Background
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hematologic disorder resulting from an ineffective and pathologic activation of the immune response system that may mimic common emergency department presentations, including sepsis, acute liver failure, disseminated intravascular coagulation, and flu-like illnesses such as coronavirus disease 2019 (COVID-19).
Objective
This narrative review provides a summary of the disease and recommendations for the recognition and diagnostic evaluation of HLH with a focus on the emergency clinician.
Discussion
Though the condition is rare, mortality rates are high, ranging from 20% to 80% and increasing with delays in treatment. Importantly, HLH has been recognized as a severe variation of the cytokine storm associated with COVID-19. Common features include a history of infection or malignancy, fever, splenomegaly or hepatomegaly, hyperferritinemia, cytopenias, coagulopathies, abnormal liver enzymes, and hypertriglyceridemia. Using specific features of the history, physical examination, laboratory studies, and tools such as the HScore, HLH-2004/2009, and hyperferritinemia thresholds, the emergency clinician can risk-stratify patients and admit for definitive testing. Once diagnosed, disease specific treatment can be initiated.
Conclusion
This review describes the relevant pathophysiology, common presentation findings, and a framework for risk stratification in the emergency department.
Background: Intubation in critically ill patients remains a highly morbid procedure, and the optimal approach is unclear. We sought to improve the safety of intubation by implementing a simulation curriculum and monitoring performance with an airway registry. Methods and Methods: This is a prospective, single-center observational study of all intubations performed by the medical intensive care unit (ICU) team over a five-year period. All fellows take part in a simulation curriculum to improve airway management performance and minimize complications. An airway registry form is completed immediately after each intubation to capture relevant patient, operator, and procedural data. Results: Over a five-year period, the medical ICU team performed 1411 intubations. From Year 1 to Year 5, there were significant increases in first-attempt success (72.6 vs. 88.0%, p<0.001), use of video laryngoscopy (72.3 vs. 93.5%, p<0.001), and use of neuromuscular blocking agents (73.5 vs. 88.4%, p<0.001). There were concurrent decreases in rates of desaturation (25.6 vs. 17.1%, p=0.01) and esophageal intubations (5 vs. 1%, p=0.009). Low rates of hypotension (8.3%) and cardiac arrest (0.6%) were also observed. Conclusions: The safety of intubation in critically ill patients can be markedly improved through joint implementation of an airway registry and simulation curriculum.
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