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  ADA Deficiency: Improvement in Immune Function after Enzyme Replacement Therapy

  Esosa Adah and Nikita Raje

  Introduction: Adenosine deaminase (ADA)-deficient severe combined immunodeficiency (SCID) is characterized by severe lymphocytopenia affecting T-, and B-,lymphocytes, with non-immunological manifestations including neurodevelopmental deficits, sensorineural deafness, and skeletal abnormalities. Treatment modalities include enzyme replacement therapy(ERT) and/or autologous gene therapy or hematopoietic stem cell treatment. We report a 9-month-old female with ADA deficient T-B-NK+ SCID treated with ERT while awaiting stem cell treatment. Case Description: A 9-month-old female with a history of failure to thrive, hypotonia and loss of developmental milestones had genetic testing done that showed variants ADA c.911 T>G, c.302G>A. She was admitted for further work up. Lymphocyte subsets showed CD3 117 mm ^3, CD4 55 mm^3, CD8 50 mm ^3, CD19 7 mm^3, and CD 16/56 cells 108 mm^3. Patient was diagnosed with T-B-NK+ SCID. ADA level noted to be

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  Bowel Cleanout Prior to Intrathecal Baclofen Pump Implantation: Effects on Acute Post- Operative Gastrointestinal Complications

  Amanda Lindenberg, Brittni Walton, Sathya Vadivelu, and Matthew J. McLaughlin

  Background: Intrathecal baclofen (ITB) pumps have been used for management of spasticity and dystonia There is limited data on immediate post-operative complications such as GI adverse events after surgery for ITB pump related procedures. This study aimed to compare patients who underwent a pre-operative bowel cleanout to those who did not and the relationship with post-operative nausea, vomiting and constipation requiring an escalation of their bowel regimen. Design: Retrospective cohort study. Setting: Pediatric acute care hospital. Participants: All patients who underwent new ITB pump and/or catheter placement from January 2015-December 2022. Interventions: Preoperative bowel clean out. Main Outcome Measure: Quantity of children requiring escalation of bowel program compared to home regimen after ITB implantation and rates of nausea and/or emesis after ITB implantation. Results: Of the 97 procedures performed in the study period, including new pump and catheter implantations and catheter revisions, 62% (n= 60) were instructed to complete a bowel clean pre-operatively. There was a significant difference between patients who had emesis in those who did not undergo a bowel cleanout preoperatively when compared to patients that did undergo a bowel cleanout (p=0.018). A chi-square test of independence demonstrated there was no significant relationship between performance of a cleanout and the need to increase their bowel regimen post-operatively (chi squared value= 0.0062, p=0.937382). Conclusions: Overall, it appears that escalation of a bowel regimen is typically required during a post-ITB implant admission regardless of if a preoperative bowel cleanout is performed. Therefore, it should be carefully considered for each individual patient. As constipation may contribute to emesis, an immediate escalation of the bowel regimen in the post-operative period should be considered.

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  Evaluating a Newborn Infant with Pancytopenia for WHIM Syndrome

  Shayan Sazdar, Thao Le, and Nikita Raje

  Introduction Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare immunodeficiency caused by a gain-of-function mutation in CXCR4. A complication of WHIM syndrome is severe neutropenia, which can result in fatal bacterial infections. Given the rarity of the disease and variability of clinical presentations, WHIM syndrome can be difficult to diagnose and manage. Case Description A full term male infant was hospitalized for hypoxic ischemic encephalopathy after delivery. Physical exam was unremarkable. During his hospitalization, he was found to have progressive pancytopenia. Initial lymphocyte subsets showed a significant T cell lymphopenia of 1325 mm3 CD3+, 920 mm3 CD4+, 390 mm3 CD8+, 42 mm3 CD19+, and natural killer (NK) cell count of 28 mm3. Naive CD4+ and CD8+ counts were also low. Immunoglobulin levels, lymphocyte to proliferation for mitogen, newborn screen, Epstein-Barr virus, and parvovirus were normal. Repeat lymphocyte subsets showed persistently low T, B, and NK cells without improvement. A comprehensive bone marrow panel showed a pathogenic variant in CXCR4. At two-years-of-age, he continues to have severe neutropenia. He has not had severe infections and has not required granulocyte-colony stimulating factor (G-CSF) therapy. Discussion WHIM syndrome may not present with all features. Treatment may include G-CSF, immunoglobulin replacement, prophylactic antibiotics, wart destruction, and stem cell transplant. A potential new treatment is plerixafor, which is a competitive antagonist of CXCR4. Its effectiveness and safety compared to G-CSF has not been established. Despite multiple treatment options, there is no standardized treatment. Early diagnosis and standardized therapy may help with preventing disease progression.

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  Evaluating Patients With Recurrent Fever and Elevated IgD

  Thao Le and Nikita Raje

  Introduction: Hyperimmunoglobulinemia D with periodic fever syndrome (HIDS) often presents within the first year of life. Symptoms include fevers, chills, headache, lymphadenopathy, and abdominal pain. Patients may be misdiagnosed as other autoinflammatory syndromes, resulting in delay of treatment. Case: A 16-year-old female presented for evaluation of recurrent fever. Her fevers started at two months of age, occurred one to two times a month, and were associated with headache, abdominal pain, and fatigue . At 18-months, she was diagnosed with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA). She was treated with prednisone and an adenotonsillectomy but her fevers persisted. . At 10-years of age, she had an immunoglobulin (Ig) D level of 936 mg/dL, normal lymphocyte subsets, tetanus, and diphtheria titers. Repeat IgD was 56 mg/dL, but other immunoglobulins were normal. Pneumococcal titers were suboptimal but showed an adequate response after administration of pneumococcal booster vaccine. She was started on cimetidine 600mg twice a day, with reduced frequency in her fevers. A repeat IgD was 52mg/dL. Genetic testing showed two pathogenic variants of mevalonate kinase (MVK), confirming the diagnosis of HIDS. She was prescribed interleukin-1 (IL-1) inhibitor therapy. Discussion: HIDS is diagnosed by an elevated IgD greater than 100 IU/mL or 14.1mg/dL on two occasions at least a month apart. However, IgD can be elevated in other autoinflammatory syndromes. Genetic testing for mutations in MVK or an elevated urinary mevalonic acid can further confirm diagnosis. This may allow for earlier diagnosis and initiation of treatment with IL-1 inhibitors.

