IntroductionPulmonary alveolar proteinosis (PAP) is an ultrarare disorder characterised by the accumulation of alveolar surfactant and the dysfunction of alveolar macrophages that results in hypoxemic respiratory failure. Whole-lung lavage (WLL) is currently the primary therapy for PAP. However, systematic evaluation of the clinical efficacy of WLL is lacking. We aim to perform a systematic review and meta-analysis of existing evidence to support WLL for the clinical treatment of PAP.Methods and analysisWe will search the PubMed (MEDLINE), Cochrane Library, Embase, Web of Science and Google Scholar databases from inception to December 2021 for observational studies using WLL for the treatment of PAP. Two authors will independently screen the eligible studies, assess the quality of the included papers and extract the required information. Review Manager V.5.4 will be used to perform the meta-analysis. We will evaluate the overall quality of evidence using the Grading of Recommendations, Assessment, Development and Evaluation approach. All steps of this protocol will be performed using the Cochrane Handbook for Preferred Reporting Items for Systematic Review and Meta-analysis statement.Ethics and disseminationThis systematic review and meta-analysis will be based on published data. Therefore, ethical approval is not required. We will publish our results in a peer-reviewed journal.PROSPERO registration numberCRD42022306221 (https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42022306221).

BackgroundBirt-Hogg-Dubé syndrome (BHD) is a rare autosomal dominant inherited disorder caused by germline mutations in folliculin (FLCN). Despite our significantly evolved understanding of BHD over the past decades, no bibliometric analyses have been conducted in this field. This study aimed to analyze and visualize the characteristics of publication outputs, the research hotspots, and scientific frontiers about BHD using bibliometric analysis.MethodsAll relevant literature on BHD was culled from the Web of Science Core Collection (WoSCC) database. Valid data were extracted from the articles and visually analyzed using CiteSpace and VOSviewer.ResultsA total of 751 qualifying papers were included. Publication outputs concerning BHD increased over time. The dominant position of the United States and Japan in BHD research field was evident. National Cancer Institute (the USA) and Yokohama City University (Japan) were the two most productive organizations. W. Marston Linehan exerted a considerable publication impact and had made the most remarkable contributions in the field of BHD. Plos One was the journal with the highest publication outputs, and half of the top 10 journals and co-cited journals belonged to Q1 or Q2. Keyword citation bursts revealed that management, tumor suppressor, flcn gene, spectrum, diagnosis, risk, computed tomography were the emerging research hotspots.ConclusionResearch on BHD is prosperous. International cooperation between countries and organizations is also expected to deepen and strengthen in the future. Our results indicated that FLCN-associated pathways involved in the pathogenesis of BHD, specific options for early diagnosis, and molecular-targeting therapies will remain research hotspots in the future.

BackgroundPulmonary alveolar proteinosis (PAP) is a rare syndrome first described by Rosen et al. in 1958. Despite our considerably evolved understanding of PAP over the past decades, no bibliometric studies have been reported on this field. We aimed to analyze and visualize the research hotspots and current trends of the PAP research field using a bibliometric analysis to help understand the future development of basic and clinical research.MethodsThe literature regarding PAP was culled from the Web of Science Core Collection (WoSCC) database. Data were extracted from the relevant articles and visually analyzed using CiteSpace and VOSviewer software.ResultsNine hundred and nine qualifying articles were included in the analysis. Publications regarding PAP increased over time. These articles mainly come from 407 institutions of 57 countries. The leading countries were the USA and Japan. University of Cincinnati (USA) and Niigata University (Japan) featured the highest number of publications among all institutions. Bruce C Trapnell exerts a significant publication impact and has made the most outstanding contributions in the field of PAP. American Journal of Physiology-Lung Cellular and Molecular Physiology was the journal with the most publications, and American Journal of Respiratory and Critical Care Medicine was the most commonly cited journal. All the top 5 co-cited journals belong to Q1. Keyword citation bursts revealed that inflammation, deficiency, tissue resident macrophage, classification, autoimmune pulmonary alveolar proteinosis, sarcoidosis, gm csf, high resolution ct, and fetal monocyte were the emerging research hotspots.ConclusionResearch on PAP is prosperous. International cooperation is also expected to deepen and strengthen in the future. Our results indicated that the etiology and pathogenesis of PAP, current and emerging therapies, especially the novel pathogenesis-based options will remain research hotspots in the future.

