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  Rare variants in renal developmental genes and the risk of hypertension and CKD: a UK Biobank study

  Benjamin Spector, Byunggil Yoo, Neil Miller, and Laurel K. Willig

  Background: Prior studies show chronic kidney disease (CKD) is heritable but only a few common variants have been associated with CKD and kidney dysfunction. Much of CKD heritability remains unknown and limited studies have explored the role of rare genetic variants in this missing heritability. Objectives: Identify rare genetic variants in renal developmental genes associated with hypertension and CKD. Methods: We examined the association between rare variants in 58 candidate genes from five renal developmental compartments and presence of CKD and elevated blood pressure (BP) in 49,989 individuals using whole exome sequencing and phenotypic data from the UK Biobank. Criteria for qualifying rare variants included a minor allele frequency < 0.1% and classification as pathogenic, likely pathogenic, or variant of uncertain significance (VUS) using in-house characterization software. Logistic regression models were generated for each compartment to determine the predictive ability of qualifying variants for the outcomes of elevated BP and CKD, with additional subgroup analysis by genetic ethnicity. Genes were selected for inclusion in regression models based on p < 0.25 using Chi-square univariate analysis. Genes included for each developmental compartment’s regression model are summarized in Tables 1 & 2. Results: Qualifying variants in 5 genes across 3 developmental compartments were significant predictors of elevated BP; qualifying variants in 4 genes across 4 developmental compartments were predictive of CKD. In subgroup analysis of individuals genetically identified as Caucasian, qualifying variants in 4 genes across 3 compartments were predictive of elevated BP; qualifying variants in 3 genes across 2 compartments were predictive of CKD. For individuals genetically identified as non-Caucasian qualifying variants in 3 genes across 2 compartments were predictive of elevated BP; qualifying variants in 6 genes across 2 compartments were predictive of CKD (Tables 1 & 2). The distribution of qualifying variant types (i.e. pathogenic, likely pathogenic, VUS) within genes containing rare variants predictive of disease were predominately VUSs (Figure 1). Conclusion: Rare variants in some renal developmental genes are associated with elevated BP and CKD and may help explain a portion of the missing heritability. However, the significances of variants differ by ethnicity, and the majority are classified as VUSs requiring further characterization.

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  Rates of Physical Abuse Screening and Detection in Infants with Brief Resolved Unexplained Events (BRUEs)

  Angela Doswell, James Anderst, Joel Tieder, and Henry T. Puls

  Background: “Apparent Life-Threatening Events” (ALTEs) have been associated with child physical abuse (CPA). In 2016, “Brief Resolved Unexplained Event” (BRUE) and the development of its clinical guidelines and risk-stratification replaced ALTE. However, it is unknown if there is a similar association between BRUEs and CPA. Objectives: To determine the rate of CPA in infants presenting with a BRUE, examine differences between infants with and without CPA, and to examine rates of diagnostic testing used to detect CPA. Methods: This study was part of the BRUE Research and Quality Improvement Network, which is composed of 15 academic and community hospitals. Subjects were infants presenting with BRUE in emergency department or inpatient settings and followed from BRUE presentation through the first year of life. The primary outcome was CPA diagnosis at either initial BRUE or subsequent presentations. The secondary outcomes were minor evidence of trauma and diagnostic testing used to detect CPA (head imaging, skeletal survey, and/or liver transaminases) at initial BRUE presentation. Chi-square tests assessed for differences. Results: Of the 2036 infants presenting with a BRUE, 7 (0.3%) were diagnosed with CPA. Only 1 (<0.1%) infant was diagnosed with CPA at initial BRUE presentation. Infants diagnosed with CPA were more likely to exhibit color change (100% vs. 51.1%, p=0.01) and have minor evidence of trauma (14.3% vs. 0.3%, p<0.001) at initial BRUE presentation. There was no difference in CPA diagnosis by BRUE risk stratification. There were 7 (0.3%) infants with minor evidence of trauma, only 1 of whom was diagnosed with CPA. Of all infants, only 6.2% underwent head imaging, 7% skeletal survey, and 12.1% liver transaminases. Skeletal survey was more likely to be performed if there was minor evidence of trauma (42.9 vs. 6.9%, P <0.001) or a concerning social history (13.9% vs. 5.9%, p <0.05). Head imaging was more often performed if infants had minor evidence of trauma (71.4% vs. 6.0%; p< 0.001), family history of sudden unexplained death (10.2% vs. 6.3%; p= 0.047) or concerning social history (22.8% vs. 5.4%; p< 0.001). Conclusion: There was a lower rate of CPA in infants at initial BRUE presentation (<0.1%) than in infants with ALTE, although testing rates at initial BRUE presentation were also low. Minor evidence of trauma and concerning social history appeared to raise suspicion for and initiate diagnostic testing to detect CPA. Further research is warranted to systematically identify infants with BRUE at increased risk for CPA.

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  Self-Reported Sexual Behavior in A Pediatric Gender Clinic Sample

