New Horizons in Emergency Department Management of Pediatric Sickle Cell Disease.

ABSTRACT: Sickle cell disease (SCD) is an important topic for emergency medicine audiences because complications of the disease account for a large proportion of hematologic emergencies that are seen in the emergency department each year. Early recognition and aggressive management of emergency complications of SCD can help to reduce the morbidity and mortality associated with this disease. Although the treatment recommendations for some complications of SCD are based on expert opinion, there has been advancement in the understanding of the pathogenesis of the disease and evidence regarding the treatment options available for managing acute complications. This continuing medical education article will provide a summary of the clinical manifestation and management of the most common acute complications of SCD: infection, vaso-occlusive episode, acute chest syndrome, splenic sequestration, stroke, and priapism.

Building a Physician-Scientist Pathway for Underrepresented Medical Students Through a Summer Research Experience.

PROBLEM: There is a need to increase the number of physician-scientists from underrepresented in medicine (URiM) groups. To engage URiM medical students, a committee of pediatric departmental leaders at the Children's Hospital of Philadelphia created the Summer Underrepresented in Medicine Medical Student Research program. This 8-week, onsite research and clinical experience takes place during the summer between students' first and second years of medical school. APPROACH: Applications were solicited between 2019-2023 through nationwide outreach to medical school deans and members of URiM student organizations. Accepted students were assigned a mentor to lead their research and clinical exposure. A curriculum highlighting aspects of academic medicine was developed. Students received a $3,000-$5,500 stipend for in-person participation. In 2020 and 2021, adjustments were made (e.g., virtual programming) to avoid interruptions during the COVID-19 pandemic. OUTCOMES: In the 2019-2023 application cycles, 298 students applied. Of 128 students who participated, 78 (61%) completed a postprogram survey. Students' survey feedback was positive. They indicated the program met expectations (mean rating = 1.3; scale: 1 = strongly agree to 5 = strongly disagree). Students reported they learned valuable information/skills (mean = 1.3) and that participation was worth time spent away from other responsibilities (mean = 1.3). The 2019 cohort (n = 12) achieved a 100% residency match rate. In addition, 4 (33%) of these students reported they are obtaining additional degrees or are performing research. After the program, many mentors continued to include students in their research projects. NEXT STEPS: Next steps include incorporating a standardized, scored rubric for selecting applicants; adding 3 lead mentors, an executive committee, and a faculty advisory board; establishing earlier pathway programming (e.g., at elementary and middle school levels); continuing to track/support alumni throughout their careers; and pursuing federal funding to expand the program.

Sickle Cell Disease Treatment with Arginine Therapy (STArT): study protocol for a phase 3 randomized controlled trial.

BACKGROUND: Despite substantial illness burden and healthcare utilization conferred by pain from vaso-occlusive episodes (VOE) in children with sickle cell disease (SCD), disease-modifying therapies to effectively treat SCD-VOE are lacking. The aim of the Sickle Cell Disease Treatment with Arginine Therapy (STArT) Trial is to provide definitive evidence regarding the efficacy of intravenous arginine as a treatment for acute SCD-VOE among children, adolescents, and young adults. METHODS: STArT is a double-blind, placebo-controlled, randomized, phase 3, multicenter trial of intravenous arginine therapy in 360 children, adolescents, and young adults who present with SCD-VOE. The STArT Trial is being conducted at 10 sites in the USA through the Pediatric Emergency Care Applied Research Network (PECARN). Enrollment began in 2021 and will continue for 5 years. Within 12 h of receiving their first dose of intravenous opioids, enrolled participants are randomized 1:1 to receive either (1) a one-time loading dose of L-arginine (200 mg/kg with a maximum of 20 g) administered intravenously followed by a standard dose of 100 mg/kg (maximum 10 g) three times a day or (2) a one-time placebo loading dose of normal saline followed by normal saline three times per day at equivalent volumes and duration as the study drug. Participants, research staff, and investigators are blinded to the participant's randomization. All clinical care is provided in accordance with the institution-specific standard of care for SCD-VOE based on the 2014 National Heart, Lung, and Blood Institute guidelines. The primary outcome is time to SCD-VOE pain crisis resolution, defined as the time (in hours) from study drug delivery to the last dose of parenteral opioid delivery. Secondary outcomes include total parental opioid use and patient-reported outcomes. In addition, the trial will characterize alterations in the arginine metabolome and mitochondrial function in children with SCD-VOE. DISCUSSION: Building on the foundation of established relationships between emergency medicine providers and hematologists in a multicenter research network to ensure adequate participant accrual, the STArT Trial will provide definitive information about the efficacy of intravenous arginine for the treatment of SCD-VOE for children. TRIAL REGISTRATION: The STArT Trial was registered in ClinicalTrials.gov on April 9, 2021, and enrollment began on June 21, 2021 (NCT04839354).

