IMPORTANCE Multisystem inflammatory syndrome in children (MIS-C) is associated with recent or current SARS-CoV-2 infection. Information on MIS-C incidence is limited. OBJECTIVE To estimate population-based MIS-C incidence per 1 000 000 person-months and to estimate MIS-C incidence per 1 000 000 SARS-CoV-2 infections in persons younger than 21 years. DESIGN, SETTING, AND PARTICIPANTS This cohort study used enhanced surveillance data to identify persons with MIS-C during April to June 2020, in 7 jurisdictions reporting to both the Centers for Disease Control and Prevention national surveillance and to Overcoming COVID-19, a multicenter MIS-C study. Denominators for population-based estimates were derived from census estimates;denominators for incidence per 1 000 000 SARS-CoV-2 infections were estimated by applying published age-and month-specific multipliers accounting for underdetection of reported COVID-19 case counts.

The Study The Medical College of Wisconsin established the SECURE-SCD Registry to collect data on COVID-19 cases occurring globally in persons living with SCD. A link to the registry (https://covidsicklecell.org) was distributed to healthcare providers caring for patients with SCD by medical professional and patient advocacy networks and was made available on the Centers for Disease Control and Prevention website. Providers were asked to report all confirmed COV-ID-19 cases among patients with SCD to this registry; they were specifically asked to report only confirmed COVID-19 cases and to report cases after resolution of acute illness or death. Persons who had the sickle cell trait were not included in this registry, nor were persons with SCD who had suspected but not confirmed COVID-19. Providers were asked to report if the patient died of COVID-19 or complications of CO-VID-19. All data were deidentified without protected health information. For each case, providers were asked to complete a short form with questions on demographics; SCD genotype; SCD-related health history; and CO-VID-19 clinical course, severity, and interventions. In addition to data on COVID-19 clinical severity indicators, such as hospitalization, ICU admission, and death, COVID-19 severity level based on patient manifestations were collected by using established criteria for asymptomatic, mild, moderate, severe, and critical (10) (Table). This analysis was limited to cases among persons with SCD living in the United States reported during March 20-May 21, 2020. We describe the reported cases and deaths caused by COVID-19 and provide

Both genetic and treatment-related risk factors contribute to the development of inhibitors in haemophilia. An inhibitor surveillance system piloted at 12 US sites has the goal of assessing risk factors through prospective data collection. This report examines the relationship of genotype and race/ethnicity to history of inhibitor in a large cohort of US haemophilia patients. Mutation analysis was performed on 676 haemophilia A (HA) and 153 haemophilia B (HB) patients by sequencing, Multiplex Ligation-dependent Probe Amplification, and PCR for inversions in F8 introns 22 (inv22) and 1 (inv1). Two HB patients with deletions had history of inhibitor. In severe HA, frequency of history of inhibitor was: large deletion 57.1%, splice site 35.7%, inv22 26.8%, nonsense 24.5%, frameshift 12.9%, inv1 11.1% and missense 9.5%. In HA, 19.6% of 321 White non-Hispanics (Whites), 37.1% of 35 Black non-Hispanics (Blacks) and 46.9% of 32 Hispanics had history of inhibitor (P = 0.0003). Mutation types and novel mutation rates were similar across ethnicities. When F8 haplotypes were constructed, Whites and Hispanics showed only H1 and H2. Within H1, history of inhibitor was 12.4% in Whites, 40.0% in Blacks (P = 0.009) and 32.4% in Hispanics (P = 0.002). Inhibitor frequency is confirmed to vary by mutation type and race in a large US population. White patients with history of inhibitor did not exhibit rare F8 haplotypes. F8 gene analysis did not reveal a cause for the higher inhibitor frequencies in Black and Hispanic patients.

We provide an updated assessment of trends in sickle cell disease (SCD)-related mortality, a significant source of mortality in the United States among black persons, using 1979 to 2017 US mortality data.Methods: SCD-related deaths were identified with International Classification of Diseases codes. Because SCD-related death is rare in other races, the analysis focused on black decedents. Age-specific and average annual SCD-related death rates were calculated. Causes of death codes were categorized into 20 groups relevant to SCD outcomes. SCD-related deaths were compared with non-SCD-related deaths after matching on race, sex, age group, and year of death.Results: There were 25,665 SCD-related deaths reported among blacks in the United States from 1979 through 2017. During that period, the annual SCD-related death rate declined in children and increased in adults, and the median age at death increased from 28 to 43 years. Acute causes of death, such as infection and cerebrovascular complications, were more common in younger age groups. Chronic complications were more common in adults. SCD-related deaths were more likely to be related to acute cardiac, pulmonary, and cerebrovascular complications; acute infections; and chronic cardiac and pulmonary complications and renal disorders; and less likely to be related to drug overdose and chronic infections than non-SCD-related deaths. Conclusion:These data indicate SCD-related deaths are now more likely to be related to chronic complications of the disease than to acute complications. More research regarding prevention and treatment of chronic complications of SCD is necessary because persons with SCD are living longer. [