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Primary central nervous system (CNS) tumors are among the most common and lethal types of cancer in children. However, the existence of health disparities in CNS tumors by race or ethnicity remains poorly understood. This systematic review sought to determine whether racial and ethnic disparities in incidence, healthcare access, and survival exist among pediatric patients diagnosed with CNS tumors. A search of MEDLINE, Embase, CINAHL, Web of Science, and Scopus was conducted. Inclusion criteria selected for studies published between January 1, 2005 and July 15, 2020 that focused on pediatric populations in the US, evaluated for potential differences based on racial or ethnic backgrounds, and focused on CNS tumors. A standardized study form was used to collect study information, population of interest, research design, and quality of analysis, sample size, participant demographics, pathology evaluated, and incidence or outcomes observed. A total of 30 studies were inlcuded. Studies suggest White children may be more likely to be diagnosed with a CNS tumor and Hispanic children to present with advanced-stage disease and have worse outcomes. The degree of influence derived from socioeconomic factors is unclear. This review was limited by few available studies that included race and ethnicity as a variable, the overlap in databases used, and unclear categorization of race and ethnicity. This review identified notable and at times contradicting variations in racial/ethnic disparities among children with CNS tumors, suggesting that the extent of these disparities remains largely unknown and prompts further research to improve health equity.

Introduction: Many children with sickle cell anemia (SCA) require blood transfusions, which carry risks and utilize a scarce resource globally, particularly in Africa. Realizing Effectiveness Across Continents with Hydroxyurea (REACH, NCT01966731) has documented the safety, feasibility, and benefits of hydroxyurea for children with SCA living in sub-Saharan Africa. In REACH, hydroxyurea escalated to maximum tolerated dose (MTD) significantly decreased vaso-occlusive events, malaria, and death; transfusions were decreased by ~70% over 30 months of treatment when compared to the 2-month screening period. Characterizing how hydroxyurea reduces transfusion needs in REACH could contribute to improved clinical understanding and lead to better outcomes, a decreased transfusion burden, and preservation of the blood supply in these limited-resource settings. Methods: Transfusions were recorded prospectively in the REACH REDCap electronic database. Using time-varying predictors and landmark analysis, transfusions during screening and treatment were analyzed by clinical site, calendar month, age, gender, splenomegaly, hydroxyurea dose, MTD status, baseline and latest laboratory values (Hemoglobin, MCV, HbF, absolute neutrophil count, and platelets, all measured at least 30 days prior to the transfusion), alpha thalassemia trait, and G6PD deficiency. Incidence rate ratios (IRR) were calculated for treatment periods compared to screening. Univariate relationships were assessed using the Anderson-Gill model, plus multiple regression to estimate Hazard Ratios (HR) with 95% confidence intervals (CI's). Results: A total of 635 children with SCA enrolled in REACH, and 606 started hydroxyurea treatment. During screening, 48 transfusions were given to 43 children, and during the treatment phase 405 transfusions were administered to 214 children over an average treatment time of 5.2 ± 1.3 years. The transfusion rate was 43.3 per 100 patient-years during screening, which decreased to 22.0 per 100 patient-years during the initial fixed dose treatment period (IRR = 0.50; 95%CI = 0.35-0.74, p Conclusion: Hydroxyurea significantly reduces blood transfusion administration in children with SCA in sub-Saharan Africa, especially when escalated to MTD. Transfusions for the sole indication of anemia decreased significantly on hydroxyurea treatment, likely due to higher treatment-associated hemoglobin levels and decreased hemolysis, and transfusions for malaria also trended toward a decrease. Splenomegaly remains a risk factor for transfusions despite hydroxyurea treatment. Overall, increased access to and implementation of hydroxyurea treatment for children with SCA across sub-Saharan Africa may not only improve individual patient outcomes through reduction in anemia, transfusion burden, and transfusion-associated complications including infections, but may also to help preserve the scarce blood supply for the benefit of the larger population. Disclosures Aygun: Global Blood Therapeutics: Consultancy; Patient Centered Outcomes Research Institute: Research Funding; National Heart, Lung, Blood Institute: Research Funding; National Institute of Nursing Research: Research Funding; bluebird bio, Inc.: Membership on an entity's Board of Directors or advisory committees, Research Funding. Stuber: ASH Research Collaborative: Consultancy. Ware: Bristol Myers Squibb: Research Funding; Addmedica: Research Funding; Hemex Health: Research Funding; Nova Laboratories: Research Funding; Novartis: Other: DSMB Chair; Editas: Other: DSMB Chair.