Return visit rates after an emergency department discharge for children with sickle cell pain episodes.

BACKGROUND: High return visit rates after hospitalization for people with sickle cell disease (SCD) have been previously established. Due to a lack of multicenter emergency department (ED) return visit rate data, the return visit rate following ED discharge for pediatric SCD pain treatment is currently unknown. PROCEDURE: A seven-site retrospective cohort study of discharged ED visits for pain by children with SCD was conducted using the Pediatric Emergency Care Applied Research Network Registry. Visits between January 2017 and November 2021 were identified using previously validated criteria. The primary outcome was the 14-day return visit rate, with 3- and 7-day rates also calculated. Modified Poisson regression was used to analyze associations for age, sex, initial hospitalization rate, and a visit during the COVID-19 pandemic with return visit rates. RESULTS: Of 2548 eligible ED visits, approximately 52% were patients less than 12 years old, 50% were female, and over 95% were non-Hispanic Black. The overall 14-day return visit rate was 29.1% (95% confidence interval [CI]: 27.4%-30.9%; site range 22.7%-31.7%); the 7- and 3-day return visit rates were 23.0% (95% CI: 21.3%-24.6%) and 16.7% (95% CI: 15.3%-18.2%), respectively. Younger children had slightly lower 14-day return visit rates (27.3% vs. 31.1%); there were no associations for site hospitalization rate, sex, and a visit occurring during the pandemic with 14-day returns. CONCLUSION: Nearly 30% of ED discharged visits after SCD pain treatment had a return visit within 14 days. Increased efforts are needed to identify causes for high ED return visit rates and ensure optimal ED and post-ED care.

A Comparative Analysis of Sensory Return in Delayed-Immediate Versus Immediate Neurotized Deep Inferior Epigastric Perforator Flap Breast Reconstruction.

BACKGROUND: Breast reconstruction patients who anticipate adjuvant radiation are not suitable candidates for immediate deep inferior epigastric perforator (DIEP) flap reconstruction due to the risk of flap fibrosis, shrinkage, and fat necrosis. Rather, many of these patients undergo delayed-immediate, or "babysitter," reconstruction, where a tissue expander is placed first as a temporizing measure during adjuvant therapy before definitive flap reconstruction. In this study, we aim to compare sensory changes in delayed-immediate to immediate DIEP flap patients. METHODS: Ninety-one patients, including 26 patients (46 breasts) with "babysitter" procedures and 65 patients (120 breasts) with immediate DIEP flaps, were prospectively identified at their preoperative visit. For both cohorts, baseline level (t = 0) is defined as before mastectomy. RESULTS: "Babysitter" patients underwent final-stage neurotized flap reconstruction on average at 12 months after initial tissue expander placement (range, 3-18 months). At 18 month after mastectomy (6 months after DIEP), delayed-immediate patients had comparable sensitivity measurements as immediate DIEP flap patients in all regions of the breast (P > 0.05). For delayed immediate patients, at 18 months postoperatively, sensitivity measurements were comparable with baseline levels only in the outer superior, outer medial, and outer lateral regions of the breast (P > 0.05). At 24 months postoperatively, cutaneous thresholds were comparable with baseline in all regions of the breast except the inner inferior region (P > 0.05), following a similar sensory recovery trajectory as immediate DIEP flap patients. CONCLUSIONS: In patients who undergo "babysitter" procedures, the combination of sensory return from the native mastectomy skin flap along with the neurotized DIEP flap yields sensory recovery comparable with immediate DIEP flap patients after definitive flap reconstruction. When final-stage flap reconstruction occurs by 12 months after mastectomy, sensation can return beginning 24 months postoperatively, or even sooner in some regions of the breast.