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  Increasing annual eye exam completion for children with cystic fibrosis receiving CFTR modulators

  Paula Capel, Claire Elson, Stephanie Duehlmeyer, Jessica Haynes, Amanda Conway, and Christopher M. Oermann

  Background: CFTR modulators are life-changing therapy for people with CF. During preclinical studies of ivacaftor, cataracts were identified in juvenile rat pups. Subsequently, noncongenital cataracts have been reported in children receiving CFTR modulator therapy [1], so baseline and annual slit lamp eye examinations for cataract development are recommended for children with CF (aged <18). At Children’s Mercy Kansas City (CMKC), our standard practice is to conduct baseline and annual ophthalmologic examinations to assess for cataracts in children with CF prescribed CFTR modulator therapy, but there was no formal process for tracking adherence to this standard and ensuring that examinations were completed. A quality improvement project was undertaken in May 2022 to determine the rate of completion of annual eye exams and increase adherence to our annual monitoring standard.

  Methods: To improve adherence to annual monitoring standards, a quality improvement taskforce including CF care team nurses and pharmacists was developed. A database was used to document baseline and follow-up eye examinations. A standardized communication process was developed and deployed that included an eye exam brochure (Figure 1), messaging to caregivers and patients through the electronic medical record (EMR), and reminders on clinic departure or discharge paperwork. Additionally, children with CF who required eye examinations were identified during preclinic huddles, and that information was communicated to the CF care team. To improve tracking of completed eye exam reports, standardized notation of the searchable term “eye exam” was included in EMR documentation.

  Results: At CMKC, at least one eye examination was documented for 116 (72.5%) of 160 children with CF prescribed CFTR modulator therapy before implementation of the quality improvement project; after implementation, eye examinations were documented for 129 (81%). 64 (40%) children with CF at CMKC had eye exams completed in the year before project implementation. After project implementation, 72 (45%) PwCF had an eye exam completed per monitoring recommendations.

  Conclusions: Using quality improvement methodology, the CMKC CF care team developed a tracking tool and standardized process for communicating annual eye examination education in children with CF prescribed CFTR modulators. An increase in the rate of annual eye exam completion of 5% was observed after implementation of the process. With continued education and awareness, we anticipate higher rates of annual eye examination completion.

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  Investigating the Association between Mast Cell Activation Syndrome and Irritable Bowel Syndrome

  Ishaan Jakhar, Maggie Urschler, Medha Singh, Suman Sahil, and Hassan Ahmad

  Investigating the Association between Mast Cell Activation Syndrome and Irritable Bowel Syndrome Introduction Mast Cell Activation Syndrome (MCAS) involves the inappropriate release of chemical mediators resulting in varied episodic symptoms. Irritable Bowel Syndrome (IBS) is a common functional abdominal disorder where mucosal immune alterations play a key role. Since mast cells function as gatekeepers for the intestinal interface, we examined what proportion of patients diagnosed with MCAS also had IBS along with comorbid risk factors. Methods All patients in our study were 18 years of age or older. We used Cerner Healthfacts to gather our cohorts of patients with MCAS, IBS. We examined potential comorbid risk factors for MCAS using multivariable regression. Results We identified 2,508 patients with MCAS, mean age of48.7 ± 17.7 years with 67.7% (1,698) being female. 79.9% (2,003) were Caucasian, 8.3% (209) were African American. 11.5% (288/2,508) of patients with MCAS had IBS as well. The most common comorbid autoimmune disorder (AiD) was autoimmune thyroid disease (220). On multivariable regression, the factors associated with a higher risk of IBS in those with MCAS included cholestatic liver disease (OR 9.95), depression (OR 2.03), anxiety (OR 1.73), female gender (OR 1.64), autoimmune disorders (OR 1.63), and asthma (OR 1.56). Conclusions MCAS and IBS have symptom overlap with diarrhea and abdominal cramping. 11.48% of MCAS patients had IBS, indicating it may be worthwhile to inquire about bowel habits. Clinicians should also consider autoimmune thyroid disease in MCAS patients. There may be merit in exploring IBS therapies for MCAS patients.

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  Privacy preserving self-service DeGAUSS Based Geomarker Portal

  Harpreet Gill, Kushal Vallambhatla, Mark A. Hoffman, Kevin Power, and Catherine Jackson

  DeGAUSS offers a privacy-preserving capability to annotate a list of addresses with geomarker information that is useful for social determinants and other research. The standard delivery of DeGAUSS requires command line proficiency, potentially limiting the group of users. We provide a self-service portal that enables researchers to load a file and receive a downloadable annotated output file. In our pilot phase, the system has processed 481 calls for more than 45,000 addresses at Children’s Mercy.

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  Understanding Macrophage Phagocytosis in Pediatric Leukemia

  Molly Leyda, Jacqelyn Nemechek, John Szarejko, Fang Tao, Tykeem Manor, Douglas Myers, and John M. Perry

  Macrophages are a diverse and widespread type of innate immune cell which play an important role in homeostasis and defense. In a process called phagocytosis, macrophages engulf dying cells, foreign substances, and pathogens. Importantly, macrophages can present antigens from phagocytosed cells allowing them to initiate an adaptive immune response against remaining cells of the same type. Despite the immunosuppressive nature of the tumor microenvironment, macrophages often infiltrate tumors where they can either promote or inhibit cancer development, though the specific conditions influencing their pro- vs anti-cancer activity remain enigmatic. Recently, macrophages have been brought into the spotlight of immunotherapy research due to the hope of harnessing their ability to migrate into tumors, phagocytose cancer cells, and mobilize an antitumor T-cell response. However, questions remain about how macrophages recognize, or fail to recognize, cancerous cells for clearance, and how they can promote vs inhibit tumor progression. To identify modulators of macrophage activation, we have developed a model to visualize and quantify phagocytosis. In this model, bone marrow-derived macrophages are stained with CMFDA, a green cell tracker dye. Cancer cells are stained with pHrodo red, a pH sensitive fluorogenic probe which is designed to fluoresce brighter when inside the acidic phagosome, indicating phagocytosis has occurred. Macrophages are co-cultured with the cancerous target cells then imaged and analyzed. Phagocytosed cells can be visualized through fluorescence microscopy and the number of fluorescent engulfed cells quantified with flow cytometry. This in vitro model allows us to test how macrophages differentially engulf target cells based on various conditions such as macrophage polarization state or cancer cell phenotype. Data acquired from these experiments helps us delineate which characteristics of cancer cells are recognized by macrophages allowing us to better understand the role of phagocytosis in cancer. Future studies will test modified macrophage states in our tumor models and determine how polarization can be exploited to alter the spectrum of pro- and anti-cancer macrophage activities.

