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ObjectiveTo compare clinical outcomes in children with hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) who were managed before and after implementation of an evidence-based guideline (EBG).MethodsA management algorithm for MAS-HLH was developed at our institution based on literature review, expert opinion, and consensus building across multiple pediatric subspecialties. An electronic medical record search retrospectively identified hospitalized patients with MAS-HLH in the pre-EBG (October 15, 2015, to December 4, 2017) and post-EBG (January 1, 2018, to January 21, 2020) time periods. Predetermined outcome metrics were evaluated in the 2 cohorts.ResultsAfter the EBG launch, 57 children were identified by house staff as potential patients with MAS-HLH, and rheumatology was consulted for management. Ultimately, 17 patients were diagnosed with MAS-HLH by the treating team. Of these, 59% met HLH 2004 criteria, and 94% met 2016 classification criteria for MAS complicating systemic juvenile idiopathic arthritis. There was a statistically significant reduction in mortality from 50% before implementation of the EBG to 6% in the post-EBG cohort (P = 0.02). There was a significant improvement in time to 50% reduction in C-reactive protein level in the post-EBG vs pre-EBG cohorts (log-rank P < 0.01). There were trends toward faster time to MAS-HLH diagnosis, faster initiation of immunosuppressive therapy, shorter length of hospital stay, and more rapid normalization of MAS-HLH–related biomarkers in the patients post-EBG.ConclusionWhile the observed improvements may be partially attributed to advances in treatment of MAS-HLH that have accumulated over time, this analysis also suggests that a multidisciplinary treatment pathway for MAS-HLH contributed meaningfully to favorable patient outcomes.

Prompt escalation to tumor necrosis factor inhibitors (TNFi) is recommended for children with juvenile idiopathic arthritis (JIA) and ongoing disease activity despite conventional disease-modifying antirheumatic drugs (cDMARDs). It is unknown if these recommendations are equitably followed for children with different insurance types. We assessed the association of insurance coverage on odds and timing of TNFi use.We conducted a retrospective study of children with newly diagnosed JIA in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. We compared the odds of starting a TNFi in the first year and time from cDMARD to TNFi between those with public and private insurance.We identified 1086 children with new JIA diagnoses. Publicly insured children had significantly higher active joint counts and parent/patient global assessment scores at enrollment visit, and were more likely to have polyarticular arthritis compared to those with private insurance. Odds of any TNFi use in first year did not differ between publicly and privately insured children. Publicly insured children were escalated from cDMARD to TNFi more quickly than privately insured children.Children who were publicly insured had more severe disease and polyarticular involvement at Registry enrollment compared to those who were privately insured. While overall TNFi use did not differ between children with different insurance types, publicly insured children were escalated more quickly, consistent with their increased disease severity. Further research is needed to determine why insurance coverage type is associated with disease severity, including how other socioeconomic factors impact presentation to care.

Objective To investigate the incidence and risk factors for hypogammaglobulinaemia and infectious complications associated with rituximab treatment in childhood-onset rheumatic diseases. Methods We performed a single-centre retrospective study of patients (n = 85) treated at Boston Children’s Hospital (BCH) from 2009 to 2019. Study subjects included patients (ages 6–24 years) who received rituximab for the treatment of a childhood-onset rheumatic disease. Results New-onset hypogammaglobulinaemia developed in 23 (27.1%) patients within 18 months of rituximab induction treatment. Twenty-two patients (25.9%) developed at least one infectious complication in the 18 months following the first rituximab infusion; of these, 11 (50%) had serious infections requiring inpatient treatment. After adjusting for potential confounders, exposure to pulse corticosteroid therapy in the month prior to rituximab use was a significant predictor of both new-onset hypogammaglobulinaemia (odds ratio [OR] 3.94; 95% CI: 1.07, 16.0; P = 0.044) and infectious complications (OR 15.3; 95% CI: 3.04, 126.8; P = 0.003). Post-rituximab hypogammaglobulinaemia was the strongest predictor of serious infectious complications (OR 7.89; 95% CI: 1.41, 65.6; P = 0.028). Younger age at rituximab use was also a significant predictor of new-onset hypogammaglobulinaemia (OR 0.83; 95% CI: 0.70, 0.97; P = 0.021). Compared with other rheumatic diseases, patients with vasculitis had a higher likelihood of developing infectious complications, including serious infections. Conclusion Although rituximab was well tolerated in terms of infectious complications in the majority of patients with childhood-onset rheumatic diseases, a substantial proportion developed new-onset hypogammaglobulinaemia and infectious complications following treatment. Our study highlights a role for heightened vigilance of rituximab-associated hypogammaglobulinaemia and infections in paediatric patients with rheumatic conditions.