Bedside exclusion of pulmonary embolism in children without radiation (BEEPER): a national study of the Pediatric Emergency Care Applied Research Network-Study protocol.

Background: The Pulmonary Embolism Rule Out Criteria (PERC) Peds rule, derived from the PERC rule, was derived to estimate a low pretest probability for pulmonary embolism (PE) in children but has not been prospectively validated. Objective: The objective of this study was to present a protocol for an ongoing multicenter prospective observational study that evaluates the diagnostic accuracy of the PERC-Peds rule. Methods: This protocol is identified by the acronym, BEdside Exclusion of Pulmonary Embolism without Radiation in children. The study aims were designed to prospectively validate, or if necessary, refine, the accuracy of PERC-Peds and D-dimer in excluding PE among children with clinical suspicion or testing for PE. Multiple ancillary studies will examine clinical characteristics and epidemiology of the participants. Children aged 4 through 17 years were being enrolled at 21 sites through the Pediatric Emergency Care Applied Research Network (PECARN). Patients taking anticoagulant therapy are excluded. PERC-Peds criteria data, clinical gestalt, and demographic information are collected in real time. The criterion standard outcome is image-confirmed venous thromboembolism within 45 days, determined from independent expert adjudication. We assessed interrater reliability of the PERC-Peds, frequency of PERC-Peds use in routine clinical care, and descriptive characteristics of missed eligible and missed patients with PE. Results: Enrollment is currently 60% complete with an anticipated data lock in 2025. Conclusions: This prospective multicenter observational study will not only test whether a set of simple criteria can safely exclude PE without need for imaging but also provide a resource to fill a critical knowledge gap about clinical characteristics of children with suspected and diagnosed PE.

A Pilot Study Comparing Sensation in Buried Versus Nonburied Deep Inferior Epigastric Perforator Flaps.

BACKGROUND: Neurotized deep inferior epigastic perforator (DIEP) flaps have been shown to improve sensory recovery after mastectomy and reconstruction. With the recent trend toward nipple-sparing mastectomies, sensation likely originates within the buried DIEP flap and then innervates the breast skin. In contrast, for patients undergoing skin-sparing mastectomies, the DIEP flap skin is preserved, brought up to the surface, and directly innervated. In this study, we aim to evaluate inner breast region sensation between patients whose DIEP flap is buried and whose DIEP flap skin is brought to the surface. METHODS: Seventy patients who underwent mastectomy with immediate reconstruction using the DIEP flap were prospectively identified. Of these, 60 patients underwent nipple-sparing mastectomy with buried DIEP flap reconstruction while 10 patients underwent skin-sparing mastectomy with nonburied DIEP flap reconstruction. Patients in both cohorts received nerve grafting using the 70 × 1-2-mm Avance Nerve Graft in identical fashion. Sensitivity evaluation was performed in five inner breast regions (corresponding to the nonburied DIEP flap area). RESULTS: In the buried DIEP cohort, at 6 months postoperatively, there was a statistically significant difference in inner breast region sensitivity measurements compared with baseline levels ( P < 0.001). In contrast, in the nonburied DIEP cohort, at 6 months postoperatively, sensation in the inner breast region was comparable with preoperative baseline levels ( P = 0.236). At 24 months postoperatively, inner breast region sensitivity measurements in both cohorts were comparable with preoperative baseline measurements ( P > 0.05). CONCLUSIONS: Neurotized DIEP flap skin raised directly to the surface confers earlier sensory recovery than buried DIEP flaps. In patients who undergo skin-sparing mastectomies with nonburied DIEP flap reconstruction, they can expect significantly better sensation in the inner regions of the breast at 6 months postoperatively. In patients who undergo nipple-sparing mastectomies with buried DIEP flap reconstruction, they can expect sensation in the inner breast to return to preoperative baseline levels at a later time point-beginning as early as 24 months postoperatively.

Faculty Perspectives on Diversity, Equity, and Inclusion: Building a Foundation for Pediatrics.

