the HFV group. No cases of pulmonary hemorrhage, pneumothorax, or death occurred during transport. HFV patients had longer ground and total transport times. Chest radiographs showed a non-significant trend toward more frequent hypo-/hyperinflation in the HFV group (Table 2). Conclusion: Our retrospective study suggests that both HFV and CV are associated with safe transport and comparable immediate post-transport physiological outcomes in VLBW neonates requiring interfacility transport with invasive ventilation. Despite being more critically ill requiring higher ventilatory support, neonates transported on HFV achieved similar ventilatory stability without increased risk of respiratory complications or mortality. These findings support the feasibility and safety of HFV during neonatal transport of VLBW infants. Future studies will define the clinical parameters that guide preference of HFV vs. CMV for choice of respiratory support. ,

• 

  Massively Dilated Aorta in a Neonate Leading to Symptomatic Right Heart Compression

  Lauren Littell and Peter LaRossa

  We present a case of a newborn, born at 32 weeks, with prenatally diagnosed massive ascending aorta (AAo) dilatation and multiple congenital anomalies with unrevealing genetic testing. Postnatal echo revealed a bicuspid aortic valve and a dilated AAo (2.2cm, z-score 12). At 2 months of age, he had progressive hypoxic respiratory failure requiring intubation, with frequent positional hypoxia despite adequate ventilation. Repeat echocardiogram showed a 9mm increase in AAo diameter over 4 weeks. Surgical intervention was indicated, but the cause of hypoxia was unclear. The dilated AAo was found to compress the right atrium, causing right-to-left shunting at the atrial level, confirmed by agitated saline study. The patient underwent hemiarch and AAo replacement with a pulmonary homograft at 11 weeks of age. Imaging Findings: Initial echo showed a bicuspid aortic valve, massively dilated AAo (2.2cm, z-score 12), and PFO with left-to-right flow. CTA on day of life 5 confirmed these findings. Repeat echo 1 month later showed stable aortic measurements with no clear atrial-level shunt. At 2 months, a follow up echocardiogram and confirmatory CTA revealed further dilation of the AAo, extending to the arch (3.1cm, z-score 17), with mild anterior tracheal mass effect. No atrial defect was seen, so an echo with agitated saline was performed, which was rapidly positive, confirming right-to-left shunting. Role of Imaging in Patient Care: CTA did not show significant airway or pulmonary artery compression. The atrial septum was challenging to image on echo due to mass effect and distortion. The agitated bubble study quickly confirmed intracardiac right-to-left shunting. Discussion This patient presented a unique challenge in differentiating the etiology of his positional hypoxia (lung vs cardiac). Multimodality imaging and agitated saline study confirmed suspicion right atrial compression from his rapidly dilating aorta, a very unusual finding in the absence of a known genetic disorder, leading to right-to-left shunting across the PFO.

• 

  No Suspicion for NOCARH: A Cautionary Tale of Anchoring Bias

  Nicole DeMarco, Julia Allyson Hays, Bonnie Sullivan, Michael Price, Kimberly A. Horii, and Shannon Haines