Purpose of review To review diagnosis, clinical characteristics and treatment of multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Recent findings MIS-C emerged in spring 2020 as a hyperinflammatory syndrome following SARS-CoV-2 exposure in children. Despite growing awareness of MIS-C, diagnosis remains challenging due to the range of phenotypes and severity. Fever accompanied by shock, cardiac dysfunction, gastrointestinal symptoms, or mucocutaneous signs suggestive of Kawasaki disease, especially in the presence of known or suspected coronavirus disease 2019 exposure, should trigger consideration of MIS-C. However, clinical presentations are highly varied and may overlap with other infectious diseases. Clinicians must maintain a high index of suspicion for MIS-C and be aware that patients may develop coronary artery aneurysms and myocarditis even with few or no Kawasaki disease symptoms. More precise diagnostic criteria and specific biomarkers are needed to aid diagnosis. Intravenous immunoglobulin (IVIG) is first-line therapy, and steroids should be considered as initial adjunctive treatment for patients with severe manifestations or other risk factors. Prompt treatment is essential, as patients may worsen acutely, though overall prognosis is reassuring. Summary MIS-C associated with SARS-CoV-2 has varied clinical manifestations. Clinicians must be aware of the common presentation and potential for decompensation and cardiac sequalae to guide appropriate evaluation and treatment.

Multisystem inflammatory syndrome in children following severe acute respiratory syndrome coronavirus 2 infection is characterized by fever, elevated inflammatory markers, and multisystem organ involvement. Presentations are variable but often include gastrointestinal symptoms. We describe 5 children with fever and gastrointestinal symptoms initially concerning for multisystem inflammatory syndrome in children who were ultimately diagnosed with bacterial enteritis, highlighting the diagnostic challenges presented by the severe acute respiratory syndrome coronavirus 2 pandemic.

ObjectivePrompt escalation to tumor necrosis factor inhibitors (TNFis) is recommended for children with juvenile idiopathic arthritis (JIA) and ongoing disease activity despite treatment with conventional disease-modifying antirheumatic drugs (cDMARDs). It is unknown whether these recommendations are equitably followed for children with different insurance types. We assessed the association of insurance coverage on the odds and timing of TNFi use.MethodsWe conducted a retrospective study of children with newly diagnosed JIA in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. We compared the odds of starting a TNFi in the first year and time from cDMARD to TNFi initiation between those with public and private insurance.ResultsWe identified 1086 children with new JIA diagnoses. Publicly insured children had significantly higher active joint counts and parent/patient global assessment scores at the enrollment visit. They were also more likely to have polyarticular arthritis compared to those with private insurance. Odds of any TNFi use in the first year did not differ between publicly and privately insured children. Publicly insured children were escalated from cDMARD to TNFi more quickly than privately insured children.ConclusionChildren who were publicly insured had more severe disease and polyarticular involvement at registry enrollment compared to those who were privately insured. Whereas overall TNFi use did not differ between children with different insurance types, publicly insured children were escalated more quickly, consistent with their increased disease severity. Further research is needed to determine why insurance coverage type is associated with disease severity, including how other socioeconomic factors affect presentation to care.

