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Generalized pustular psoriasis: immunological mechanisms, genetics, and emerging therapeutics.
Trends Immunol
December 2024
College of Medicine, Chang Gung University, Taoyuan, Taiwan; Department of Dermatology, Chang Gung Memorial Hospital, Keelung, Taiwan; Department of Dermatology, Chang Gung Memorial Hospital, Linkou, Taiwan; Department of Dermatology, Chang Gung Memorial Hospital, Taipei, Taiwan; Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Linkou, Taiwan; Cancer Vaccine and Immune Cell Therapy Core Laboratory, Department of Medical Research, Chang Gung Memorial Hospital, Linkou, Taiwan; Chang Gung Immunology Consortium, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan; Department of Dermatology, Xiamen Chang Gung Hospital, Xiamen, China; Xiamen Chang Gung Allergology Consortium, Xiamen Chang Gung Hospital, Xiamen, China; Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan; Immune-Oncology Center of Excellence, Chang Gung Memorial Hospital, Linkou, Taiwan; Whole-Genome Research Core Laboratory of Human Diseases, Chang Gung Memorial Hospital, Keelung, Taiwan; Genomic Medicine Core Laboratory, Chang Gung Memorial Hospital, Linkou, Taiwan; School of Medicine, National Tsing Hua University, Hsinchu, Taiwan. Electronic address:
Generalized pustular psoriasis (GPP) is a rare human autoinflammatory disorder with life-threatening systemic effects. Keratinocyte-derived interleukin (IL)-36 signaling has been identified as a key mediator of immune response in the skin of affected individuals. Recognition of various mutations along the IL-36 axis and the downstream nuclear transcription factor κB (NF-κB) signaling have established GPP as genetically, immunologically, and histopathologically distinct and amenable to immunomodulation, which is epitomized by the recent success of IL-36 antagonism.
View Article and Find Full Text PDFMapping the current status and outlook of research on noonan syndrome over the last 26 years: a bibliometric and visual analysis.
Front Genet
December 2024
Department of Pediatrics, Affiliated Hospital of Nanjing University of Chinese Medicine, Nanjing, China.
Background: Noonan syndrome (NS) is a rare group of autosomal genetic disorders. In recent years, with the exploration and development of molecular diagnostic techniques, more and more researchers have begun to pay attention to NS. However, there is still a lack of reports on the bibliometric analysis of NS worldwide.
View Article and Find Full Text PDFTOURISM study (Treatment Outcomes in UteRIne SarcoMa): a 10-year retrospective evaluation of practice in the UK.
BMJ Open
December 2024
Edinburgh Cancer Centre, Western General Hospital, Edinburgh, UK.
Background: Although rare, uterine sarcomas account for a high proportion of uterine cancer mortality. Treatment options and robust trial data are limited.
Objectives: The TOURISM study (Treatment Outcomes in UteRIne SarcoMa) is a UK-wide study by the National Oncology Trainees Collaborative for Healthcare Research which aimed to characterise this patient cohort.
The rollout of electric vehicles and photovoltaic panels is essential to mitigate climate change. However, they depend on technology-critical elements (TCEs), which can be harmful to human health and whose use is rapidly expanding, while recycling is lacking. While mining has received substantial attention, in-use dissipation in urban areas has so far not been assessed, for example, corrosion and abrasion of vehicle components and weather-related effects affecting thin-film photovoltaic panels.
View Article and Find Full Text PDFScurvy incidence trend among children hospitalised in France, 2015-2023: a population-based interrupted time-series analysis.
Lancet Reg Health Eur
February 2025
Department of General Paediatrics, Paediatric Internal Medicine, Rheumatology and Infectious Diseases, National Reference Centre for Rare Paediatric Inflammatory Rheumatisms and Systemic Autoimmune Diseases (RAISE), Robert-Debré University Hospital, Assistance Publique-Hôpitaux de Paris, F-75019, Paris, France.
Background: Scurvy, historically rare in-high income countries, has re-emerged as an indicator of socioeconomic and dietary disparities. Limited data exist on scurvy trends among European children, particularly following socioeconomic changes since the COVID-19 pandemic. This study analysed scurvy incidence trends among French children over a nine-year period, examining potential post-pandemic increases.
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