  Mirae J. Fornander, Anna Egan, and Christine Moser

  Introduction Previous studies of transgender/gender diverse (TGD) youth indicate differences in rates of sexual behaviors between TGD youth and their cisgender peers. However, few studies have reported the sexual behavior and preferences of TGD youth in a large clinic sample, and no studies, to our knowledge, have utilized questionnaires that do not assume the sex or gender of one’s sexual partner. The current study aims to explore the self-reported sexual behavior of TGD youth presenting for gender-affirming medical care. Methods Retrospective chart review of 589 patients (ages 12-22, M= 15.92, SD= 1.54; 72.9% assigned female sex at birth (SAB)) presenting for an initial visit at a pediatric transgender clinic from 2015-2021. Patients self-reported their gender identity, sexual behavior, sexual partners, and sexual interests via a REDCap electronic survey. Results Rates of dating (39.3% v 68.3%; X2=216.06, p< 0.0001) and sexual intercourse (22.9% vs 39.5% X2=65.69, p< 0.0001) among TGD youth were significantly lower than national norms (Kann et al., 2018). Rates of self-reported being forced to have sex among TGD youth was 7.6% and did not differ significantly from national norms (6.9%, ns; Kann et al., 2018). In the last year, TGD youth reported having one sexual partner (58.2%), 2-3 partners (19.4%), no partners (11.2%), 4-7 partners (8.2%), 8-10 partners (2.2%), and more than 10 partners (0.7%). Most TGD reported they were not likely to have sex in the next year (60.2%). TGD youth reporting using their mouth (11.2%), vagina (7.1%), penis (2.5%), anus (2.2%), or none of these body parts (0.7%) during sex. The gender of TGD youth partners included cisgender female (6.6%), cisgender male (5.8%), gender nonconforming female SAB (2.2%), transgender male (2.0%), gender nonconforming male SAB (1.4%), and transgender female (0.8%). Conclusions Results suggest that rates of dating and sexual intercourse among TGD youth are significantly lower than national norms. Clinically, results support screening for sexual behaviors and health among TGD youth and utilizing measures that do not assume the sex or gender of one’s partners. Future research should aim to develop a standardized measure of sexual behavior that addresses the unique needs of TGD youth to improve the accuracy of reporting in this population.

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  Trends in Proportions of Female Representation at American Society of Pediatric Nephrology Pediatric Academic Society Meetings 2012-2021

  Bahar Barani Najafabadi, Dave Selewski, Danielle Soranno, and Darcy Weidemann

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  Ultrasound Guided Peripheral IV Access Curriculum for the Pediatric Emergency Department: A Pilot Study.

  Samuel Dillman, Frances Turcotte Benedict, and Christopher S. Kennedy

  Ultrasound Guided Peripheral IV Access Curriculum for the Pediatric Emergency Department: a pilot study. Background: Up to 50% of children have difficult venous access. Studies in the Pediatric Emergency department (PED) have shown that ultrasound guided peripheral IV (USGPIV) access has decreased IV access time and ED length of stay. Barriers for use include lack of training and comfort with the procedure. Objectives/Goal: Our objectives are to pilot and evaluate USGPIV training for Pediatric Emergency Medicine (PEM) physicians and nurses. Methods/Design: The course included a 4-hour, hands-on session with US IV training using a blue phantom task trainer in conjunction with the Vascular Access Team (VAT) for PEM physicians and nurses. Participants’ skills were accessed by the trainers in two ways: 1. Consensus-based stepwise checklist (345-460 Excellent, 230-344 Good, 229-116 Fair, 0-115 Poor) 2. Global assessment of performance (1=Novice to 5= Expert). Obtained pre and post course and participant self-assessments by web-based survey (RedCap). Self-reported confidence with USGPIV was collected pre and post training and 3 months after training. Pre-post confidence mean scores were calculated for each participant and compared using a paired t-test (p value of <.05). Data collection included discipline and prior traditional IV or ultrasound experience. Course-based questions included if the training was an appropriate use of time, would be recommended to colleagues, and if it would be utilized in future practice. (5-point Likert scales: e.g. Novice to expert). Results: 23 providers were trained, 65% PEM physicians and 35% nurses. Skills assessment results for all participants scored an excellent rating (mean of 439) with subjective ratings most frequently indicating a competent trainee. There was a statistically significant increase in self-reported confidence in USGPIV placement post training (p < 0.0003). Three months post survey, confidence in skills significantly decreased. Learners recommend training to other providers, thought it was a good use of their time, and agreed they would utilize USGPIV access in their clinical practice. Conclusions: The training to date has shown acceptability, perceived increase in confidence, and excellent scores on training evaluations. Confidence decreased 4 months post training. Further steps include quality improvement of decreasing time to IV access in difficult access patients through utilization of USGPIV with patient data showing a 56-minute time to IV access for difficult venous access patients at our institution.

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  Evaluation Of The Outcomes Of Oral Challenges To Azithromycin, Cephalexin And Trimethoprim-Sulfamethoxazole In Pediatrics

  Hannah N. Neuhaus, Jordan Heath MD, and Salman Aljubran

  Rationale: Antibiotic hypersensitivity complicates treatment for various infections and leads to long-term healthcare costs and antibiotic resistance. Data regarding the outcomes of oral challenges to trimethoprim-sulfamethoxazole, azithromycin and cephalexin are limited in Pediatrics. The goal of this study was to characterize the outcomes and safety of oral challenges to these antibiotics in Pediatrics.

  Methods: A retrospective chart review was performed of pediatric patients who underwent oral challenges to cephalexin, azithromycin and trimethoprim-sulfamethoxazole in Allergy Clinic over the last 12 years.

  Results: Ten patients underwent oral challenge to trimethoprim-sulfamethoxazole and all were successful. Thirteen patients underwent oral challenge to azithromycin and twelve were successful. One patient failed the oral challenge with development of urticaria within 5 minutes on first dose. Twelve patients underwent oral challenge to cephalexin. Ten patients successfully passed the oral challenge while two failed. Of the two who failed, one patient later developed signs of viral illness and the second developed pruritic rash shortly after first dose and was transitioned to a desensitization protocol.

  Conclusions: Hypersensitivity to trimethoprim-sulfamethoxazole, azithromycin and cephalexin have significant impact on treatment for infections and require evaluation and de-labeling if possible. Overall, this study demonstrated that oral challenge to azithromycin, cephalexin and trimethoprim-sulfamethoxazole is a safe procedure to perform in select pediatric patients and can be done safely in the outpatient setting.

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  Extubated VV-ECMO for COVID-19 ARDS in an Immunosuppressed Pediatric Renal Transplant Patient.