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  Anesthetic Management of Clamshell Thoracotomy for Large Tumor

  Kasey Brooks and Nathaniel Lata

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  Candida Chorioretinitis in Immunocompromised Patient with Candida Tropicalis Fungemia Secondary to Chemotherapy

  Jourdan Valkner Krause

  Summary: An immunocompromised patient with candida tropicalis fungemia secondary to chemotherapy presented for follow up of candida chorioretinitis. This infection puts the patient at high risk for CNVM formation and must be followed closely. Case History: • 3-year-old Hispanic female • CC: Candida chorioretinitis follow up • POH: Bilateral orbital chloromas and optic nerve atrophy • PMH: Acute myeloid leukemia, Candida tropicalis fungemia • Meds: o Amphotericin B liposomal 85 mg 42.5 mL, IV, q24hr o Flucytosine 500 mg Capsule *NF* 1,000 mg 2 capsule, PO/NG, q6hr o Micafungin 100 mg 100 mL, IV, q24hr *Plus numerous chemotherapy, pain and other systemic medications. Pertinent Findings: •Clinical: o DFE: One small, <1/2 disc diameter, white lesion inferior to macula OD. Slightly elevated with pigmented borders • Physical: o Immunocompromised • Laboratory testing: o Culture confirmation of Candida tropicalis fungemia o MRI: innumerable foci of nodular and ringlike contrast enhancement involving the brain, brainstem, and cerebellum most compatible with CNS dissemination of fungal disease. There is additional dissemination of fungal disease with involvement of the orbits and scalp. The presence of restricted diffusion within the rim-enhancing collection of the right orbit as described on the CT examination is most compatible with an abscess. Differential Diagnosis: • Toxoplasmosis • White dot syndrome • Cotton wool spot • Retinal tuft Diagnosis Discussion: Immunocompromised patients are susceptible to life threatening infections that are otherwise rare in the immunonormal. With any concern for infection, a thorough work up is needed to initiate proper treatment quickly. In this patient, cultures were positive for Candida tropicalis fungemia. Amphotericin B, micafungin and flucytosine were started. A baseline eye exam was performed and bilateral candida chorioretinitis was diagnosed. Two lesions near the macula OD and one lesion in the periphery OS were observed. After continuous antifungal treatment, only one lesion with pigmented edges remained near the macula OD and no lesions OS at 1 month follow up. With no additional signs to suggest active infection, pigmentation around the remaining lesion led to diagnosis of inactive candida chorioretinitis. Treatment/Management Discussion: Although the infection appears to be inactive, it is crucial to follow this patient every 6 weeks while the patient remains immunocompromised due to high risk of reactivation. There is also risk of CNVM formation. With the lesion’s close proximity to the macula, this must be carefully monitored with DFE and fundus imaging at every 6 week exam. In addition, it is anticipated the reflex seen during retinoscopy will be altered if changes to the macula occur. Alteration in the reflex would be an additional sign leading to the assumption the disease has progressed. The patient should continue taking systemic antifungals as prescribed by infectious disease team until instructed otherwise. Due to this patient’s young age, and still being within the visual development period, it is important to monitor for amblyopia since the lesion resides within the macula. With the patient having equal 20/50 acuity OD and OS, no amblyopia treatment is indicated at this time, but will be closely watched. Conclusion: Immunocompromised patients are predisposed to life threatening infections. In individuals with a history of infection, it is imperative to follow them closely, no matter if the infection is active or inactive. This group is at a higher risk of reactivation, thus should be monitored until the immune system recovers. In patient’s with any form of chorioretinitis, there is a risk of CNVM formation, even once immunonormal. Careful DFE and imaging should be performed at every follow up.

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  Dexamethasone for BPD while on Non-invasive positive pressure ventilation

  Priya Tiwari, Jansynn Radford, Michael Norberg, and Alain Cuna

  Background: Postnatal dexamethasone (DEX) has traditionally been reserved for preterm infants on prolonged mechanical ventilation, with reported rates of successful extubation at 47% to 76%. Recent emphasis on avoiding mechanical ventilation has resulted in preterm infants being increasingly supported with chronic non-invasive ventilation (NIV). The use of DEX for weaning preterm infants on chronic NIV remains largely unexplored. Objective: To determine efficacy of postnatal DEX in weaning infants on chronic NIV, and to identify factors and outcomes associated with successful weaning. Methods: Single-center retrospective study (2010-2022) of preterm infants ≤32 weeks gestation treated with postnatal DEX while on chronic NIV. Data on baseline demographics, respiratory characteristics at start and end of treatment, and clinical outcomes were collected. Primary outcome was rate of successful weaning from NIV to high-flow nasal cannula (HFNC). Other outcomes included factors and outcomes associated with successful weaning. Results: A total of 56 infants (mean gestational age 26 weeks, mean birth weight 890 grams) received postnatal DEX while on NIV. The average postnatal day and postmenstrual age at time of treatment were 78 days and 37 weeks, respectively. DEX treatment resulted in a significant decrease in oxygen and ventilation support from baseline to end of treatment (Fig 1). Overall, the rate of successful weaning to HFNC was 46% (26/56). Infants treated with postnatal DEX for the first time had similar rates of successful weaning compared to infants who had received prior DEX treatment (43% vs 39%, P =0.79). Factors associated with successful weaning to HFNC were earlier age and lower NIV and oxygen support at time of DEX treatment (Table 1). Infants with successful weaning were more likely to be discharged earlier with less bronchopulmonary dysplasia and less retinopathy of prematurity compared to infants with unsuccessful weaning (Table 2). Other complications of prematurity were similar between the two groups (Table 2). Conclusion: In our level IV NICU, efficacy of postnatal DEX to wean infants on chronic NIV was 46%. Our results suggest that DEX may also be useful in infants on chronic NIV, but further studies are needed to fully determine efficacy and safety.