  Introduction: Neonatal onset of cytopenia autoinflammation rash and hemophagocytic lymphohistiocytosis syndrome (NOCARH), a subset of hemophagocytic lymphohistiocytosis (HLH), is a rare and life-threatening condition characterized by uncontrolled immune response. It presents a diagnostic challenge in neonates due to the overlap of clinical features with other conditions, particularly congenital infections. We report a case of NOCARH in a late pre-term neonate, highlighting an atypical age group and the diagnostic difficulty of this syndrome. Case Presentation: A 1 day old ex-35 week er neonate presented to a level 4 NICU with chief complaints of pancytopenia,; an extensive petechialmaculopapular rash of on the her extremities, torso, and face ; e, abdominal distention;, hepatomegaly ; abnormal findings on abdominal film ; , and a systolic murmur . Initial symptoms prompted concern for congenital infection s, as well asand necrotizing enterocolitis. Comprehensive infectious disease testing yielded negative results. She was subsequently evaluated for immunodeficiency, liver, and thyroid associatedrelated diseases. Persistent profound pancytopenia, lymphadenopathy, elevated inflammatory markers, episodic rashes, growth failure, worsening cholestasis, and new onset lymphadenopathy episodic rashes at three weeks of age prompted expansion of differential and evaluation for NOCARH despite not fitting clearly into diagnostic criteria . Bone marrow biopsy confirmed the diagnosis with findings of hemophagocytosis without malignancy. Genetic evaluation identified a heterozygous pathogenic CDC42 mutation consistent with NOCARH syndrome. Management: Treatment included the administration of dexamethasone, intravenous immunoglobulin, and emapalumab, a monoclonal antibody targeting IFN-γ. Prophylactic Aantimicrobials were initiated for prophylaxis in the setting of her immunosuppression. Outcomes and Follow-up: The patient demonstrated clinical stabilization with improved resolving cytopenia and reduction in inflammatory markers. Her health ultimately improved , ultimately being able to beenough to discharged home with close follow-up with specialized care and ongoing multidisciplinarymultiple subspecialists , including Gastroenterology and Oncology follow-up. Bone marrow transplantation is planned to achieve long-term disease control. Conclusion: This case highlights the hazards of anchoring bias when evaluation for initial suspected medical condition is inconclusive. Early diagnosis and targeted therapy are critical to improving outcomes in this rare, high-mortality condition. Pediatricians and neonatologists should be aware of the potential for HLH to present in neonates, particularly in association with genetic syndromes like NOCARH. Introduction: Neonatal onset of cytopenia, autoinflammation, rash, and hemophagocytic lymphohistiocytosis syndrome (NOCARH), a subset of hemophagocytic lymphohistiocytosis (HLH), is a rare and life-threatening disorder driven by uncontrolled immune activation. Diagnosis in neonates is challenging because its features overlap significantly with congenital infections and other systemic inflammatory conditions. We present a case of NOCARH in a late preterm neonate, illustrating the diagnostic complexity and importance of early recognition in an atypical age group. Case Presentation: A 1-day-old ex-35-week neonate was admitted to a level IV NICU with pancytopenia, widespread petechiae, abdominal distention, hepatomegaly, and a systolic murmur. The initial differential diagnosis included congenital infections. Broad infectious workup was unrevealing. Over the next three weeks, the infant exhibited persistent cytopenia, lymphadenopathy, elevated inflammatory markers, and recurrent episodic rashes, raising suspicion for HLH/NOCARH despite not fully meeting diagnostic criteria. Bone marrow biopsy revealed hemophagocytosis without malignancy,

• 

  Pursuit of A Full Night's Sleep

  Kennison Adams, Andrew Donaldson, and Brian S. Harvey

  This project was a literature review and reflection upon the importance of having an osteoid osteoma on the differential for adolescent hip pain. The primary objective highlighted key attributes of this pathology and instructed readers to be vigilant in their assessment so that quicker diagnosis, management, and return to sport could occur.

• 

  Recombinant erythropoietin use for anemia of prematurity: a three year since center experience

  Hannah Darland, Krishna Dummula, Chaitali Mahajan, and Vishal Pandey

  Background: Anemia is a common disorder of prematurity secondary to limited blood volume, excessive blood draws, inadequate nutrition, poor iron reserves, and insufficient endogenous erythropoietin. While many NICUs use recombinant erythropoietin (rEpo) to treat anemia of prematurity, the absence of universal guidelines has led to variability in dosing and schedules. Here we present our experience on the use of rEpo in a level 3 NICU. Our unit protocol uses 400 IU/kg three times a week for a total of 9 doses if hematocrit < 30%. Objectives: The purpose of this study was to evaluate the response to rEpo in infants with anemia of prematurity. Methods: Charts of infants born between 2018 and 2020 with gestation ≤34 weeks who received rEpo for anemia of prematurity were retrospectively reviewed. We defined response to therapy as an increase of hematocrit by 5% from pre-treatment baseline. Infants who did not achieve the therapeutic goal or who received blood transfusions were considered non-responders. Neutropenia, thrombocytopenia, and retinopathy of prematurity (ROP) requiring treatment were treated as adverse events. Results: A total of 44 infants were treated with rEpo during the study period. Clinical characteristics are described in Table I. Average hematocrit at birth was 44.8±7.5 (32.7 to 60.8). Infants were started on rEPO at a median corrected age of 33.3 ±3 weeks and a weight of 1674±622 g. Average DOL was 37±18 at initiation of therapy. Average baseline hematocrit at the start of therapy was 27.9±1.8%. 80% of patients (35/44) responded with increase of hematocrit by >5%, 20% (9/44) did not respond. 75% of infants (33/44) received all 9 doses. 11 patients received between 1-8 doses. Reasons for discontinuation of rEpo were: discharge prior to therapy completion (36%, 4/11), blood transfusion (27%, 3/11), neutropenia (27%, 3/11), and ROP progression (9%, 1/11). Average increase of hematocrit in the responders was 7.3±2.9%. In all, 11.4% of patients (5/44) developed neutropenia- none with ANC< 500 neutrophils/µl. None developed thrombocytopenia but 13.6% (6/44) had thrombocytosis >450,000 platelets/µl. 4.5% (2/44) required treatment for severe ROP, and no deaths occurred. Conclusion: rEpo was efficacious in increasing the hematocrit in the majority (80%) of convalescent preterm infants. Neutropenia observed during the therapy was mild-to-moderate (between 501-1500 neutrophils/µl) and did not result in invasive sepsis. Larger prospective comparative-dosing studies are needed to determine the ideal dose and timing of rEpo therapy in growing preterm infants to prevent late blood transfusions.