  Johanna I. Orrick, Cara Holton, Tara Benton, and Jenna Miller

  Extracorporeal membrane oxygenation (ECMO) for coronavirus disease 2019 (COVID-19) associated acute respiratory distress syndrome (ARDS) in adults is common, and outcomes are similar to those for non-COVID-19 ARDS on ECMO1. However, children are much less likely to have severe COVID-19 disease, and thus the need for extracorporeal support is rare2,3. Even pediatric solid organ transplant recipients with active COVID-19 infections typically do not require ECMO.4 We present the case of an immunosuppressed pediatric patient with COVID-19-related ARDS who had an excellent outcome on VV-ECMO. An 11-year-old female with a history of polycystic kidney disease status post renal transplant three years earlier was admitted to the pediatric intensive care unit (PICU) with COVID-19 pneumonia. She developed severe ARDS and required intubation on hospital day (HD) 19. The patient's condition deteriorated despite trials of prone positioning, nitric oxide, and neuromuscular blockade. Due to her refractory hypoxemia, she was cannulated to venovenous (VV) ECMO on HD 28 utilizing a 27 french Avalon bi-caval dual lumen cannula. Laboratory testing demonstrated severely impaired immune function due to her baseline immunosuppressive regimen of mycophenolate, prednisone, and tacrolimus. In addition, she had received B cell depleting therapy with Rituximab several months prior to admission for management of transplant rejection. During her ECMO run, she developed pulmonary aspergillus in addition to her COVID-19 infection. Thus, the overall treatment goal was to minimize immunosuppression sufficiently to manage COVID-19 and aspergillus infections while not triggering rejection of her transplanted kidney. On admission, her mycophenolate dose and tacrolimus trough goal were reduced. When her status worsened shortly after intubation, mycophenolate was discontinued, and tacrolimus trough goal was further decreased. For treatment of acute COVID-19 pneumonia she received remdesivir, dexamethasone, tocilizumab, and convalescent plasma. Farther into her course, she was also treated for suspected MIS-C with anakinra, IVIG, and methylprednisolone. COVID-19 polymerase chain reaction (PCR) and cycle threshold times were monitored weekly. Gradually her cycle threshold times increased, and her COVID-19 PCR became negative on HD 61. With this laboratory and clinical improvement, her mycophenolate was restarted at 50% of the previous dose, and the tacrolimus trough goal was increased to its previous level. There were no concerns for graft rejection during her hospitalization. However, continuous renal replacement therapy (CRRT) was required in tandem for the first four days of extracorporeal support. Following this, she maintained good renal function with her transplanted kidney for the remainder of her hospital course. Five days after VV-ECMO cannulation, she was extubated to a high-flow nasal cannula. With weaning of neurosedative infusions, she was able to participate in pulmonary clearance and physical rehabilitation while on ECMO. With aggressive rehab she could sit on the edge of her bed and even transfer to a chair with assistance. After 33 days of ECMO support, she was decannulated on high-flow nasal cannula. She left the PICU on HD 83 and was discharged home without supplemental oxygen on HD 104. Our case experience suggests that extubated VV-ECMO can be a safe and effective rescue therapy for COVID-19-related ARDS in children, even in the setting of immunosuppression. Modification of immunosuppressive regimens in the setting of acute COVID-19 disease is not well described in the pediatric population.3 Our case study demonstrates it is possible to balance the risk of transplant rejection with infection control and ECMO therapy. Immunocompromised pediatric patients with COVID-19 can be considered candidates for ECMO support.

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  Timing Of Testing For IgE-Mediated Food Allergy After Systemic Reaction

  Jordan Pitt, Paul J. Dowling, Christopher Miller, Ashley Sherman, and Salman Aljubran

  Rationale: A refractory period of falsely negative testing can occur following a systemic allergic reaction to Hymenoptera sting. As a result, blood specific IgE and/or skin prick tests (SPT) for other allergens are often delayed. This retrospective chart review aims to identify the proportion of patients with falsely negative test results in the 6 weeks following an allergic reaction to food, and factors that may affect it. Methods: One hundred fourteen pediatric subjects met inclusion criteria. Each had a convincing history of food allergy with a systemic allergic reaction and was tested to the culprit food within 6 weeks. The proportion of negative tests for each testing modality was compared. Subjects testing negative were also compared to those testing positive. Chi-square and Fisher’s exact tests identified differences between groups. Results: Seventeen of 79 blood IgE tests (21.5%) and 6 of 35 SPT (17.1%) were negative, with no significant difference between the two tests (p-value 0.591). The distribution of trigger foods was significantly different in subjects with negative versus positive tests. Importantly, there were no subjects who tested falsely negative to tree nuts out of the 38 blood IgE tests and 13 SPT for tree nut allergy. Conclusions: The proportion of falsely negative tests in the 6 weeks following systemic allergic reactions to food is similarly low in both testing modalities. This proportion was even lower in subjects with tree nut allergy. Thus, testing can be considered to confirm food allergy in most patients with a convincing history during the weeks following a systemic reaction.

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  Getting Back to Global Health; Lake Atitlan, Guatemla

  Kyra L. McCarty

  Describes pediatric resident's exposure to the Guatemala health service and medical education system.