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  Factors associated with respiratory pathogen panel utilization in children hospitalized with acute respiratory illness — New Vaccine Surveillance Network, Kansas City, 2017–2021

  Edward Lyon, Brian R. Lee, Benjamin Clopper, Heidi L. Moline, Rangaraj Selvarangan, and Jennifer Schuster

  Background: Respiratory pathogen panels (RPP) are multiplex PCR platforms that detect several respiratory viruses from one specimen. For most children hospitalized with acute respiratory illness (ARI), management is supportive, and detection of a specific virus from RPP does not impact clinical care. Therefore, clinical RPP use is not standardized, and ordering is at the discretion of the clinician. We sought to understand factors associated with RPP utilization among pediatric patients hospitalized with ARI. Methods: From October 2017 to September 2021, participants < 18 years hospitalized with ARI were enrolled at a single site in the New Vaccine Surveillance Network (NVSN). Eligible patients were residents of Jackson County, MO, had one or more ARI symptoms (e.g., cough, fever, nasal congestion) lasting < 14 days, and were enrolled within 48 hours of admission. Parent interviews and medical chart reviews were conducted. All participants had a research RPP, but results were not available to the clinical providers. Clinical providers were able to order a clinical RPP (cRPP), for which they received test results. Characteristics of NVSN enrollees hospitalized with ARI with and without a cRPP are described. Lastly, medical complexity was assessed via the pediatric complex chronic conditions classification system (CCC) then analyzed via chi- square test between groups. Findings: During the study period, 1,038 participants were enrolled, and 555 (53.4%) received a cRPP. Most, 299 (53%), cRPPs were ordered in the emergency department or urgent care before admission. Age was a significant factor associated with cRPP use (Table 1). cRPP participants were more likely to have complex chronic conditions, and/or technology dependence. No difference in cRPP use was observed by race/ethnicity, payer status, or sex. More participants were enrolled in 2020-2021, but the overall usage of cRPP is similar between years (Table 2). Conclusions: In this large cohort of children hospitalized with ARI, medical complexity, technological dependence, and age < 2 months were associated with increased utilization of cRPPs. Understanding the impact of cRPP on clinical care requires further investigation to better understand the utility of these tests.

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  Impact of Fetal Health Center on Mode of Neonatal Death

  Erin Bolen, Ashley Sherman, Julie Weiner, and Jotishna Sharma

  Introduction There has been significant innovation and technological advancement in prenatal diagnosis, fetal therapeutic intervention, and establishment of fetal health centers (FHC) at children’s hospitals with dedicated delivery services for high-risk neonates and expedited access to level IV neonatal intensive care units (NICU). Morality trends have been examined on individual bases and within diagnostic groups, but there have been few studies that examine overall modes of mortality in the NICU population across diagnoses after the implementation of a dedicated FHC. This study aims to compare modes of death at a level IV children’s hospital NICU between infants born at the fetal health center (inborn) and those transferred from referral centers (outborn). Study Design/Methods Patients who died at the Children’s Mercy Hospital NICU from January 2012-December 2022 were identified. FHC deliveries started in 2011. Via retrospective chart review, data were collected on place of birth and mode of death (CPR within six hours of death, withholding of potential interventions, or withdrawal of existing interventions). Results were evaluated with a 2-tailed z test. Further analysis will be undertaken. Results Over the 10-year study period, there were 10,862 admissions and 563 (5.2%) deaths. Of the deaths, 35.6% were inborn and 64.5% outborn. Of total admissions, 1,857 (17%) were inborn with 200 (10.7%) deaths. Outborn infants accounted for 9,005 admissions with 363 (4%) deaths. Data were available for 561 deaths, including all inborn deaths. Figures 1 (inborn) and 2 (outborn) show modes of death by year as a percentage. Modes of death were compared based on place of birth in Figure 3. Differences for withdrawal and withholding of interventions between outborn and inborn infants were significant with p=<0.00001 while the difference for receiving CPR approached but did not reach significance with p=0.073. Discussion The data demonstrate a significant difference in the percentage of withdrawal of intervention and withholding of intervention as the primary mode of death between inborn and outborn infants. This could be secondary to initial stabilization at outside hospitals prior to transport or indicate differences in the underlying diagnoses. This could also be secondary to inborn births receiving extensive prenatal counselling at the FHC with a do not resuscitate (DNR) at birth or limited intervention plan already in place. Further analyses based on diagnoses and type of birth plan will be undertaken. The presence of immediate post-natal withholding of care will be further evaluated with multivariate analysis based on age of death. Year-by-year data also demonstrates a trend of increased rates of CPR prior to death in the outborn group. FHC programs focus on congenital abnormalities that are amenable to prenatal diagnosis which may lead to an overrepresentation of devastating congenital abnormalities in this group. Further multivariate analysis will be undertaken to assess if this or other variables may be responsible for the differences in modes of death between populations. Conclusion Inborn newborns born at a fetal health center had a higher mortality compared to outborn infants and were more likely than outborn infants to have interventions held.