Oligoarticular juvenile idiopathic arthritis (oligo JIA) is the most common form of chronic inflammatory arthritis in children, yet the cause of this disease remains unknown. To understand immune responses in oligo JIA, we immunophenotyped synovial fluid T cells with flow cytometry, bulk RNA-Seq, single-cell RNA-Seq (scRNA-Seq), DNA methylation studies, and Treg suppression assays. In synovial fluid, CD4+, CD8+, and gamma delta T cells expressed Th1-related markers, whereas Th17 cells were not enriched. Th1 skewing was prominent in CD4+ T cells, including Tregs, and was associated with severe disease. Transcriptomic studies confirmed a Th1 signature in CD4+ T cells from synovial fluid. The regulatory gene expression signature was preserved in Tregs, even those exhibiting Th1 polarization. These Th1-like Tregs maintained Treg-specific methylation patterns and suppressive function, supporting the stability of this Treg population in the joint. Although synovial fluid CD4+ T cells displayed an overall Th1 phenotype, scRNA-Seq uncovered heterogeneous effector and regulatory subpopulations, including IFN-induced Tregs, peripheral helper T cells, and cytotoxic CD4+ T cells. In conclusion, oligo JIA is characterized by Th1 polarization that encompasses Tregs but does not compromise their regulatory identity. Targeting Th1-driven inflammation and augmenting Treg function may represent important therapeutic approaches in oligo JIA. Joint Biology Consortium [P30 AR070253]; NIH National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)United States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Arthritis & Musculoskeletal & Skin Diseases (NIAMS) [K08 AR077037, T32 HL007633-35]; NIHUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USA [5T32AI007512-34]; NIAMSUnited States Department of Health & Human ServicesNational Institutes of Health (NIH) - USANIH National Institute of Arthritis & Musculoskeletal & Skin Diseases (NIAMS) [P30 AR070253, R01 AR075906, R01 AR073201, K08 AR073339]; Fundacion Bechara; Arbuckle Family Fund for Arthritis Research; Rheumatology Research Foundation; Arthritis National Research Foundation; Office of Faculty Development at Boston Children's Hospital Published version We thank the patients and volunteers for participation in this study. We thank Steve Moskowit for support in preparing the graphical abstract for this study. The graphical abstract makes use of images available at Servier Medical Art (https://smart.servier.com).AMJ was supported in part by a microgrant from the Joint Biology Consortium (P30 AR070253). KW is supported by NIH National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) K08 AR077037. JN was funded by T32 HL007633-35. JER was supported by the NIH grant 5T32AI007512-34. PAN was funded by NIAMS awards R01 AR075906, R01 AR073201, P30 AR070253; the Fundacion Bechara; and the Arbuckle Family Fund for Arthritis Research. LAH was funded by NIAMS K08 AR073339, NIAMS P30 AR070253, an Investigator Award of the Rheumatology Research Foundation, an All Arthritis grant from the Arthritis National Research Foundation, and a Career Development Award from the Office of Faculty Development at Boston Children's Hospital.

The transition from pediatric to adult care is the focus of growing research. It is important to identify how to direct future research efforts for maximum effect. Our goals were to perform a scoping review of the transition literature, highlight gaps in transition research, and offer stakeholder guidance on the importance and feasibility of research questions designed to fill identified gaps. The transition literature on rheumatic diseases and other common pediatric-onset chronic diseases was grouped and summarized. Based on the findings, a survey was developed and disseminated to pediatric rheumatologists and young adults with rheumatic diseases as well as their caregivers. The transitional care needs of patients, healthcare teams, and caregivers is well described in the literature. While various transition readiness scales exist, no longitudinal posttransfer study confirms their predictive validity. Multiple outcome measures are used alone or in combination to define a successful transition or intervention. Multimodal interventions are most effective at improving transition-related outcomes. How broader health policy affects transition is poorly studied. Research questions that ranked highest for importance and feasibility included those related to identifying and tracking persons with psychosocial vulnerabilities or other risk factors for poor outcomes. Interventions surrounding improving self-efficacy and health literacy were also ranked highly. In contrast to healthcare teams (n = 107), young adults/caregivers (n = 23) prioritized research surrounding improved work, school, or social function. The relevant transition literature is summarized and future research questions prioritized, including the creation of processes to identify and support young adults vulnerable to poor outcomes.