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  Elevated stool inflammatory markers in early onset polymorphic post-transplant lymphoproliferative disease following orthotopic heart transplant

  Jeremy Stewart, Keith August, and Thomas M. Attard

  Acute gastrointestinal hemorrhage in the context of a recent organ transplant with immunosuppression, antibiotic exposure, drug exposures and other comorbidities is a challenging clinical scenario with coordinated multidisciplinary effort needed to optimize management and outcomes. We present a patient with recent solid organ transplant with severe acute gastrointestinal bleeding attributable to EBV associated post-transplant lymphoproliferative disease. The patient is a 13-year-old Female with Williams syndrome who is EBV negative and CMV positive who received an EBV/CMV positive orthotopic heart transplant 5.5 months prior to presentation. She presented with bloody diarrhea, anemia (7.8 gm/dL), hypovolemia, and acute kidney injury (BUN 25 mg/dL - baseline 8-12) and admitted for IV fluid replacement, blood transfusion, and IV Iron infusion. Fecal calprotectin was 938 mcg/g and stool lactoferrin was 379.8 mcg/mL. MR enterography demonstrated inflammatory changes of the terminal ileum without evidence of stricture and prominent (reactive) mesenteric lymph nodes. EGD and Colonoscopy on day 8 of admission revealed multiple, sessile, semi-pedunculated, moderately vascular and raised lesions with central umbilication and granulation tissue at the apex ranging in size from 0.3 to 1 cm in the stomach, duodenum, terminal ileum, and the colon with similar findings in the small bowel on capsule endoscopy. She also had esophagitis, non-specific mild, chronic gastritis, normal terminal ileal and colonic mucosal biopsies with no inflammatory changes. Margin biopsies were infiltrated with a variable lymphoid cell population throughout the lamina propria, glandular architectural distortion, and focal gland destruction. The lymphoid cells were variable in size with small (CD3/CD43 positive) and large (CD20/CD43 positive) lymphocytes with vesicular chromatin and occasional prominent nuclei. EBER CISH was positive in many of the abnormal cells. She was diagnosed with EBV positive, polymorphic post-transplant lymphoproliferative disease (PTLD). Following a pre-treatment PET/CT scan revealing abnormal activity throughout the neck, chest, abdomen, and pelvis, she was started on rituximab, maintained tacrolimus with a goal serum level of 5-10, whereas MMF was withheld. PTLD is caused by proliferation of lymphoid or plasma cells and is the most common malignancy in the pediatric transplant population. The hallmark is EBV infection in the context of immunosuppression, occurs in approximately 2-15% of organ transplant recipients, can present with fever, lethargy, weight loss, generalized malaise, and lymphadenopathy but may also present with symptoms specific to the organ affected. Outside the gastrointestinal tract, PTLD can develop in the lymph system, central nervous system, lung, and liver. Gastrointestinal involvement includes bleeding, anemia, hypoalbuminemia, protein-losing enteropathy, and diarrhea. Treatment includes reduction in immunosuppression followed by a chemotherapeutic agent such as rituximab. Treatment can also involve surgical resection, radiation, anti-CD20 antibody therapy, or cytotoxic T-cell therapy. Outcomes vary by transplanted organ and can result in organ failure secondary to rejection and even death but with higher overall survival rates in children. The case presented is interesting insofar as the early presentation is atypical and it also illustrates the elevation in stool inflammatory markers, both lactoferrin and calprotectin, which has not previously been reported in the literature. The elevated stool markers are also interesting given the lack of gross morphologic or biopsy proven inflammation in non-lesion pathology. Our report underscores the utility of stool inflammatory markers in gastrointestinal bleeding in the solid-organ transplant recipient.

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  Grading of Improvement in Hypsarrhythmia with Standard Epileptic Spasms Treatment at a Large Pediatric Tertiary Care Center

  Julie Grace Gianakon, Roha Khalid, and Mohammed Ilyas

  Background Epileptic spasm is a peculiar type of epileptic seizure, entailing the clinical spasms and a characteristic electroencephalogram (EEG) abnormality often called hypsarrhythmia or its variants. The main goal of epileptic spasm treatment with standard therapy is to suppress clinical spasms and abolish the hypsarrhythmia and its variant EEG pattern. This interictal EEG pattern frequently heralds developmental regression. The elimination of hypsarrhythmia is a principal goal of therapy and a key outcome measure in clinical trials. There have been several studies in the interpretation or grading of hypsarrhythmia (Watanabe et al.1993 & Jeavons & Bower et al.1961). Still, there have been no studies in the grading of electrographic improvement with the standard treatment and its effects on the outcome, mainly in terms of remission vs. relapse. Objective: We aim to assess the electrographic improvement of hypsarrhythmia and its variants with standard hormonal (ACTH or prednisone) and Vigabatrin therapy based on the standard EEG scoring system (Kramer et al.1998). It is also generally accepted that outcome depends upon the underlying disease, so we selected patients with common etiologies (Cryptogenic, Down Syndrome, and Tuberous Sclerosis) and determined the EEG features predicting remission or relapse of epileptic spasms.

  Methods: This is a retrospective chart review examining several EEGs before and after treatment using a standard scoring system (Kramer et al.1998) for a follow-up period of at least 6months to 1year. We analyzed different EEGs and graded them based on a standard scoring system on pretreatment EEG (at the time of diagnosis) and post-treatment EEG changes (1-2wks, 2-3 months, or 6-12 months) in patients with epileptic spasms who received standard treatment.

  Results: Of 24 patients with epileptic spasms, 18 had EEG improvement of over 75% with resolution of spasms. Four had less than 50% improvement with refractory or recurrent spasms. Two patients had improvement between 50 and 75%. One had resolution of spasms, while the other had recurrent spasms.

  Conclusions: Patients with sustained improvement in EEG over at least 3-6 months of about > 75% were associated with spasms remission. Patients in whom the improvements were less than 50% continued to have ongoing spasms. These results highlight the need for the level of aggressiveness and close follow-up depending on the degree of EEG improvements with standard therapy.