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  Increasing the Rate of Infants Rooming In with their Mothers with Low Acuity Congenital Heart Disease

  Anna Nelson, Amy Marks, and Ekta Patel

  Background The Children’s Mercy Fetal Health Center (FHC) was created to be able to deliver infants with complex fetal diagnoses including congenital heart disease. Mothers are referred from all over the Midwest region for their babies to have access to specialized care immediately after birth. Nearly all babies born in the FHC are admitted to our neonatal intensive care unit (NICU), as there are not well-established guidelines for keeping babies with their mothers if they have low-risk congenital heart disease that do not need intensive care continuous monitoring. Maternal infant bonding starts before an infant is born and becomes stronger in those first few moments after birth. Maternal infant bonding is important to help establish breastfeeding if desired and for early childhood development. Previously, only 27% of the patients with these diagnoses that would have been safe to room in with their mothers did. We aim to increase the rate of neonates born with low acuity congenital heart disease that room-in with their mothers in the FHC from 27% to 47% by October 2023. Methods We targeted infants with prenatal diagnoses of likely asymptomatic vascular rings, balanced AV canal, low risk intermittent fetal supraventricular tachycardia, tetralogy of Fallot without concern for pulmonary obstruction or low risk coarctation of the aorta. The first PDSA cycle implemented a protocol that was developed in collaboration with Pediatric Cardiologists to help providers assess the appropriateness of allowing a patient to room in with the mother with intermittent monitoring of vital signs. This protocol also dictates how frequently vital signs should be assessed by nursing once a baby is rooming-in with its mother. Education was provided to the physicians, NNPs and nurses about implementing the protocol. The second PDSA cycle included Fetal Cardiology APRNs discussing the appropriateness of patients rooming in with their mothers at weekly Fetal Health Center meetings. The quantitative data is collected monthly based upon FHC weekly patient e-mails and review of the medical records. Results The rate of infants rooming-in increased to 38% following implementation of the first PDSA cycle and up to 52% following implementation of the second PDSA cycle. Discussion We were able to increase the number of infants with low acuity congenital heart disease that roomed-in with their mothers in the FHC without any rapid responses. The next step is to survey families to better understand their experience of having their infant room-in with them in the FHC.

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  Increasing vaccination rates of 23-valent pneumococcal polysaccharide vaccine among patients at high-risk for invasive pneumococcal disease.

  Edward Lyon, Tracey Wetzel, Ann L. Wirtz, Douglas Swanson, Rachel Moran, Jessica Peters, Christine Symes, Liset Olarte, and Rana El Feghaly

  Introduction: Pneumococcal disease causes significant morbidity and mortality in children. Routine childhood immunizations protect for 13 or 15 pneumococcal serotypes via two pneumococcal conjugated vaccines. Patients with immunocompromising and chronic medical conditions are at high risk of invasive pneumococcal disease. A 23-valent pneumococcal polysaccharide vaccine (PPSV23) is recommended in these patients to protect against more pneumococcal serotypes, but many patients have not received it. Our AIM is to increase PPSV23 vaccination rates among eligible patients in both the inpatient and outpatient infectious diseases (ID) settings from a baseline of 44% to 55% by October 2024. Methods: In collaboration with Children’s Mercy integrated care solutions, we created a report of eligible patients evaluated by ID in the inpatient and outpatient settings. This report captures pneumococcal immunization history of patients eligible for PPSV23. In October 2022, we formed a multi-disciplinary team of ID nurses, a pharmacist, providers, and a patient advocate. We identified several potential causes of low PPSV23 vaccination rate and developed countermeasures (figure 1). Since October 2022, we have discussed the project weekly during our divisional huddle. In January 2023, we matched outpatient and inpatient reports to include maximal information and created a 2-way communication strategy where outpatient nursing staff notifies ID providers of patients who qualify for PPSV23. In February 2023, we created an EMR shared phrase to include in ID provider notes and a badge buddy with qualifying conditions for PPSV23 vaccination. Results: Following the initiation of the project we have seen an increase in qualifying patients who have received PPSV23 that has so far been sustained for 6 months (48-56%); we also see narrowing of the control limits implying improved process (figure 2). Discussion: We were able to increase PPSV23 vaccination of eligible high-risk patients in both the outpatient and inpatient settings. If this trend is sustained, we will be able to move our center line in 2 months. We have multiple additional plan-do-study-act cycles planned over the next several months to include electronic medical record changes, collaboration with different specialties and family education.

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  Latency Time From Prior Authorization Submission To Initiation Of Biologic Therapy In Inflammatory Bowel Disease

  Shannon Capraro, Janelle R. Noel-Macdonnell PhD, Lauren Disselhoff, Layne Sieve, and Rachel Chevalier

  Background For pediatric patients with a diagnosis of inflammatory bowel disease (IBD), initiation of biologic therapy can be essential to attain disease control. Unfortunately, patients often experience delays in receiving the first dose of these medications due to the need for insurance companies to approve medication prior authorizations (PA). Physicians and support staff spend many working hours collating and transferring information regarding urgency to payors. To our knowledge, no studies exist to evaluate the factors that contribute to how quickly a medication is approved in pediatric patients. We set out here to evaluate which demographic and disease factors contribute to the lag time between submission of medication prior authorization for IBD biologic therapy and administration of that therapy. Methods Our pilot cohort consists of 102 patients with IBD who initiated biologics as an outpatient at our tertiary care hospital between the years of 2018-2023. Clinical information regarding disease status, previous therapies, surgical history, laboratory information, and medication administration information was obtained from the chart. Descriptive statistics were performed and followed by bivariate summaries for insurance status (Medicaid users vs. not), initial denial of biologic, and type of biologic. Wilcoxon Rank Sum Tests and Kruskal-Wallis Test were used to compare time from PA to first dose depending on variable of interest. Results Demographics are included in Table 1. Patients were median age of 14.7 years (interquartile range [IQR] 11.5-17.0) and were 52.9% male. Most patients (80/102; 78.4%) have Crohn’s disease. In this cohort, the median time from submission of prior authorization to administration of medication (lag time) was 15.0 days (IQR 12.0-24.0) with maximum times up to 53 days. When evaluating between medications, infliximab had a significantly lower median lag time (median 14d; IQR 10-16d) compared to ustekinumab (17.5d; IQR 13-28) or adalimumab (18d; IQR 14-25d) but not vedolizumadb (16d; IQR 11-26) (Figure 1). No other pairings showed a significant difference. Pediatric Ulcerative Colitis Activity Index (PUCAI) decreased the lag time 1.5 days for every 10-point increase in PUCAI. Hemoglobin, albumin, ESR, and CRP contributions to lag time varied between medications. Lag time does not vary significantly between state insurance (Medicaid) and private insurance (Figure 2). Additionally, an initial denial of the prior authorization requiring appeal does not prolong lag time. Conclusion Early initiation of biologic therapy can delay the progression of disease to stricturing or penetrating disease 2 . However, providers experience significant challenges administering these medications in a timely manner. Notably, pediatric patients with IBD often wait at least two weeks to receive their first dose of biologic therapy. Our preliminary analysis does not indicate that severity of illness or type of insurance make clinically significant differences. It is important to remark that our population was limited in the number of Medicaid patients, so more data will be essential to corroborate this analysis. Infliximab shows the quickest availability perhaps due to its long history on the market, availability of biosimilars, or widespread use. Further analysis of our collected cohort of approximately 400 patients is pending including analysis of outcomes.