Objective Features of multisystem inflammatory syndrome in children (MIS-C) overlap with other febrile illnesses, hindering prompt and accurate diagnosis. The objectives of this study were to identify clinical and laboratory findings that distinguished MIS-C from febrile illnesses in which MIS-C was considered but ultimately excluded, and to examine the diseases that most often mimicked MIS-C in a tertiary medical centre. Study design We identified all children hospitalised with fever who were evaluated for MIS-C at our centre and compared clinical signs and symptoms, SARS-CoV-2 status and laboratory studies between those with and without MIS-C. Multivariable logistic LASSO (least absolute shrinkage and selection operator) regression was used to identify the most discriminative presenting features of MIS-C. Results We identified 50 confirmed MIS-C cases (MIS-C+) and 68 children evaluated for, but ultimately not diagnosed with, MIS-C (MIS-C-). In univariable analysis, conjunctivitis, abdominal pain, fatigue, hypoxaemia, tachypnoea and hypotension at presentation were significantly more common among MIS-C+ patients. MIS-C+ and MIS-C- patients had similar elevations in C-reactive protein (CRP), but were differentiated by thrombocytopenia, lymphopenia, and elevated ferritin, neutrophil/lymphocyte ratio, BNP and troponin. In multivariable analysis, predictors of MIS-C included age, neutrophil/lymphocyte ratio, platelets, conjunctivitis, oral mucosa changes, abdominal pain and hypotension. Conclusions Among hospitalised children undergoing evaluation for MIS-C, children with MIS-C were older, more likely to present with conjunctivitis, oral mucosa changes, abdominal pain and hypotension, and had higher neutrophil/lymphocyte ratios and lower platelet counts. These data may be helpful for discrimination of MIS-C from other febrile illnesses, including bacterial lymphadenitis and acute viral infection, with overlapping features., This single centre cohort study identifies clinical and laboratory findings that distinguish multisystem inflammatory syndrome in children (MIS-C) from other febrile illnesses in which MIS-C was considered but ultimately excluded. The most discriminative features of MIS-C included older age, conjunctivitis, oral mucosa changes, abdominal pain and hypotension, higher neutrophil/lymphocyte ratios and lower platelet counts.

Background Multisystem Inflammatory Syndrome in Children (MIS-C) is a pediatric complication of SARS-CoV-2 infection characterized by multiorgan inflammation and frequently, cardiovascular dysfunction. It occurs predominantly in otherwise healthy children. We previously reported haploinsufficiency of Suppressor of Cytokine Signaling 1 (SOCS1), a negative regulator of Type I and II interferons, as a genetic risk factor for MIS-C. Objectives We aimed to identify additional genetic mechanisms underlying susceptibility to SARS-CoV-2-associated MIS-C. Methods In a single center, prospective cohort study, whole exome sequencing was performed on patients with MIS-C. The impact of candidate variants was tested using patients’ peripheral blood mononuclear cells obtained at least seven months after recovery. Results We enrolled 18 patients with MIS-C (median age: 8 years, IQR 5 – 12.25 years), of whom 89% had no conditions other than obesity. In two boys with no significant infection history, we identified and validated hemizygous, deleterious defects in XIAP, encoding X-linked inhibitor of apoptosis, and CYBB, encoding cytochrome b-245. Including the previously reported SOCS1 haploinsufficiency, a genetic diagnosis was identified in three (17%) of 18 patients. Even after recovery, patients with defects in SOCS1, XIAP, or CYBB exhibit an inflammatory immune cell transcriptome with enrichment of differentially expressed genes in pathways downstream of IL-18, oncostatin M, and NF-κB, compared to those with mild COVID-19. Conclusions Although inflammatory disorders are rare in the general population, our cohort of patients with MIS-C was enriched for monogenic susceptibility to inflammation. Our results support the use of next-generation sequencing in previously healthy children who develop MIS-C., Graphical abstract, Capsule Summary: Hypomorphic inborn errors of immunity may predispose children to SARS-CoV-2-associated Multisystem Inflammatory Syndrome in Children (MIS-C).

OBJECTIVES: To characterize the socioeconomic and racial and/or ethnic disparities impacting the diagnosis and outcomes of multisystem inflammatory syndrome in children (MIS-C). METHODS: This multicenter retrospective case-control study was conducted at 3 academic centers from January 1 to September 1, 2020. Children with MIS-C were compared with 5 control groups: children with coronavirus disease 2019, children evaluated for MIS-C who did not meet case patient criteria, children hospitalized with febrile illness, children with Kawasaki disease, and children in Massachusetts based on US census data. Neighborhood socioeconomic status (SES) and social vulnerability index (SVI) were measured via a census-based scoring system. Multivariable logistic regression was used to examine associations between SES, SVI, race and ethnicity, and MIS-C diagnosis and clinical severity as outcomes. RESULTS: Among 43 patients with MIS-C, 19 (44%) were Hispanic, 11 (26%) were Black, and 12 (28%) were white; 22 (51%) were in the lowest quartile SES, and 23 (53%) were in the highest quartile SVI. SES and SVI were similar between patients with MIS-C and coronavirus disease 2019. In multivariable analysis, lowest SES quartile (odds ratio 2.2 [95% confidence interval 1.1–4.4]), highest SVI quartile (odds ratio 2.8 [95% confidence interval 1.5–5.1]), and racial and/or ethnic minority background were associated with MIS-C diagnosis. Neither SES, SVI, race, nor ethnicity were associated with disease severity. CONCLUSIONS: Lower SES or higher SVI, Hispanic ethnicity, and Black race independently increased risk for MIS-C. Additional studies are required to target interventions to improve health equity for children.