Objectives Acute pancreatitis is a significant toxicity of L-asparaginase, a chemotherapeutic agent used to treat acute lymphoblastic leukemia. This case series describes the short-term clinical course and disposition of patients who developed asparaginase-associated pancreatitis (AAP) at one quaternary pediatric center. Methods Clinical data, including laboratory data, inpatient and intensive care unit (ICU) days, imaging findings, presence of complications such as need for ventilation, dialysis, and the development of pleural effusions, and mode of nutrition were abstracted from the medical record of patients with AAP. Pediatric criteria were used to classify episode severity based on the development of organ failure and local complications, such as pancreatic necrosis. Results Between 2005 and 2015, 34 patients had AAP with 43 distinct episodes of pancreatitis. The median inpatient length of stay was 10 days (range 2 to 65). Seven episodes (16.3%) required ICU-level care. Seventeen episodes (39.5%) were severe based on the development of organ failure or presence of pancreatic necrosis. Total parenteral nutrition (TPN) was used in 17 episodes (39.5%); for 34 episodes (79.1%), patients were discharged on entirely oral feeds. Antibiotics were administered in 20 episodes (46.5%). Pancreatic necrosis was identified within the first week in 12 episodes (27.9%). There were no deaths due to AAP. Conclusions The clinical course varies widely among patients with AAP. Over 1/3 of the patients in this series developed severe pancreatitis. Although the prognosis of AAP is generally good, many patients develop systemic complications of AAP, requiring TPN or ICU-level care. An infographic is available for this article at:http://links.lww.com/MPG/C5554.

Primary central nervous system (CNS) tumors are among the most common and lethal types of cancer in children. However, the existence of health disparities in CNS tumors by race or ethnicity remains poorly understood. This systematic review sought to determine whether racial and ethnic disparities in incidence, healthcare access, and survival exist among pediatric patients diagnosed with CNS tumors.A search of MEDLINE, Embase, CINAHL, Web of Science, and Scopus was conducted. Inclusion criteria selected for studies published between January 1, 2005 and July 15, 2020 that focused on pediatric populations in the US, evaluated for potential differences based on racial or ethnic backgrounds, and focused on CNS tumors. A standardized study form was used to collect study information, population of interest, research design, and quality of analysis, sample size, participant demographics, pathology evaluated, and incidence or outcomes observed.A total of 30 studies were inlcuded. Studies suggest White children may be more likely to be diagnosed with a CNS tumor and Hispanic children to present with advanced-stage disease and have worse outcomes. The degree of influence derived from socioeconomic factors is unclear. This review was limited by few available studies that included race and ethnicity as a variable, the overlap in databases used, and unclear categorization of race and ethnicity.This review identified notable and at times contradicting variations in racial/ethnic disparities among children with CNS tumors, suggesting that the extent of these disparities remains largely unknown and prompts further research to improve health equity.

Population-level estimates of the survival of children from low- and middle-income countries diagnosed with solid tumors do not currently exist, in contrast to outcomes of hematologic and central nervous system cancers, which have been collated from population-based cancer registries and published by the CONCORD Programme. To fill this knowledge gap, we conducted a systematic review of PubMed Legacy, Embase, Web of Science, Cochrane Central Registry of Controlled Trials, and three regional databases for publications that reported survival of children diagnosed age 0-14 years with any malignancy from January 2011 to December 2016. The search identified 4695 original records; 51 articles met inclusion criteria. The range of survival reported was broad; for instance, 5-year overall survival for retinoblastoma ranged from 48% in Central America to 98% in China. However, the paucity of published statistics prevented approaches for meta-analyses and emphasizes the need for more standardized data collection and reporting.

BACKGROUND In sickle cell disease (SCD), high emergency department (ED) utilization is associated with worse outcomes and increased costs. A metric called ED reliance (EDr), the percentage of healthcare visits that occur in the ED, attempts to identify ED overutilization. It is unknown if household material hardships (HMH)-housing, utility, or food insecurity-impact reliance on the ED. As these may represent modifiable risk factors for high ED utilization, we aimed to estimate the association between HMH and EDr in pediatric patients with SCD. METHODS We reviewed the electronic medical records of pediatric patients with SCD who received care in the Boston Medical Center network in Massachusetts, USA, to collect data on HMH and healthcare utilization. Using linear regression to control for potential confounders, we modeled the association between material hardships and EDr. RESULTS Of 101 eligible patients, 60 (59%) reported one or more HMH. The mean EDr was 12% overall, with significant differences between those with and without HMH (15.9 vs 5.9, P = 0.0001). Each additional hardship experienced was associated with an increased average EDr of 7.7 percentage points (R2 = 0.34, P