• 

  Severe Thiopurine-Induced Myelosuppression in a Pediatric ALL Patient with the NUDT15 *1/*6 Genotype

  Jillian Fry, Keith August, Terrie Flatt DO, and Laura B. Ramsey

  Introduction: Genetic variants in TPMT and NUDT15 that affect the metabolism of mercaptopurine (6MP) are routinely tested for to guide 6MP dosing. Nudix hydroxylase 15 (NUDT15) deficiency can cause myelosuppression when affected patients are treated with 6MP. The NUDT15 *6 allele is classified as having uncertain function by the Clinical Pharmacogenetics Implementation Consortium Guidelines given insufficient data to determine its phenotype. Here, we present the case of a pediatric patient with the NUDT15 *1/*6 genotype experiencing significant mercaptopurine-induced myelosuppression. Case Description: We present a 4-year-old female with standard risk-average precursor B-cell acute lymphoblastic leukemia treated per AALL1731. Exome sequencing reported TPMT *1/*1 (Normal Metabolizer) and NUDT15 *1/*6 (Indeterminate). In Maintenance Cycle 1, 6MP was started at standard dosing of 75 mg/m2/day. On Cycle 1 Day 29, her absolute neutrophil count (ANC) and platelets had decreased but remained within goal range. At that time, 6-thioguanine nucleotides (6-TGN) were at the higher end of normal at 445 pmol/8 x 108 RBC, and 6-methylmercaptopurine (6-MMP) was elevated at 9200 pmol/8 x 108 RBC. On Cycle 1 Day 47, she developed increased bruising and was found to have severe neutropenia with an ANC of 0.03 x 103 mcL and thrombocytopenia with platelets of 5 x 103 mcL. 6-TGN were relatively unchanged at 440 pmol/8 x 108 RBC, but 6-MMP had significantly increased to 16,177 pmol/8 x108 RBC. Given the degree of myelosuppression, 6MP was held. Her ANC and platelets did not recover until Cycle 1 Day 64, at which time 6MP was restarted at 50% of the original dose. She has not required any additional dose changes or disruption in Maintenance Cycle 2. Conclusions: This case suggests that patients with the NUDT15 *1/*6 genotype could be treated as intermediate metabolizers with higher risk of toxicity than those with wild-type NUDT15 and require dose reduction.

• 

  A Multipronged Approach to Developing & Marketing Asynchronous Resources to Faculty

  Heather McNeill; Emilie Keeton, MSOD; Enam Haddad; Myles Chandler; Eric T. Rush; and Jacqueline Hill

  What suggested GFA topic does your submission align with? Organizational Policies, Practices and Procedures Intended/Target Audience Seasoned Faculty and Staff Mid-Career Faculty and Staff New Faculty and Staff Description Faculty have limited time for professional development. We piloted an approach to create and actively promote curated asynchronous professional development resources in five targeted topic areas and recorded resource use over two academic years (July 2023-July 2025). Since July 2023, we had 4,543 unique daily views; more than half (n=2,319) were views of academic promotion resources. Asynchronous resources engaged faculty in professional development topics, especially academic promotion. Additional information: Background: Faculty have limited time for professional development necessary for career advancement. Goal: Pilot an approach to develop and promote asynchronous faculty development resources at our pediatric teaching hospital. Methods: We created resources (e.g., how-to guides, templates, recordings) in topic areas such as academic promotion and professional development. The resources are hosted on SharePoint sites and cataloged on intranet pages, allowing us to track daily resource usage by area. We promoted the resources through targeted communications and presentations and recorded views from July 2023 to July 2025. Outcomes: From July 2023 to December 2024, we had 4,543 unique daily views. More than half (n=2,319) were views of promotion resources. Full data will be available at the conference. Conclusion: Our approach engaged faculty in asynchronous learning on faculty development topics, especially academic promotion. These data will inform changes to our resource catalogs. Learning Objectives After participating in this session, attendees should be able to… 1. Recognize the need for asynchronous resources among faculty 2. Explain how asynchronous resources can engage faculty in professional development 3. Identify opportunities to develop and promote asynchronous resources for faculty