Clinical features of Multisystem Inflammatory Syndrome in Children (MIS-C) associated with COVID-19 are nonspecific. In this retrospective cohort study of 39 patients evaluated for MIS-C, 11 had non-SARS-CoV-2 infections, 3 of whom were also diagnosed with MIS-C. Clinical features were similar in patients with MIS-C and patients with non-SARS-CoV-2 infections. Clinicians should consider non-SARS-CoV-2 infections in patients undergoing MIS-C evaluation.

BACKGROUND: Pediatric SARS–CoV-2 infection can be complicated by a dangerous hyperinflammatory condition termed multisystem inflammatory syndrome in children (MIS-C). The clinical and immunologic spectrum of MIS-C and its relationship to other inflammatory conditions of childhood have not been studied in detail. METHODS: We retrospectively studied confirmed cases of MIS-C at our institution from March to June 2020. The clinical characteristics, laboratory studies, and treatment response were collected. Data were compared with historic cohorts of Kawasaki disease (KD) and macrophage activation syndrome (MAS). RESULTS: Twenty-eight patients fulfilled the case definition of MIS-C. Median age at presentation was 9 years (range: 1 month to 17 years); 50% of patients had preexisting conditions. All patients had laboratory confirmation of SARS–CoV-2 infection. Seventeen patients (61%) required intensive care, including 7 patients (25%) who required inotrope support. Seven patients (25%) met criteria for complete or incomplete KD, and coronary abnormalities were found in 6 cases. Lymphopenia, thrombocytopenia, and elevation in inflammatory markers, D-dimer, B-type natriuretic peptide, IL-6, and IL-10 levels were common but not ubiquitous. Cytopenias distinguished MIS-C from KD and the degree of hyperferritinemia and pattern of cytokine production differed between MIS-C and MAS. Immunomodulatory therapy given to patients with MIS-C included intravenous immune globulin (IVIG) (71%), corticosteroids (61%), and anakinra (18%). Clinical and laboratory improvement were observed in all cases, including 6 cases that did not require immunomodulatory therapy. No mortality was recorded in this cohort. CONCLUSION: MIS-C encompasses a broad phenotypic spectrum with clinical and laboratory features distinct from KD and MAS. FUNDING: This work was supported by the National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases; the National Institute of Allergy and Infectious Diseases; Rheumatology Research Foundation Investigator Awards and Medical Education Award; Boston Children’s Hospital Faculty Career Development Awards; the McCance Family Foundation; and the Samara Jan Turkel Center.

Objectives Pneumocystis jirovecii pneumonia (PJP) is associated with significant morbidity and mortality in adult myositis patients; however, there are few studies examining PJP in juvenile myositis [juvenile idiopathic inflammatory myopathy (JIIM)]. The purpose of this study was to determine the risk factors and clinical phenotypes associated with PJP in JIIM. Methods An research electronic data capture (REDCap) questionnaire regarding myositis features, disease course, medications and PJP infection characteristics was completed by treating physicians for 13 JIIM patients who developed PJP (PJP+) from the USA and Canada. Myositis features and medications were compared with 147 JIIM patients without PJP (PJP–) from similar geographic regions who enrolled in National Institutes of Health natural history studies. Results PJP+ patients were more often of Asian ancestry than PJP– patients [odds ratio (OR) 8.7; 95% CI 1.3, 57.9]. Anti- melanoma differentiation associated protein 5 (MDA5) autoantibodies (OR 12.5; 95% CI 3.0, 52.4), digital infarcts (OR 43.8; 95% CI 4.2, 460.2), skin ulcerations (OR 12.0; 95% CI 3.5, 41.2) and interstitial lung disease (OR 10.6; 95% CI 2.1, 53.9) were more frequent in PJP+ patients. Before PJP diagnosis, patients more frequently received pulse steroids, rituximab and more immunosuppressive therapy compared with PJP– patients. Seven PJP+ patients were admitted to the intensive care unit and four patients died due to PJP or its complications. Conclusions PJP is a severe infection in JIIM that can be associated with mortality. Having PJP was associated with more immunosuppressive therapy, anti-MDA5 autoantibodies, Asian race and certain clinical features, including digital infarcts, cutaneous ulcerations and interstitial lung disease. Prophylaxis for PJP should be considered in juvenile myositis patients with these features.