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  Standardizing Resident Education on GI service

  Jennifer Halma and Kenneth Schmidt

  Introduction: The pediatric residency curriculum at Children’s Mercy Hospital is standardized to offer each trainee the same rotational experience. As a result, each resident spends one month on the inpatient gastroenterology (GI) service during training. This invites variability in the quantity and quality of GI education that residents receive. Therefore, there is a need to design a standard curriculum to provide comprehensive GI education. The aim of this project is to standardize resident education by identifying most requested topics and providing didactic education to increase resident general GI knowledge. Methods: Each month, residents were provided with a survey to assess comfort level in taking care of GI patients and writing total parenteral nutrition (TPN) orders, along with requesting their most desired didactic topics. These topics were then prioritized to occur as early in the month as possible. A similar survey was provided at the end of the month to measure the experience received. Results: 16 residents were surveyed between July 2020 and March 2021. The mean reported level of general GI knowledge at the start of the month was 4.0 and increased to 7.2 at the end of the month. TPN knowledge was rated a mean of 4.6 at the beginning of the service month and increased to a mean of 6.2 at the end of the service month. Using a pareto diagram (figure 1), it was determined that inflammatory bowel disease teaching and TPN teaching were among the most requested topics, along with short bowel syndrome and indications for endoscopy. The monthly curriculum was built to incorporate these four topics along with other highly requested teaching as early in the rotation as possible. Conclusion/Discussion: Our QI project has helped to standardize the education that all residents receive while on the GI inpatient service by identifying the most highly demanded teaching topics and prioritizing them early in each service month to meet their educational goals. Further investigation into the impact of these changes is necessary, including continued assessment of resident knowledge/comfort metrics between the start and end of each service month as well as resident satisfaction with the standardized teaching model.

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  A case of an elevated tryptase

  Sonya Parashar and Nikita Raje

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

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  Antimicrobial susceptibility testing practices at cystic fibrosis care centers

  Christopher M. Oermann, Claire Elson, Ellen Meier, and Megan Gripka

  Background: Published data suggest a lack of correlation between antimicrobial susceptibility testing (AST) of respiratory cultures and clinical outcomes in people with cystic fibrosis (CF). Nevertheless, AST is recommended by the CF Foundation (CFF) and the Cystic Fibrosis Trust. A survey of CF center program directors was conducted to understand how susceptibility testing is performed in North American CF centers. Methods: A survey was sent by the CFF to North American CF program directors and pharmacists via a CFF email distribution list. A reminder email was sent 2 weeks later. The survey was conducted using the online platform Survey Monkey, and responses were anonymous. Results: The survey of pharmacists is to be completed. The survey was completed by 36 of the 111 (32%) program directors who opened the email invitation. Two program directors provide care for fewer than 50 people with CF (6%),13 for 50 to 100 people (36%),10 for 100 to 200 people (28%), 4 for 200 to 300 people (11%), and 7 for more than 300 people (19%). In describing current AST practices, 3 CF centers (8%) had all susceptibility testing performed by a reference microbiology laboratory rather than an internal laboratory. AST methods included automated susceptibility testing (22, 67%), disk diffusion (17, 52%), and broth/microbroth dilution (14, 42%). Regarding frequency of obtaining respiratory cultures, 34 respondents reported obtaining respiratory cultures at each ambulatory care visit (94%), and 19 respondents obtained respiratory cultures during inpatient admission (53%). Thirty-three respondents (92%) reported obtaining identification and susceptibility testing for each respiratory culture collected. The remaining 3 respondents reported variable practices for susceptibility testing based on previous culture history. When asked about ideal AST practices, 21 respondents (58%) recommended susceptibility testing on every respiratory culture. If susceptibility testing was not performed on every culture, respondents recommended susceptibility testing at least once per year (8, 50%), each time a new isolate is identified (10, 63%), and as needed for a change in symptoms or clinical status during ambulatory care visits (8, 50%). Respondents reported using susceptibility data to inform clinical decisions, including initiation of antibiotic therapy for new isolates (30, 83%), initiation of antibiotics to treat pulmonary exacerbations (31, 86%), and for individuals with multidrug-resistant infections or a history of adverse drug reactions (31, 86%). Conclusion: The association between AST and clinical outcomes in CF is unclear, but most CF centers conduct routine susceptibility testing for each isolate as part of their standard of care. Almost half of program directors believed that AST was not needed for every culture. Finally, most program directors reported using AST data to inform clinical decisions. For these reasons, additional studies are needed to assess correlations more fully between ASTand clinical outcomes, and the cost-effectiveness of AST for all isolates should be assessed.

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  COVID-19 vaccination in individuals with cystic fibrosis at a pediatric cystic fibrosis center

  Christopher M. Oermann, Claire Elson, Ellen Meier, Paula Capel, Jessica Haynes, Michelle Fischer, and Stephanie Duehlmeyer

  Background: Observational data suggest that most people with cystic fibrosis (PwCF) who contract COVID-19 have outcomes similar to those of the general population, although PwCF who are older or have CF-related diabetes, poor lung function, or a history of lung transplantation may be at greater risk for more severe disease. Therefore, the CF Foundation advocates for PwCF to discuss vaccination with care teams. At present, the FDA has authorized emergency use of 3 COVID-19 vaccines. ACIP/CDC guidance allows each state to determine vaccine distribution based on an individual’s exposure and risk for severe disease. This study describes the attitudes of adolescents with CF followed by Children’s Mercy Kansas City (CMKC)’s CF center whowere eligible for COVID-19 vaccination in the state of Missouri. Methods: The CMKC Cystic Fibrosis Center is located in Missouri but provides care for 234 PwCF from Missouri and Kansas. COVID-19 vaccine was received from Missouri’s and Kansas’ allocation, with distribution based on state-wide, phased, and tiered systems. Phase 1B–Tier 2 in Missouri included, among others, individuals with chronic obstructive pulmonary disease (COPD). CMKC used the inclusion of COPD to advocate for PwCF to qualify for vaccination. Phase 4 in Kansas included PwCF. CMKC was allotted doses (first and second) to be administered over 7 vaccine clinic days for all CMKC patients meeting vaccination criteria. Center staff contacted (telephone and electronic medical record messaging) and documented vaccine status of all PwCF aged 16 and older receiving care at CMKC. Results: Of the 234 individuals followed at CMKC, 56 (24%) were aged 16 and older and eligible for COVID-19 vaccination. The median age was 18.0 (16. 1–20.8), and 31 (55%) were female. Of the 56 vaccine-eligible patients, we were unable to contact 10 (18%), 18 (32%) declined, and 28 (50%) scheduled vaccination. For thosewho declined, logistical issues were most common; 2 could not travel, and 3 had scheduling conflicts. Other reasons for decliningwere mistrust in the vaccine or pandemic severity (n = 3,17%), concerns about adverse reactions (n = 1), perceived lack of susceptibility to infection (n = 1), and current SARS-COV-2 infection (n = 2); 5 (28%) individuals refused without stating a reason, and one caregiver desired consent from a minor child. Of those receiving the vaccine, 25 (89%) had received an influenza vaccination in the 2 years prior; of those that refused, 16 (89%) had received an influenza vaccination. Conclusion: Of the 56 PwCF contacted, the majority agreed to COVID-19 vaccination. Avariety of reasons were given for declining vaccination. Most troubling of these were skepticism regarding the pandemic and vaccine necessity and misconceptions about safety and efficacy. As access to SARSCoV- 2 vaccination is expanded nationally and includes younger children with CF, it will be critical for CF care center staff to proactively address issues surrounding vaccination hesitancy.

