Internal and emergency medicine
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Acid sphingomyelinase deficiency (ASMD) is an ultra-rare disease, and several gaps of knowledge on various issues remain, particularly at a regional/national level. Expert opinions collected through well-defined consensus methodologies are increasingly used to make available reliable information in the context of rare/ultra-rare diseases. ⋯ During two Delphi rounds, a high ratio of agreement was found on several topics related to ASMD characteristics, diagnosis, management and disease burden. Our findings may provide valuable indications for management of ASMD at a public health level in Italy.
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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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Pulmonary embolism (PE) is the third-leading cause of cardiovascular death in the United States, and several studies suggest PE shows seasonal variation. Variation in monthly PE diagnosis may be due to pathophysiologic factors or confounding/bias. However, severe PE may be less prone to diagnostic bias. To address this gap, we analyzed two registries from 1/2013-12/2018 with the aim of describing temporal trends in PE diagnosis and severity. ⋯ Our results indicate correlation between calendar month and PERT activation; however, this pattern was not observed for PE diagnoses. This finding warrants further investigation into the causes of calendar month variation of PERT activations.