Background: Many patients live with serious chronic or terminal illnesses. Multicomponent palliative care interventions have been increasingly utilized in patient care; however, it is unclear what is being implemented and who is delivering these interventions. Objectives: To (1) describe the delivery of multicomponent palliative care interventions, (2) characterize the disciplines delivering care, (3) identify the components being implemented, and (4) analyze whether the number of disciplines or components being implemented are associated with positive outcomes. Design: Systematic review. Study Selection: English-language articles analyzing multicomponent palliative care interventions. Outcomes Measured: Delivery of palliative interventions by discipline, components of palliative care implemented, and number of positive outcomes (eg, pain, quality of life). Results: Our search strategy yielded 71 articles, which detailed 64 unique multicomponent palliative care interventions. Nurses (n = 64, 88%) were most often involved in delivering care, followed by physicians (n = 43, 67%), social workers (n = 33, 52%), and chaplains (n = 19, 30%). The most common palliative care components patients received were symptom management (n = 56, 88%), psychological support/counseling (n = 52, 81%), and disease education (n = 48, 75%). Statistical analysis did not uncover an association between number of disciplines or components and positive outcomes. Conclusions: While there has been growth in multicomponent palliative care interventions over the past 3 decades, important aspects require additional study such as better inclusion of key groups (eg, chronic obstructive pulmonary disease, end-stage renal disease, minorities, older adults); incorporating core components of palliative care (eg, interdisciplinary team, integrating caregivers, providing spiritual support); and developing ways to evaluate the effectiveness of interventions that can be readily replicated and disseminated.

Background Multisystem inflammatory syndrome in children (MIS-C) has been described in areas with high Severe Acute Respiratory Syndrome coronavirus 2 (SARS-CoV-2) burden. Clinical features included in the MIS-C case definition (e.g fever, elevated inflammatory markers) overlap with features of other childhood infections. The prevalence of non-SARS-CoV-2 infection in patients evaluated for MIS-C has not been described. Patients evaluated for MIS-C, and therapies administered. Methods Retrospective cohort study of patients < 21 years of age admitted to a freestanding children’s hospital in Boston, MA from May 14-June 6, 2020 who were evaluated for MIS-C. We identified patients undergoing Rheumatology consultation and echocardiogram (per the hospital’s protocol for evaluating children with suspicion for MIS-C). We tabulated patients evaluated for MIS-C found to have non-SARS-CoV-2 infection detected on standard microbiologic testing. Results 39 patients met inclusion criteria. Median age was 5 years (IQR 2–12 years). Of evaluated patients, 19/39 (49%) were diagnosed with MIS-C according to the Massachusetts Department of Public Health case definition; 10/39 (26%) required ICU admission. Non-SARS-CoV-2 infections were identified in 7/39 (18%), of whom 5/7 (71%) had bacterial infections, 1/7 (14%) had viral infection, and 1/7 (14%) had viral and bacterial co-infections; no fungal or parasitic infections were identified. Of patients diagnosed with MIS-C, 2/19 (11%) were found to have non-SARS-CoV-2 infection. Additionally, 5/19 (26%) had a positive polymerase chain reaction test for SARS-CoV-2 at time of MIS-C diagnosis, of whom 4/5 (80%) received remdesivir. Of patients evaluated for MIS-C, 17/39 (44%) received intravenous immune globulin, 14/39 (36%) aspirin, 4/39 (10%) anakinra, and 14/39 (36%) methylprednisolone. Additionally, 21/39 (54%) received antibacterial and 5/39 (13%) antiviral therapy (Table). Conclusion In this study, non-SARS-CoV-2 infections were diagnosed in 18% of children evaluated for MIS-C. Clinicians should consider alternative or concomitant infectious diagnoses in patients undergoing MIS-C evaluation. Research is needed to identify clinical and laboratory features that may distinguish patients with MIS-C from those with non-SARS-CoV-2 infection. Disclosures All Authors: No reported disclosures