ABSTRACT: Pediatric departments and children's hospitals (hereafter pediatric academic settings) increasingly promote the tenets of diversity, equity, and inclusion (DEI) as guiding principles to shape the mission areas of clinical care, education, research, and advocacy. Integrating DEI across these domains has the potential to advance health equity and workforce diversity. Historically, initiatives toward DEI have been fragmented with efforts predominantly led by individual faculty or subgroups of faculty with little institutional investment or strategic guidance. In many instances, there is a lack of understanding or consensus regarding what constitutes DEI activities, who engages in DEI activities, how faculty feel about their engagement, and what is an appropriate level of support. Concerns also exist that DEI work falls disproportionately to racial and ethnic groups underrepresented in medicine, exacerbating what is termed the minority tax. Despite these concerns, current literature lacks quantitative data characterizing such efforts and their potential impact on the minority tax. As pediatric academic settings invest in DEI programs and leadership roles, there is imperative to develop and use tools that can survey faculty perspectives, assess efforts, and align DEI efforts between academic faculty and health systems. Our exploratory assessment among academic pediatric faculty demonstrates that much of the DEI work in pediatric academic settings is done by a small number of individuals, predominantly Black faculty, with limited institutional support or recognition. Future efforts should focus on expanding participation among all groups and increasing institutional engagement.

Patterns of Change in Race Category in the Electronic Medical Record of a Pediatric Population.

This cross-sectional study sought to quantify the frequency of change in race category in the electronic medical record (EMR) of a pediatric population.

Diversity among research coordinators in a pediatric emergency medicine research collaborative network.

We conducted a survey study of clinical research coordinators (CRCs) at the member institutions of the Pediatric Emergency Care Applied Research Network, to determine the demographic and linguistic characteristics of CRCs around the network, and any perceived impact of those characteristics on their duties. A total of 53/74 CRCs completed the survey. Most respondents identified as "female," "white," and "not Hispanic/Latino." Most respondents felt that their race/ethnicity and their ability to speak a language other than English would positively impact recruitment. Four female respondents felt that their gender hindered their recruitment efforts and their sense of belonging within the research team.

A Qualitative Systematic Review of Pediatric Patient and Caregiver Perspectives on Pain Management for Vaso-Occlusive Episodes in the Emergency Department.

OBJECTIVES: The primary objective of this study is to describe the experiences of pediatric patients with sickle cell disease (SCD) and their caregivers who have presented to the emergency department (ED) for management of vaso-occlusive pain events. METHODS: We conducted a qualitative systematic review. The search protocol was developed to identify both published and unpublished literature that met inclusion/exclusion criteria. Included articles were primary hospital-based research with study populations that included (but were not limited to) pediatric patients aged 21 years or younger and qualitative or mixed-method analysis. RESULTS: Four themes were identified: (1) patients and caregivers perceive the ED as the last resort; (2) health care professionals in the ED lacked knowledge about SCD but rejected patients' and caregiver's attempts to share experience or advocate for their needs; (3) patients' accounts of pain are doubted because they do not always have "typical" signs of pain; and (4) caregivers identify racism as a reason for suboptimal care in the ED. CONCLUSIONS: There are multiple opportunities to improve management for vaso-occlusive pain events in the ED, including education of health care providers about SCD and complications, partnership between patients/caregivers and providers, and efforts to reduce the impact of systemic racism on health care delivery.

Intranasal fentanyl and discharge from the emergency department among children with sickle cell disease and vaso-occlusive pain: A multicenter pediatric emergency medicine perspective.

Children with sickle cell disease (SCD) commonly experience vaso-occlusive pain episodes (VOE) due to sickling of erythrocytes, which often requires care in the emergency department. Our objective was to assess the use and impact of intranasal fentanyl for the treatment of children with SCD-VOE on discharge from the emergency department in a multicenter study. We conducted a cross-sectional study at 20 academic pediatric emergency departments in the United States and Canada. We used logistic regression to test bivariable and multivariable associations between the outcome of discharge from the emergency department and candidate variables theoretically associated with discharge. The study included 400 patients; 215 (54%) were female. The median age was 14.6 (interquartile range 9.8, 17.6) years. Nineteen percent (n = 75) received intranasal fentanyl in the emergency department. Children who received intranasal fentanyl had nearly nine-fold greater adjusted odds of discharge from the emergency department compared to those who did not (adjusted odds ratio 8.99, 95% CI 2.81-30.56, p < .001). The rapid onset of action and ease of delivery without intravenous access offered by intranasal fentanyl make it a feasible initial parenteral analgesic in the treatment of children with SCD presenting with VOE in the acute-care setting. Further study is needed to determine potential causality of the association between intranasal fentanyl and discharge from the emergency department observed in this multicenter study.

