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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.

Globally, over the past two decades, many countries have significantly reduced the rate of infant mortality. Yet, in Africa, Nigeria remains one of the countries with the highest infant mortality rate (IMR).We conducted a population-level study using the 2018 Nigeria Demographic Health Survey (NDHS). A total of 41,668 household data were analyzed retrospectively. The association between each exposure and infant mortality was analyzed in logistic regression models (independently adjusted by demographic and socioeconomic status variables) and confirmed by the multiple comparisons analysis.The overall IMR of 2013-2017 was 61.5 (95% CI 58.0, 65.3) per 1000 live births. In general, the North-West and North-East regions had the highest IMR, whereas the South-West, South-East and South-South regions had the lowest IMR. The regression analysis found women who delivered their babies at the age=18 years old (odds ratio (OR): 1.37 [1.17, 1.62]), had religion of Islam (OR: 1.35 [1.10, 1.65]), no ANC visit (OR: 1.69 [1.21, 2.35]),4 ANC visits (OR: 1.70 [1.23, 2.34]), ANC not at home or skilled provider (0.40 [0.35, 0.46]) and the babies as the first child (OR: 1.23 [1.07, 1.42]) to be associated with higher IMR.Our findings imply that Nigeria is not on track to achieving the SDG target of reducing child mortality by 2030. Sustainable interventions are urgently needed to address the challenges for women of reproductive age, particularly those that are living in the rural areas and Northern regions, having limited/no access to health care/skilled providers, and delivered their first child.None.