• 

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

  Charles Maloy, Jessica L. Bettenhausen, Y Raymond Chan, Leah Jones, Kathryn Kyler, Laura Plencner, Jessica Markham, Jacqueline Walker, Smit Shah, Sian Best, Jonathan Ermer, Shelby Chesbro, Maria Newmaster M.D., and Henry T. 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.

• 

  A Case of Early Pediatric-Onset Multiple Sclerosis

  Mellanie De Guzman and Marcy Vandament

  Pediatric-onset multiple sclerosis (POMS) is a central nervous system neurodegenerative disease impacting individuals who experience symptom onset prior to the age of 18-years-old.17 Children and adolescents diagnosed with POMS prior to 16-years-old are considered as early POMS cases. 1,6,10,11, 18 The current case study presents an 11-year-old boy, “Patient A,” who was diagnosed with POMS at 10-years-old. Common neurocognitive weaknesses and behavioral concerns seen in this patient population include deficits in language (receptive, expressive, verbal fluency), memory, attention, executive functions, processing speed, visual-motor/fine-motor coordination/dexterity, depression, anxiety, and fatigue.2-5,15,20 Results from testing demonstrated mild weaknesses in abstract reasoning and fine motor coordination/dexterity. Qualitative concerns surrounding executive functioning and mild depression were reported. Overall, no significant cognitive or behavioral concerns were revealed. Therefore, no additional diagnoses were provided aside from Patient A’s historical diagnosis of POMS. Establishing a neurocognitive baseline for patients diagnosed with early POMS is pivotal for the monitoring of their cognitive, behavioral, and adaptive functioning over time. Incorporation of a pediatric neuropsychologist as part of the patient’s treatment team and the implementation of comprehensive neuropsychological evaluations can support this goal of longitudinal comparison.

• 

  Scoring Large Muscle Movements in Pediatric Sleep Studies: An Educational Module for Sleep Technologists

  Shauna Michelle VanDoren and David G. Ingram

  Introduction Restless Sleep Disorder (RSD) has recently been recognized as a distinct sleep disorder, with Large Muscle Movement (LMM) scoring being a crucial component of its diagnostic criteria. This project aimed to develop and implement an online educational module to enhance the knowledge and skills of sleep technologists to accurately score LMMs. Methods We constructed an online learning module that included training on the identification and scoring of LMMs in pediatric sleep studies. The module consisted of a 10-question pre-assessment, the educational content (which explained the background of RSD diagnosis, the role of LMMs in diagnostic criteria, and current scoring rules), a repeat of the 10 questions for post-assessment, and a feedback section regarding the module and LMM scoring in general. Results Preliminary results indicate a significant improvement in the accuracy and consistency of LMM scoring among sleep technologists who completed the module. The average post-module assessment scores showed a marked increase compared to pre-module scores, with total scores on the 10-question learning assessment increasing from 63.3+/-20.6% pre-module to 90.0+/- 8.9% post-module (p=0.017). Eighty percent of technologists agreed or strongly agreed with the statement that “As a result of taking this course I am confident I can recognize the clinical characteristics, symptoms, and diagnostic criteria of RSD.” Similarly, 60% agreed or strongly agreed with the state that “As a result of taking this course, I am confident I can accurately apply scoring guidelines for LMM events in sleep studies.” Feedback from technologists regarding the module itself, as well as questions and suggestions regarding LMM scoring in general, provided valuable information for potential future revisions of scoring rules. Conclusion An online educational module significantly improved the knowledge and ability of sleep technologists to score LMMs accurately, thereby aiding in the diagnosis of RSD. This initiative highlights the importance of continuous education and training in maintaining high standards of care in pediatric sleep studies.