Serious Bacterial Infections in Young Febrile Infants With Positive Urinalysis Results.

It is unknown whether febrile infants 29 to 60 days old with positive urinalysis results require routine lumbar punctures for evaluation of bacterial meningitis. OBJECTIVE: To determine the prevalence of bacteremia and/or bacterial meningitis in febrile infants ≤60 days of age with positive urinalysis (UA) results. METHODS: Secondary analysis of a prospective observational study of noncritical febrile infants ≤60 days between 2011 and 2019 conducted in the Pediatric Emergency Care Applied Research Network emergency departments. Participants had temperatures ≥38°C and were evaluated with blood cultures and had UAs available for analysis. We report the prevalence of bacteremia and bacterial meningitis in those with and without positive UA results. RESULTS: Among 7180 infants, 1090 (15.2%) had positive UA results. The risk of bacteremia was higher in those with positive versus negative UA results (63/1090 [5.8%] vs 69/6090 [1.1%], difference 4.7% [3.3% to 6.1%]). There was no difference in the prevalence of bacterial meningitis in infants ≤28 days of age with positive versus negative UA results (∼1% in both groups). However, among 697 infants aged 29 to 60 days with positive UA results, there were no cases of bacterial meningitis in comparison to 9 of 4153 with negative UA results (0.2%, difference -0.2% [-0.4% to -0.1%]). In addition, there were no cases of bacteremia and/or bacterial meningitis in the 148 infants ≤60 days of age with positive UA results who had the Pediatric Emergency Care Applied Research Network low-risk blood thresholds of absolute neutrophil count <4 × 103 cells/mm3 and procalcitonin <0.5 ng/mL. CONCLUSIONS: Among noncritical febrile infants ≤60 days of age with positive UA results, there were no cases of bacterial meningitis in those aged 29 to 60 days and no cases of bacteremia and/or bacterial meningitis in any low-risk infants based on low-risk blood thresholds in both months of life. These findings can guide lumbar puncture use and other clinical decision making.

Prepectoral Breast Reconstruction Without the Use of Acellular Dermal Matrix: A 3-Year Review.

INTRODUCTION: Acellular dermal matrix (ADM) is frequently used during prepectoral tissue expander-based breast reconstruction. However, there has been a paucity of literature describing the experience of prepectoral reconstruction without the accompanying use of ADM. We seek to highlight our institutional experience with immediate prepectoral tissue expander placement without the use of ADM in breast reconstruction. METHODS: A retrospective, single-institution review of patient records was performed to identify all patients who underwent either skin sparing or nipple-sparing mastectomy with immediate tissue expander placement without the use of ADM. Demographics including age, body mass index, comorbidities, history of smoking or steroid use, perioperative radiation or chemotherapy, intraoperative details, and complication profiles during the tissue expander stage were retrospectively collected and analyzed. At the time of tissue expander placement, all mastectomy flaps were evaluated clinically and with indocyanine green laser angiography. Postoperative outcomes were tracked. RESULTS: Between 2017 and 2020, 63 patients (for a total of 108 breasts) underwent either skin sparing (16%) or nipple-sparing mastectomy (84%) with immediate prepectoral tissue expander without ADM placement. Fourteen percent of breasts developed postoperative cellulitis, 19% of breasts developed skin compromise, and 5% required a postoperative revisional procedure that did not result in immediate expander explant. There was a 13% (n = 14 breasts) explant rate occurring at a mean time of 74 days. Of those breasts that developed skin compromise, 45% went on to require eventual explant. Patients in the study were followed for an average of 6.3 months. CONCLUSIONS: Immediate prepectoral breast reconstruction using tissue expanders without ADM offers a viable alternative to established reconstructive paradigms. The major complication rate for prepectoral reconstruction without the use of ADM (17%) was found to be comparable with our historical subpectoral tissue expander reconstruction with ADM use. Tissue expander explant rates were also comparable between the prepectoral without ADM (13%) and the subpectoral with ADM cohorts. These preliminary data suggest that immediate breast reconstruction with tissue expander placement without accompanying ADM is viable alternative in the breast reconstructive algorithm.