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  Life Threatening Anemia and Acidosis Secondary to Systemic Loxoscelism in a Pediatric Patient

  Rohan Akhouri, Vincent J. Calleo, and Leslie Hueschen

  Introduction Rashes are common in pediatric patients and most resolve without significant patient illness. In rare cases, they can represent life-threatening disease entities. History is crucial to determine a rash’s etiology, and barriers like developmental delay and language can complicate accurate diagnosis. We present a case of hemodynamic instability and hemolysis without known envenomation, which was diagnosed as systemic loxoscelism. Case Description A 10-year-old male with developmental delay presented with decreased activity and reduced oral intake for 4 days. An interpreter was used due to a language barrier. Initial vital signs were T 36.6°C, HR=120, RR=20, BP=117/66, and SpO2 96%. The child was listless with 1+ peripheral pulses and a capillary refill of 4 seconds. Skin exam showed a warm, hyperpigmented rash with rough texture on his trunk and upper extremities. Initial VBG showed a pH=7.04 and pCO2=27. His CBC showed hemolytic anemia with Hg=3.2g/dL and extreme leukocytosis with WBC=61x10*3/uL. After initial fluid bolus the patient became hypotensive, and labs showed worsening acidosis with a pH=6.89, pCO2=22, and lactate of 27mmol/L. Resuscitative efforts included fluid boluses, antibiotics, blood transfusion, sodium bicarbonate, and high flow supplemental O2. He was subsequently admitted to the PICU. After initial stabilization, further history revealed the patient had a rash and cellulitis on his left shoulder and completed treatment with amoxicillin one week prior to presentation. During his PICU course, multiple subspecialty services were consulted to assist with his workup. He received additional packed red blood cells (PRBC), rasburicase, steroids, and antibiotics. His rash evolved with desquamating areas and eschar formation at the left shoulder. Based on the extensive systemic symptoms, negative work-up for other etiologies, and an evolving eschar, the diagnosis of systemic loxoscelism was made. After 5 days, the patient recovered with supportive measures and is currently being followed for hemolytic anemia. Discussion Only 5 % of Brown recluse spider (Loxosceles reclusa) bites develop severe systemic manifestations. Life threatening toxicity can include severe hemolysis, acidosis, and autonomic instability. Treatments are largely supportive and include IV fluids, PRBC transfusions, and steroids. Antibiotics are often indicated if there are concerns for infection, and hemodialysis may be indicated for life threatening acidosis. Often loxoscelism patients do not know they have a spider bite which can delay diagnosis. Some other challenges with the initial diagnosis and management of this case included language barrier and the patients developmental delay with decreased mentation. Conclusion Systemic loxoscelism is a rare and life-threatening condition that should be considered in patients with hemolysis and acidosis, especially in the setting of eschar formation. Diagnosing rare conditions in patients with language barriers and developmental delay can be challenging, and provider awareness can increase the chances of a proper diagnosis.

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  National Prescribing Practices for Dystonia Among Providers in the United States

  Sarah Paige Davis, Natalie J. Kane, Haley Botteron, and Rose N. Gelineau-Morel

  Objective: While multiple oral medications are used to treat dystonia, limited information exists on current prescribing practices. This study analyzes prescribing practices for dystonia in the United States, evaluating variations in dosing and impact of co-morbidities. Methods: Querying the Cerner Real World database from 2014 to 2019 for children age 0-18 with an ICD-10 diagnosis containing “dystonia” resulted in 11,300 inpatient and outpatient encounters. Information extracted included current dystonia medications (baclofen, clonidine, carbidopa-levodopa, gabapentin, tetrabenazine, trihexyphenidyl, and benzodiazepines including diazepam, clonazepam, midazolam, and lorazepam), medication dosing, and co-morbid diagnoses of cerebral palsy, epilepsy, or spasticity. Encounters without current weight were excluded from medication dosage analysis. Results: Benzodiazepines were most commonly prescribed for dystonia (61.2%), then baclofen (12.0%) and clonidine (9.5%). Patients with cerebral palsy (n=5639) were prescribed baclofen more than clonidine (16.2% vs 7.0%), while patients without cerebral palsy (n=5731) were prescribed clonidine more frequently than baclofen (11.7% vs 7.6%). All medications showed decreased mean dosing in milligram per kilogram (mg/kg) as patient weight range increased. Mean weight-based dosing for baclofen varied by weight group from 0.162 mg/kg/dose (110-120 kg group, mean dose 18.3 mg) to 0.662 mg/kg/dose (0-20 kg group, mean dose 9.3 mg). Conclusion: Benzodiazepines, baclofen, and clonidine are the most common medications prescribed for dystonia in the United States. Medication dosing is variable, with patients of higher weights receiving smaller weight-adjusted doses. There is a need for dystonia medication selection and dosing guidelines to standardize care and inform dosing recommendations for clinical trials.