• 

  When Macroglossia is not Beckwith-Wiedemann: A Case of an Infant with Congenital Central Hypoventilation Syndrome

  Shauna Michelle VanDoren and Samira Naime

  Introduction Congenital central hypoventilation syndrome (CCHS) is a rare genetic disorder caused by the mutation of the paired-like homeobox 2B (PHOX2B) gene that leads to alveolar hypoventilation during sleep and at times while awake. The majority of patients with CCHS have polyalanine repeat mutations (PARM) whereas 10% have non-PARM (NPARM). Of those with NPARM, less than 1% are caused by whole gene deletion. NPARM is associated with more severe phenotypes; however, some may have variable penetrance causing variable presentation within families. Report of case(s) Our patient is a 4-month-old infant born at 32 weeks in an outside hospital and transferred to our tertiary NICU at 10 weeks of age due to feeding difficulties, respiratory distress, hypoxemia, macroglossia and concern for Beckwith-Wiedemann syndrome. Upon transfer, she was on 2L of oxygen (FiO2: 0.3) via high flow nasal cannula. During her stay, she was noted to have hypoventilation which was thought to be secondary to upper airway obstruction. Upper airway evaluation by ENT revealed mild laryngomalacia for which supraglottoplasty was performed. Drug induced sleep endoscopy (DISE) revealed macroglossia with posterior displacement of the epiglottis causing obstruction. Due to concerns for obstruction and persistent hypoxemia, she was referred for a polysomnogram (PSG). The study was started on room air, but 1/4L of oxygen via low flow nasal cannula (LFNC) was initiated after 6 minutes due to persistent hypoxemia and hypoventilation. PSG showed an apnea hypopnea index (AHI) of 18/hour, central apnea index (CAI) of 11/hour with persistent nocturnal hypoventilation based on ETCO2, transcutaneous CO2 monitor (TCM) and blood gas (ETCO2 > 50mmHg for 85% of total sleep time (TST), max ETCO2: 62mmHg, TCM>50mmHg for 96% TST, peak TCM: 70mmHg). Given these findings, recommendations were made to obtain PHOX2B gene testing and obtain neurologic workup. Exome sequencing showed deletion of the PHOX2B gene consistent with CCHS. Conclusion A high suspicion for CCHS should be present in patients with central apnea and hypoventilation. Many children with CCHS may be missed due to complex genetic testing and variation in clinical presentation.

• 

  A single center qualitative study to improve coordination of follow-up care for patients diagnosed with Abusive Head Trauma

  Ruairi Smith-Dewey, Elise Wright, Daniella Donofrio, Hannah Goebel, and Sara Kilbride

  Introduction: Children diagnosed with abusive head trauma (AHT) have subsequent long-term developmental, behavioral, and medical consequences resulting in increased medical system utilization. There were 16 overall potential needs identified, with a single patient having 12. In 2023 at Children’s Mercy Hospital (CMH), 47% (8/17) of patients with AHT had < 50% of follow-up needs addressed at time of discharge. None had 100% of follow-up needs addressed. Needs not being addressed included need not identified or written documentation for need of follow-up without subsequent referral placed/appointment scheduled. Project goal was all follow-up needs to be addressed for at least 50% of children with AHT by January 2025. Methods (include PDSA cycles): Children admitted to CMH from January 2024-December 2024 and diagnosed with AHT by the Safety, Care & Nurturing (SCAN) team were included. Interventions were designed and implemented by a multidisciplinary team to address barriers including variation in scheduling process per specialty, lack of knowledge, and changing caregivers. • PDSA 1 (1/1/24): SCAN added standardized list of potential follow-up needs to documentation. • PDSA 2 (2/1/24): Trauma NP placed ophthalmology outpatient referrals. • PDSA 3 (3/1/24) Social work placed and documented early intervention referrals. Transitioned to SCAN RN responsibility. • PDSA 4 (4/10/24): AHT caregiver handouts with description of all potential follow-up needs added to discharge instructions. • PDSA 5 (12/11/24): AHT Evidence Based Practice guideline created including follow-up needs. Appointments and referrals were tracked by the author. QI tools used included fault tree, fishbone, and PICK Chart. Results: In 2024, 27 children were diagnosed with AHT by SCAN team at CMH. Five children were excluded from the study as 3 died and 2 were transferred to another hospital. The number of patients with < 50% of follow-up needs addressed was reduced from 47% to 43%. All follow-up needs were addressed in 13% (3/23) of patients. Conclusions: While project aims weren’t met, there was progress. Next steps include educating all discharging teams at CMH as there is no single team discharging these patients. Overall, there are few of these patients so standardization of discharge practices is challenging. SCAN created a working group and point person for appointment tracking to ensure follow-up needs established at discharge.