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  Delayed-Onset Anaphylactic Reaction With High Fever After Amoxicillin Oral Challenge And Negative Penicillin Skin Testing

  Jordan Pitt, Paul J. Dowling, Christopher Miller, and Aarti Pandya

  Introduction: Immunologic adverse drug reactions can be categorized based on Gell and Coombs’s classification system. Anaphylaxis is generally considered a type I, immediate, IgE-mediated reaction and typically occurs independent of other immunologic reactions. However, the child presented here reacted after amoxicillin challenge with features of type I and type III or IV hypersensitivity reactions. Case Description: A 12-year-old female presented for amoxicillin allergy evaluation after treatment for scarlet fever with amoxicillin. After the second dose she developed rash with varied features, fatigue, edema, and joint swelling. Labs included a persistently low C4 level, eosinophilia, normal inflammatory markers, and normal tryptase level. It was unclear if symptoms were due to infection or drug reaction, so she underwent skin testing to benzylpenicillin, benzylpenicilloyl polylysine, and ampicillin which was negative. Two hours after a graded amoxicillin oral challenge, she developed shortness of breath, diffuse erythema, and pruritus. Epinephrine was administered with symptom resolution. One hour later, she developed diffuse erythema, periorbital/lip edema, nausea, delayed capillary refill, and high fever. Epinephrine and intravenous fluids were given with symptom improvement. Tryptase level was elevated from baseline. She was admitted and discharged asymptomatic the next day. Discussion: The patient’s symptoms and elevated tryptase are consistent with delayed-onset, biphasic anaphylaxis, however the presence of high fever suggests a co-existing type III or IV hypersensitivity reaction. The literature has reported rare cases of mixed hypersensitivity drug reactions that include multiple reaction types. Recognition of this phenomenon is important when evaluating patients with adverse drug reactions involving mixed features.

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  Evaluation of the Outcomes of Trimethoprim-Sulfamethoxazole Oral Challenges in the Pediatric Population

  Hannah N. Neuhaus and Salman Aljubran

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

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

  Results: Eleven patients were identified who underwent trimethoprim-sulfamethoxazole skin testing and/or oral challenge. Two reactions were consistent with an IgE-mediated process while the remainder were non-IgE-mediated/indeterminate. Two underwent skin testing to trimethoprim-sulfamethoxazole; both negative. Ten patients underwent successful oral challenge to trimethoprim-sulfamethoxazole; the eleventh patient was lost to follow-up after skin testing.

  Conclusion: Trimethoprim-sulfamethoxazole hypersensitivity is infrequently evaluated in the Pediatrics. However, trimethoprim-sulfamethoxazole is an effective antibiotic and reported allergy limits treatment for infections. Evaluation of the initial reaction is necessary, as many patients have non-IgE-mediated/indeterminate reactions. Severe cutaneous adverse reactions should be evaluated for when considering candidates for oral challenge to trimethoprim-sulfamethoxazole as oral challenge is contraindicated in these patients. Overall, this study demonstrated that oral challenge to trimethoprim-sulfamethoxazole is a safe procedure to perform in select pediatric patients and can be done safely in the outpatient setting. One limitation was small sample size.

  Educational Objective: Upon completion of this session, participants should be able to discuss the overall safety of oral challenges to Bactrim in the pediatric population.

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  Impact of Early Tracheostomy on Neurodevelopmental Outcome in Infants with Severe Bronchopulmonary Dysplasia Exposed to Postnatal Steroids

  Amjad Taha, Gangaram Akangire, Janelle R. Noel-Macdonnell, Tiffany Willis, and Winston Manimtim

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  Optic Disc Pit Maculopathy Leading to Vision Loss in a Pediatric Patient

  Allyson Hall

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

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  Optimizing oral glucose tolerance test completion at a pediatric cystic fibrosis care center: A 10-year continuing quality improvement effort

  Christopher M. Oermann, Paula Capel, Jessica Haynes, Michelle Fischer, and Jill Kohmetscher

