Objectives Prone positioning is widely used in mechanically ventilated patients with COVID-19; however, the specific clinical scenario in which the individual is most poised to benefit is not fully established. In patients with COVID-19 respiratory failure requiring mechanical ventilation, how effective is prone positioning in improving oxygenation and can that response be predicted? Design This is a retrospective observational study from two tertiary care centers including consecutive patients mechanically ventilated for COVID-19 from 3/1/2020 – 7/1/2021. The primary outcome is improvement in oxygenation as measured by PaO2/FiO2. We describe oxygenation before, during and after prone episodes with a focus on identifying patient, respiratory or ventilator variables that predict prone positioning success. Setting 2 Tertiary Care Academic Hospitals Patients 125 patients mechanically ventilated for COVID-19 respiratory failure. Interventions Prone positioning Main Results One hundred twenty-five patients underwent prone positioning a total of 309 times for a median duration of 23 hours IQR (14 – 49). On average, PaO2/FiO2 improved 19%: from 115 mm Hg (80 – 148) immediately before proning to 137 mm Hg (95 – 197) immediately after returning to the supine position. Prone episodes were more successful if the pre-prone PaO2/FiO2 was lower and if the patient was on inhaled epoprostenol (iEpo). For individuals with severe acute respiratory distress syndrome (ARDS) (PaO2/FiO2 < 100 prior to prone positioning) and on iEpo, the median improvement in PaO2/FiO2 was 27% in both instances. Conclusions Prone positioning in mechanically ventilated patients with COVID-19 is generally associated with sustained improvements in oxygenation, which is made more likely by the concomitant use of iEpo and is more impactful in those who are more severely hypoxemic prior to prone positioning.
Introduction: Hemophagocytic lymphohistiocytosis is a rare condition with dysregulated multi-organ inflammation that may cause acute liver failure. It often presents with non-specific clinical features and can be difficult to diagnose.
Case Report: We present the case of a 2-year-old girl with lethargy, encephalopathy, poor oral intake, vomiting, and jaundice. Her labs indicated acute liver failure with additional findings of bicytopenia and elevated ferritin. Given high concern for hemophagocytic lymphohistiocytosis, we promptly consulted the hematology/oncology, transplant hepatology, and pharmacy services. Bone marrow biopsy initially showed no evidence of hemophagocytosis. Given our high clinical index of suspicion for hemophagocytic lymphohistiocytosis and its high mortality and morbidity, we initiated treatment with high dose dexamethasone, etoposide, and the recently approved biologic drug emapalumab, a monoclonal antibody against interferon gamma, despite not meeting full HLH-2004 diagnostic criteria. One day after treatment initiation, the final review of the bone marrow biopsy showed evidence of hemophagocytosis. Ultimately, with implementation of multidisciplinary rounds, close neurologic examinations, aggressive management of evolving hyperammonemia and cerebral edema, and early initiation of treatment, our patient achieved full liver recovery.
Conclusion: This patient’s presentation emphasizes the importance of having a broad differential when a patient presents with liver failure and cytopenias. Our management of this patient showcases the importance of expedient, multidisciplinary management for a complex critically ill pediatric patient. The patient’s survival and complete liver recovery with the treatment protocol given suggests emapalumab should be studied in future clinical trials as an important adjunctive treatment for patients with hemophagocytic lymphohistiocytosis with acute liver failure.
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