• 

  A Rare Case Of Adolescent Epiglottitis Secondary To Streptococcus Dysgalactiae

  Kedar Tilak, Mohamed Aashiq Abdul Ghayum, Douglas Swanson, and Rana El Feghaly

  Streptococcus dysgalactiae causes pharyngitis, cellulitis, necrotizing soft tissue infections, sepsis, and streptococcal toxic shock syndrome • It can lead to both toxin-mediated and immune-mediated diseases • Mostly seen in adults, rare in pediatric patients • Here we present a 15-year-old with S. dysgalactiae- associated septicemia, and epiglottitis with extensive neck infection

• 

  Bisphosphonate Treatment in Pediatric Patients with Rare Ovarian Malignancy and Hypercalcemia: A Case Series

  Seth Lamb, Samhita Bhattarai, and Jennifer Boyd

  Objective: To describe the presentation, management, and outcomes of severe hypercalcemia in two adolescent females secondary to ovarian malignancy. Methods: The case series includes two adolescent female patients, aged 11 and 16, diagnosed with rare ovarian malignancies and associated hypercalcemia. The 11-year-old female was diagnosed with small cell carcinoma of ovary while the 16-year-old was diagnosed with dysgerminoma. Both patients presented with abdominal pain, nausea and constipation. The 11-year-old received zoledronic acid while the 16-year-old received pamidronate. Serum calcium level, symptoms and adverse effects were monitored before and after both medications. Results: Both patients presented with severe hypercalcemia. The 11-year old presented with serum calcium of 15.0 mg/dL while the 16-year old presented with a serum calcium of 15.9 mg/dL. Both patients were refractory to hyperhydration with isotonic normal saline and 3 or more doses of calcitonin over a span of 3 days. Patients were then given bisphosphonates to decrease severe hypercalcemia. Patient 1 received one dose of zoledronic acid 0.1 Unit/kg while patient 2 received pamidronate. Calcium decreased to 10.4 mg/dl in 56 hours after zoledronic acid administration. Patient also had her tumor resection 3 days after medication administration which further decreased serum calcium to a lowest of 6.9 mg/dl. This was improved after 1 dose of intravenous calcium gluconate. Patient 2 received 1 dose of pamidronate 0.37 mg/kg. Serum calcium decreased to 9.1 mg/dl in 41 hours. Patient had surgical debulking surgery the same day. Lowest post operative calcium level for patient 2 was 7.5 mg/dl 3 days post pamidronate and 1 day post tumor debulking. Diagnosis was confirmed by pathology to be small cell carcinoma of ovary-hypercalcemia type and dysgerminoma respectively for patient 1 and 2. Patient 1 required calcium supplementation for 5 days post-surgery while patient 2 needed no calcium supplementation. No adverse effects noted on both patients. Conclusion: Small cell carcinoma of ovary and dysgerminoma are rare causes of hypercalcemia in adolescents. These cases highlight the importance of recognizing paraneoplastic hypercalcemia, safety, efficacy, and monitoring for hypocalcemia after a single dose of bisphosphonate. Close monitoring is needed if patients plan to undergo tumor resection within the next week as both medications are known to have a long half-life.

• 

  Dental Considerations for a Pediatric Patient with Osteopetrosis: Case Report

  Bethany Castro, Robin Onikul, Neena Patel, Jenna Sparks, Amy Burleson, and Brenda S Bohaty

  Introduction: Osteopetrosis is comprised of a group of rare hereditary disorders characterized by impaired osteoclast development or activity resulting in altered bone metabolism and high bone mineral density. Resulting bone is denser and heavier but more fragile than healthy bone. This condition can have oral manifestations such as malformed teeth, hypomineralization, or agenesis of teeth. The treatment for osteopetrosis can include hematopoietic stem cell transplantation and recommendations for prophylactic dental treatment7. The purpose of this report is to present a review of the literature regarding the dental considerations for patients with osteopetrosis. Case Report: This case report details a six-year-old male who initially presented to the Children’s Mercy Dental Clinic in 2020 for dental clearance prior to a bone marrow transplant and the subsequent follow up care over the next four years. The patient’s medical history is significant for osteopetrosis status post bone marrow transplant in 2020 as well as numerous medical diagnoses. Clinical and radiographic examination revealed carious primary teeth and multiple malformed, hypoplastic, or unerupted successor teeth. Clinical considerations for this patient include managing carious teeth as well as providing the parent with recommendations regarding future care are presented in this report.