  Background: Cystic fibrosis–related diabetes (CFRD) is a common comorbidity among people with CF (PwCF). It is associated with weight loss, protein catabolism, lung function decline, and increased mortality. Nutritional status and pulmonary function begin to decline in PwCF several years before the diagnosis of CFRD. Early CFRD detection and aggressive insulin therapy have been shown to reduce the mortality gap between PwCF who have CFRD and those who do not. The Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes recommend annual screening for people with CF starting at age 10 [1]. Methods: In 2011, team members at Children’s Mercy Kansas City (CMKC) embarked on a quality improvement (QI) project focused on improving oral glucose tolerance test (OGTT) completion rates in PwCF. During the initial phase of this project, QI methodology including fishbone diagrams and process flowcharts were employed to identify barriers to obtaining OGTTs. Patient education materials (English and Spanish) detailing the importance of and process for completing OGTTs were developed and distributed annually. A database for tracking PwCF who are greater than 10 years old and require OGTT was created. Weekly monitoring of upcoming appointments helped ensure that testing opportunities were not missed. Efforts were made to schedule OGTTs with annual laboratory testing to reduce phlebotomy. PwCF who wished to schedule with a local laboratory or provider were encouraged to do so and were provided with outside orders as needed. When PwCF in this group were admitted to the hospital, every attemptwas made to complete OGTTs near the end of their hospitalization. Results: Due to the lack of a standardized process and education, previous OGTT screening rates were poor: 9% in 2008, 13% in 2009, and 25% in 2010. During the first year of standard interventions (2011), the rate rose to 77%. By identifying barriers and standardizing our process, OGTT completion rates have continued to rise. In 2019 our OGTT completion rate was 92%, and in 2020—despite the COVID-19 pandemic which eliminated 3 months of testing opportunities—it was 81%. In recent years, endocrinology has partnered with the CF team in monthly CF/endocrinology “combo clinics,” which allow PwCF who have impaired glucose tolerance or CFRD to be evaluated by an endocrinology provider during their routine CF clinic visit. Conclusion: This QI project was initiated in 2011 and quality improvement work has continued to the present day. Continued education of PwCF and their families, tracking of testing, and commitment to sustained quality have allowed CMKC to attain high rates of OGTT completion. Earlier identification of impaired glucose tolerance and CFRD has allowed for earlier interventions, including dietary modifications, exercise recommendations, and endocrinology involvement in the plan of care.

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  Vancomycin AUC monitoring in individuals with cystic fibrosis at a pediatric institution

  Christopher M. Oermann, Stephanie Duehlmeyer, Ellen Meier, and Claire Elson

  Vancomycin AUC monitoring in individuals with cystic fibrosis at a pediatric institution S. Duehlmeyer1, C. Oermann1, E. Meier1, E. Elson1. 1Pulmonology, Children’s Mercy Kansas City, Kansas City, USA Background: Antibiotic therapy is essential for the treatment of cystic fibrosis (CF) lung infections. Methicillin-resistant Staphylococcus aureus (MRSA) infects 20% to 25% of people with CF (PwCF) and is associated with increased morbidity. Treatment of pulmonary exacerbations (PEs) often requires hospitalization including respiratory treatments and intravenous (IV) antimicrobials. IV vancomycin, which is commonly used for MRSA infections, requires serum concentration monitoring to ensure efficacy and minimize toxicity. Previous guidelines recommended trough concentrations to monitor efficacy and toxicity. Updated guidelines now recommend area under the curve (AUC) modeling as the optimal parameter for monitoring IV vancomycin. Methods: Children’s Mercy Kansas City (CMKC) changed IV vancomycin monitoring from trough to AUC/minimum inhibitory concentration (MIC) modeling on 01 May 2020 for PwCF. Two serum concentrations, a postdistributive and a trough, are obtained to estimate the AUC/MIC. A retrospective chart review collected trough monitoring data for all PwCF that received IV vancomycin at CMKC from01 January 2019 to 31 December 2019. Data for all PwCF treated with IV vancomycin after the AUC monitoring change were collected through 19 March 2021. Information on patient demographics, details of IV vancomycin therapy (dose, frequency, total exposure, nephrotoxicity), and monitoring data (serum concentrations, AUC modeling) were collected. Descriptive statistics were used to assess pre- and post-implementation data. Results: Before AUC monitoring, 25 patients received 42 courses of IV vancomycin; 14 were female (56%), and the median age was 14.02 years (4.25–20.25). Median treatment duration was 9.62 days (1.79–26.54), and median daily vancomycin exposure was 71.43 mg/kg/day (49.58–99.29). Target vancomycin trough concentration (≥15 μg/mL) was reached during 18 courses (43%). The median time to therapeutic trough was 83.58 hours (11.55–273.55) and required a median of 3 phlebotomies (1–9). Post-AUC there have been 15 courses of IV vancomycin in 8 PwCF; 5 were female (63%), and the median agewas 17.96 years (7.60–20.10). Median treatment duration was 9.52 days (5.68–14.63), and median daily vancomycin exposure was 75 mg/kg/day (48.63–92.80). All treatment courses reached target vancomycin AUC/MIC (400–600 μg/mL*h); median time to therapeutic AUC/MIC was 20.13 hours (11.6–106.12) and required a median of 3 phlebotomies (2–8). A median trough of 10 μg/mL (7–15 μg/mL) correlated with an AUC within target range. Conclusion: Changing to AUC monitoring for IV vancomycin in PwCF was not associated with a significant change in vancomycin daily exposure or duration. Fifty-seven percent more individuals achieved therapeutic targets with AUC monitoring (n = 15, 100%) than with trough monitoring (n = 18, 43%). AUC monitoring decreased time to therapeutic target by 63.45 hours. Trough concentrations of 15 μg/mL or less correlated with target AUC/MIC. A difference in nephrotoxicity was not seen. Study limitations include short postimplementation period (10 months) and small sample size. Ongoing data collection is planned.