Hemodialysis (HD) patients experience significant symptom burden that is often undertreated.This study identified (1) barriers to symptom management in the HD population, (2) potential targets for improving symptom burden, (3) provider attitudes toward palliative care for HD patients, and (4) perceptions of how transplant eligibility impacts care.Semistructured, one-on-one interviews were conducted, audiotaped, and transcribed. Data were analyzed qualitatively by two investigators to identify discrete themes.Health care providers (HCPs) and caregivers were recruited (June to October 2012) from three outpatient HD units in New York City.Open-ended questions were used with follow-up probes.Interviews were completed with 34 HCPs (8 physicians, 2 nurse practitioners, 4 social workers, 13 registered nurses, 7 patient care technicians) and 20 caregivers (14 family members, 5 home health aides, 1 friend). Barriers to symptom control were identified in three areas: (1) provider unawareness of symptoms, (2) provider's uncertainty as to whose responsibility it is to treat symptoms, and (3) inherent difficulty in symptom management. Ideas for ameliorating symptoms included enhancing systems for patient/caregiver education, improving systems for HCP communication with other disciplines, and encouraging alternative methods of dialysis when appropriate. HCPs also expressed discomfort with the term "palliative care."Renal HCPs are often unaware of the magnitude of symptom burden in their HD patients. This study found that there is lack of ownership for assessing and treating these symptoms. Providers also feel certain symptoms are "untreatable." Research is needed to develop effective treatment strategies for HD populations.

Patients with end-stage renal disease (ESRD) on hemodialysis (HD) suffer from a high symptom burden. However, there is significant heterogeneity within the HD population; certain subgroups, such as the elderly, may experience disproportionate symptom burden.The study's objective was to propose a category of HD patients at elevated risk for symptom burden (those patients who are not transplant candidates) and to compare symptomatology among transplant ineligible versus eligible HD patients.This was a cross-sectional study.English-speaking, cognitively intact patients receiving HD and who were either transplant eligible (n=25) or ineligible (n=32) were recruited from two urban HD units serving patients in the greater New York City region.In-person interviews were conducted to ascertain participants' symptom burden using the Dialysis Symptom Index (DSI), perceived symptom bother and attribution (whether the symptom was perceived to be related to HD treatment), and quality of life using the SF-36. Participants' medical records were reviewed to collect demographic and clinical data.Transplant ineligible (versus eligible) patients reported an average of 13.9±4.6 symptoms versus 9.2±4.4 symptoms (p0.01); these differences persisted after adjustment for multiple factors. A greater proportion of transplant ineligible (versus eligible) patients attributed their symptoms to HD and were more likely to report greater bother on account of the symptoms. Quality of life was also significantly lower in the transplant ineligible group.Among HD patients, transplant eligibility is associated with symptom burden. Our pilot data suggest that consideration be given to employing transplant status as a method of identifying HD patients at risk for greater symptom burden and targeting them for palliative interventions.

Effective communication is essential for shared decision making with families of critically ill patients in the intensive care unit (ICU), yet there is limited evidence on effective strategies to teach these skills.The study's objective was to pilot test an educational intervention to teach internal medicine interns skills in discussing goals of care and treatment decisions with families of critically ill patients using the shared decision making framework.The intervention consisted of a PowerPoint online module followed by a four-hour workshop implemented at a retreat for medicine interns training at an urban, academic medical center.Participants (N=33) completed post-intervention questionnaires that included self-assessed skills learned, an open-ended question on the most important learning points from the workshop, and retrospective pre- and post-workshop comfort level with ICU communication skills. Participants rated their satisfaction with the workshop.Twenty-nine interns (88%) completed the questionnaires. Important self-assessed communication skills learned reflect key components of shared decision making, which include assessing the family's understanding of the patient's condition (endorsed by 100%) and obtaining an understanding of the patient/family's perspectives, values, and goals (100%). Interns reported significant improvement in their comfort level with ICU communication skills (pre 3.26, post 3.73 on a five-point scale, p=0.004). Overall satisfaction with the intervention was high (mean 4.45 on a five-point scale).The findings suggest that a brief intervention designed to teach residents communication skills in conducting goals of care and treatment discussions in the ICU is feasible and can improve their comfort level with these conversations.