• 

  Differential Diagnosis of Oral Ulcerations: a Case Report

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

  The diagnosis of oral lesions in children can be challenging as it can be an unspecified symptom of several diseases. Differential diagnoses can range from infectious diseases of childhood, inflammations, nutritional deficiencies, and chronic diseases. Because of this, children with oral ulcers may be treated by different specialists such as pediatric dentists and pediatricians. The purpose of this poster is to report the case of a twenty-one-month-old female patient who presented to Children’s Mercy Hospital Kansas City Emergency Department with chief concern for acute onset of intraoral oral ulcers, extraoral blisters near the lower lip, and fetid oral odor. Patient has a complex contributory medical history. This poster presents a review of the literature regarding various diseases associated with oral ulcers along with the potential development of acute necrotizing ulcerative gingivitis

• 

  Increasing incentive spirometry use in patients with sickle cell disease admitted to CMH

  Juan Nario Ramirez, Alexandra Prosser-Dombrowski, Thomas R. Cochran, and Joel Thompson

  Acute chest syndrome is one of the leading causes of morbidity and mortality in patients with sickle cell disease. The National Heart, Lung, and Blood Institute (NHLBI) recommends that patients with sickle cell disease perform ten breaths of incentive spirometry (IS) every two hours while awake to prevent development of acute chest syndrome. By not meeting these guidelines, patients are more likely to develop this potentially life-threatening acute complication. To increase the use of IS in patients with sickle cell disease admitted to the hospital for vaso-occlusive pain. Patients were identified and IS occurrences were measured via review of electronic medical record. Of those patients admitted with sickle cell vaso-occlusive pain, the frequency that IS was offered or performed each hospital day was recorded. Partial admission and discharge days were excluded. For PDSA cycle 1, all IS orders in Cerner powerplans were updated to reflect current NHLBI recommendations. Additionally, education was provided to residents and nursing staff regarding the importance of the use of IS to prevent severe complications such as acute chest syndrome. Following PDSA cycle 1, a second meeting with stakeholders led to changing IS documentation to coincide with the required task of patient-controlled analgesia (PCA) assessment every two hours. Furthermore, as it is difficult for younger children to use IS appropriately, a new PCA form was created to document the use of alternatives to IS such as pinwheels or bubbles. Data obtained from 10/2023-12/2023 and 10/2024-12/2024 was then reviewed to evaluate the impact of PDSA cycle 2. From 8/2021-11/2021, there were 191 hospital days with patients admitted with sickle cell vaso-occlusive pain, of which 53% had IS documented. When IS was documented, 0% of patients received the recommended frequency of IS and the median hours between IS occurrences was 6.3 hours. From 12/2021-3/2022, 164 hospital days had IS documented 40% of the time with 2% of hospital days meeting the recommended frequency and 5.9 median hours between IS occurrences. From 8/2022-11/2022, 167 hospital days had IS documented 26% of the time with 12% of hospital days meeting the recommended frequency and 3.0 median hours between IS occurrences. From 10/2023 - 12/2023, 181 hospital days had IS documented 12.7% of the time with 9% of hospital days meeting the recommended frequency and 5.6 median hours between IS occurrences. From 10/2024 - 12/2024, 177 hospital days had IS documented 11% of the time with 15.7% of hospital days meeting the recommended frequency and 4.1 hours between IS occurrences. Implementation of uniform IS orders that match NHLBI recommendations resulted in an increase in the provision and frequency of IS used among the studied population. In PDSA cycle 2, implementation of IS documentation along with analgesia assessments resulted in an overall improvement of the median hours between IS occurrences and the number of hospital days with IS given at the recommended frequency, when IS was documented. It is important to note that the overall number of times in which IS was documented decreased compared to the first two cycles, however, the percentage of patients using IS at the recommended frequency continued to improve. For the next PDSA cycle, we will plan to address other potential barriers to IS documentation, such as accessibility to IS in the Hematology/Oncology inpatient unit . Further research will also focus on evaluating the impact of appropriate use of IS on the development of acute chest syndrome in our patient population.