Internal and emergency medicine
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The objective of the study was to evaluate all available systematic reviews on the use of prone positional ventilation in adult patients with acute respiratory distress syndrome (ARDS). An umbrella review on the efficacy of prone positional ventilation in adult patients ventilation in adult patients with acute respiratory distress syndrome was conducted. We performed a systematic search in the database of Medline (Pubmed), Scopus, Cochrane Library, Web of Science, and Epistemonikos. ⋯ Most reviews had severe methodological flaws that led to results with very low certainty of evidence. The review with the lowest risk of bias presented results in favor of prone positional ventilation compared with high-frequency oscillatory ventilation and lung-protective ventilation. There is a need to update the available reviews to obtain more accurate results.
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VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a recently described pathological entity. It is an acquired monogenic autoinflammatory disease caused by somatic mutations of the UBA1 gene in blood cells precursors; the gene encodes one of the two E1 enzyme isoforms that initiates ubiquitylation in cell's cytoplasm. VEXAS syndrome leads to systemic inflammation, with all organs and tissues potentially involved. ⋯ The syndrome deserves a multidisciplinary approach to reach the diagnosis and ensure the best management of a potentially very challenging condition. To quickly describe the clinical course, long-term outcomes, and the optimal management of this new syndrome it is essential to join forces internationally. To this end, the international AutoInflammatory Disease Alliance (AIDA) registry dedicated to VEXAS syndrome has been developed and is already active.