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  Oscillometry in Term Neonates without Respiratory Disease

  Anna Nelson, Cheri Gauldin, Brooke Smith, Venkatesh Sampath, and Winston M. Manimtim

  Background Bronchopulmonary dysplasia (BPD) is one of the most common complications of prematurity. While severity of BPD is defined by need and type of respiratory support at 36 weeks, it does not distinguish between endotypes of disease. Recent studies suggest that infants with severe BPD have parenchymal, airway and pulmonary vascular disease. Determining the BPD phenotype could help guide future individualized therapy. Oscillometry is a bedside tool that measures the resistance of the airways and the reactance of the pulmonary parenchyma. Normative values of oscillometry need to be established to serve as a comparison prior to establishing oscillometry characteristics of the different types of BPD. This study aims to determine normative values of oscillometry for neonates without respiratory disease. Methods Neonates born at a gestational age of ≥ 36 weeks and in room air were recruited at < 29 days of age. Exclusion criteria included current supplemental oxygen or positive pressure support. Neonates were tested at their bedside while asleep, supine, with head and neck in neutral positioning. The Tremoflo N-100 oscillometry device was applied to the neonate’s face with a simple face mask and 2Liters/min bias flow was introduced into the system. Neonates were breathing spontaneously while the oscillometry device introduced 6 different frequencies (7 Hz, 13 Hz, 20 Hz, 29 Hz and 35 Hz) into the neonate’s respiratory system. The device then determined the reactance and resistance of the respiratory system at each frequency. Neonates were tested 3 times, in 30 second intervals, with a few minutes rest allowed between each test. Results Eight patients have been recruited to participate at this time. The participant group has an average gestational age of 38.1 weeks, average birth weight of 3176 grams and average birth length of 50.9 cm. Preliminary data demonstrates consistent reactance values at 37, 29 and 20 Hz, with reactance spacing out some as the frequency decreases. The measured resistance at these frequencies (37 and 29 Hz) is also clustered, although the resistance values remain consistent at 13 Hz as well. The resistance is greatest at the lowest frequency of 7 Hz. Two test subjects have outlying data, but this does not appear to be related to gestational age or size of the subject. There was no difference in reactance or resistance based on gestational age, birth weight or birth length. Discussion Our findings are consistent with previous studies that have demonstrated resistance in the airways is highest at the lowest frequency of 7 Hz and resistance decreases with increasing frequency (Klinger et al.) Reactance is most negative at the lowest frequencies and increases with increasing frequency. Oscillometry provides consistent and reliable data for evaluating the pulmonary function of term, healthy newborns.

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  Rumination Syndrome Diagnosis Supported by Colon Manometry in a Pediatric Patient

  Alex Biller, John Rosen, and Amornluck Krasaelap

  Introduction: Rumination syndrome is a disorder of gut-brain interaction characterized by effortless repetitive postprandial regurgitation, reswallowing, and/or spitting.(1) This condition is caused by increased intragastric pressure due to abdominal muscle contractions, leading to the opening of the lower esophageal sphincter and the return of gastric content into the esophagus.(1) Rumination is a clinical diagnosis after the exclusion of other etiologies of symptoms. The diagnosis of rumination is clinical, but supportive evidence can be obtained through esophageal or antroduodenal manometry.(2) We present a unique case report of rumination syndrome diagnosis supported by data obtained during colon manometry in a pediatric patient. Case: We describe the case of a 13-year-old male with autistic spectrum disorder who presented with chronic constipation, fecal soiling, and poor response to standard laxative treatments. An anorectal and colon manometry was performed prior to an antegrade continence enema procedure. The anorectal manometry showed normal resting anal pressures and recto-anal inhibitory reflex but revealed dyssynergic defecation. During the colon manometry, normal contractions were observed in the proximal colon, but during the postprandial phase, a simultaneous increase in pressure ("r" waves) was detected across all sensors in the colon. (Figure 1) Coinciding with this increase in pressure, the patient was observed to regurgitate and rechew food without experiencing any pain or discomfort. The family reported that the patient had been exhibiting this behavior since early childhood. In addition to modifying the patient's bowel regimen, diaphragmatic breathing exercises were recommended to address the rumination syndrome. Discussion: This is the first reported case in which rumination syndrome was diagnosed using colon manometry. The presence of repetitive regurgitation and reswallowing during the postprandial phase alone would have been sufficient for establishing the diagnosis. Additionally, the simultaneous increase in pressure ("r" waves) observed across multiple colon sensors supports the diagnosis of rumination syndrome. These pressure waves likely originate from skeletal abdominal muscle contractions rather than from the stomach or intestines. Conclusion: Rumination syndrome is diagnosed based on the clinical presentation of effortless repetitive postprandial regurgitation, reswallowing, and/or spitting. Colon manometry, which detects spontaneous increases in pressure across multiple sensors due to abdominal muscle contractions, can be a valuable tool in supporting the diagnosis of rumination syndrome.

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  Sangrado del Tubo Digestivo Alto Secundario a Infección de Adenovirus

  Moises Alatorre-Jimenez, Sarah T. Edwards, and Thomas M. Attard

  Titulo: Sangrado del Tubo Digestivo Alto Secundario a Infección de Adenovirus Introducción: El Sangrado del tubo digestivo alto (STDA) es causado por múltiples etiologías incluyendo gastritis, varices, esofagitis, anomalidades vasculares, cuerpos extraños, y ulceras (1). Dentro de estas causas se conoce que infecciones virales pueden producir ulceras causando sangrado los más comunes son el virus de Epstein- Barr, Covid-19, Herpesvirus-6, Herpesvirus-7 y Citomegalovirus (1,2,3,4,5). Presentamos el caso de un paciente con STDA severo a causa de infección por adenovirus. Reporte de Caso: Paciente de 22 meses de edad, previamente sano que se presenta al servicio de Urgencias debido a un día de fatiga, anorexia y melena. El paciente presento a la hora de ingreso presento episodios de hematemesis. Laboratorios al momento demostraron anemia con hemoglobina de 8.2gm/dL, leucopenia 2.7 x 10x 103/mL, acidosis, elevación de las transaminasas (AST 553 U/L, ALT 417 U/L) y TP/INR normal. El paciente requirió varios paquetes de sangre, elevando su hemoglobina a 10.4 gm/dL y empezó tratamiento con infusión continua de octreotida y pantoprazol IV. A pesar del tratamiento continuo con hematemesis y la hemoglobina disminuyo a 6.7 gm/dL en menos de 8 horas post transfusión. El paciente fue llevado al quirófano para control hemorrágico con endoscopia. Se identifico de inicio un coagulo de gran tamaño (fig.1) que fue succionado demostrando múltiples ulceraciones serpiginosas profundas predominantemente en el fondo gástrico (fig 2). Múltiples de las ulceraciones tenían sangrado lento y se procedió a control con Hemospray®. Múltiples estudios virales incluyendo sangre, heces, o respiratorios concluyeron positivo para Adenovirus en PCR. Posterior al procedimiento el sangrado se detuvo y los laboratorios regresaron a valores normales. Se repitió la endoscopia tres meses visualmente normales. Discusión: Se han presentado varios casos en la literatura de STDA causado por en algunos casos por una infección viral (1). Los casos de ulceras por adenovirus son reportados en pacientes adultos y/o inmunocomprometidos (6). No hay casos reportados en pacientes pediátricos previamente sanos con infección de adenovirus causando STDA. La identificación de adenovirus en ulceras gástricas pudiera ayudar en un futuro a evitar iniciar tratamiento con antivirales, aunque pocos casos en la literatura (5). Conclusión: Reportamos este caso de pacientes pediátrico STDA causado por ulceras por adenovirus donde no se han encontrado casos reportados previamente en la literatura.