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  A Proposal for Developing Academic Partnerships between American Clinical Institutions and NGOs

  Anik Patel, Nahreen Ahmed, Alfredo Mena Lora, and Riley Jones

  "Humanitarian and health-focused non-governmental organizations, such as MedGlobal, and American clinical institutions can mutually benefit from the formation of academic partnerships. MedGlobal has a longitudinal presence in ongoing humanitarian disasters that can provide rich clinical experiences for academic institutions looking to provide more diverse and equity-driven training for its residents and fellows. American institutions can provide resources, personnel with special skills and knowledge, as well as research assistance to MedGlobal and its partner sites as it continues to promote evidence-based clinical care for refugees. We propose an innovative model that addresses 3 areas of opportunities: education, research/QI, and clinical care. By regularly distributing a "menu" of opportunities dictated by needs on the ground, US academic programs can participate up to their capabilities and skill sets. These opportunities could include virtual lectures and modules, tele-health consultations, creating trainee rotations, participating in rigorous research and QI projects, holding in-person train-the-trainer sessions such as with neonatal resuscitation or ultrasound, and creating a vetted roster of American providers from partnered programs that could be deployed in the setting of acute disaster response. This model could be replicated by other NGOs working in the humanitarian space to improve education, knowledge, and clinical care for some of the most vulnerable patients in the world."

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  Assessing Early Use and Complications of Gastrostomy Blended Feeds.

  James Fraser, Kristen L. Sayers, Amy L. Pierce, Beth A. Orrick, Wendy Jo Svetanoff, Tolulope A. Oyetunji MD MPH, and Shawn D. St Peter

  Providers are hesitant to recommend using blended tube feeds (BF) after gastrostomy tube (GT) placement due to increased risk of bacterial contamination, nutrition inadequacy, tube blockages, and lack of data addressing clinical outcomes. Caregivers often feel that BF are more natural, better tolerated, and more cost-effective. We studied early use of BF, potential complications, and satisfaction among caregivers.

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  Descriptive Study of the Safety Behaviors and Attitudes of Portable Pool Owners

  Kristyn Jeffries, Kathy W. Monroe, Alicia Webb, Kristin L. Chancellor, Justina C. Goldman, and David C. Schwebel

  Background Drowning is the leading cause of injury death for children 1-4 years old and the second leading cause for children 5-9 years old. Most prior epidemiology work has focused on submersions in below-ground swimming pools and natural bodies of water. Portable pools pose a new and emerging risk for drowning due to their affordability, convenience, and easy assembly. Successful drowning prevention consumer products, such as 4-sided fencing, may prove more difficult to implement with portable pools, and currently are not marketed for such use. Furthermore, parental perceptions and knowledge of drowning risks associated with portable pools has not yet been well studied.

  Methods We performed a prospective study of caregivers to children aged 9 months to 6 years in an urban pediatric emergency department during summer 2021. Enrolled caregivers were given a QR code that directed them to complete a self-administered questionnaire on their mobile device. Survey questions assessed the caregivers’ access to portable pools and their safety behaviors and attitudes related to portable pools. Frequencies of portable pool ownership, caregivers’ safety practices while using them, and caregivers’ behavioral perceptions were calculated.

  Results Of the 85 caregivers enrolled in the study, 54% reported either owning or having access to a portable pool. Of the subset who owned portable pools, a majority (n=23/28) bought their portable pool in 2021, but only 28% (n=8/28) used any safety products with their pool and only 10% had previously enrolled their child in formal swim lessons. The primary reasons portable pool owners did not use safety products included perceived lack of necessity of such products for portable pools and confidence in close supervision while their child is swimming. While all caregivers (n=85/85) responded they would always watch their child in the shallow end of a below-ground pool, 14% (n=12/85) of caregivers responded they would only watch their child intermittently while in a portable pool. Over 48% (n=41/85) of caregivers thought they would hear their child if he/she was drowning in a portable pool.

  Conclusions Parents may underestimate the risk associated with portable pools, which could contribute to young children’s risk of drowning in these pools. These results provide insights that could be used in the development of drowning prevention messaging and the development of prevention strategies specifically targeting portable pool users.

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  Pharmacogenetic Testing In Patients with Autism Spectrum Disorder Evaluated in a Pediatric Precision Medicine Clinic

  Rachel Goodson, Cy Nadler, Jennifer A. Wagner, Sarah Soden, Sarah Nyp, and Tracy L. Sandritter

  Pharmacogenetic Testing In Patients with Autism Spectrum Disorder Evaluated in a Pediatric Precision Medicine Clinic Purpose The purpose of this study is to investigate the demographic and presentation profiles of children with autism spectrum disorder (ASD) who present for evaluation in a pediatric precision medicine clinic. Methods: This retrospective, observational cohort study utilized data extracted from a pediatric precision medicine clinic database between 2010 and 2021 with recorded ICD9/10 codes of Autism Spectrum Disorder, Autistic Disorder, Pervasive Developmental Disorder, or Asperger’s Syndrome. Extracted variables included demographic data, presenting medication regimens and concerns to be addressed by precision medicine. Results: A sample of 202 patients was identified (see Table 1). Patients referred for precision medicine services were primarily due to poor medication response (64.8%) and/or adverse drug reactions (48.5%). Referrals were made by subspecialists (78.2%), primary care providers (16.3%), and via self-referral (4.95%). At presentation to the clinic, patients were already prescribed between 1-10 medications (Mean = 6.15, Median 5; see Figure 1). Medications with indication commonly used for sleep, gastrointestinal disorders, and psychiatric/behavioral disorders were among the most common medications taken at the time of evaluation (see Figure 2). At time of presentation to the clinic, males and females did not differ in terms of age (t= 1.22, p = 0.22) or number of medications taken (t = 0.994, p = 0.323). Age was also not significantly associated with number of medications (F = 0.277, p = 0.527). Conclusions: Youth with ASD presenting for precision medicine consultation experienced notable degrees of polypharmacy, with no clear differences associated with sex or age. Trends may emerge with the addition of a typically developing control group, and the robustness of associations must be evaluated in samples drawn from a wider variety of practice settings. While these findings are primarily descriptive, the data fill a critical gap regarding the characteristics of youth with ASD referred for precision medicine. Few dedicated precision medicine clinics exist, yet this service is increasingly recommended for youth with autism due to higher rates of adverse drug reactions and clinical nonresponse. More research is needed to establish how precision medicine fits within existing evidence-based guidelines and can most effectively serve this population.