Background: With the Coronavirus disease 2019 epidemic, wearing a mask has become routine to prevent and control the virus's spread, especially for healthcare workers. However, the impact of long-term mask wear on the human body has not been adequately investigated. This study aimed to investigate whether Powered Air Purifying Respirators and N95 masks impact the olfaction in healthcare workers.Methods: We recruited fifty-six healthcare workers and randomly divided them into 2 groups, wearing a powered air purifying respirator (PAPR) (experiment group, N = 28) and an N95 mask (control group, N = 28). Olfactory discrimination and threshold tests were performed before and after wearing the masks. SPSS 26.0 (SPSS Inc., Chicago, Illinois) software was used for the statistical analyses.Results: There was a statistical difference in the olfactory threshold test after wearing the mask in both PAPR Group (Z = -2.595, P = .009) and N95 Group (Z = -2.120, P = .034), with no significant difference between the 2 (χ 2 = 0.29, P = .589). There was no statistical difference in the discrimination test scores in both 2 groups after wearing the masks. Conclusion:Wearing a mask affects the healthcare workers' olfaction, especially odor sensitivity. Healthcare workers have a higher olfactory threshold after long-term mask wear, whether wearing PAPRs or N95 masks.

Background: Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency disorder. Despite our enormous progress in the strategies used to diagnose, treat, and cure WAS, no bibliometric studies have been performed in this research field. This study explored the trends in WAS research through a bibliometric analysis evaluating relevant literature quantitatively and qualitatively.Methods: The literature concerning WAS from 2001 to 2021 was retrieved from the Science Citation Index Expanded (SCIexpanded) of the Web of Science Core Collection database. Acquired data were then visually analyzed using CiteSpace and VOSviewer.Results: 2036 papers were included in the final analysis. The annual publication outputs reached its peak in 2013 but declined in recent years. The dominant position of the United States in WAS research was quite obvious. Harvard University (USA), University College London (UK), and Inserm (France) were the three most prolific institutions. Adrian J. Thrasher exerted significant publication impact and made the most notable contributions in the field of WAS. Blood was the most influential journal with the highest publication outputs, and nearly all the top 10 journals and co-cited journals belonged to Q1. Immune dysregulation, thrombocytopenia, syndrome protein deficiency, stem cell, mutation, and diagnosis were the keywords with the strongest citation burst. Conclusion:From 2001 to 2021, the United States was a global leader in the WAS research. Collaboration between countries and institutions is expected to deepen and strengthen in the future. Research hotspots included pathogenesis, clinical manifestations, diagnosis, and therapy. Our results suggest a greater understanding of the mechanistic underpinnings of immune dysfunction in WAS patients, the application of targeted therapies for individual complications, and the development of curative approaches, which will remain research hotspots in the future.Abbreviations: Nc = number of citations, Np = number of publications, WAS = Wiskott-Aldrich syndrome, WASp = WAS protein, XLT = X-linked thrombocytopenia.

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Background: With the COVID-19 epidemic, wearing a mask has become routine to prevent and control the virus's spread. Wearing masks for long periods can have various adverse effects on the human body, but little attention has been paid to the impact on olfaction. This study aimed to investigate whether wearing a mask affects healthcare workers' olfactory function and provide a reference for clinical olfactory research and mask wear regulations.Methods: We recruited fifty-six healthcare workers and randomly divided them into two groups, wearing a Powered Air Purifying Respirator (PAPR, experiment group, N=28) and an N95 mask (control group, N=28) for 8h. Olfactory discrimination tests and threshold tests were performed before and after wearing the masks. SPSS 26.0 software was used for the statistical analyses.Results: Current results suggested no statistical difference in the discrimination test scores between the PAPR Group (Z=-0.707 P =0.480) and N95 Group (Z=-0.828 P =0.408) before and after wearing the masks. The olfactory threshold test revealed a statistical difference in threshold scores before and after wearing the mask in the PAPR Group (Z= -2.595 P = 0.009) and N95 Group (Z= -2.120 P= 0.034). Both PAPRs and N95 masks reduce the sensitivity of healthcare workers to odors, with no significant difference between the two (χ2= 0.292，p = 0.589).Conclusion: Wearing a mask affects the healthcare workers’ olfaction, especially odor sensitivity. Healthcare workers wearing masks have a higher olfactory threshold than before， whether wearing PAPRs or N95 masks. More attention needs to be paid to the effect of masks on the olfactory function.

Condition being studied: Pulmonary alveolar proteinosis (PAP), first described by Rosen et al. in 1958, is a rare pulmonary surfactant homeostasis disorder resulting in buildup of lipo-proteinaceous material within the alveoli, whose annual prevalence INPLASY 1