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  Teaching and Tomorrow: Children's Mercy Hospital Pediatric Gastroenterology Fellowship Program

  Children's Mercy Kansas City

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  The Implementation of a Pectus Bar Insertion ERAS Pathway

  Todd A. Glenski, Emily Weisberg, Christian M. Taylor, Brandon Layton, Andrea Melanson, and Sameer Lalani

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  A Genomics Driven Pluripotent Stem Cell Model of Infant Acute Lymphoblastic Leukemia

  Meagan Vacek, Jacqelyn Nemechek, Julia Draper, Irina Pushel, Bradley Thornton, Molly Leyda, Priyanka Prem Kumar, Midhat S. Farooqi, Jay L. Vivian, Erin M. Guest, and John M. Perry

  Acute lymphoblastic leukemia in infants (iALL) is a high-risk subtype of childhood leukemia, with poor survival outcomes despite intensive therapies. Rearrangement of KMT2A (KMT2A-r) on chromosome 11q23 is the most frequent cytogenetic abnormality in iALL, occurring in 70% of cases. KMT2A-r is associated with chemotherapy resistance, early relapse, and rapid leukemic progression, all of which contribute to poor survival outcomes. The most common KMT2A-r in iALL is KMT2A::AFF1 (MLL::AF4), derived from t(4;11)(q21;23) and arises in utero as shown in multiple clinical studies. Infant ALL’s cell of origin is thought to be a very early hematopoietic precursor, with transcriptomic studies of iALL blasts showing similarities to hematopoietic stem and progenitor cells (HSPCs), multipotent progenitors and early lymphoid progenitors (ELPs). Despite this, much is unknown regarding how KMT2A::AFF1 transforms early hematopoiesis, the cell types of origin, or how it alters the severity of the disease. Additionally, existing iALL models fail to mimic human disease, which creates an essential, ongoing need for the development of representative model systems for this devastating disease. To understand the development and progression of iALL, we have created a highly controlled human induced pluripotent stem (iPS) cell model to uncover the genomic and epigenetic landscape and cellular evolution of KMT2A::AFF1 iALL. Preliminary studies reveal our ability to differentiate human iPS cells into functional HSPCs with multilineage differentiation capacity and successfully express the doxycycline inducible KMT2A::Aff1 transgene in a controlled manner. We have also generated an in-house reference library of single cell RNA sequencing data and have compared transcriptomes of KMT2A-r blasts from iALL patients. This data revealed KMT2A-r cells are transcriptionally similar to ELPs. The goal for future investigations is to use single cell genomics to investigate transcriptomic changes during hematopoietic differentiation of our KMT2A:Aff1 iPS cell line. We also plan to compare these cells to publicly available single cell RNA sequencing data from multiple datasets that encompass fetal bone marrow and HSPC development. Through this research we expect to discover unique mechanisms of iALL emergence and progression with the long-term goal to uncover targets specific to iALL for the development of new therapies.

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  Exploring bidirectionality in US pediatric global health programs

  Anik Patel, Pauline Kamau, Ashley Combs, and Megan McHenry

  Introduction: Over the last two decades, GH programs have offered experiences at partner sites through established global health partnerships (GHPs). Historically, GHPs have been one-directional, favoring partners from resource-rich settings. These partnerships are now being reevaluated through the lens of equity and decolonization. We seek to understand how US pediatric GH faculty define the concept of “bidirectionality”, assess perspectives on their institution’s practice of bidirectional activities, and explore perceived benefits and challenges to prioritizing bidirectionality within their own GHPs. Methods: This is a cross-sectional qualitative survey study on bidirectionality of 206 AAMC-accredited US pediatric residency programs. 24 survey responses from global health faculty or staff at these programs were received. The REDCap-based online survey was developed by iterative repetition with a diverse author group. Data was collected from January 18, 2023 to February 20, 2023. Free-text responses were reviewed and coded through an inductive approach. Results: The majority of participants were “very familiar” with bidirectionality. Bidirectionality was described in terms of reciprocity, equity, and collaboration. Examples of programs’ own efforts to be bidirectional varied, and examples given varied to the degree of achieving bidirectionality. Perceived benefits of bidirectionality included friendship and mutual respect, educational opportunities, and opportunities to achieve equity. Perceived challenges of bidirectionality included technical barriers, cultural barriers and mismatched expectations, and financial barriers. Despite challenges, participants expressed persistence and the ability to be flexible in order to create and maintain bidirectional GHPs. Conclusions: A shared concept of bidirectionality exists, whereby partners collaborate at the personal and institutional level, forming a relationship based on mutual respect and understanding, resulting in a reciprocal and equitable exchange of benefits. Perceived benefits of bidirectionality can be difficult to quantify and justify in comparison to perceived challenges of bidirectionality. US pediatric GH programs should continue to reevaluate their own GHPs to assess if they are achieving true bidirectionality.