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.

Genetic susceptibility to Multisystem Inflammatory Syndrome in Children (MIS-C) SARS-CoV-2 SOCS1 XIAP CYBB Asymptomatic MIS-C inflammation Monogenic variants in: No genetic risk factors XIAP, X-linked inhibitor of apoptosis CYBB, Cytochrome b(-245), beta subunit SOCS1, Suppressor of cytokine signaling 1

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Background: Hemodialysis (HD) patients experience significant symptom burden that is often undertreated. Objective: 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. Design: Semistructured, one-on-one interviews were conducted, audiotaped, and transcribed. Data were analyzed qualitatively by two investigators to identify discrete themes. Setting/subjects: Health care providers (HCPs) and caregivers were recruited ( June to October 2012) from three outpatient HD units in New York City. Measurements: Open-ended questions were used with follow-up probes. Results: 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.'' Conclusions: 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.

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.