Purpose of review Over the past several decades, hydroxyurea has emerged as a well tolerated and potent disease-modifying therapy for children and adults with sickle cell anemia (SCA). Strong, evidence-based recommendations from the National Institutes of Health, American Society of Hematology, and British Society of Haematology document that hydroxyurea is now standard of care treatment for SCA. In low-resource settings, however, hydroxyurea is rarely utilized due to lack of availability, inadequate treatment guidance, and excessive costs. Recent findings Research trials conducted within the Caribbean and sub-Saharan Africa confirm the efficacy of hydroxyurea as a well tolerated, feasible, and beneficial treatment in low-resource countries. Hydroxyurea is therefore vital to reaching the targets for control of SCA outlined by the WHO. To maximize its utilization toward real-world effectiveness, specific attention must be given to healthcare provider education and training, public and institutional awareness, and medication access and affordability. Summary Efforts to introduce hydroxyurea effectively into low-resource countries should urgently address the lack of treatment guidelines, gaps in knowledge and clinical infrastructure, and medication inaccessibility. Partnerships among governmental, academic, pharmaceutical, and charitable organizations must tackle these barriers so that all individuals living with SCA worldwide can benefit from hydroxyurea.

Racism and Sickle Cell Disease As medical leaders around the United States issue statements denouncing racial injustice and calling for the dismantling of racism, we must ensure that these pledges ...

Introduction The medical community recognizes the importance of confronting structural racism and implicit bias to address health inequities. Several curricula aimed at teaching trainees about these issues are described in the literature. However, few curricula exist that engage faculty members as learners rather than teachers of these topics or target interdisciplinary audiences. Methods We developed a longitudinal case conference curriculum called Health Equity Rounds (HER) to discuss and address the impact of structural racism and implicit bias on patient care. The curriculum engaged participants across training levels and disciplines on these topics utilizing case-based discussion, evidence-based exercises, and two relevant conceptual frameworks. It was delivered quarterly as part of a departmental case conference series. We evaluated HER's feasibility and acceptability by tracking conference attendance and administering postconference surveys. We analyzed quantitative survey data using descriptive statistics and qualitatively reviewed free-text comments. Results We delivered seven 1-hour HER conferences at our institution from June 2016 to June 2018. A mean of 66 participants attended each HER. Most survey respondents (88% or more) indicated that HER promoted personal reflection on implicit bias, and 75% or more indicated that HER would impact their clinical practice. Discussion HER provided a unique forum for practitioners across training levels to address structural racism and implicit bias. Our aim in dissemination is to provide meaningful tools for others to adapt at their own institutions, recognizing that HER should serve as a component of larger, multifaceted efforts to decrease structural racism and implicit bias in health care.

BACKGROUND: Social determinants of health (SDoH) are socioeconomic factors that influence health outcomes. Guidelines recommend universal screening for SDoH at clinic visits, however, models that do not require additional resources are limited in subspecialty clinics. Individuals with sickle cell disease (SCD) face the burdens of chronic illness and often racial disparities, both of which may increase their vulnerability to adverse SDoH. Hematologists can impact both quality of life and clinical outcomes for their patients by implementing screening and referral programs addressing SDoH. METHODS: Through prospective, quality improvement methods, we introduced universal screening for SDoH into our pediatric hematology clinic. The intervention was a paper screener followed by a referral to local community organizations for the specific needs endorsed. The aims of this study were to determine the feasibility of universal screening for SDoH in a busy subspeciality clinic using pre-existing resources, to identify the needs of our patients, and to facilitate referrals between our patients and community organizations via this low touch intervention. RESULTS: Between August, 2017 and November, 2018, 156 screens were completed. Sixty-six percent were positive for at least one unmet social need for which 80% were referred to a relevant community organization. Forty-five percent of patients available via follow-up phone call reached out to the community organization. CONCLUSIONS: There is a high burden of SDoH in families of children with SCD. Universal screening in a pediatric hematology clinic with the subsequent connection of patients with SCD to community resources is feasible using existing clinic resources.

Progress has been made in resource-limited countries in treating acute lymphoblastic leukemia, but advances in solid malignancies have been slower. Multidisciplinary care coordination is challenging; assessing adherence to guidelines through quality improvement initiatives is essential. We characterized deviations from guidelines in the delivery of radiation in a middle-income country program as a pilot for evaluating adequacy of local control and as surrogate for integration of multidisciplinary care. One-third of patients for whom it was indicated did not receive radiation. Of the patients who received radiation, 95% had a deviation. This study underscores the importance of quality assessment in resource-limited settings.

Progress has been made in resource-limited countries in treating acute lymphoblastic leukemia, but advances in solid malignancies have been slower. Multidisciplinary care coordination is challenging, assessing adherence to guidelines through quality improvement initiatives is essential. We characterized deviations from guidelines in the delivery of radiation in a middle-income country program as a pilot for evaluating adequacy of local control and as surrogate for integration of multidisciplinary care. One-third of patients for whom it was indicated did not receive radiation. Of the patients who received radiation, 95% had a deviation. This study underscores the importance of quality assessment in resource-limited settings.